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A three-month-old female born full-term and without any significant past medical history is brought to the clinic with episodes of head-nodding for the past one week. Episodes last for only a few seconds and only occur while awake. On physical exam, there is an abnormal head tilt to the left and intermittent head nodding. Intermittent, horizontal, and bilateral nystagmus was also appreciated. Routine EEG and MRI of the brain with and without contrast were normal. Which of the following is the most likely diagnosis?
A 78-year-old male with a past medical history of depression and chronic alcohol use presents to the clinic with his wife for evaluation of a tremor. He states that the tremor is most severe at rest and disappears with purposeful movement. His wife states that he has been having slower movements over the past year. On physical exam, a “pill-rolling” resting tremor is noted in his left hand and the patient has difficulty with gait initiation and takes short steps when walking forward. Which dopaminergic pathway is most likely affected in this patient?
Which of the following is the mechanism of action of ropinirole?
A patient with a past medical history of Parkinson’s disease is brought to the emergency room with the chief complaint of a severe headache, chest pain, and shortness of breath. His blood pressure on arrival was 196/122 mmHg. The patient states that he was in his usual state of health earlier in the day but started feeling unwell after attending a wine and cheese pairing event at a local winery. Which of the following medications is likely the cause of the patient’s symptoms?
A 9-year-old boy presents to the clinic with complaints of gait and speech trouble. His mother reports he started walking “like he was drunk” a few months ago. He had genetic testing that confirmed a trinucleotide GAA repeat expansion in one of his genes. Which of the following findings on physical exam is least likely to be present?
A 67-year-old male presents to the clinic with his wife due to a gradual decline in his balance over the past two years. His balance issues have resulted in him falling twice in the past month. His wife states that she notices that he has been walking more slowly than usual. The patient also complains of double vision when reading the newspaper. On physical exam, the patient is noted to have a vertical gaze palsy and impaired smooth ocular pursuit, and a subtle right hand resting tremor. A brain MRI is ordered which shows atrophy in the midbrain tegmentum. What is the most likely diagnosis?
A nine-month-old boy with no significant past medical history is brought to the clinic by his mother because of paroxysmal episodes of unknown etiology. His mother states that since he was six months old, she noticed that his head would intermittently tilt to his right lateral side when he woke up. His head tilt would always disappear during sleep. During these “attacks” the patient would become irritable and would occasionally vomit. The symptoms last up to 48 hours before resolution. Imaging of the brain and EEG do not demonstrate any abnormalities. Which of the following is the most likely cause of these symptoms?
Carbidopa is given concurrently with levodopa to reduce the risk of which of the following side effects?
You are caring for a patient with Lewy body dementia who is having difficulty with myoclonic jerks. Which of the following is considered the first-line treatment for cortical myoclonus?
A 58-year-old male presents with a few months of progressive symptoms. He reports limb rigidity, left greater than right, as well as neck stiffness. On exam, you note bradykinesia and cervical dystonia. He does not have a tremor. Speech is hesitant, and there is evidence of speech apraxia. You also note that the patient has trouble controlling the location of his left hand in space, and he grabs for your stethoscope without realizing it.
What is the most likely diagnosis?
Which of the following direct dopamine agonists are the most effective in the management of severe “off” phenomenon related to chronic levodopa use in the management of advanced Parkinson’s disease?
Which of the following is the most appropriate initial screening test for suspected restless leg syndrome?
A 67-year-old man with a past medical history of obstructive sleep apnea is being evaluated for bilateral arm tremors. His symptoms started insidiously 5 years ago. It is best appreciated when the patient brushes his teeth or eats with a fork. Oddly, he has noticed that a glass of wine at night can help with his symptoms. What is the most appropriate therapy for his movement disorder?
You are caring for a 38-year-old man with schizophrenia since his early twenties. He has had many years of uncontrollable irregular facial movements including tongue writhing, lip smacking, and frequent blinking. His psychiatrist stopped the medication suspected to cause this problem a year ago, yet he is still having the symptoms and they are especially bothersome. Which of the following medications is FDA approved for the treatment of this problem?
An 80-year-old female presents with abnormal eye movements and cognition. She has also been experiencing problems with gait and memory. An MRI of the brain revealed midbrain atrophy, with relative sparing of the other brainstem structures. Which of the following is the most likely diagnosis?
Which of the following is the most appropriate pharmacologic agent for type II episodic ataxia?
A 67-year-old woman suffers from sustained involuntary contraction causing her head to be forcibly turned to the right and significant discomfort. On exam, her left sternocleidomastoid is significantly hypertrophied. She receives treatment with Botulism Toxin A. Subtype A of botulism toxin acts to reduce synaptic action of acetylcholine by what mechanism?
A 76-year-old man with a five-year history of idiopathic Parkinson’s disease presents to your office accompanied by his daughter. She remarks that he has started talking to his wife who passed away 5 years ago. She also frequently finds him frightened, looking around the room at “small people” who are not there. Which of the following is a treatment of choice that is correctly paired with its associated monitoring parameters?
A 74-year-old male with a past medical history of hypertension and hyperlipidemia is brought to the physician by his daughter who states that he has been exhibiting abnormal shaking of his hands and difficulty walking for the past year. On exam, the patient exhibits a shuffling gait, a tremor of his left hand at rest, and difficulty initiating movements. Which of the following is the most appropriate first-line therapy?
A 65-year-old man with a past medical history of chronic liver disease and diabetes presents with progressive falls and a tremor. On exam, he is rigid throughout with significant gait instability, a tendency to turn his head to the right, and a hypertrophied left sternocleidomastoid muscle. He fails initial treatment with carbidopa-levodopa. His brain MRI reveals T1 hyperintensities of the bilateral globi pallidi. Which of the following is the most likely diagnosis?
Which of the following medications is the most appropriate pharmacologic agent for paroxysmal kinesigenic dyskinesia?
A 64-year-old man with idiopathic Parkinson’s disease being treated with carbidopa-levodopa is experiencing significant wearing-off despite increasing frequency and dose of levodopa-carbidopa, which is now producing significant peak dose dyskinesia. Which of the following would be a reasonable next agent to prescribe to reduce this wearing-off effect?
A 40-year-old woman is hospitalized at an inpatient psychiatric facility for new behavioral disturbances and a suicide attempt. She has also begun to develop unusual movements of her bilateral upper extremities. The movements are non-rhythmic, high-amplitude, low-frequency, and involve the entire extremity. Her brain MRI is shown below. Which of the following may help to treat her movements?
You are interested in initiating tetrabenazine for a patient with refractory chorea. You should educate the patient that this medication carries a black box warning for which of the following?
A 63-year-old female presents to the office, accompanied by her husband who has become increasingly worried about his wife’s ability to perform tasks at home. He states that for the past three months she has not been able to tie her shoes, and fumbles with the shoelaces in her hands. Additionally, within the past month, she has not been able to brush her teeth, and simply holds the toothbrush in her hand, without moving. Lastly, her husband has noticed that the patient moves slower on their morning walks together, and her arms and legs seem more tight and rigid. A brain MRI is ordered which shows asymmetric atrophy of the superior parietal lobule. Which of the following is the most likely diagnosis?
A 7-year-old boy was brought to the ED by his mother for a three-week history of involuntary movements. He was initially seen by his pediatrician two weeks ago. MRI of the head was performed and was unremarkable. Methylphenidate was trialed for a presumed tic disorder but it had no benefit. The Movements resolve with sleep. On examination, he has difficulty controlling facial and extremity movements as well as maintaining tetanic contraction of his hands. There was also a newly appreciated cardiac murmur. What is the most likely diagnosis?
A 10-month-old male with no significant past medical history has had a 2-month history of abnormal movements. The family describes these events as head drops and head-turning. On examination, horizontal nystagmus is appreciated. MRI revealed no abnormal findings. What is the most likely diagnosis?
On microscopic evaluation, which of the following is a classic finding present in the Parkinson-plus syndromes of both progressive supranuclear palsy and corticobasal degeneration, but not Parkinson’s disease?
Stiff person syndrome is associated with which of the following auto-antibodies?
An 8-year-old boy is brought to the neurology clinic due to an 18-month history of repetitive, involuntary movements of his face and body. His mother states that he has been bullied at school because of it, which is causing him significant distress and anxiety. His birth history and developmental history are normal. On physical exam, the patient has excessive blinking and occasionally jerks his right shoulder upwards at rest. The physical exam is otherwise within normal limits. which of the following is the most likely diagnosis?
Deep brain stimulation has been effective in the management of Parkinson’s disease when it targets which of the following structures?
Which of the following botulinum serotypes cleaves synaptosomal-associated protein (SNAP)-25.
A 4-year-old male is brought to your clinic by his father who states that he has been experiencing random jerky movements of his arms and his neck for the past year. His father states that the jerky movements have not gotten worse over time but tend to appear more when the boy is agitated or under stress. The movements cease when he is asleep. The patient has otherwise been meeting all of his developmental milestones. Genetic testing reveals a mutation in TITF1, a thyroid transcription factor. What is the diagnosis?
A 69-year-old male presents to the clinic with his wife who states that for the past 10 months the patient has become increasingly slow and more clumsy with daily activities. He has also been complaining of dizziness, especially in the morning when he first gets out of bed and has fallen once in the past month. On physical exam, it is observed that the patient has a 21 mmHg drop in systolic blood pressure when going from a sitting to a standing position. A brain MRI is ordered and shows a cruciform hypointensity in the pons. What is the most likely diagnosis?
A two-month-old boy is brought to the Emergency Room by his mother because of lethargy for the past 3 hours. His mother states that earlier today the child rolled off the couch and hit his head on the hardwood floor. Physical exam shows excessive somnolence, tense anterior fontanelle, and fundoscopic exam shows bilateral retinal hemorrhages. A non-contrast CT of the head shows subdural hematomas of varying ages. A CT head is ordered and pending. Otherwise, what is the next most appropriate step in the management of this patient?
Which of the following is NOT known to cause a drug-induced tremor?
A 42-year-old woman with major depressive disorder and anxiety who has recently completed treatment for gambling addiction presents with a unilateral upper extremity resting tremor and the feeling that she is “stiff all of the time”. Her husband remarks that she has become much more soft-spoken lately and that five years ago she began acting out her dreams. Which of the following medications should be avoided in this patient?
A 32-year-old female with a history of schizophrenia is brought to the emergency room by her husband for acute psychosis. She receives a medication while in the emergency room to aid in her agitation. Soon after administration, the patient develops involuntary contractions of muscles of her face and neck. Which of the following medications was most likely administered to cause these symptoms?
Which of the following is the mechanism of action for selegiline?
Which of the following is the mechanism of action of entacapone?
A 68-year-old female presents to your clinic with the chief complaint of daytime somnolence. She endorses an unpleasant feeling in her lower extremities while trying to go to bed which prevents her from sleeping. Moving her limbs gives transient relief. What is the most appropriate first diagnostic test?
A 71-year-old female with a past medical history of hypertension presents to the clinic with a 1-year history of a progressive right tremor. She only notices the tremor when she is sitting still. On physical exam, you notice that her movements are not smooth, and she seems rigid in her extremities. She also demonstrates a slow, shuffling gait. This patient’s condition is most likely due to the loss of which neurotransmitter?
Which of the following is a common side effect of levodopa therapy?
A 55-year-old male with a 3-year history of postural hypotension, incontinence, and ataxia comes to the clinic for evaluation. An MRI brain shows degeneration of the pontocerebellar and olivocerebellar fibers. What is the most likely diagnosis?
A 72-year-old female presents to the clinic with her husband who states that she is “seeing things that aren’t there”. For the past two weeks, she has been complaining of bats flying around her room at night. Comparable visual disturbances have occurred over the past year, but have gotten progressively worse recently. She has also been acting out a lot at night; her husband states that she has been violently kicking and flailing her arms while asleep. Finally, she has been experiencing cognitive decline, including forgetting where she lives, what year it is, and the names of her grandchildren. On exam, there is symmetric cog-wheel rigidity appreciated. What is the most likely diagnosis?
Which of the following is the mechanism of action of tetrabenazine?
An 11-year-old female presents with worsening stiffness. She had no complications while in utero or at birth, yet ever since she started kindergarten she noted to have stiffness in her feet and ankles, which worsens by the end of the day. She eventually started to notice modest stiffness in the legs and mild stiffness in the neck. She underwent physical therapy for this and remained stable. Over the last 2 years, however, the symptoms have been worsening. Which of the following next steps is most indicated?
A 61-year-old male with no past medical history presents to the clinic with difficulty moving for the past year. He states that it takes him a lot longer to do daily tasks such as walking to the mailbox and that it is getting worse. He is accompanied by his daughter who states that she notices that he shuffles his feet when walking. On physical exam, the patient is observed to have a 4-6 Hz resting tremor in his hand. Pathologic examination of this patient’s brain tissue would most likely show which of the following?
A 66-year-old patient with bradykinesia, hypomimia, dementia, postural instability, cogwheel rigidity, shuffling gait, and resting tremor presents to your clinic. He has been on carbidopa/levodopa for several years, which has helped, but now his wearing-off phenomenon is becoming more frequent despite attempted medication adjustments. He asks about deep brain stimulation (DBS).
Which of the following is a contraindication for DBS in Parkinson’s disease (PD)?
A 30-year-old man with a past medical history of migraines presents to the emergency department for his typical migraine symptoms, which have become intractable. He is treated with a “migraine cocktail” (normal saline, acetaminophen, and prochlorperazine) with some improvement. You are then called to the bedside for concern that he is having a possible seizure. His head is turned forcibly to the right without eye deviation and he is able to follow commands and verbalize. Which of the following is the best next step in management?
A 50-year-old male with a 1-year history of behavioral changes and abnormal movements has a CT head performed after having a fall at his nursing home. Based on the imaging and brief clinical history what is the likely genetic abnormality associated with his neurological disease?
A 20-month-old female presents for regression of milestones. She was walking, and now crawls or refuses to move. The parents are concerned she may be having seizures because they notice she has intermittent jerks of the limbs and trunk, especially when she is startled. on physical exam, you note conjugate random darting of the eyes in vertical and horizontal directions. Tone, strength, and deep tendon reflexes are normal.
What test is most likely to identify the underlying etiology of this problem?
A 14-year-old female presents to the clinic with frequent falls, clumsiness, and stumbling on her feet. She also reports progressive difficulty with simple tasks such as buttoning her shirts and a loss of feeling in her feet. On physical exam, she has high-arched feet and loss of position and vibration sensation of her great toe. Thoracolumbar MRI shows atrophy of the cervical cord. This condition is associated with which genetic mutation?
A 3-year-old female with a past medical history of recurrent sinus infections is brought to the clinic by her father because of unsteadiness on her feet for the past 4 months. Her father states that she began walking at 12 months. However, more recently, she has been losing her balance and has been falling. On physical exam, she has truncal ataxia and an ataxic gait. An ocular exam reveals dilation of the retinal capillaries. Labs are ordered that show an elevated alpha-fetoprotein. Which of the following is the most likely diagnosis?
A 57-year-old man with a past medical history of bipolar disorder, atrial fibrillation, and type 2 diabetes presents to the clinic with complaints of a tremor. Symptoms have been present for years but they have not seemed to progress significantly over time. His current medications are warfarin, metformin, low-dose aspirin, and lithium. His neurological examination was unremarkable except for the presence of a fine, high-frequency tremor in his bilateral upper extremities. Based on the most likely diagnosis, what is the most appropriate next step in medical management?
A 51-year-old woman with a past medical history of migraines and irritable bowel syndrome comes to the physician because of abnormal shaking of both hands for the past two months. The shaking improves after two glasses of wine. Examination shows symmetric hand tremors that worsen when the patient reaches for objects in front of her. What is the mechanism of action of the most appropriate first-line treatment?
Which of the following symptoms is atypical for essential tremor?
Which of the following is the mechanism of action of pimavanserin?
A 68-year-old male with a 10-year history of Parkinson’s disease comes to the clinic for routine follow-up. He currently is taking levodopa/carbidopa, entacapone, and ropinirole. The patient is complaining of significant dyskinesias and unpredictable motor fluctuations between doses of levodopa/carbidopa. What is the most appropriate next best step in management?
An 80-year-old woman with a history of well-controlled Parkinson’s disease presents with worsening movements at night. Her husband states that she often punches and yells during her sleep. Some of these movements have been so dramatic that she punched a hole in the wall beside her bed. Melatonin was tried but did not lead to a significant improvement in symptoms. Which of the following is the most appropriate next step in treatment?
Which of the following is the mechanism of action of tolcapone?
A 56-year-old man with idiopathic Parkinson’s disease presents to the emergency department for hypertensive emergency and tachycardia. You suspect that this may be an adverse effect related to one of his medications. Patients that are prescribed MAO-B Inhibitors should be counseled on avoiding which of the following?
A 35-year-old female with a diagnosis of bipolar disorder is being seen in the clinic for the first time with a history of progressive difficulties walking. On examination, there was difficulty in fine motor movements and decreased arm swings with ambulation. A CT scan was performed and shown below. What is the most likely diagnosis?