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Buddhism and the Rite Resident Friday AM Lecture What is Buddhism • A religion (ish) that dates back 2,500 years and has approximately 300 million followers around the world • Founded by Siddhartha Gotama upon his enlightenment under a fig tree at the age of 35 • Buddhism teaches that the solution to our problems can only be found within ourselves • When attachment to all worldly desires including the idea of self is relinquished Nirvana can be attain • The core of Buddha's teachings are encompassed in the four noble truths and the eightfold path The Four Noble Truths 1. Dukkha – life is suffering, impermanent and fleeting 2. Samudaya – the arising of dukkha is contained in thirst, carving, desire and attachment 3. Nirodha – the cessation of dukkha is nirvana 4. Magga – the eightfold path towards cessation of dukkha The Eightfold Path 1. Right Understanding 2. Right Thought 3. Right Speech 4. Right Action 5. Right Livelihood 6. Right Effort 7. Right Mindfulness 8. Right Concentration The Four Truths of the RITE 1. The RITE is suffering 2. The RITE is predictable 3. You have to take the RITE 4. Yojana (skrt) – the plan to do well on the RITE The Eightfold Plan 1. RITE Attitude - you’re the smartest! 2. RITE Approach – start studying early 3. RITE Material – prior answers keys are gold 4. RITE Practice – go through old images 5. RITE Bathroom breaks – don’t break the seal! 6. RITE Fuel – nuts, berries, grains, water, eggs and greens 7. RITE Energy - bring your stamina 8. RITE celebration (when it’s over) A patient underwent surgery to remove a colloid cyst which was causing obstructive hydrocephalus. Following the procedure she was unable to create new memories. Her ability to name objects and perform tasks was unaffected. Which part of the brain was likely damaged during the surgery? 1. Anterior temporal lobe 2. Basal ganglia 3. Fornix 4. Orbito-frontal lobe A patient underwent surgery to remove a colloid cyst which was causing obstructive hydrocephalus. Following the procedure she was unable to create new memories. Her ability to name objects and perform tasks was unaffected. Which part of the brain was likely damaged during the surgery? 1. Anterior temporal lobe 2. Basal ganglia 3. Fornix 4. Orbito-frontal lobe Damage to the fornix can occur with transcallosal surgery to remove a colloid cyst of the third ventricle, which interrupts the Papez circuit and results in loss of the ability to form new memories. The other structures, while important for memory, would not be affected by a transcallosal procedure. This individual has problems with procedural memory, that is the ability to acquire (with practice) cognitive and behavioral skills that subsequently operate automatically. Procedural memory is associated with lesions of the basal ganglia-cerebellum supplementary motor area system. Procedural memory problems are commonly reported in patients with Parkinson disease (PD)and affect tasks such as playing a musical instrument or golfing. Episodic memory localizes to the medial temporal lobe-Papez circuit system, semantic memory to the anterior and inferior temporal lobes, and working memory to the prefrontal cortex subcortex-parietal-association cortex system. Balint syndrome localizes to the posterior parietal regions and consists classically of the triad of optic ataxia, ocular dysmetria and asimultagnosia. Episodic memory deficits are typical of Alzheimer disease (AD), as is Balint syndrome. Semantic memory deficits are typical of Alzheimer disease and frontotemporal lobar degeneration (FTD). Working memory deficits can result from multiple cerebrovascular events, AD, FTD, PD, dementia with Lewy Bodies, and traumatic brain injury. Papez Circuit - Part of the limbic system - Important for control of emotion and episodic memory A lesion at what level of the brainstem can cause decerebrate posturing? 1. 2. 3. A lesion at what level of the brainstem can cause decerebrate posturing? 1. 2. 3. The lateral vestibulospinal tract facilitates extensor muscle tone, whereas the rubrospinal tract facilitates flexor tone. A lesion between the red nucleus and vestibular nuclei can result in decerebrate posturing. A 55-year-old male is recovering from a left ACA ischemic infarct. He has severe RLE weakness and his family notes that the he is quite apathetic and not engaged in rehab. Which of the following medications can be used to treat his behavioral changes. 1. Quetiapine 2. Methylphenidate 3. Sertraline 4. Mirtazapine A 55-year-old male is recovering from a left ACA ischemic infarct. He has severe RLE weakness and his family notes that the he is quite apathetic and not engaged in rehab. Which of the following medications can be used to treat his behavioral changes. 1. Quetiapine 2. Methylphenidate 3. Sertraline 4. Mirtazapine Dopaminergic agonists and inhibitors of dopamine reuptake have been used to treat the behavioral traits associated with medial frontal syndrome. Methylphenidate is such a drug. This syndrome may be seen after ischemia in the distribution of the anterior cerebral artery. A 75 year old man suffers are large L MCA stroke. PET scan reveals hypometabolism in the left MCA territory. A PET scan would also be expected to show hypometabolism in what structure? 1. Contralateral frontal eye field 2. Contralateral cerebellum 3. Contralateral midbrain 4. Contralateral thalamus A 75 year old man suffers are large L MCA stroke. PET scan reveals hypometabolism in the left MCA territory. A PET scan would also be expected to show hypometabolism in what structure? 1. Contralateral frontal eye field 2. Contralateral cerebellum 3. Contralateral midbrain 4. Contralateral thalamus The contralateral cerebellar hemisphere (in this case, the left cerebellar hemisphere) is engaged with motor tasks planned and executed from the opposite cerebral hemisphere. Activation of a cerebral cortical motor strip is accompanied by activation of the contralateral cerebellar hemisphere. Conversely, if the motor areas of a cerebral hemisphere are damaged, the contralateral cerebellar hemisphere shows a reduction in activity. This is also called cerebellar diaschesis. The synapse for deep tendon reflexes is located where in the nervous system? 1. Intermediate column of the spinal cord 2. Substantia gelatinosa 3. Doral root ganglion 4. Ventral horn of the spinal cord The synapse for deep tendon reflexes is located where in the nervous system? 1. Intermediate column of the spinal cord 2. Substantia gelatinosa 3. Doral root ganglion 4. Ventral horn of the spinal cord The synapse of the afferent axons for the muscle stretch reflex is located in the anterior (ventral) horn. The muscle stretch reflex is monosynaptic directly from sensory neuron to an alpha motor neuron. The dorsal root ganglion contains cell bodies of the pseudounipolar sensory neurons. The intermediate column contains synapses related to sympathetic function while the substantia gelatinosa is the site of synapse for afferent fibers which will form the spinothalamic tract. The substantia gelatinosa sits within the zone of Lissauer. A 75 year old women is brought to your office by her husband for evaluation of memory changes. On your exam you notice that the relaxation phase of her reflexes is prolonged. Which additional lab finding could suggest an etiology to explain her exam and subjective memory concerns? 1. Elevated 24 hour urine copper 2. Elevated TSH 3. Anemia 4. Elevated MCV A 75 year old women is brought to your office by her husband for evaluation of memory changes. On your exam you notice that the relaxation phase of her reflexes is prolonged. Which additional lab finding could suggest an etiology to explain her exam and subjective memory concerns? 1. Elevated 24 hour urine copper 2. Elevated TSH 3. Anemia 4. Elevated MCV Decreased hemoglobin and elevated mean corpuscular volume are compatible with a macrocytic anemia that may be secondary to vitamin B12 deficiency. Vitamin B12 deficiency, in turn may cause combined system degeneration (affecting posterior columns and lateral corticospinal tracts). Dementia and peripheral neuropathy may also be seen. Copper metabolism is altered in Wilson's disease, which is accompanied by a movement disorder. In hypothyroidsim, the relaxation phase of reflexes is prolonged, the reflexes are not hyperactive. This patient most likely has hypothyroidism, which may present with a dementing illness and systemic symptoms including weight gain, hoarseness, lethargy, and cold intolerance. Screening for hypothyroidism is considered standard for patients presenting with dementia. An 8 year boy is brought to your office for evaluation of abnormal movements. 9 months ago the patient started snapping followed by jerking of his head. Later in the course he was noted to make inappropriate squeeking sounds. The child stated that if he did not perform these movements and make these sounds he would feel very uncomfortable until he finally perform the action. Which of the following is the correct diagnosis? 1. Tourette’s syndrome 2. Motor stereotopy 3. Transient Tic Disorder 4. complex motor and vocal tic disorder An 8 year boy is brought to your office for evaluation of abnormal movements. 9 months ago the patient started snapping followed by jerking of his head. Later in the course he was noted to make inappropriate squeeking sounds. The child stated that if he did not perform these movements and make these sounds he would feel very uncomfortable until he finally perform the action. Which of the following is the correct diagnosis? 1. Tourette’s syndrome 2. Motor stereotopy 3. Transient Tic Disorder 4. complex motor and vocal tic disorder A diagnosis of Tourette's disorder is made when a patient has a history of tics for over year and at some point in the course has had the presence of motor tics and vocal tics. The onset of symptoms has to be before the age of 18. Regardless of whether or not there are motor or vocal tics (or both), if symptoms have been under a year, the diagnosis of Transient Tic Disorder is given. There is no diagnosis of "Complex motor and vocal tic disorder," but the tics in the patient are all simple tics anyway. A 65-year-old male with an extensive psychiatric history is seen in your clinic for evaluation of memory loss. On exam you notice that he has acne and a very fine resting tremor, intention tremor and action tremor. Which of the following medications can produce side effects to explain his symptoms? 1. Clonazepam 2. Buproprion 3. Lamotrigine 4. Lithium A 65-year-old male with an extensive psychiatric history is seen in your clinic for evaluation of memory loss. On exam you notice that he has acne and a very fine resting tremor, intention tremor and action tremor. Which of the following medications can produce side effects to explain his symptoms? 1. Clonazepam 2. Buproprion 3. Lamotrigine 4. Lithium Well-known side effects of lithium therapy include dyspepsia, nausea, vomiting, diarrhea, hair loss, acne, tremor, decreased cognition, and incoordination. You are seeing a 55 year old patient in clinic referred by her PCP with specific concern for dementia. You are struck by her language difficulties without deficits in any other cognitive domain. On exam she can name single objects. Her speech is generally very fluent, but she will occasionally pause to find a word. Repetition of multi-clause sentences is quite impaired. Post-mortem pathology is most likely to show? 1. NF tangles and amyloid plaques 2. TDP-43 inclusions 3. Lewy bodies 4. Negri bodies You are seeing a 55 year old patient in clinic referred by her PCP with specific concern for dementia. You are struck by her language difficulties without deficits in any other cognitive domain. On exam she can name single objects. Her speech is generally very fluent, but she will occasionally pause to find a word. Repetition of multi-clause sentences is quite impaired. Post-mortem pathology is most likely to show? 1. NF tangles and amyloid plaques 2. TDP-43 inclusions 3. Lewy bodies 4. Negri bodies Logopenic progressive aphasia (LPA) is one of several different progressive language disorders (semantic dementia, progressive anarthria, progressive nonfluent aphasia are some of the others). The pathologic findings in LPA are generally the same of patients with Alzheimer's disease. Hypometabolism is seen in the left temporal/parietal regions. Bilateral anterior temporal hypometabolism is more common in patients with semantic dementia, whereas the left frontal opercular region is affected in patients with progressive nonfluent aphasia. - Semantic variant is most commonly TDP-43 pathology. - Non-fluent variant is usually FTD-tau pathology - Logopenic is usually AD pathology A 59 year old male presents to clinic with his wife with behavior changes. He is sleeping all day, not changing his clothes and has become very impulsive. On exam you notice wasting of his intrinsic hand muscles and fasciculations throughout the muscles of his arms and legs. His reflexes are quite brisk. Post-mortum pathology is most likely to show inclusions composed primarily of what? 1. Tau 2. beta-amyloid 3. TDP-43 4. alpha-synuclein A 59 year old male presents to clinic with his wife with behavior changes. He is sleeping all day, not changing his clothes and has become very impulsive. On exam you notice wasting of his intrinsic hand muscles and fasciculations throughout the muscles of his arms and legs. His reflexes are quite brisk. Post-mortum pathology is most likely to show inclusions composed primarily of what? 1. Tau 2. beta-amyloid 3. TDP-43 4. alpha-synuclein This man best fits the diagnosis of frontotemporal dementia (FTD) with motor neuron disease. This condition is characterized by personality changes prior to memory disturbances typically before age 65 and development of upper and lower motor neuron deficits (amyotrophic lateral sclerosis). Frontotemporal dementias are heterogeneous with the two most common protein accumulations in these cases consisting of tau or TDP-43. Nearly all of the cases of FTD with motor neuron disease have TAR DNA-binding protein 43 (TDP-43) protein accumulations. Alphasynuclein inclusions are mostly seen in Parkinsonian dementias. Amyloid is seen in Alzheimer disease cases. Vascular dementia will show ischemic changes. Which of following tests is recommend for all children with autism spectrum disorder? 1. CSF profile 2. MRI brain 3. 2 hour sleep deprived EEG 4. Genetic testing Which of following tests is recommend for all children with autism spectrum disorder? 1. CSF profile 2. MRI brain 3. 2 hour sleep deprived EEG 4. Genetic testing Routine genetic testing is recommended for all children with autism spectrum disorder. Brain imaging, EEG and CSF analysis are not routinely indicated unless there is a clear history of regression, or there are focal deficits or possible seizure like episodes. The most common cause of inherited mental retardation is fragile X syndrome. Nearly all affected boys manifest attention deficit disorder and have learning disabilities. The most frequent neurocognitive symptoms are difficulty with abstract reasoning, complex problem solving, and expressive language. Many will also show manifestations of autism, with 33% meeting criteria for autism. Female carriers can have a milder form of the disease, with learning disabilities; about 50% of them will manifest attention deficit disorder. Characteristic physical features include a long thin face, prominent forehead and jaw, protuberant ears, hip dislocation, and club feet. A 70 year old male presents with falls. On exam you notice mild bradykinesia. He has a wide based ataxic gait. He scores a 24/30 on MMSE. Family history is notable for two grandsons with mental retardation. His MRI is show. Which of the following is the most likely diagnosis? 1. Freidriech ataxia 2. Charcot Marie Tooth 3. FAXTAS 4. Spinocerebellar ataxia 5. Dentatorubral-pallidoluysian atrophy A 70 year old male presents with falls. On exam you notice mild bradykinesia. He has a wide based ataxic gait. He scores a 24/30 on MMSE. Family history is notable for two grandsons with mental retardation. His MRI is show. Which of the following is the most likely diagnosis? 1. Freidriech ataxia 2. Charcot Marie Tooth 3. FAXTAS 4. Spinocerebellar ataxia 5. Dentatorubral-pallidoluysian atrophy This patient most likely has fragile X-associated tremor ataxia syndrome (FXTAS). He is not likely to have Friedreich ataxia, dentatorubralpallidoluysian atrophy, or spinocerebellar ataxia type 3 given his age, absence of other clinical features, and the history of mental retardation in his daughter's son. Patients with CharcotMarie-Tooth disease may have postural and action tremor but should not have cerebellar ataxia. FAXTAS - FMR1 gene CGG repeats 55-200 can present with FAXTAS - > 200 repeats = MR - Can present in males or females usually > 55 years of age - Classic features: kinetic tremor, cerebellar ataxia, cognitive decline, parkinsonism, psychiatric symptoms (anxiety, mood lability, apathy) - Other less common features: impotence and autonomic dysfunction, neuropathy - “MCP” sign : bilateral T2 middle cerebellar peduncle hyperintensities, present in ~ 60% cases - Another clue in women is premature ovarian failure Of the following anti-psychotics, which of the following is associated with the most weight gain? 1. Olanzapine 2. Quetiapine 3. Haldol 4. Clozapine Of the following anti-psychotics, which of the following is associated with the most weight gain? 1. Olanzapine 2. Quetiapine 3. Haldol 4. Clozapine Although all atypical antipsychotics and many typicals are known to cause weight gain and diabetes mellitus, numerous studies in both dementia and schizophrenia have shown that olanzapine causes the most weight gain. Which of the following antipsychotics is most commonly associated with seizures? 1. Olanzapine 2. Quetiapine 3. Haldol 4. Clozapine Which of the following antipsychotics is most commonly associated with seizures? 1. Olanzapine 2. Quetiapine 3. Haldol 4. Clozapine Although much emphasis is placed on its causing agranulocytosis, seizures occur more frequently with the use of clozapine than with other atypical antipsychotics. An 80 year old female was referred to your clinic for hallucinations. She has a long history of macular degeneration and poor vision at baseline. With minimal assistance, however she is able to live mostly independently. She has no history of any cognitive issues. Her neurologic exam other than poor vision is normal and her MMSE score is 28/30. Which of the following is the most likely diagnosis for her hallucinations? 1. Charles bonnet syndrome 2. Anton syndrome 3. Peduncular hallucinosis 4. Diffuse Lewy body disease An 80 year old female was referred to your clinic for hallucinations. She has a long history of macular degeneration and poor vision at baseline. With minimal assistance, however she is able to live mostly independently. She has no history of any cognitive issues. Her neurologic exam other than poor vision is normal and her MMSE score is 28/30. Which of the following is the most likely diagnosis for her hallucinations? 1. Charles bonnet syndrome 2. Anton syndrome 3. Peduncular hallucinosis 4. Diffuse Lewy body disease Charles Bonnet syndrome involves visual hallucinations without other psychopathology. It is seen in the elderly and is due to visual loss, with the most common pathology being macular degeneration. The other clinical exam findings are not usually identified with Charles Bonnet syndrome. - Anton syndrome: cortical blindness but agnostic to the fact that they are blind - Peduncular hallucinosis: damage to the midbrain which causes well formed hullicinations. Usually patient knows they are hallucinations (similar to CBS). Thought to be due to release of reticular activating system control. A 43 year old women is recovering from HSV encephalitis. While in acute rehab her providers notice that she has an excessive fascination with objects and has to touch them and often puts them in her mouth. Prior to her hospitalization she was a very strong willed CEO of a major company and prided herself on her physical appearance. She is now very docile, eating an excessive amount and gaining weight. Her symptoms are best described by which of the following? 1. Foix-Chavany-Marie 2. Foster-Kennedy 3. Klein-Levin 4. Kluver-Bucy syndrome A 43 year old women is recovering from HSV encephalitis. While in acute rehab her providers notice that she has an excessive fascination with objects and has to touch them and often puts them in her mouth. Prior to her hospitalization she was a very strong willed CEO of a major company and prided herself on her physical appearance. She is now very docile, eating an excessive amount and gaining weight. Her symptoms are best described by which of the following? 1. Foix-Chavany-Marie 2. Foster-Kennedy 3. Klein-Levin 4. Kluver-Bucy syndrome This syndrome is known as Klüver-Bucy syndrome and occurs as a result of bilateral temporal lesions involving amygdalae. Hypermetamorphosis, a common feature, involves excessive fascination with objects in the patient's environment and the urge to touch them all. -Foster-kennedy : optic atrophy and anosmia due to sphenoid or parasellar tumor -Foix-chavany-Marie: bilateral opercular damage which causes facial diplegia and drooling - Klein-Levin: sleeping beauty syndrome A 41 year old female was hospitalized for the treatment of paranoia and personality changes. She had a history of medically refractory epilepsy that came under very good control with lacosamide. There was no prior psychiatric history. A routine EEG was obtained which had a normal background rhythm and no epileptiform activity. A thorough medical work up was negative for any toxic/metabolic/infectious cause of her symptoms. Structural brain imaging was unchanged from previous evaluations. Which of the following could be an explanation for her symptoms. 1. Ictal psychosis 2. Forced normalization 3. Limbic encephalitis 4. medication induced psychosis A 41 year old female was hospitalized for the treatment of paranoia and personality changes. She had a history of medically refractory epilepsy that came under very good control with lacosamide. There was no prior psychiatric history. A routine EEG was obtained which had a normal background rhythm and no epileptiform activity. A thorough medical work up was negative for any toxic/metabolic/infectious cause of her symptoms. Structural brain imaging was unchanged from previous evaluations. Which of the following could be an explanation for her symptoms. 1. Ictal psychosis 2. Forced normalization 3. Limbic encephalitis 4. medication induced psychosis Forced normalization refers to a psychosis occurring after achievement of good clinical seizure control, or resolution of interictal epileptiform discharges. Seizures are basically ECT? An 18 year old female presents with ptosis, difficulty breathing, and poor pupillary light response that progressed over the course of 24-48 hours. Her symptoms are most concerning for which of the following etiologies? 1. Botulism 2. Myasthenia-gravis 3. Guillain-Barre 4. Diptheria An 18 year old female presents with ptosis, difficulty breathing, and poor pupillary light response that progressed over the course of 24-48 hours. Her symptoms are most concerning for which of the following etiologies? 1. Botulism 2. Myasthenia-gravis 3. Guillain-Barre 4. Diptheria Botulism causes pupil dilation due to its effect on both muscarinic and nicotinic cholinergic nerve terminals. Other disorders that may acutely produce this constellation of symptoms, for example myasthenia gravis, Guillain Barre syndrome and diptheria, are typically not associated with pupillary abnormalities. Autonomic dysfunction can be seen with Guilain Barre syndrome and diptheria but usually not early on. The same 18 year old patient with botulism is admitted to the hospital and you were rightly concerned about her ability to ventilate. Which of the following is the best predictor of the need for intubation? 1. Hypoxia on blood gas 2. Hypercarbia on blood gas 3. FVC 4. Drop in O2 Sat The same 18 year old patient with botulism is admitted to the hospital and you were rightly concerned about her ability to ventilate. Which of the following is the best predictor of the need for intubation? 1. Hypoxia on blood gas 2. Hypercarbia on blood gas 3. FVC 4. Drop in O2 Sat The best predictor to assess the need for intubation in patients with neuromuscular respiratory failure (NMRF) is the forced vital capacity (FVC). It accurately predicts diaphragmatic dysfunction and the development of atelectasis due to diminished cough. A drop in FVC is an early indicator of respiratory failure and the need for possible intubation. Drops in arterial oxygen saturation and hypercarbia occur late in the setting of NMRF. Although anti-MAG antibodies and repetitive nerve stimulation studies may assist with the diagnosis of demyelinating polyneuropathy and myasthenia gravis, neither predicts the clinical severity of disease or the need for intubation. A 25 year old male with a history of IV drug use and a prior head injury presents for evaluation of abnormal pupils which he noticed several months ago. On exam he has anisicoria L>R with a great difference in ambient light. The left pupil reacts poorly to light, but reacts very slowly to accommodation. Instillation of dilute pilocarpine does not affect the right eye but causes constriction of the left eye. Which of the following is the most likely diagnosis? 1. Ocular trauma 2. Horner’s syndrome 3. Neurosyphillis 4. Tonic pupil A 25 year old male with a history of IV drug use and a prior head injury presents for evaluation of abnormal pupils which he noticed several months ago. On exam he has anisicoria L>R with a great difference in ambient light. The left pupil reacts poorly to light, but reacts very slowly to accommodation. Instillation of dilute pilocarpine does not affect the right eye but causes constriction of the left eye. Which of the following is the most likely diagnosis? 1. Ocular trauma 2. Horner’s syndrome 3. Neurosyphillis 4. Tonic pupil This patient's history is most compatible with a unilateral Adie's tonic pupil. An Adie's pupil is typically initially large although becomes smaller over time. It shows minimal reaction to light but normal constriction to near, ie, lightnear dissociation. Unlike normal pupils, which will show no response to instillation of dilute pilocarpine, Adie's pupils will constrict to this agent due to denervation super sensitivity from ciliary ganglion dysfunction. Unlike Adie's pupils, ArgyllRobertson pupils due to neurosyphilis are typically bilaterally small. Adie's pupils are often associated with impaired muscle stretch reflexes; this combination of findings is referred to as the Holmes-Adie syndrome. Apraclonidine is used to detect a Horner pupil which should dilate in response to this agent. 0.125% Pilocarpine •Normal pupil will constrict to 1% pilocarpine, but not to dilute pilocarpine •A pupil that has lost parasympathetic denervation for any reason will become hypersensitive to dilute pilocarpine Tonic Pupil • One large poorly reactive pupil • Caused by damage to ciliary ganglion or short ciliary nerve • Can see near light dissociation and pilocarpine hypersensitivity but these take weeks to develop • When associated with hypoactive deep tendon reflexes: Adie-Holms syndrome • Differential includes: - idiopathic - trauma - infection (syphilis) - Sjogren’s - space occupying lesion - migraine (transient tonic pupil) • For an uncomplicated tonic pupil, neuro-imaging is not warranted The most likely cause of death in acute liver failure is what? 1. GI hemorrhage 2. Diffuse ischemic brain injury 3. Cerebral edema 4. Intraparenchymal brain hemorrhage The most likely cause of death in acute liver failure is what? 1. GI hemorrhage 2. Diffuse ischemic brain injury 3. Cerebral edema 4. Intraparenchymal brain hemorrhage Patients who develop liver failure acutely are more prone to development of hepatic encephalopathy. In patients with hepatic encephalopathy-induced coma, up to 80% develop cerebral edema, which is the leading cause of death in these patients. Which of the following tests would be most useful in identifying frontal lobe pathology? 1. Wisconsin Card Sort Test 2. Facial Recognition Test 3. Letter fluency 4. Clock drawing Which of the following tests would be most useful in identifying frontal lobe pathology? 1. Wisconsin Card Sort Test 2. Facial Recognition Test 3. Letter fluency 4. Clock drawing The Wisconsin Card Sort Test (WCST), which challenges a patient to change cognitive sets without warning, is particularly sensitive to frontal damage. Language skills may be unaffected, and vocabulary is often spared. Face recognition and visual perception abnormalities such as hemi-inattention syndromes are most often associated with damage to the parietal, temporal, or occipital lobes, rather than the frontal lobes. Therefore, the best answer is the WCST. Inhibition of unwanted or inappropriate responses falls under the rubric of executive function. In the widely-used Stroop test, subjects are presented with names of colors written in colors other than the one spelled out. After reading the words, subjects then must report the color of the word seen, rather than its meaning (e.g., the word red written in green ink), necessitating inhibition of the immediate response to read the word as written. Trail making tests the ability to shift sets, while letter fluency primarily tests generation of new items. Digit span subtest tests working memory, while the CVLT (California verbal learning test) is a test of episodic memory Wisconsin Card Sort Test Stroop Test Which of the following lesions is most likely to produce a congruous homonymous field defect? 1. 2. 3. 4. 5. Which of the following lesions is most likely to produce a congruous homonymous field defect? 1. 2. 3. 4. 5. In the retrochiasmatic visual pathways, homonymous hemianopias are most likely to be incongruous when caused by lesions of the optic tracts or lateral geniculate body, and more congruous in the optic radiations and occipital lobe. A lesion in an optic chiasm would cause a bitemporal or junctional visual field defect. A patient on long term isoniazid develops diarrhea and profound memory difficulties. Which of the following vitamin deficiencies may be to blame? 1. Vitamin D 2. Niacin 3. vitamin E 4. B12 A patient on long term isoniazid develops diarrhea and profound memory difficulties. Which of the following vitamin deficiencies may be to blame? 1. Vitamin D 2. Niacin 3. vitamin E 4. B12 Niacin deficiency, commonly found in malnourished individuals and in those taking isoniazid, classically presents with the 3 D's: dementia, dermatitis, and diarrhea. Other neurologic manifestations include peripheral neuropathy and myelopathy. Vitamin E deficiency typically presents with cognitive dysfunction, ataxia, and neuropathy. Can also get a B6 deficiency with INH. Pellagra You are seeing a 30 year old operation Iraqi Freedom male verteran at the VA in evaluation for TBI and headaches. When discussing sleep he endorses that it is very interrupted because he wakes up in the middle of the night multiple times with vivid nightmares. Which of the following medications may be effective in reducing this symptoms? 1. Quetiapine 2. Prazosin 3. Zolpidem 4. Clonazepam You are seeing a 30 year old operation Iraqi Freedom male verteran at the VA in evaluation for TBI and headaches. When discussing sleep he endorses that it is very interrupted because he wakes up in the middle of the night multiple times with vivid nightmares. Which of the following medications may be effective in reducing this symptoms? 1. Quetiapine 2. Prazosin 3. Zolpidem 4. Clonazepam This individual has traumatic brain injury (TBI) along with flashbacks, nightmares and social avoidance characteristic of posttraumatic stress disorder (PTSD). Prazosin, an alpha-adrenergic blocking agent which penetrates the blood-brain barrier, is the medication of choice to improve sleep and to block nightmares associated with TBI and PTSD. It may also help other adrenergic symptoms of PTSD as well. The other medications listed are not as useful for this purpose and may produce cognitive slowing and worsening daytime sleepiness. You are seeing an 8 year old boy in clinic for difficulty walking. On exam you notice four limb and truncal ataxia. He has weakness in his legs and absent deep tendon reflexes throughout. An EMG reveals absent sensory response in the arms and legs. His brother had a similar condition. Which of the following will be the most likely cause of death in this child? 1. Cardiomyopathy 2. Non-ketotic hyperosmolar hyperglycemia 3. Aspiration 4. Respiratory failure You are seeing an 8 year old boy in clinic for difficulty walking. On exam you notice four limb and truncal ataxia. He has weakness in his legs and absent deep tendon reflexes throughout. An EMG reveals absent sensory response in the arms and legs. His brother had a similar condition. Which of the following will be the most likely cause of death in this child? 1. Cardiomyopathy 2. Non-ketotic hyperosmolar hyperglycemia 3. Aspiration 4. Respiratory failure 5. Immunodeficiency Hypertrophic obstructive cardiomyopathy is the characteristic cause of death in Friedreich ataxia. Smaller numbers of patients may succumb to complications of diabetes. Lymphoma and other consequences of immunodeficiency occur at increased rates in ataxia-telangiectasia, another recessively inherited progressive ataxic syndrome. Friedreich Ataxia • Loss of function mutation in Frataxin gene, usually GAA repeats in both alleles • Protein normally functions in iron trafficking around the cell, expressed in high levels in brain, heart and pancreas • Age of onset usually adolescence 2-70 • Most common hereditary ataxia • Clinical features: ataxia (limb and truncal), sensory neuronopathy (absent reflexes and sensory loss), posterior column disease (upgoing toes and sensory loss), LE weakness, dysarthria/dysphagia, degeneration of betz cells (UMN weakness) • Aside from neurologic problems also have diabetes and cardiomyopathy (leading cause of death) Which of the following EEG patterns portends the worse prognosis after cardiac arrest with resulting hypoxic ischemic encephalopathy? 1. TIRDA 2. Burst suppression 3. Reactivity 4. Diffuse beta Which of the following EEG patterns portends the worse prognosis after cardiac arrest with resulting hypoxic ischemic encephalopathy? 1. TIRDA 2. Burst suppression 3. Reactivity 4. Diffuse beta Burst suppression pattern in hypoxic ischemic coma carries an extremely poor prognosis. Diffuse beta activity is most often associated with drugs/medications, most commonly benzodiazepines, the effects of which are reversible. Diffuse polymorphic delta is a nonspecific finding indicative of generalized cerebral dysfunction/encephalopathy and IRDA (intermittent rhythmic delta activity) does not clearly portend poor prognosis. EEG reactivity following cardiac arrest is a relatively favorable prognostic sign compared with the absence of reactivity, which typically carries a poor prognosis. Excess Beta (13-30 Hz) Burst Suppression A neonate presents clinically with meningitis. Their imaging is shown. What is the most likely organism? 1. Citrobacter 2. Pseudomonas 3. E. Coli 4. Group B strep A neonate presents clinically with meningitis. Their imaging is shown. What is the most likely organism? 1. Citrobacter 2. Pseudomonas 3. E. Coli 4. Group B strep Several microorganisms including Citrobacter species, Serratia marcescens, Proteus, Pesudomaonas and Enterobacter can result in neonatal central nervous infection complicated by necrosis and abscess formation. Of these, the Citrobacter species are the most common microorganism. A 45 year old male presents with facial droop upon awakening in the morning. On exam he has weakness of lower face and forehead. There is no visible rash nor vesicles. He has no prior history of rash and lives in New York City and has never left. What is the most appropriate treatment? 1. Prednisone 2. Valacyclovir 3. Doxycycline 4. Acyclovir A 45 year old male presents with facial droop upon awakening in the morning. On exam he has weakness of lower face and forehead. There is no visible rash nor vesicles. He has no prior history of rash and lives in New York City and has never left. What is the most appropriate treatment? 1. Prednisone 2. Valacyclovir 3. Doxycycline 4. Acyclovir This patient has signs and symptoms of an idiopathic (Bell) facial palsy. The 2001 AAN Practice Parameter on treatment of Bell palsy concluded that, based on the available evidence, steroids are probably effective for improving outcome. Subsequent class I and II studies have not shown clear additional benefit from either acyclovir or valacyclovir for this condition, as underscored by the updated 2012 AAN Practice Parameter on the treatment of Bell Palsy. However, some experts believe that the addition of antivirals to steroids provide a marginal increased benefit. Even in Lyme endemic regions idiopathic facial palsy is more common than Lyme related facial palsy. In the absence of a tick bite, erythema migrans rash, or meningitis-like prodrome, Lyme neuroborreliosis is unlikely and doxycycline is therefore not warranted unless Lyme serologies are positive A marathon runner is brought to the ED at the end of a race with confusion and then has a seizure. She ate a normal meal prior to the event and then stayed well hydrated throughout the race by drinking water. What is the most likely etiology the seizure? 1. Hyponatremia 2. Hyperkalemia 3. Hyperuremia 4. Hypocalcemia A marathon runner is brought to the ED at the end of a race with confusion and then has a seizure. She ate a normal meal prior to the event and then stayed well hydrated throughout the race by drinking water. What is the most likely etiology the seizure? 1. Hyponatremia 2. Hyperkalemia 3. Hyperuremia 4. Hypocalcemia Hyponatremia has been documented in 13% of marathon runners. Patients can have both systemic and neurologic symptoms related to hyponatremia including delirium, weakness and seizures. While hypocalcemia and hypoglycemia could also cause altered mental status they are less likely in this setting. Increased or decreased potassium, while concerning from a cardiac standpoint, would not be expected to cause prominent mental status alterations. A 5 year old boy presents with progressive behavioral disturbances and cognitive decline. His parents bring him to the ED after a tonic clonic seizure. His imaging is show. What additional work up should be obtained at this time? 1. ECHO 2. Audiogram 3. TSH 4. Serum cortisol A 5 year old boy presents with progressive behavioral disturbances and cognitive decline. His parents bring him to the ED after a tonic clonic seizure. His imaging is show. What additional work up should be obtained at this time? 1. ECHO 2. Audiogram 3. TSH 4. Serum cortisol Hypoadrenalism (Addison's) is a common feature of adrenoleukodystrophy. This is a typical history of the progression of adrenoleukodystrophy, once the cortical form becomes manifest. The MRI typically shows posterior to anterior progression of leukodystrophy, with enhancement of the advancing rim. In contrast, PRES due to hypertension typically is acute, involves both posterior gray and white matter, and has no enhancement of the rim. Cardiomyopathy is associated with several neurodegenerative conditions, most notably several mitochondrial cytopathies, but this is not consistent either clinically or radiographically. Hashimoto's encephalopathy may be associated with abnormal thyroid function tests but this would not be either the clinical or radiographic presentation. But whosoever in this world overcomes this wretched craving so difficult to overcome, his sorrows fall away from him like water-drops from a lotus leaf. Calm is the thought, calm the word and deed of him who, rightly knowing, is wholly freed, perfectly peaceful and equipoised.