The Kaufman
Courses

PCP intoxication is characterized by unpredictable and violent behavior. Dissociative and psychotic symptoms can occur. Individuals intoxicated with PCP are frequently agitated, impulsive, and insensitive to pain. Physical findings include autonomic hyperactivity, muscle rigidity, and ataxia. Although rotary or horizontal nystagmus may be observed they are nonspecific, but vertical nystagmus in this setting is virtually pathognomonic of PCP intoxication. Vertical nystagmus is not seen with any other drug of abuse. Treatment includes placing the patient in a quiet area with low stimulation. Haloperidol and/or a benzodiazepine may reduce the agitation and psychosis.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Pharmacological management strategies for serotonin syndrome include the use of benzodiazepines (for control of agitation) and 5-HT2A antagonists, such as cyproheptadine. In severe cases, especially to control hyperthermia, if the excessive muscular activity is not suppressed by a benzodiazepine, the use of a non-depolarizing agent, such as vecuronium (with appropriate supportive measures), may be necessary. Since the hyperthermia is a direct result of muscular hyperactivity, antipyretics, such as acetaminophen have no benefit.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins.

Major Depressive Disorder is twice as common in women, and it tends to have a later age of onset than bipolar disorder. Bipolar disorder occurs with equal frequency in men and women. Rapid cycling occurs much more frequently in women than men.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins

In Piaget’s cognitive development theory, the period from 7 years to puberty coincides with the Concrete Operational Stage. Piaget defines this stage as the one during which operational thought and logical thinking dealing with a large amount of information replace egocentric thought. Also, according to Piaget, this is the stage when the child can take another’s perspective. In the psychodynamically-informed Developmental Stage Theory of Erik Erikson, the stage of Industry versus Inferiority is described as extending from age 5 years to adolescence and coinciding with the onset of latency. During this phase, the child learns new skills, enters systematic instruction, and discovers the joy of productivity.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Carbamazepine, phenytoin, phenobarbital all induce CYP 450 3A4 enzymes, leading to decreased levels of co-administered drugs that are also metabolized by this enzyme. Important substrates are oral contraceptives, warfarin, atypical antipsychotics, imipramine, methadone, statins, and some antineoplastic agents. In addition, since carbamazepine is also metabolized by 3A4, it can “auto-induce” its own metabolism, leading to its own decreased serum levels. Valproic acid can increase the levels of several co-administered drugs through a variety of metabolic pathways; however, it has no induction properties. Of most significance in psychiatry, valproic acid can increase levels of lamotrigine (thereby increasing the risk of developing a rash), carbamazepine, and amitriptyline.

Schacter S. (2023). Antiseizure medications: Mechanism of action, pharmacology, and adverse effects. In Dashe, LF (Ed), UpToDate. Retrieved February 11, 2023 from https://www.uptodate.com

Choreoathetotic movements of the head, neck, extremities and trunk characterize tardive dyskinesia. Oral-buccal-lingual movements are characteristic. Older females are at greatest risk. Other risk factors include early onset of extrapyramidal symptoms (EPS), mood disorders, and high doses of medication. In contrast, tardive dystonia is characterized by slow, sustained, powerful muscular contractions of the neck, trunk, and limb muscles. Blepharospasm and facial grimacing frequently occur. Tardive dystonia usually has an earlier onset than tardive dyskinesia, and males are at increased risk. Unlike in tardive dyskinesia, anticholinergic medications may improve symptoms of tardive dystonia. Although not FDA approved, botulinum toxin and dopamine depleting agents may also be effective in the treatment of tardive dystonia. VMAT-2 inhibitors (e.g., valbenazine) show efficacy in reducing symptoms of tardive dyskinesia. Lowering the dose of medication or switching to a second-generation antipsychotic may be helpful in both conditions.

Kaufman DM, Geyer HL, Milstein MJ, Rosengard JL. (2023). Kaufman’s Clinical Neurology for Psychiatrists (9th ed). Elsevier

Psychiatric symptoms may be the first or most prominent manifestation of NMDA-receptor encephalitis, a recently described cause of delirium and first psychotic break. In addition to mood and thought disturbances, seizures, and involuntary movements, typically called “dyskinesias,” occur regularly. Ovarian teratomas frequently give rise to the disorder. Removing a teratoma, if present, will reverse all the synptoms. Conditions with similar symptoms include infectious encephalitis, non-infectious inflammatory encephalitis, such as lupus, and abuse of PCP or ketamine. NMDA-receptor encephalitis may develop in children as well as men predominantly, older than 45 years. However, it most commonly affects adult females between 25 and 35 years, and females four times more then males.

Dalmau J, Armangue T, Planaguma J, et al. (2019). An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol, 18(11); 1045-1057.
Debopam S, Forshing L. Anti-NMDA Receptor Encephalitis. StatPearls. 2022. Ncbi.nlm.nih.gov/books/NBK551672/

Sarin is an organophosphate, a group of chemicals that constitute the active element of poison gases as well as insecticides and pesticides. Organophosphates inhibit acetylcholinesterase and thereby allow acetylcholine to accumulate, precipitating a cholinergic crisis.

Hulse EJ, Halsam J, Emmett SR, et al. (2019). Organophosphorus nerve agent poisoning: managing the poisoned patient. British Journal of Anesthesia, 123;457-463.

Nelson LS, Howland MA, Lewin NA, et al. (2018). Goldfrank’s Toxicologic Emergencies. McGraw-Hill.

Salient features of this case are dementia, parkinsonism, and visual hallucinations. These three symptoms constitute the core features of dementia with Lewy body disease. In addition, its suggestive features are rapid eye movement (REM) disorder and hypersensitivity to antipsychotic agents. By way of contrast, dementia usually does not complicate Parkinson disease until it has been present for five or more years. Parkinsonism does not complicate Alzheimer disease. Unlike dopaminergic substances, such as cocaine, dopamine-blocking substances do not produce euphoria and are unlikely to be taken surreptitiously.

Kaufman DM, Geyer HL, Milstein MJ, Rosengard JL. (2023). Kaufman’s Clinical Neurology for Psychiatrists, 9th edition. Philadelphia, PA. Elsevier

Because SSRIs have a flat dose response curve, higher doses of SSRIs will not necessarily offer additional therapeutic benefit, although some patients may show a response to higher doses. Premature dose increases can also increase the risk of side effects. In general, partial response in the first 4 weeks predicts more complete response over the next 8 weeks, even if the dose is held constant. If a given dose is tolerated and not producing partial benefits at 4 weeks (unlike in this patient), the dose should be increased rather than the medication switched.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

This man had a several hour period of almost entirely anterograde amnesia that developed after vigorous exercise. During that time, he retained personal, well-learned information. This is the well-known syndrome of transient global amnesia (TGA). Physically stressful activities, including sexual intercourse and exposure to cold may precipitate an episode of TGA, but episodes rarely recur. Postulated mechanisms have included transient ischemia, cerebral venous insufficiency, and seizures. Although research has not identified a specific cause, the consensus has always been that TGA is a physiologic disorder. In contrast to TGA, the diagnostic criteria for Dissociative Amnesia require loss of important autobiographical information, even if only for a specific event. Dissociative identity disorder’s criteria essentially require two or more personalities as well as amnesia for autobiographical information or traumatic events. Because he was fully alert and attentive, this patient could not have been having a partial complex seizure.

Mahler M. (2020). Transient global amnesia. in Wilterdink, JL (Ed), UpToDate. February 11, 2023, from https://www.uptodate.com

When doctors go to court for medication over objection, they need to demonstrate that the patient lacks the capacity to make a refusal and that the treatment is in the patient’s best interests. Involuntary hospitalization does not negate the presumption that a patient is competent to refuse medication. If the patient represents an acute danger to self or others, this situation constitutes an emergency for which medication can be given over a patient’s objection without going to court. Even if the patient were willing to contract for safety, the contract would not resolve the issue of a refusal to take medication.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins.

Appelbaum PS, Gutheil TG. (2020). Clinical Handbook of Psychiatry and the Law. Lippincott Williams & Wilkins.

While suicide or attempted suicide represents the most frequent cause of loss related to malpractice claims against psychiatrists, the listed choices are each represented to varying degrees. Breach of confidentiality was cited in 15 percent of malpractice claims, the highest among the answer choices. Boundary violation was named as the primary allegation in 3 percent of cases, whereas a “duty to warn/protect” (e.g., a Tarasoff case), and abandonment are each cited in only 1 percent.
Frierson RL, Joshi KG. (2019). Malpractice law and psychiatry: an overview. Focus: the journal of lifelong learning in psychiatry, 17(4): 332-336.

Appelbaum PS, Gutheil TG. (2020). Clinical Handbook of Psychiatry and the Law. Lippincott Williams & Wilkins

A socially harmful act is not enough to have committed a crime. To be found guilty, the accused must also have mens rea (evil intent) and actus reus (voluntary conduct). Because the man is psychotic, he could be deemed not-guilty-by-reason-of-insanity. Respondeat superior states that a person occupying a high position in a hierarchy is responsible for those in lower positions. Non-malfeasance is the duty of physicians to do no harm. Parens patriae is a doctrine that allows the state to intervene and act as a surrogate parent for those who are unable to care for themselves.

Appelbaum PS, Gutheil TG. (2020). Clinical Handbook of Psychiatry and the Law. Lippincott Williams & Wilkins.

The paraphilias that most commonly lead to legal consequences include pedophilia, exhibitionism, and sexual sadism resulting in rape or murder. Persons with fetishism rarely become entangled in the legal system.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

The McGarry instrument is a clinical guide that evaluates 13 areas of functioning in order to determine a patient’s competence to stand trial. The McNaughton (M’Naghten) rule states that people are not guilty by reason of insanity if they have a mental disease such that they are unaware of the nature, quality, and consequences of the actions, or if they are incapable of realizing that their acts are wrong. The McGann instrument and the McInnis instrument are not real and are just distractors.

Appelbaum PS, Gutheil TG. (2020). Clinical Handbook of Psychiatry and the Law. Lippincott Williams & Wilkins.

Iloperidone (Fanapt) is metabolized by CYP 2D6 and CYP 3A4 P450 enzymes. It is necessary to reduce the dosage of iloperidone by 50% if it is given concomitantly with a strong inhibitor of either enzyme. Paroxetine is a strong inhibitor of 2D6. When the inhibitor is discontinued, the dosage should be returned to its previous level. Side effects of iloperidone include sedation, orthostatic hypotension, dizziness, and tachycardia. Iloperidone can also cause QTC prolongation and EPS. The usual starting dose is 1 mg. BID with a gradual titration to a 6-12 mg. BID.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

MRI studies in patients with PTSD show decreased hippocampal volume. The answer choice involving the putamen and caudate is a distractor. Increased size of the lateral ventricles is seen in schizophrenia. Decreased metabolic activity in the prefrontal cortex is seen in schizophrenia, not PTSD. In addition, metabolic activity is evaluated using fMRI or PET, not a standard MRI.

Ben-Zion Z, Artzi M, Niry D, et al. (2020). Neuroanatomical risk factors for posttraumatic stress disorder in recent trauma survivors. Biological Psychiatry: Cognitive Neuroscience and Neuroimaging, 5; 311-319.

B.F. Skinner is associated with operant conditioning. In operant conditioning, voluntary behavior is modified using positive and negative reinforcement to shape the desired behavior. Ivan Pavlov is associated with classical conditioning. In classical conditioning a neutral (conditioned) stimulus is paired with a stimulus that evokes a response (unconditioned), such that the neutral stimulus eventually comes to evoke the same response. Martin Seligman is associated with learned helplessness, which is a model of depression in which an individual repetitively fails at tasks and eventually stops trying, adopting a hopeless apathetic position. Salvador Minuchin is associated with structural family therapy. Structural family therapy focuses on the organization of the family and how that organization promotes or impedes the family from functioning successfully.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins.

CBT has been well-studied and shown to be very effective for panic disorder. It often includes psychoeducation, continuous panic monitoring, breathing retraining, cognitive restructuring, and exposure to fear cues. Developing discrepancy between the patient’s goals and current behavior is an important element of Motivational Interviewing, which is used in substance abuse treatment; however, it is not considered a central element of CBT.

Wright JH, Thase ME, Beck AT. (2019). The American Psychiatric Publishing Textbook of Psychiatry (7th ed). American Psychiatric Publishing, Inc.

In the treatment guidelines for children with ADHD and moderate tic disorders, guanfacine and clonidine are considered first line treatments. These medications are effective both for tic control and decrease in impulsivity and hyperactivity. Moreover, they have a favorable safety margin compared with the antipsychotic medications.

Ogundele MO, Ayyash HF. (2018). Review of the evidence for the management of co-morbid tic disorders in children and adolescents with attention deficit hyperactivity disorder. World Journal of Clinical Pediatrics, 7(1);36-42.

Osland ST, Steeves TD, Pringsheim T. (2018). Pharmacological treatment for attention deficit hyperactivity disorder (ADHD) in children with comorbid tic disorders. The Cochrane database of systematic reviews, 6(6);CD007990

The most important first intervention in a child who presents with language delay is a full hearing evaluation.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins

Problems with attention and behavior typically occur in several settings and may vary depending on the level of structure and supervision provided, the interest in and/or novelty of an activity or setting, as well as the level of concentration and effort required. Therefore, to make the diagnosis of ADHD, the patient must exhibit symptoms during at least two settings.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins

Velocardiofacial Syndrome (VCFS), also called Shprintzen syndrome or DiGeorge syndrome, is caused by a microdeletion in the long arm of chromosome 22 at band 22q11.2. The phenotypic spectrum of VCFS is wide and includes congenital cardiac anomalies, abnormal facies, palatal abnormalities, hypocalcemia, T-cell immunodeficiencies, cognitive deficits, and psychiatric manifestations including schizophrenia.

Cirillo A, Lioncino M, Maratea A, et al. (2022). Clinical manifestations of 22q11.2 deletion syndrome. Heart Failure Clinics, 18(1):155-164.

The CATIE study was a randomized controlled, double-blind trial comparing the effectiveness of second-generation antipsychotics to the conventional antipsychotic perphenazine. More than 1400 patients were enrolled. The results indicated that 74% of patients in the trial discontinued their medication during the 18 months of the study duration, confirming that adherence is a substantial problem in the treatment of patients with schizophrenia. The most likely cause of perphenazine discontinuation was EPS; however, there was no overall significant difference in the proportion of patients with EPS across groups. Olanzapine was associated with more weight gain and metabolic abnormalities. Regarding efficacy, perphenazine was found to be similar to quetiapine, risperidone, and ziprasidone. Olanzapine was found to be the most effective of the agents tested based on longest time to discontinuation, greater decrease in psychopathology, and lower rates of rehospitalization.

Kim J, Ozzoude M, Nakajima S. (2019). Insight and medication adherence in schizophrenia: an analysis of the CATIE trial. Neuropharmacology, 168;107634

Lithium is excreted in the urine. Because lithium levels can be increased 30%-50% when taken with thiazide diuretics, adjustments in its dosage are required when the two medicines are used concomitantly. NSAIDs, ACE inhibitors, and calcium channel blockers can also increase lithium levels. Lithium levels are decreased by theophylline and osmotic diuretics. Salt and water intake can also affect lithium levels. If GFR is increased, for example with pregnancy, lithium levels will decrease. On the other hand, if GFR is decreased, for example in the elderly or in patients with renal disease, lithium levels will increase.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc

When patients treated with nonreversible MAOIs ingest tyramine containing foods, they are at risk to develop a hypertensive crisis. They should avoid foods with high tyramine content, including beer, red wine, aged cheeses, aged or fermented meats, fava beans, smoked fish, and yeast extracts. Chocolate, caffeine, and other alcoholic beverages can be a problem if ingested in large amounts but are acceptable if used in moderation. Foods that are safe to ingest include cottage cheese, cream cheese, figs, and raisins.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Schizoid personality disorder is characterized by social detachment, isolation, and constricted affect. Affected individuals lack close friends, do not take pleasure in activities, and appear cold, aloof, and indifferent to others. Suspiciousness and mistrust is a core feature of paranoid personality disorder.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Medications with proven efficacy in acute mania include antipsychotics (both typical and atypical, with generally equal benefit) and certain mood stabilizers, namely lithium, valproic acid, and carbamazepine. Lamotrigine, while an effective treatment for bipolar depression, has not been shown to be effective in treatment of acute mania.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Due to fluoxetine’s long half-life, when switching from it to an MAOI, a 5-week washout period is advised to reduce the risk of developing serotonin syndrome. When transitioning from an MAOI to fluoxetine, the recommended washout period is two weeks. When switching between an MAOI and other SSRIs, the washout period is two weeks.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

According to 2020 data published by Center for Disease Control, firearms account for 52.8% of completed suicides in the United States. Suffocation or hanging accounts for 27.2% and poisoning resulted in 12.9 % of suicides. Worldwide, ingestion of pesticide, hanging, and firearms are the most common methods of suicide, according to the World Health Organization.

CDC (2022, June 28). Suicide prevention: Suicide data and statistics. Retrieved February 11, 2023, from https://www.cdc.gov/suicide/suicide-data-statistics.html

WHO (2021, June 17). Suicide. Retrieved February 11, 2023 from https://www.who.int/news-room/fact-sheets/detail/suicide.html

Serotonin syndrome can present with a wide array of symptoms that vary in severity. In a patient who has recently received serotonergic agents or has had a dosage adjustment, hyperreflexia, myoclonus, and tremor are highly suggestive of the disorder. Other symptoms of the serotonin syndrome include diarrhea, tachycardia, elevated temperature, diaphoresis, shivering, elevated BP, and muscular rigidity (which can mask the other neuromuscular symptoms). Mental status changes, ranging from agitation and confusion to frank delirium, can also occur. Lithium toxicity would be unlikely to occur at a level of 0.7meq.l. In contrast, in neuroleptic malignant syndrome, severe muscle rigidity (“lead pipe rigidity”), mutism, and decreased bowel sounds would be seen. Anticholinergic toxicity is characterized by hyperthermia, tachycardia, dry mouth, decreased or absent bowel sounds, agitation and delirium, normal neuromuscular tone, and normal reflexes.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Valproic acid can double serum levels of lamotrigine. This interaction is of particular concern because of the increased risk of rash that occurs in patients taking lamotrigine. While most often the rash is benign, it can herald Steven’s Johnson syndrome, a potentially fatal reaction. The risk of developing a rash is increased by rate of titration and starting dosage. Therefore, lower dosage and slower titration of lamotrigine is recommended if valproic acid is used together with lamotrigine. Neither paroxetine, a 2D6 inhibitor, nor iloperidone affects lamotrigine levels.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

The long-acting injectable formulation of olanzapine has a black box warning because it is associated with the development of post-sedation delirium syndrome (PDSS). Symptoms can range from sedation, which may be severe, to coma or delirium. This formulation of olanzapine can only be administered in a registered health care facility, and patients must be observed for at least 3 hours before they can be sent home.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Anticholinergic agents do not improve and in some cases may exacerbate symptoms of tardive dyskinesia and should be discontinued or avoided. Switching to clozapine or a second-generation antipsychotic agent may decrease symptoms of tardive dyskinesia. Lowering the dose of a medication may also be effective, but initially the symptoms may appear to worsen, as a result of withdrawal emergent dyskinesias. Benzodiazepines may offer some partial or temporary relief.

Kaufman DM, Geyer HL, Milstein MJ, Rosengard JL. (2023). Kaufman’s Clinical Neurology for Psychiatrists (9th ed). Elsevier.

There is an increased risk of schizotypal personality disorder in the relatives of people diagnosed with schizophrenia. This relationship provides support for the concept that schizotypal personality disorder and schizophrenia are spectrum disorders. In addition, an increased risk for developing schizophrenia occurs in the relatives of people with schizotypal personality disorder. Paranoid personality disorder can also be found in relatives of people with schizophrenia, but it is not as common as schizotypal personality disorder.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Carbamazepine is both a substrate and an inducer at CYP 450 3A4. It can induce its own metabolism, which leads to decreased plasma levels even though dosing remains constant.
Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Fluoxetine is a potent inhibitor of the cytochrome P450 enzyme CYP2D6 and a less potent inhibitor of CYP3A4, which can reduce the clearance of risperidone and increase plasma concentrations of risperidone. This interaction may lead to toxic plasma risperidone concentrations and parkinsonism and EPS. In addition to careful clinical observation, monitoring plasma risperidone levels may be of value in patients given adjunctive therapy with fluoxetine.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Sedation is the most common side effect of trazodone. Orthostatic hypotension and dizziness also frequently occur. Priapism and cardiac arrhythmias are uncommon adverse effects.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Psychotic symptoms are seen in patients during a manic episode but not during hypomania. All of the other symptoms are consistent with both mania and hypomania. Another distinction between hypomania and mania is that social and occupational functioning is not significantly impaired in the hypomania.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Fetal Alcohol Syndrome, caused by maternal excessive alcohol consumption during pregnancy, is the most common acquired and potentially preventable, cause of intellectual disability. Down’s syndrome is the most common genetic cause of intellectual disability. Because trisomy 21 (Down’s syndrome) is not present in either parent, it is not inheritable. Fragile X, in contrast, is probably the most common inheritable rather than acquired cause of intellectual disability. The mutation (excessive trinucleotide repeats) is often present in parents, but in small enough size that symptoms occur in only mild forms. When the size of the mutation is large, children are at risk for the full-blown illness. Autism spectrum disorder is associated in ~70% of cases of Fragile X.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

VCFS is best known for its association with psychiatric disorders. It is particularly well-known for representing a genetic risk for schizophrenia. In VCFS, early loss of temporal grey matter seems to predict the early signs of significant psychiatric problems. Except for having two parents with schizophrenia or being the monozygotic twin of someone with schizophrenia, VCFS is the highest risk factor for developing schizophrenia. VCFS is also associated with autism, ADHD, mood disorders, and phobias. In the very young, management is related to treatment of cardiac, immune, and palatal difficulties along with developmental delays. In the school years, cognitive difficulties are prominent. In young adulthood, psychiatric difficulties may develop. VCFS is not a sex-linked condition; it is caused by a micro-deletion at 22.11.2. Answer A refers to tuberous sclerosis, which presents with nodules seen on the malar surface of the face, refractory epilepsy, and progressive intellectual disability. It is associated with autism-like symptoms. The microdeletion syndromes at 15Q are Prader-Willi (usually paternal deletion) and Angelman (usually maternal deletion). In general, abnormal sex chromosomes produce physical findings and not necessarily intellectual dysfunction, whereas abnormal autosomal chromosomes are usually associated with intellectual disability.

Cirillo A, Lioncino M, Maratea A, et al. (2022). Clinical manifestations of 22q11.2 deletion syndrome. Heart Failure Clinics, 18(1):155-164.

The vast majority of paraphilias occur in men. Close to 100% of exhibitionists are males exposing themselves to women. Fetishism, a disorder in which sexual arousal involves the use of inanimate objects that are typically associated with the body – e.g., shoes, gloves, undergarments – also almost always occurs in men. About 95% of pedophiles are heterosexual. Most pedophiles also have engaged in voyeurism, exhibitionism and rape.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Angelman Syndrome is a heritable cause of intellectual disability that occurs when the deletion at 15q is maternally inherited. It occurs in boys and girls and is comprised of profound intellectual disability, microcephaly, paroxysms of laughter, ataxia, and autistic features. Rett syndrome is inheritable but present exclusively in girls (and XXY boys) so this is an incorrect diagnosis. Young girls with Rett have characteristic hand stereotypies. They do have microcephaly but not bursts of laughter or ataxia. Prader- Willi is an inheritable cause of intellectual disability (paternal deletion at 15q) characterized by hyperphagia, obesity, nonfood-related obsessions, and severe behavior problems. Children with Williams syndrome often have “elfin” facies, loquaciousness, and some level of intellectual disability.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry (10th ed). Lippincott Williams & Wilkins.

Mild elevations of pulse and blood pressure are a common side effect of stimulants. A recommendation for pediatric or cardiology consultation and EKG or further work-up is indicated if there is a family history of early/sudden death or congenital cardiac condition or dysrhythmia in the child. Stimulants do not cause evening sedation. Lithium may cause increased thirst. SSRIs and antipsychotics increase appetite (stimulants typically decrease appetite.)

Storeb OJ, Pedersen N, Ramstad E. (2018). Methylphenidate for attention deficit hyperactivity disorder (ADHD) in children and adolescents – assessment of adverse events in non-randomized studies. Cochrane Database of Systematic Reviews, 5(5) CD012069.

This woman suffers from Social Anxiety Disorder. Paroxetine, sertraline and venlafaxine have FDA approval for the treatment. Off-label, all SSRIS, MAOIs, and benzodiazepines have demonstrated efficacy. β-blockers are effective off-label treatments for performance anxiety but are not useful in generalized social anxiety. Tricyclic antidepressants, which are effective in the treatment of both GAD and panic disorder, have not been shown to be helpful in the treatment of social anxiety disorder.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Guanfacine (and extended release guanfacine) as well as clonidine, both alpha 2-adrenergic agonists, commonly present with side effects of drowsiness, dizziness, and dry mouth. Bradycardia and decreased appetite are also seen with these medicines. Increased urination is seen with lithium, but not guanfacine, treatment.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology, (9th ed). American Psychiatric Publishing, Inc.

Bupropion acts to enhance transmission of dopamine and norepinephrine and does not exert influence on serotonin receptors. Common side effects include headache, anxiety, insomnia, GI upset, and sweating. Tremor and akathisia may also occur. Bupropion is not associated with weight gain (and may cause weight loss), sexual dysfunction, or cardiac conduction problems. It does not cause daytime sedation or orthostatic hypotension. Bupropion is not associated with anticholinergic side effects. Daily dosage should not exceed 450 mg. as dose dependent-seizures can occur above this level. Because it facilitates dopamine transmission, bupropion may be a good choice for use in patients with Parkinson’s disease.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Since 2007, the FDA has approved 3 medications for the treatment of fibromyalgia: pregabalin (Lyrica), duloxetine (Cymbalta), and milnacipran (Savella). Pregabalin acts as an alpha2-delta ligand, while the latter two are serotonin-norepinephrine reuptake inhibitors (SNRI). Selective-serotonin reuptake inhibitors and tricyclic antidepressants have been shown to be helpful. However, bupropion, a norepinephrine-serotonin reuptake inhibitor, is not among medications with demonstrated efficacy.

Sadock BJ, Sadock VA, Ruiz P. (2017). Kaplan and Sadock’s Comprehensive Textbook of Psychiatry, 10th edition. Philadelphia, PA. Lippincott Williams & Wilkins.

The use of antipsychotics has been recognized to increase the risk for markers of cardiac dysfunction (e.g., prolonged QTc), ventricular arrhythmias (including torsade de pointe), and even sudden cardiac death. The association may be more common in elderly patients and with the use of typical antipsychotic agents.

Beach SR, Celano CM, Sugrue AM. (2018). QT prolongation, torsades de pointes, and psychotropic medications: a 5-year update. Psychosomatics, 59(2);105-122

Adrenal insufficiency or Addison’s Disease is an often-overlooked endocrine condition with prominent fatigue that can mimic depression. Other common symptoms include nausea, vomiting, diarrhea, muscle pain, and sweating. Weight loss and postural hypotension are common signs. The treatment involves replacement corticosteroids.

Kumar R, Wassif W. (2022). Adrenal insufficiency. J Clin Pathol. 75(7):435-442.

Bupropion doses should not exceed 450mg. daily due to increased risk of seizures at higher doses. Bupropion use is contraindicated in patients with bulimia and anorexia, as these patients demonstrated an increased susceptibility to seizures in premarketing trials. Bupropion has been shown to be effective in treating patients with seasonal affective disorder and is a second line treatment for ADHD. Marketed under the name Zyban, it is an approved treatment for smoking cessation. Bupropion may be useful in the treatment of depression in bipolar disorder because it is less likely to induce a switch to mania. Bupropion may exacerbate anxiety symptoms in patients with panic disorder or other anxiety disorders.

Sadock BJ, Sussman N, Sadock VA. (2019). Kaplan & Sadock’s Pocket Handbook of Psychiatric Drug Treatment (7th ed.). Wolters Kluwer

Compared to those with bulimia, patients with anorexia nervosa have a lower nutrition status and thus higher rates of medical complications, including effects on thyroid (euthyroid sick syndrome) and sex hormones (including decreased LH and FSH), amenorrhea, electrolyte abnormalities, osteoporosis, cardiac malfunction (including bradycardia and arrhythmia), and overall mortality. An elevated amylase is associated with purging in either condition. In this case the bulimic patient who purges is more likely to have an elevated amylase than a non-purging anorexic patient.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

Obstructive sleep apnea (OSA) occurs in up to 17% of men. OSA can lead to symptoms that mimic depression: fatigue, lack of energy, and poor concentration. Compared to controls, patients are more likely to be involved in car accidents. In patients with OSA, medications may be used to increase alertness or to treat associated depressive symptoms, but continuous positive airway pressure (CPAP) has been shown to decrease somnolence and to improve the quality of life, mood, and alertness. First, however, patients must be evaluated with polysomnography. Morning cortisol should be checked in patients with presentations suspicious for adrenal insufficiency, which would include those with prominent fatigue, nausea/anorexia, weight loss, and hypotension.

Akashiba T, Inoue Y, Uchimura N. (2022). Sleep apnea syndrome (SAS) clinical practice guidelines 2020. Respiratory Investigation, 60(1):3-32.

The SSRIs and SNRIs can all cause sexual dysfunction. Mirtazapine and bupropion are notable for their lack of association with sexual dysfunction. Common side effects of mirtazapine include weight gain, sedation, and dizziness.

Sadock BJ, Sussman N, Sadock VA. (2019). Kaplan & Sadock’s Pocket Handbook of Psychiatric Drug Treatment (7th ed.). Wolters Kluwer.

Psoriasis is a chronic, relapsing skin disease presenting with erythematous, scaling papules, and indurated plaques, arising preferentially on the elbows, knees, and scalp. Common triggers include psychological stress, physical trauma, infections, certain drugs (lithium, antimalarials, interferon), corticosteroid withdrawal, and alcohol use.

Sadock BJ, Sussman N, Sadock VA. (2019). Kaplan & Sadock’s Pocket Handbook of Psychiatric Drug Treatment (7th ed.). Wolters Kluwer.

HIV-associated dementia (HAD) is one of the leading causes of dementia in individuals under 60 years of age. Risk factors for HAD include high viral load, low educational level, anemia, older age, and female sex. The incidence of HAD has significantly decreased since the advent of highly active antiretroviral medication regimens.

Kolson D. (2022). Developments in neuroprotection for HIV-associated neurocognitive disorders (HAND). Current HIV/AIDS Reports, 19: 344-357

FDA approved medications for nicotine dependence include varenicline (Chantix), bupropion SR (Zyban), and nicotine replacement therapy. Physician advice has been shown to be effective as part of the “5 A’s” (Ask, Advise, Assess, Assist, Arrange). Acupuncture has not been shown to demonstrate efficacy.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Lippincott Williams & Wilkins.

The most likely cause of her delirium is MDMA (Ecstasy, Molly), which can cause agitation and paranoia; however, the greatest concern is her hypertension and hyperthermia, which can lead to cardiovascular collapse. The other drugs do not produce this combination of symptoms.

Hoffman R. (2020). MDMA (ecstasy) intoxication. In Grayzel, JG (Ed), UpToDate. Retrieved February 11, 2023 from https://www.uptodate.com

When benzodiazepines are given to patients with cirrhosis, those with short half-lives and without active metabolites, like lorazepam and oxazepam, are preferrable. Benzodiazepines that undergo oxidative metabolism in the liver can accumulate to toxic levels in patients with cirrhosis. Despite its short half-life, alprazolam, which also undergoes oxidation, can also accumulate to dangerous levels in patients with cirrhosis.

Sadock BJ, Sussman N, Sadock VA. (2019). Kaplan & Sadock’s Pocket Handbook of Psychiatric Drug Treatment (7th ed.). Wolters Kluwer.

Conduct disorder both precedes and predicts substance abuse in adolescents. Its risk is greater than for ADHD, PTSD, and Bipolar Disorder.

Taylor BP, Schlussel D, Hollander E. (2019). The American Psychiatric Publishing Textbook of Psychiatry, (7th ed). American Psychiatric Publishing, Inc.

Patients with co-occurring disorders my benefit from self-help groups (e.g., “Double Trouble”). Clinicians should treat psychosis, irrespective of etiology, with neuroleptics. No medications have proven to be effective for cocaine use. Benzodiazepines may be effective in anxiety disorders where patients have not responded to SSRIs, buspirone, etc, where they are stable in an Methadone Maintenance Treatment Program (MMTP) with a good therapeutic alliance. Avery J, Adam T, Levounis P. (2019). The American Psychiatric Publishing Textbook of Psychiatry, (7th ed). American Psychiatric Publishing, Inc.

Kaye A, Vadivelu N, Urman RD. (2015). Substance Abuse Inpatient and Outpatient Management for Every Clinician. Springer. New York.

Ruiz P, Strain E (eds). (2011). Lowinson and Ruiz’s Substance Abuse: A Comprehensive Textbook, 5th Edition. Lippincott Williams & Wilkins. Philadelphia.

Her dementia, accompanied by myoclonus and an EEG showing periodic complexes, progressing to death in 6 months characterizes Creutzfeldt-Jakob disease. Finding a 14-3-3 protein in her CSF would have given further diagnostic support. A brain biopsy would have shown spongiform changes, but clinicians do not routinely perform biopsies in suspected Creutzfeldt-Jakob disease cases because the material, which is infectious, would contaminate the surgical instruments and expose the surgical and pathology teams. Another reason for not performing a biopsy is that the illness remains untreatable. Creutzfeldt-Jakob disease is a frequently occurring neurodegenerative cause of dementia that follows a rapid, fatal course. As Dr. Stanely Prusiner, the Nobel- prize winning discoverer of the mechanism underlying Creutzfeldt-Jakob disease and related illnesses, explained at the American Psychiatric Association’s 2013 meeting, prions cause these illnesses by conformational conversion of prion proteins.

Uttley L, Carroll C, Wong R. (2020). Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis, 20; 2–10

ADHD is always more impairing than tics when the two are co-morbid. The severity of ADHD, not tics, is a good predictor of social difficulties, particularly with peers. Methylphenidate and clonidine can be used either alone or in combination when patients have comorbid tics/Tourette’s and ADHD, no matter which condition is worse. By history, ADHD will precede tics, usually by 2-3 years.

Osland ST, Steeves TD, Pringsheim T. (2018). Pharmacological treatment for attention deficit hyperactivity disorder (ADHD) in children with comorbid tic disorders. Cochrane Database Syst Rev (2018 06 26) 6:CD007990

While methadone and buprenorphine have both been demonstrated to be safe during pregnancy for the mother and fetus, the National Institute of Drug Abuse (NIDA)–sponsored MOTHER study showed milder neonatal abstinence with buprenorphine compared to methadone.

Lemon LS, Caritis SN, Venkataramanan R, et al. (2018). Methadone versus buprenorphine for opioid use dependence and risk of neonatal abstinence syndrome. Epidemiology, 29(2);261–268.

Frontotemporal dementia, a neurodegenerative illness, causes dementia that is initially overshadowed by personality changes and behavioral disturbances. The diagnostic criteria require 3 of 6 of the following disturbances: disinhibition, apathy, loss of sympathy, perseveration or compulsive behaviors, hyperorality, and impaired executive ability. Physicians might easily misdiagnosis frontotemporal dementia for bipolar disorder or late-life depression. Frontotemporal dementia is a common cause of dementia that characteristically has a relatively young age of onset (53 years), follows a rapidly fatal course (4 years), develops in men more than women, and occurs in multiple family members.

Kaufman DM, Geyer HL, Milstein MJ, Rosengard JL. (2023). Kaufman’s Clinical Neurology for Psychiatrists (9th ed). Elsevier.

Illness anxiety disorder is characterized by a preoccupation with having or developing a serious illness. Despite these concerns, somatic symptoms are not present or, if so, are minimal. These individuals have excessive anxiety about their health and engage in excessive health related behaviors (e.g., frequently checking body parts). Yet, they exhibit maladaptive avoidance of doctor appointments and hospitals. Attempts by physicians to provide reassurance generally do not alleviate anxiety and may heighten the individual’s concerns.
American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders Fifth Edition DSM-5-TR, 2022.

Trisomy 21, also known as Down syndrome, includes mild to moderately severe intellectual disability; characteristic physical findings including simian palm creases, stunted growth, low muscle tone, small genitalia, and facial dysmorphia; and risk of dementia in later life. This risk is of concern because people with Down syndrome now have a relatively long life expectancy. Their intellectual decline in their 40s and 50s is associated with an excess of beta-amyloid. In contrast, Down syndrome is negatively associated with autism. Prader-Willi syndrome, caused by a paternally-inherited translocation at 15Q, consists of hyperphagia, non-food-related compulsive behavior, and borderline-moderate intellectual disability. Fragile X syndrome, caused by a mutation on the X chromosome (Xq27.3), is the most common inherited cause of intellectual disability. This syndrome consists primarily of moderate to severe intellectual disability but also ADHD, autism, and social anxiety. Most boys with the disorder have a long face with large ears and macro-orchidism.

Fortea J, Vilaplana E, Carmona-Iragui M, et al. (2020). Clinical and biomarker changes of Alzheimer’s disease in adults with Down syndrome: a cross-sectional study. Lancet, 395(10242):1988-1997.

This recognition is an example of the concept of operational thought, described by Piaget. It typically emerges between the ages of 7-11 years. Normally developing children in this developmental stage are able to use logical thought to order, seriate, and group objects based on common characteristics. The concept of conservation attained in this developmental phase refers to the ability to recognize that even when the shape of an object changes, the amount of matter is conserved.

Boland R, Verduin M, Ruiz P. (2022). Kaplan & Sadock’s Synopsis of Psychiatry (12th ed.). Wolters Kluwer. Philadelphia.

High-potency conventional antipsychotics, like haloperidol, are most likely to cause extrapyramidal side effects. In contrast, low-potency agents, like thioridazine, are more likely to cause anticholinergic side effects (blurred vision, dry mouth, urinary hesitancy, constipation, tachycardia), alpha-adrenergic side effects (orthostasis), weight gain, and sedation (mediated by histamine and alpha-adrenergic receptors). Perphenazine and thiothixene, both midrange in potency, are less likely to cause anticholinergic side effects.

Schatzberg AF, Debattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology (9th ed.). American Psychiatric Publishing. Washington, DC.

Hemorrhagic changes at the base of the frontal and temporal lobes characterize herpes simplex encephalitis. The incidence of SSPE, which had been the most common cause of dementia of childhood, has dramatically fallen with widespread, complete measles immunization.

Jafri SK, Kumar R, Ibrahim S. (2018). Subacute sclerosing panencephalitis – current perspectives. Pediatric Health, Med Therapy, 9;67–71

This patient is most likely using crystal methamphetamine. The presentation is similar to cocaine, but with cocaine use, the urine would be positive for benzoylecgonine, which is the metabolite of cocaine. PCP does not cause dilated pupils. Schizophrenia in and of itself would not cause increased heart rate or dilated pupils, but otherwise may be similar.

Courtney K, Ray LA. (2014). Methamphetamine: An update on epidemiology, pharmacology, clinical phenomenology, and treatment literature. Drug Alcohol Depend, 143;11–21

At one to six months after bariatric surgery, particularly when they have vomiting, patients are at risk for Wernicke encephalopathy and other nutritional deficiencies. In this case, she had the classic signs of Wernicke encephalopathy. In many cases, atypical features are also present. Surprisingly, Wernicke encephalopathy rarely develops in patients with anorexia with or without bulimia.

Oudman E, Wijnia JW, Dam MV. (2018). Preventing Wernicke encephalopathy after bariatric surgery. Obesity Surgery, 28(7);2060–2068

Prescription drug abuse is a major epidemic resulting in loss of life now exceeding motor vehicle accidents. Its use is enhanced by its perceived safety and increased availability. Because of prescription opiates’ greater expense, people transition to heroin.

Substance Abuse and Mental Health Services Administration (SAMHSA). (2011). Results from the 2010 National Survey on Drug Use and Health: Summary of National Findings. NSDUH Series H-41, HHS Publication No. (SMA) 11-4658

His physical outbursts during REM periods indicates REM sleep behavior disturbance. Seizures would have been reflected in paroxysmal EEG activity, such as bursts of spike-and-wave activity.

Arnulf I. (2012). REM sleep behavior disorder: motor manifestations and pathophysiology. Move Dis, 27(6);677–689

The most consistent PSG changes in depression are decreased REM latency, increased total REM sleep time, and decreased slow wave sleep. Even with remission of symptoms, these changes may persist. If so, they are a risk factor for recurrence of depression.

Palagini L, Baglioni C, Ciapparelli A, et al. (2013). REM sleep dysregulation: State of the art. Sleep Med Rev, 17; 377-390

Ataxia, confusion and ocular abnormalities are the hallmarks of acute thiamine deficiency (Wernicke’s Disease). In contrast, anterograde amnesia is a defining symptom of chronic thiamine deficiency (Korsakoff’s Dementia).

Kosten T, Haile C, Woods S. (2015). The American Psychiatric Association Publishing Textbook of Neuropsychiatry and Clinical Neurosciences (5th ed). APA Press. Washington, D.C.

Sublimation is a mature defense mechanism in which an individual channels unacceptable impulses into socially acceptable alternatives. In this case, the man’s unacceptable aggressive behavior has been redirected into sports, which allows him to discharge his aggressive impulses in this socially acceptable context. Displacement is a defense mechanism in which unacceptable feelings or impulses are redirected towards a less threatening object. Reaction formation is when an unacceptable thought or feeling is converted into its opposite. Projection is when one’s own unwanted or unacceptable thoughts or impulses are attributed to someone else.

Roberts LW (eds). (2019). The American Psychiatric Publishing Textbook of Psychiatry, 7th ed. American Psychiatric Publishing. Philadelphia.

The patient is in the locked-in syndrome or state. He characteristically can communicate with eye movements and eyelid blinks. Most important, his cognitive function is preserved. Because he is in the locked-in syndrome, he retains his autonomy and decisional capacity. Most cases of locked-in syndrome result from a brainstem stroke due to an occlusion of the basilar artery. Other causes are brainstem trauma, amyotrophic lateral sclerosis (ALS), and, at least temporarily, Guillain-Barré syndrome.

Kaufman D, Milstein M, Geyer HL. (2017). Kaufman’s Clinical Neurology for Psychiatrists. 8th edition,
Elsevier, Philadelphia.

Kuehlmeyer K, Racine E, Palmour N, et al. (2012). Diagnostic and ethical challenges in disorders of consciousness and locked-in syndrome. J Neurol, 259;2076-2089

REM sleep behavior disorder often develops along with or up to 15 years before the onset of either Parkinson disease or dementia with Lewy bodies. It ultimately affects almost one-third of Parkinson disease patients. In both of these illnesses, which neurologists classify as synucleinopathies, REM sleep behavior disorder correlates with cognitive impairment.

Arnulf, I. (2012). REM sleep behavior disorder: Motor manifestations and pathophysiology. Move Disord, 27(6);677–689

Sixel-Doring F, Trautmann E, Mollenhauer B, et al. (2011). Associated factors for REM sleep behavior disorder in Parkinson disease. Neurology, 77;1048-1054

Approximately 50% of patients diagnosed with schizophrenia have a co-morbid substance use disorder. Cannabis is found to be the most commonly used substance of abuse by first episode patients, but with long term illness, alcohol use is most frequent. Nicotine use is extremely common with rates as high as 90% of patients with schizophrenia.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Publishing Textbook of Psychopharmacology. American Psychiatric Publishing. Philadelphia.

This boy has the presumptive diagnosis of Fragile X, which is associated with ADHD, learning problems, some language dysfluency and social anxiety. There may be a history of infertility or autism spectrum disorder in the family. Physically, as the child grows, there is progressive elongation of the head and ears. DNA testing will reveal between 230 and >1000 trinucleotide repeats when the syndrome is present with fragility at
Xq27.3. Rett Syndrome is an X-linked dominant disorder with a mutation at MECP2 and is seen in girls only – and in XXY boys – so would not likely be seen in this teenage boy. An ADOS evaluation would not be
specific for Rett but the patient could score in the autistic range if she was evaluated during that part of her clinical course. Uric acid testing might help diagnose Lesch Nyhan syndrome, but the boy would have characteristic self-mutilation, spasticity and psychomotor retardation. The patient with tuberous sclerosis can present with CNS tubers; tubers are characteristically seen across the malar surface of the face and the syndrome is associated with autism, epilepsy and worsening dementia.

Dulcan MK. (2016). Dulcan’s Textbook of Child and Adolescent Psychiatry (2nd ed.). American Psychiatric Association. Arlington.

Approximately 50% of children diagnosed with ADHD will continue into adulthood with the diagnosis. Typically, as children age into adolescence and adulthood, the gross manifestations of hyperactivity become less prominent, though restlessness can still remain as a residual symptom. Whereas DSM-IV-TR required that symptoms be present prior to the age of 7, DSM-5 only requires that symptoms (“several”) be present prior to the age of 12. In childhood, the diagnosis of ADHD is made far more often in boys than girls; adult men with ADHD seem to still outnumber women, though previously the ratio had thought to be equal; women are likely oversampled in treatment groups as they seem to preferentially seek help, making these statistics variable from study to study.

American Psychiatric Association. (2013). Diagnostic and Statistical Manual of Mental Disorders, 5th Ed. American Psychiatric Association Press. Washington, D.C.

The vignette describes a patient with classic symptoms of Somatic Symptom Disorder (according to DSM-V): She has four pain symptom (abdominal, head, pelvic and back), two gastrointestinal complaints (N/V), a pseudoneurologic problem (memory difficulty) and a sexual complaint (anorgasmia). Since her symptoms are already chronic at the age of 32, she meets the DSM-IV TR requirement that the symptoms begin before the age of 30. In his 2007 review of treatments for somatoform disorders, Kroenke found a total of 34 randomized controlled trials. Most of the studies involved patients with somatization disorder or similar lower threshold variants. Cognitive behavioral therapy (CBT) was effective in 11 of 13 studies, while antidepressants were helpful in 4 of 5 of studies. Only 1 of 3 RCTs for conversion disorder showed benefit. There were no studies of pain disorder.

American Psychiatric Association. (2017). Diagnostic and statistical manual of mental disorders: DSM-5 (5th ed.). American Psychiatric Association. Arlington.

Kurlansik SL, Maffei MS. (2016). Somatic Symptom Disorder. Amer Fam Physician, 93(1), 49–54

Learning Disorders are defined as persistent difficulties learning keystone academic skills that are chronic, well below average for age, and not attributable to intellectual disability. The full assessment of such a learning disability requires IQ testing (such as the WISC or Stanford Binet) and psychoeducational testing of the relevant learning area, for example reading (Kaufman), mathematics (standardized mathematics examination) or writing( Test of Written Language/ TOWL). Frame LB, Vidrine, SM, Hinojosa R. (2016). Test review: Kaufman, A. S, Kaufman, N. L. (2014). Kaufman Test of Educational Achievement, 3rd Ed. J Psychoeduca Assess, 34(8);811–818

The following medications are approved by the U.S. Food and Drug Administration (FDA) for the treatment of ADHD: dextroamphetamine, D- and D, L-methylphenidate, mixed salts amphetamine, and atomoxetine. The American Academy of Pediatrics (AAP) and the American Academy of Child and Adolescent Psychiatry (AACAP) have recommended stimulants as the first line of treatment for ADHD. After two failed trials of
each of the two stimulant classes, it is recommended that the third line choice for ADHD treatment is atomoxetine. However atomoxetine, a noradrenergic reuptake inhibitor, can be considered as the first line medication for ADHD in individuals with an active substance abuse problem, comorbid anxiety, or tics.

Schatzberg AF, DeBattista C. (2015). Manual of clinical psychopharmacology, 8th ed. American Psychiatric Publishing, Inc. Washington, D.C.

Bupropion’s mechanism of action is believed to involve reuptake inhibition of norepinephrine and dopamine. There is an increased risk of seizures at doses above 450 mg. Because patients with eating disorders are particularly susceptible to developing seizures while taking bupropion, its use in these patients is contraindicated. Due to its amphetamine like properties, appetite tends to be suppressed, so weight gain is not a problem. In addition, it is not associated with sexual dysfunction.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Publishing Textbook of Psychopharmacology. American Psychiatric Publishing. Philadelphia.

Use in physically hazardous situations and persistent desire or unsuccessful effort to cut down have been retained from DSM-IV-TR to DSM-5. Recurrent legal problems have been deleted. The only new criteria is craving or a strong desire or urge to use the substance and can even persist in remission.

American Psychiatric Association. (2017). Diagnostic and statistical manual of mental disorders: Dsm-5 (5th ed.). American Psychiatric Association. Arlington.

Sleeping shorter or longer than the optimal sleep duration imperils health. The optimum nightly sleep duration is 7-9 hours. Nevertheless, one-third of the U.S. population sleeps less than 7 hours at night. Sleep difficulties impair numerous activities. From most to least, sleep difficulties impair concentrating (about 30%), remembering, working on a hobby, driving, and performing work (about 12%). Sleep deprivation has a cumulative detrimental effect on both mental and physical well-being. Moreover, it exacerbates chronic disease.

Roberts LW (eds). (2019). The American Psychiatric Publishing Textbook of Psychiatry, 7th ed. American Psychiatric Publishing. Philadelphia.

This vignette describes several diagnostic features of schizotypal personality disorder including odd beliefs or magical thinking, having few or no close friends other than first degree relatives, suspiciousness or paranoid ideation, and social anxiety. While each feature may be seen in other personality disorders (absence of close friends-schizoid; suspiciousness-paranoid; social anxiety-avoidant personality) the cluster of these 4 features occurring together is consistent with a diagnosis of schizotypal personality disorder.

American Psychiatric Association. (2017). Diagnostic and statistical manual of mental disorders: DSM-5 (5th ed.). American Psychiatric Association. Arlington.

Of the choices, oxcarbazepine (most closely) and carbamazepine (second most closely) are linked with hyponatremia. One study (Dong) found serum sodium levels <128 mEq/L in 12% of patients taking oxcarbazepine and 3% of ones taking carbamazepine. The mechanism of hyponatremia probably involves renal dysfunction rather than either pituitary insufficiency or inappropriate secretion of antidiuretic hormone (ADH). Risk factors include high doses of the medicine, concurrent use of other medicines, and advanced age.

Pimozide is a classic antipsychotic agent that blocks D2 dopamine receptors. Its distinguishing features are that it has an indication for treatment of Tourette's disorder, for which it is still used, and its half-life is one of the longest of all of the antipsychotics (55 hours). As with other D2 dopamine receptor antagonists, pimozide use may be complicated by acute dystonic reactions, other extrapyramidal side effects, and the neuroleptic malignant
syndrome. Likewise, it may also dangerously prolong the QTc interval.

Lithium itself does not induce hyponatremia. However, if patients taking lithium or the other medicines develop psychogenic polydipsia or take diuretic, they may develop hyponatremia.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Publishing Textbook of Psychopharmacology. American Psychiatric Publishing. Philadelphia.

Lin CH, Lu CH, Wang FJ, et al. (2010). Risk factors of oxcarbazepine-induced hyponatremia inpatients with epilepsy. Clin Neuropharmacol, 3;293-296

Signs and symptoms of opiate withdrawal can occur within minutes to several days after cessation following heavy or prolonged use, or receiving an opiate antagonist. They include stomach cramps, nausea, vomiting, and /or diarrhea. Individuals may report dysphoria, muscle aches and difficulty sleeping. Yawning , lacrimation, rhinorrhea can occur, as does pupillary dilation, piloerection, diaphoresis and elevated temperature. On PE, needle “tracks” may be visible. Seizures can occur during withdrawal from sedatives, hypnotics or alcohol.

Galanter M, Kleber HD, Brady K. (2015). The American Psychiatric Publishing Textbook of Substance Abuse Treatment. American Psychiatric Publishing. Washington, D.C.

The neural tube forms in the first trimester of gestation. If the lower (caudal) end of the neural tube fails to close, the failure will cause a meningomyelocele, meningocele, or spina bifida. If the upper (cephalad) end fails to close, the failure will cause anencephaly, encephalocele, or similar defect. Supplementing breakfast cereals with folic acid or taking prenatal vitamins with folic acid has significantly lowered the incidence of neural tube defects. Nevertheless, the mother’s taking folic acid while taking medicines associated with the defect remains unsafe. Neural tube defects are readily detectable with MRI or ultrasound. Elevated levels of maternal serum alpha-fetoprotein indicate the presence of a neural tube defect. Not all mood stabilizers taken during pregnancy have been associated with neural tube defects. Valproate and, to a less extent, carbamazepine have been linked
to neural tube defect. Other medical teratogens and genetic mutations have also been linked to the defect.

Gool JDV, Hirche H, Lax H, et al. (2018). Folic acid and primary prevention of neural tube defects: A review. Reprod Toxicol, 80;73–84

Orthostatic hypotension is a side effect of trazodone, iloperidone, and phenelzine. Venlafaxine is associated with hypertension, especially diastolic blood pressure.

Roberts LW (eds). (2019). The American Psychiatric Publishing Textbook of Psychiatry, 7th ed. American Psychiatric Publishing. Philadelphia

Behavioral Signs and symptoms of amphetamine intoxication include initial euphoria or affective blunting that gives way to changes in sociability, hypervigilance, and interpersonal sensitivity in which individuals can become easily angered. Anxiety, tension and stereotyped behaviors can also occur. Impairment in judgment and function frequently result. Individuals can exhibit psychomotor agitation or retardation. Other clinical manifestations may include changes in heart rate and blood pressure, nausea and vomiting, and/or dilated pupils. Individuals may also present with respiratory depression, muscular weakness and/or chest pain. Weight loss is common. Confusion, seizures, dyskinesia, cardiac arrhythmias and coma can occur.

Galanter M, Kleber HD, Brady K. (2015). The American Psychiatric Publishing Textbook of Substance Abuse Treatment. American Psychiatric Publishing. Washington, D.C.

Cerebral amyloid accumulation and other biomarkers, such as abnormal concentrations of CSF Aβ42 and tau, precede cognitive impairment in Alzheimer’s disease by several years. Cerebral tau accumulation and then atrophy also precede cognitive impairment in Alzheimer’s disease.

Palomo MSM. (2019). Amyloid hypothesis in Alzheimer´s disease. Alzheimer’s disease: pathological and clinical findings. Recent Advances in Alzheimer Research, 3;1–15

He is punding – performing mindless, repetitive, purposeless, and unrewarding activities. Punding, which is similar to stereotypies, but more complex, would include incessantly arranging clothing in a drawer, building and taking apart paper constructions, and repeatedly aligning pens and pencils into diagrams. Parkinson’s disease patients, as well as individuals who regularly use amphetamine or cocaine, engage in punding. Excessive dopamine activity is its most likely cause.

Sanchez-Ramos J. (2015). Neurologic complications of psychomotor stimulant abuse. International review of neurobiology. The Neuropsychiatric Complications of Stimulant Abuse, 4;131–160.

Spencer AH, Rickards H, Fasano A, et al. (2011). The prevalence and clinical characteristics of punding in Parkinson’s disease. Mov Disord, 26;578-86

Quetiapine has very low rates of EPS. Studies indicate that when used in patients with Parkinson’s disease and Lewy Body disease treatment with quetiapine results in improvement in psychosis without exacerbation of motor function. Low doses of clozapine have also been used with similar benefit, but its side effect profile renders it less desirable.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Publishing Textbook of Psychopharmacology. American Psychiatric Publishing. Philadelphia.

Taking lurasidone on an empty stomach can decrease absorption and result in plasma levels that are up to 50% lower than when taken with food. This is also true for ziprasidone. Patients prescribed either of these medications should be advised to take their doses with meals.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Publishing Textbook of Psychopharmacology. American Psychiatric Publishing. Philadelphia.

Depersonalization is a sense of detachment from oneself and one’s identity; whereas derealization is a sense that people and the environment are not real. In contrast, an illusion is a misperception of an actual sensory output.

Roberts LW. (2019). The American Psychiatric Association Publishing Textbook of Psychiatry (7th ed). American Psychiatric Association Publishing. Washington, D.C.

Patients with schizotypal personality disorder have been shown to have deficits on tests of executive function. They also have deficits in tests of attention (including the Wisconsin Card Sorting Test), working memory, and learning. Because of these findings, schizotypal personality disorder is classified with schizophrenia in ICD-10.

Roberts LW. (2019). The American Psychiatric Association Publishing Textbook of Psychiatry (7th ed). American Psychiatric Association Publishing. Washington, D.C.

In this scenario, the patient has clearly indicated a choice that he was able to communicate to the team. He was also able to state his condition, treatment, risks, and benefits, which demonstrate that he has understanding of the situation. However, he is unable to acknowledge the medical condition and likely outcomes, which shows the patient lacks appreciation. Delusions, distortions, and denial are common causes of impairment in appreciation. Competency is not a component of a capacity evaluation.

Appelbaum PS. (2007). Assessment of patients’ competence to consent to treatment. NEJM, 357(18);1834–1840.

Although all of the medications can be used to treat chronic insomnia, clonazepam is not approved for chronic use.

Schatzberg AF, Nemeroff CB. (2017). The American Psychiatric Association Publishing Textbook of Psychopharmacology (5th ed). American Psychiatric Association Publishing. Arlington.

The primary advantages of (SGAs) given IM are fewer extrapyramidal side effects (EPS), including dystonic reactions, and more rapid onset of action. Studies have shown SGAs to be not more effective than FGAs and that they are more costly.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology. American Psychiatric Association Publishing. Washington, D.C.

The HLA-B1502 gene substantially increases the risk of Stevens Johnson syndrome in Han Chinese individuals being treated with carbamazepine. In 2007, the FDA recommended pharmacogenomic testing in at-risk populations.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology. American Psychiatric Association Publishing. Washington, D.C.

Although several studies indicate potential use for stimulants as an adjunctive treatment for depression, they do not have an FDA indication.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology. American Psychiatric Association Publishing. Washington, D.C.

Paroxetine has an association with fetal cardiac abnormalities, including atrial and septal defects, and thus is generally avoided in pregnancy. Most of the data on other SSRI exposure during pregnancy does not show a higher risk of birth anomalies.

Schatzberg AF, DeBattista C. (2019). Schatzberg’s Manual of Clinical Psychopharmacology. American Psychiatric Association Publishing. Washington, D.C.

Although all of the answer choices can be seen in NMS, hyperthermia with oral temperature of >100.4F or >38.0C on at least 2 occasions will distinguish NMS from other drug-induced side effects.

American Psychiatric Association. (2017). Diagnostic and Statistical Manual of Mental Disorders: DSM-5 (5th ed). American Psychiatric Association. Arlington.

Children 2-4 years of age will begin to express gender-specific interest. This is also the age of onset of cross-gender behaviors, including expressed desire to be the other gender or labeling oneself as the other gender.

American Psychiatric Association. (2017). Diagnostic and Statistical Manual of Mental Disorders: DSM-5 (5th ed). American Psychiatric Association. Arlington.

Diagnostic criteria for Avoidant/Restrictive Food Intake Disorder including eating or feeding disturbance associated with one of the following: significant weight loss, nutritional deficiency, dependence on nutritional supplementation, marked interference with functioning. There is no disturbance in body image as seen in anorexia. Patients with autism may have sensory issues; however, they do not result in significant weight loss/nutritional deficiency. Rumination Disorder includes repeated regurgitation of food.

American Psychiatric Association. (2017). Diagnostic and Statistical Manual of Mental Disorders: DSM-5 (5th ed). American Psychiatric Association. Arlington.

Cultural idioms of distress are ways of expressing distress that may not involve specific symptoms or syndromes but provide a shared expression of personal or social concerns. Cultural syndromes are clusters of symptoms that tend to co-occur among individuals in a particular community. A cultural explanation is a model for a culturally recognized meaning or etiology for a symptom or illness.

American Psychiatric Association. (2017). Diagnostic and Statistical Manual of Mental Disorders: DSM-5 (5th ed). American Psychiatric Association. Arlington.

Bipolar disorder is more common in high versus low-income countries (1.4% vs 0.7%). Family history is one of the strongest risk factors for bipolar disorder. Although women have increased risk for rapid cycling and mixed episodes, gender ratio is almost equal in the prevalence of bipolar I disorder.

American Psychiatric Association. (2017). Diagnostic and Statistical Manual of Mental Disorders: DSM-5 (5th ed). American Psychiatric Association. Arlington.

. Bromocriptine is a dopamine agonist, that decreases elevated prolactin levels caused by antipsychotic medications. This return to normal levels may improve sexual functioning; however, there may be concern for increase of psychotic symptoms given this mechanism of action.

Krysiak R, Szkróbka W, Okopień B. (2018). The effect of bromocriptine treatment on sexual functioning and depressive symptoms in women with mild hyperprolactinemia. Pharm Report, 70(2), 227–232.

Steffens DC, Blazer DG, Thakur ME. (2015). The American Psychiatric Publishing Textbook of Geriatric Psychiatry, Fifth Edition. American Psychiatric Publishing, Inc. Arlington.

Several studies have shown that ECT is a highly effective treatment for major depression in older adults and several studies have shown at least equivalent improvement among older adults in comparison to middle-age patients.

Steffens DC, Blazer DG, Thakur ME. (2015). The American Psychiatric Publishing Textbook of Geriatric Psychiatry, Fifth Edition. American Psychiatric Publishing, Inc. Arlington.

As per the APA practice guidelines, an attempt to taper or withdraw an antipsychotic medication in a patient with dementia with behavioral/psychological symptoms should begin at 4 months. During this taper, the APA also recommends that the patient be assessed for reemergence of symptoms at least monthly during the taper and for at least 4 months after discontinuation.

American Psychiatric Association. (2016). The American Psychiatric Association practice guideline on the use of antipsychotics to treat agitation or psychosis in patients with dementia. Amer J Psychiatry, 5(173);543–546.

Both anxiety and mood disorders increase after a TBI, whereas rates of psychotic, somatoform, eating and adjustment disorders appear comparable to the general population.

Silver JM, McAllister TW, Arciniegas DB. (2019). Textbook of Traumatic Brain Injury. American Psychiatric Association Publishing. Washington, D.C.

Engaging is a crucial process of establishing and building rapport in MI. Ways to improve rapport in MI include the use of
OARS (open questions, affirmations, reflections and summaries).

Levounis P. (2017). Motivational Interviewing for Clinical Practice. American Psychiatric Association. Arlington.

There are several instances when a psychiatrist can and is required to break confidentiality, such as the following: 1. When a patient presents a danger to themselves or others, 2. Mandatory reports of child/elder abuse, 3. Protecting a third party and 3. Proceedings where mental health issues are raised. Patients also waive their right to confidentiality when they ask to bring others into sessions, explicitly sign a release of information, or when they sue their providers.

Roberts LW. (2019). The American Psychiatric Association Publishing Textbook of Psychiatry (7th ed). American Psychiatric Association Publishing. Washington, D.C.

Schema or core beliefs are deep cognitive structures that contain the basic rules for screening, coding, and filtering information from the environment that allow an individual to process information and facilitate decision making. Although the schemas are largely influenced from early childhood experiences, subsequent formative influences can alter them.

Roberts LW. (2019). The American Psychiatric Association Publishing Textbook of Psychiatry (7th ed). American Psychiatric Association Publishing. Washington, D.C.

If a patient has comorbid schizophrenia and ADHD, it is important first to treat the symptoms of schizophrenia. If the patient continues to have significant ADHD symptoms, it is best to choose a non-stimulant option because stimulants may precipitate psychosis. Although stimulants can be trialed with patients with schizophrenia, non-stimulants would be the next best step.

Gough A, Morrison J. (2016). Managing the comorbidity of schizophrenia and ADHD. J Psychiatry Neurosci, 41(5);79–80.

Low potency phenothiazines (thioridazine, chlorpromazine, mesoridazine) are most closely associated with QTc prolongation. IV haloperidol has been shown to increase QTc. The oral formulation of haloperidol can as well, although to a much lesser extent. Atypical antipsychotics, while less likely, can also cause QTc prolongation. Ziprasidone and iloperidone have the greatest propensity.

Leucht S, Cipirani A, Spineli L, et al. (2013). Comparative efficacy and tolerability of 15 antipsychotic drugs in schizophrenia: a multiple treatments meta-analysis. Lancet, 382(9896);951-962.

. Lamotrigine is transmitted through breast milk in relatively high concentrations. Carbamazepine is considered safe, but there have been reports of hepatic dysfunction and transient seizure-like activity. Lithium is associated with several adverse effects, including cyanosis, poor muscle tone, and EKG changes. Most atypical antipsychotics have low milk/plasma ration and low infant serum levels.

Medications most commonly associated with weight gain include olanzapine, clozapine, risperidone, quetiapine, gabapentin, divalproex, and lithium. Medications associated with less weight gain include carbamazepine, lamotrigine, and ziprasidone.

Yatham LN, Kennedy SH, Parikh SV, et al. (2018). Canadian Network for Mood and Anxiety Treatments (CANMAT) and International Society for Bipolar Disorders (ISBD) 2018 guidelines for the management of patients with bipolar disorder. Bipolar Disorders, 20(2);140–144.

A meta-analysis of 16 randomized controlled trials comparing the above agents demonstrated a reduction in length of stay, duration of mechanical ventilation, and delirium incidence in patients who received dexmedetomidine versus the other agents.

Levenson JL. (2019). The American Psychiatric Association Publishing Textbook of Psychosomatic Medicine and Consultation-Liaison Psychiatry. American Psychiatric Association Publishing. Washington, D.C.

Usually after having their illness for longer than five years, Parkinson disease patients are at risk of developing cognitive impairment and psychosis manifested by hallucinations and delusions. The illness, its treatments that increase dopamine activity, or a combination of those factors may be responsible for the psychosis. The FDA has recently given pimavanserin (Nuplazid), a novel selective inverse agonist of the serotonin 5-HT2A receptor, an indication for suppression of hallucinations and delusions in Parkinson disease. Unlike most dopamine receptor antagonists used in this situation, pimavanserin does not increase Parkinson motor disability. Nevertheless, pimavanserin carriers a Black Box warning about the risk of death in elderly individuals with dementia. Clozapine (Clozaril) may be effective, but it has not received FDA indication for hallucinations and delusions in Parkinson disease. Moreover, potential dangers and rigorous monitoring requirements probably prevent physicians and their patients from using it. Quetiapine has received mixed reviews as to its effectiveness in suppressing hallucinations and delusions in Parkinson disease. Benzodiazepines may help anxiety and insomnia, but they carry a risk of amnesia, confusion, falls, and other side effects, especially in the elderly. Additional carbidopa-levodopa will likely increase hallucinations and delusions. In fact, one of the first strategies in combating hallucinations and delusions in Parkinson disease patients is to reduce the total dose of carbidopa-levodopa or, at least, avoid administering it during the evening.

Coombs BL, Coz AG. (2017). Update on the treatment of Parkinson disease psychosis. Neuropsychiatr Dis Treat, 13;737-744.

Mathis MV, Muoio BM, Anfreaso P, et al. (2017). The US Food and Drug Administration’s perspective on the new antipsychotic pimavanserin. J Clin Psychiatry, 78;e668-e673.

Moreno G, Gandhi R, Lessig S, et al. (2018). Mortality in patient with Parkinson disease psychosis receiving pimavanserin and quetiapine. Neurology, 91;797-799.

Probably because of their inattention and impulsiveness, student athletes with ADHD may have five times the risk of sustaining a concussion and they are more apt to have multiple concussions compared to non-ADHD student athletes. The sports with the greatest frequency of traumatic brain injury are boxing, football, wrestling, soccer, and hockey.

Iaccarino MA, Fitzgerald M, Pulli A, et al. (2018). Sport concussion and attention deficit hyperactivity disorder in student athletes. Neurol Clin Practice, 8;403-411.

Liou YJ, Wei HT, Chen, et al.(2018). Risk of traumatic brain injury among children, adolescents, and young adults with attention-deficit hyperactivity disorder in Taiwan. J Adolesc Health, 63;233-238.

He has evolved from coma, possibly through a vegetative state, to the minimally conscious state. Patients in both the persistent vegetative state and the minimally conscious state have their eyes open and maintain sleep-wake cycles. The essential difference in the first three levels is that those in coma have their eyes closed, are unaware of their surroundings and their situation, and they are unresponsive except for reflex activity; those in the persistent vegetative state have their eyes open, show roving eye movements that may briefly alight on the examiner or relative but do not establish eye contact, cannot communicate, and do not respond except for reflex activity; and those in the minimally conscious state also have their eyes open, but they show a modicum of ability to follow verbal or gestured requests and to communicate, and exhibit some purposeful behavior. In each of these altered levels of consciousness, patients lack capacity for making informed decisions.

Giacino JT, Ashwal A, Childs N, et al. (2002). The minimally conscious state: Definition and diagnostic criteria. Neurology, 58;349-353.

Rosenbaum AM, Giacino JT. (2015). Clinical management of the minimally conscious state. Handb Clin Neurol, 127;395-410.

This patient experiences visions, which she recognizes as hallucinations, in a hemianopic visual field. This constitutes the essence of Charles Bonnet syndrome, a classic neuropsychiatric disorder. Neurologists generally require that the patient has no confabulations, denial, or dementia. The Charles Bonnet syndrome most often follows a stroke that causes a homonymous hemianopsia, but visual loss from ocular disease, injury, or surgery may lead to this phenomenon. The hallucinations, which appear in blind areas, may be frightening or bothersome in some other way. Neurologists often suppress them with small doses of dopamine-blocking antipsychotic medicines, but ones that block serotonin have been suggested. Neurologists usually attribute the hallucinations to a deafferentation phenomenon in which the lack of visual input allows spontaneous visual cortex neuronal activity.

In contrast, Anton syndrome is a perceptual disorder in which patients with blindness, from either ocular or cerebral injury, believe and often act as if their vision were intact. Sometimes called visual agnosia, Anton syndrome induces denial, confabulations, and other defense mechanisms. Medications may calm the patient while physicians and family reassure and orient the patient.

Visual hallucinations also occur in the context of dementia or delirium. They are a hallmark of dementia with Lewy bodies disease and Parkinson disease dementia.
The clinical correlate of these relationships is that the physician who encounters a patient with visual hallucinations should test the visual acuity, visual fields, and cognitive function.

Chen JJ, Chang HF, Hsu YC, et al. (2015). Anton-Babinski syndrome in an old patient: A case report and literature review. Psychogeriatrics, 5;58-61.

Frenke M, Rauschenberger L. (2018). Challenges of diagnosing and treating Charles Bonnet syndrome. Neurology Practice 8;359-361.

Gold K, Rabins PV. (1998). Isolated visual hallucinations and the Charles Bonnet syndrome: A review of the literature and presentation of six cases. Compre Psychiatry, 30;90-98.

Schadlu AP, Schadlu R, Shepherd JB. (2009). Charles Bonnet syndrome: A review. Current Opinion Ophthalmology 20;219-222.

Vesicular monoamine transporter (VMAT), a transporter protein, brings dopamine from the cellular cytosol into vesicles of the presynaptic neuron’s terminal. Valbenazine (Ingrezza), tetrabenazine (Xenazine), and deutetrabenazine (Austedo) inhibit VMAT. By depleting presynaptic dopamine storage, these medicines reduce dopamine activity. Their primary clinical action reduces involuntary movements, such as oral-buccal-lingual form of tardive dyskinesia and chorea in Huntington disease. Haloperidol blocks dopamine receptors and thereby, in the short run, reduces the involuntary movements of tardive dyskinesia. In the long run, however, that strategy will reset the receptors and cause additional movement. Nevertheless, the general idea is that reduction in dopamine activity reduces involuntary movements. Thus, physicians prescribe medications that inhibit VMAT on an off-label basis to patients with Tourette disorder, hemiballismus, and athetosis.

Of the choices offered, valbenazine’s potential effect on cardiac rhythms is the most important. This and similar medications may also cause sleepiness, anticholinergic effects, parkinsonism, and even akathisia. Some medicines in this group carry a risk of suicidality and parkinsonism.

Caroff SN, Aggarwal S, Yonan C. (2018) Treatment of tardive dyskinesia with tetrabenazine or valbenazine. J Comp Eff Res, 7;135-148.

Epping EA, Kim JI, Craufurd D, et al. (2016). Longitudinal psychiatric symptoms in prodromal Huntington’s disease: A decade of data. Am J Psychiatry, 173;184-192.

Hauser RA, Factor SA, Marder SR, et al. (2017). KINECT 3: A phase 3 randomized, double-blind, placebo-controlled trial of valbenazine for tardive dyskinesia. Am J Psychiatry, 174;476-484.

Kenney C, Hunter C, Davidson A, et al. (2007). Short-term effects of tetrabenazine on chorea associated with Huntington’s disease. Mov Disord, 22;10-13.

Kenney C, Hunter C, Jankovic J. (2007). Long-term tolerability of tetrabenazine in the treatment of hyperkinetic movement disorders. Mov Disord 22;193-197.

Müller T. (2017). Valbenazine for the treatment of tardive dyskinesia. Expert Rev Neurother, 17;1135-1144.

Ondo WG, Hanna PA, Jankovic J. (1999). Tetrabenazine treatment for tardive dyskinesia: Assessment by randomized videotape protocol. Am J Psychiatry, 156;279-1281.
 

Blood lead levels should be determined because of the wide-spread behavioral, developmental, and cognitive disorders in these young children. The neurocognitive symptoms, their variable nature, and the constipation are consistent with lead poisoning. Although neuropsychologic and other testing is advisable, additional tests once a poisoning is suspected would delay diagnosis, identifying the source, and instituting treatment. The most common cause of lead poisoning had been young children eating lead-containing paint, but nowadays the most common cause is probably industrial pollution. Incidentally, the radiation required for head CT, which may be small, has nevertheless been associated with a significant risk for cancer in later life.

Bellinger DC, Stiles KM, Needleman HL. (1992). Low-level lead exposure, intelligence and academic achievement: A long-term follow-up study. Pediatrics, 90;855-861.

Canfield RL, Kreher DA, Cornwell C, et al. (2003). Low-level lead exposure, executive functioning, and early learning in childhood. Child Neuropsychol, 9;35-53.

Chandramouli K, Steer CD, Ellis M, et al. (2009). Effects of early childhood lead exposure on academic performance and behavior of school age children. Arch Dis Child, 94;844-848.

Hanna-Attisha M, Kuehn BM. (2016) Pediatrician sees long road ahead for Flint after lead poisoning crisis. JAMA, 315;967-969.

Liu J, Liu X, Wang W, et al. (2014). Blood lead concentrations and children’s behavioral and emotional problems. JAMA Pediatr, 168;737-745.