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Behavioral Neurology and Psychiatry ANDREW STEIN Question 1 A 63-year-old man is brought to the physician by his daughter because she is concerned about his memory loss over the past year. Yesterday, he could not remember his 18-month-old granddaughter's name. Although he denies any problem, she says he has been forgetful and becomes easily confused. He has lost his way twice while driving the last few months. There is no history of alcohol abuse. His temperature is 37°C, blood pressure is 118/84, pulse is 77 beats per minute, and respirations are 12 per minute. On mental status examination, his mood is normal. He is oriented to person and place, but initially gives the wrong month, which he is able to correct. He recalls from his youth in great detail, but only recalls 1 of 3 words after 5 minutes. He has difficulty recalling names of common objects and does not remember the name of the current US president. Physical examination, laboratory studies, and thyroid function tests show no abnormalities. What is the most likely diagnosis? A. masked depression B. Alzheimer's dementia C. Normal age associated memory decline D. Delirium because of her medical condition E. Depression Answer 1 Correct answer B. Alzheimer's dementia Typical early symptoms of Alzheimer's dementia include memory loss, inability to use judgment and make decisions, getting lost in familiar places, difficulty learning and remembering new information, difficulty expressing oneself, and decreased ability to full perform tasks like cooking a meal or managing finances. They also develop new behavior changes and withdrawal from friends and family. Hallucinations, delusions, and paranoia can occur in some people become verbally or physically aggressive or abusive. Eventually they become unable to perform basic tasks such as eating, dressing, and bathing. Some patients (less than 5%) can have seizures and end-stage disease. Question 2 "Witzelsucht" is a neurological symptom occurring secondary to lesions affecting which the following locations? A. Left parietal B. Right parietal C. Hypothalamic area D. Orbital frontal cortex E. Anterior singular gyrus Answer 2 answer D. Orbital frontal cortex "Witzelsucht"is a set of neurological symptoms characterized by the patient's uncontrollable tendency to pun, tell inappropriate jokes, and pointless or other irrelevant stories at inappropriate times. The patient usually finds these utterances particularly amusing. It is associated with small lesions of the orbital frontal cortex. Question 3 When a person acts intentionally to produce a symptom without obvious personal or financial benefit, it is called: A. malingering B. Factitious disorder C. Conversion D. Gancer syndrome E.Munchausene by proxy Answer 3 Correct answer: B. Factitious disorder It is important to distinguish between these diagnoses. factitious disorder is an umbrella term to describe conditions were patient's intentionally act as physically or mentally ill without obvious benefits. Malingering is present when the person has obvious secondary benefits from his or her sick role. Conversion is when the patient unconsciously produces psychological symptoms. Gancer syndrome is a disorder that has been classified as a factitious disorder or dissociative disorder. It is characterized by pseudo-stupidity, and it has also been named "the syndrome of approximate answers". Answers given by the patient will not be correct, but approximately close enough to the question to give an indication that the patient understood the question. Munchausen syndrome is when the person makes up symptoms of illness in him or herself to obtain sympathy and comfort for others. It is by proxy, when a parent, usually the mother, presents with frequent hospital visits for nonexisting complaints with her child. mother is often a healthcare provider and a dysfunctional family situation is present. The mother seems to be gaining sympathy or comfort from others. Question 4 A 68-year-old male is referred to you in clinic for progressive memory loss and visual hallucinations. His wife accompanies him today at the visit and explains that his symptoms started about one year ago. At that time, he insisted there was a parked outside his house and he was sure that it was his son's car. The wife explains there was no car outside and that his son lives 100 miles away and has not visited them in a while. She also describes that he has been having difficulty managing the finances. One peculiar aspect of her husband's behaviors that at times he appears completely lucid, but a couple hours later he would be staring at her not responding to her questions. He has a history of hypertension and asthma. His vital signs are normal. He is well groomed and pleasant and cooperative. It is remarkable for mild rigidity of the upper extremities. He is unable to copy a clock or 2 intersecting pentagon figures. The type of dementia that you are suspecting has, pathologic features associated with which of the following disorders? A. Alzheimer's disease B. Inclusion body myositis C. Parkinson's disease D. Cortical Basal ganglionic degeneration E. Pick's disease Answer 4 C. Parkinson's dementia with Lewy bodies (DLB) is being described.both this and Parkinson's disease has Lewy bodies. With DLB lewy bodies are in the substantia nigra and in the locus coeruleus, dorsal raphe nuclei, and throughout the neocortex. Symptoms result mainly from frontal lobe dysfunction. Distinguishing features of this are 1. fluctuations in cognitive function 2. visual and non-visual hallucinations 3, extrapyramidal features (such as rigidity, tremor) Question 5 A 40-year-old woman comes to you with sadness and frequent crying spells. she reports difficulty sleeping, decreased appetite, and generalized fatigue. She reports loss of pleasure for daily activities. She denies suicidal ideations. There is no apparent immediate precipitating factor for the symptoms. According to the DSM-IV criteria, how long do these symptoms have to be present for the diagnosis of major depression? A. 1 week B. 2 weeks C. 1 month D. 2 months E. 6 weeks Answer 5 Answer: B: 2 weeks. Question 6 Psychiatry was consulted for 26-year-old mother of 2. She is admitted under the trauma service after suffering second-degree burns to the back of her neck and arms. 3 days back she was returning home from the corner store after buying groceries when she was assaulted by an unknown male and a chemical, likely acid, was poured on her neck causing the burns. She says that during these events, she felt she was dissociated from the events and felt that these events were happening to "another person". once in the hospital, she started developing flashbacks. She would wake up from sleep in a cold sweat and could feel his fingers on her neck and his breath on her face. She found it difficult to go back to sleep and reports decreased appetite. There is no apparent primary or secondary gain. Examination reveals a mildly obese lady who is dysphoric. She has a labile affect, but has no delusions or hallucinations. Her thought content is appropriate and she has a clear sensorium. She has second-degree burns on the back of her neck. Which of the following is the likely diagnosis? A. acute stress reaction B. PTSD C. Malingering D. Schizophrenia E. Somatization Disorder Answer 6 The correct answer is A. Acute stress reaction Acute stress reaction: requires a dramatic event, which was experienced or exposed to and a feeling of helplessness. There also must be a feeling of dissociation from the event (depersonalization, amnesia, derealization) . There is also reexperience as intrusive thoughts of flashbacks, avoidance of the stimulus, marked anxiety, and resulting in significant social or functional disability. symptoms occur within 4 weeks and need to be present for 2 days to 4 weeks. PTSD is similar , but dissociation is not needed to make the diagnosis. The symptoms must also be present for more than 1 month. Question 7 A 72-year-old man is brought to attention because of his unusual behavior for the past 6 months. His family noticed a change from previously normal behavior. He now frequently curses, has sudden mood changes, and bursts easily until after tears without apparent cause. Neuro imaging reveals prominent frontotemporal atrophy. This is characteristic of which are the following conditions? A. Alzheimer's dementia B. schizophrenia C. factitious disorder D. diffuse lewy body disease E. Pick disease Answer 7 Answer: E Pick Disease Pick disease is one of frontotemporal dementias. It presents with behavioral changes and difficulty with organization and executive function. Imaging usually shows atrophy predominantly the frontal and temporal lobes. Question 8 Aprosodia has been associated to lesions involving which of the following brain regions? A. left parietal B. right occipital C. left frontal D. right frontal E. anterior cingulate Answer 8 Correct answer D: Right frontal Aprosodia is lack of variation and normal speech, characterized just, such as speed, tone, and varied since. Clinical and imaging studies suggest that this localizes to the posterior inferior frontal lobe of the nondominant hemisphere. Question 9 Which of the following is the most common psychiatric condition associated with catatonia? A. Bipolar disorder B. major depression C. Schizophrenia D. dementia not otherwise specified E. bereavement Answer 9 Correct Answer: A. Bipolar Disorder An excited state of catatonia is usually associated with bipolar disorder. This is generally characterized by impulsivity, combativeness, and autonomic instability. Catatonia is a symptom which can be seen in psychiatric diagnosis is characterized by immobility and rigid tone in the property of "waxy flexibility", which implies that you can change the position of a limb, the patient maintains that position. patient's can remain in abnormal flexed positions for hours to months. Commonly associated conditions with catatonia are schizophrenia, mood disorders, and general medical conditions. The most common is mood disorders (25-50%) followed by schizophrenia (10-15%). Among the mood disorders, bipolar is most commonly associated with catatonia. Question 10 Which findings has been most consistently seen in schizophrenic patients? A. increase in ventricular size B. decrease in ventricular size C. frontal atrophy D. temporal and occipital atrophy E. frontal and hippocampal atrophy Answer 10 Correct answer: A Increase in ventricular size studies have demonstrated anatomical abnormalities in patients with schizophrenia. Bilateral ventriculomegaly occurs medial temporal areas such as the hippocampus and amygdala show decreased brain volume. Question 11 65-year-old right-handed man presents with an acute onset of inability to speak. He has hypertension and diabetes and was in otherwise good state of health. His wife who accompanies him reports that he was playing chess in the morning when he suddenly could not speak or make intelligible sounds. On examination, his vitals are pulse of 92 bpm and blood pressure of 130/70. His fundus and eye exam otherwise normal. There is mild right facial paresis of the upper motor neuron type and a 4+ out of 5 weakness in the right arm. He is unable to speak any words, but has intact comprehension. He is able to repeat sentences. He is able to clearly write what a full sentences with intact grammar. Which of following describes this phenomena? A. Broca's aphasia B. Wernicke's aphasia C. Aphemia D. Amusia E. Aprosodia Answer 11 Correct answer: C. Aphemia. This is caused by a lesion, usually vascular, involving the dominant frontal lobe. This is characterized by complete inability to speak, but preserved comprehension and repetition and ability to read and write. There is a good prognosis usually and there is often complete recovery in aperiod of weeks to months. Amusia is an inability appreciate music is caused by a lesion in the nondominant hemisphere analogous to the one just area on the dominant side. Aprosodia is the lack of musicality of speech, and is caused by a lesion in the non-dominant hemisphere analagous to the Broca's area on the dominant side. Question 12 You are seeing a 35-year-old woman in your clinic with the diagnosis of epilepsy. She has been suffering from complex partial seizures since the age of 5. Today, she complains of sadness and frequent crying spells, difficulty sleeping, decreased appetite and generalized fatigue. She denies suicidal ideation. Which of the following statements is true regarding the likely diagnosis? A. depression is the most common psychiatric disorder among patients with epilepsy B. after anxiety disorder, depression is the 2nd most common psychiatric disorder among patients with epilepsy C. epilepsy patients rarely have depression D. depression and bipolar are the most common psychiatric disorders among patients with epilepsy E. after bipolar disorder, depression is the 2nd most common psychiatric disorder among patients with epilepsy Answer 12 Correct answer: A. Depression is most common depression is a common disorder among epileptic patients and constitutes the most frequent psychiatric condition in these patients. The relationship between depression and epilepsy is bidirectional, since patients with major depression also have a high-risk of epilepsy. Question 13 An 8-year-old boy is brought to office by his mother who states that he is constantly getting in trouble at school. His teacher states that he keeps getting out of his seat at inappropriate times, fidgets with things in his tests and does not pay attention in class. He does poorly on examinations, but his mother thinks he is very smart. At home he is very active, was playing outside, but only watches TV for a few minutes at time and does not appear to be listening when she reads him stories. He constantly forgets his backpack at school and sometimes forgets to get off the bus at his normal stop. He has a normal physical examination and is age-appropriate developmentally. He does not stay still in the examination room and is contantly running and climbing on and off the examination table. He seems very bright and inquisitive, but is easily distracted. An appropriate step in treatment would be A. behavioral modifications including goalsetting, and some punishments B. methylphenidate C. behavioral modifications and methylphenidate D. no treatment is necessary at this time as this is normal behavior for an 8-year-old boy E. psychiatric consult Answer 13 Correct answer: C Behavioral modifications and methylphenidate this is classic ADHD. The standard treatment is with stimulant drugs and behavioral modifications. Question 14 The 58-year-old wife of this 60-year-old patient is contemplating divorce from her husband of almost 40 years. She says that beginning about a year ago, they had to stop going out with her friends as her husband was an embarrassment to be around him public. He will be combative when he disagreed with friends opinions and would yell obscenities at waiters when kept waiting to have his order taken. In the past month, he has stopped showering, and has reread his favorite novel over 10 times. In a last ditch effort to save their marriage, his wife brings him to couples counseling, and the psychologist refers her to note the neurology clinic at the local hospital. Which of the following is a likely diagnosis? A. Tourette syndrome B. schizophrenia C. normal aging D. Alzheimer's disease E. frontotemporal dementia Answer 14 Correct answer: E. frontotemporal dementia This usually presents from age 40-60 with gradual progressive changes in behavior or gradual, progressive language dysfunction. The most common presentation is change in social and personal contact, characterized by difficulty modulating behavior to social commands. As the disease progresses, criminal behavior like shoplifting may develop Question 15 Which of the following types of dementia is associated with the development of new musical and artistic abilities? A. Alzheimer's disease B. Lewy body dementia C. frontotemporal dementia D. Huntington disease E. corticobasal ganglionic degeneration Answer 15 Answer: C. Frontotemporal dementia Frontotemporal dementias characterized by apathy, disinhibition, planning and organizing difficulties. Curiously the development of new musical and artistic ability has been associated with frontal temporal dementia. Question 16 Which are the following is most characteristic feature of borderline personality disorder? A. obsessive-compulsive disorder B. dependency C. irritability D. instability of mood E. flat affect Answer 16 Answer D. Instability of mood Borderline personality disorder includes instability of mood, extreme splitting, unstable interpersonal relationships. Question 17 Familial forms of Alzheimer's disease associated with presenilin 1 and 2 are linked to which of the following chromosomes? A. Chromosome 21 B. Chromosome 1 and 14 C. Chromosome 19 D. Chromosome 21 and 19 E. Chromosome 7 Answer 17 Correct answer B: Chromosome 1 and 14. this accounts for less than 7% of all cases of Alzheimer's disease. Mutations of presenilin 1 gene (on chromosome 14) and prenilin 2 (on chromosome 1) are linked to familial cases of Alzheimer's disease. Question 18 A 45-year-old HIV positive man presents to the office with a six-month history of progressive memory loss and difficulty writing. He is currently taking Zidovudine and a protease inhibitor. CD4 count is 25. Neurological exam elicits psychomotor delay and impairment in fine motor control. MRI demonstrates moderate brain atrophy, but no focal lesions. Laboratory results of his CSF analysis are within normal limits. What is the most likely diagnosis? A. CMV encephalitis B. cryptococcal meningoencephalitis C. HIV encephalopathy D. primary primary lymphoma E. progressive multifocal leukoencephalopathy Answer 18 Answer: C. HIV encephalopathy HIV encephalopathy is also known his AIDS dementia complex, which is a subacute inflammatory infiltration of the brain caused by direct spread of HIV to the CNS. It is characterized by cognitive impairment, psychomotor delay, incontinence, impairment of motor skills, and confusion. CMV encephalitis typically affects the periventricular white matter and retina. CSF analysis in cryptococcal meningitis would demonstrate organisms. Primary brain lymphoma would present as a mass on MRI. Progressive multifocal leukoencephalopathy would present with multifocal white matter lesions on MRI. Question 19 A 55-year-old male presents the doctor with headache and personality change over the past 3 months. Neurologic exam reveals right upper extremity weakness. Neuroimaging reveals a 5 cm, welldefined mass in the left frontal white matter. T1-weighted postcontrast MRI images of the brain reveal heterotopic jean Easley ring enhancing lesion, while T2 demonstrates significantly surrounding edema. Laboratory data are within normal limits, including a negative HIV test. Which of the following is the most likely diagnosis? A. cerebral toxoplasmosis B. demyelinating disease C. glioblastoma multiforme D. infarct E. primary brain lymphoma Answer 19 Correct answer C. Glioblastoma multiforme. This is a classic presentation of glioblastoma multiforme. Typically patients present with symptoms less than 3 months duration. Metastasis and abscess should also be in the differential. cerebral toxoplasmosis is unlikely given the patient is HIV negative, Question 20 20. which are the following is included in the cluster B personality disorders? A. schizoid B. schizotypal C. paranoid D. borderline E. dependent Answer 20 Correct answer: D. Borderline in the DSM-IV, personality disorders are classified into A, B, and C clusters. Cluster A disorders are the "odd" or "eccentric" group and include schizoid, schizotypal, and paranoid. Cluster B disorders are the "dramatic" group and include borderline, histrionic, antisocial, and narcissistic varieties. Cluster C disorders are the "fearful/anxious" group and include Avoidant, Depentent, and Obsessive-compulsive types. Thank you for coming. Questions are from neuroprep Any questions?