movement/tic

Question 1

42 y/o M with a gradually progressive cognitive deficit also develops jerking movements of the extremities, head, and trunk. Imaging shows atrophy of the caudate nuclei bilaterally. Pt’s father had a similar illness that started at age 50. Which of the following tests is most likely to confirm the diagnosis? (6x)

Answer 1

DNA ANALYSIS FOR CAG REPEATS

Question 2

79 y/o pt w/ decreasing mental state over 3 weeks has an exaggerated startle response with violent myoclonus that is elicited by turning on the room lights, speaking loudly, or touching the patient. Myoclonic jerks occur spontaneously, ataxia, EEG: sharp waves. Dx: (5x)

Answer 2

SUBACUTE SPONGIFORM ENCEPHALOPATHY

Question 3

Parkinson’s Disease treated w/ levodopa. Visual hallucinations. Recommendations? (5x)

Answer 3

REDUCE DOSE OF LEVODOPA

Question 4

What MRI finding would most specifically indicate a diagnosis of Huntington’s Disease? (5x)

Answer 4

CAUDATE HEAD ATROPHY

Question 5

65y/o w/ h/o asthma presents for treatment of essential tremor. Which for first-line? (4x)

Answer 5

PRIMIDONE

Question 6

Medication useful in management of orthostatic hypotension in Parkinson’s (4x)

Answer 6

FLUDROCORTISONE

Question 7

98 y/o M in ER, unconscious after choking, had progressive neuro condition presented in his early 30’s w involuntary irregular movements of all extremities & face but after 15 yr course evolved into rigid, akinetic condition w diff swallowing, speaking. Also progressive dementia & full time care. After obstruction was relieved pt remained unconscious, had cardiac arrest & died. PM exam showed generalized brain atrophy. (Pathology picture showing brain atrophy). Dx: (3x)

Answer 7

HUNTINGTON’S DISEASE

Question 8

Treatment of Huntington’s chorea (3x)

Answer 8

HALOPERIDOL

Question 9

Gait consisting of: postural instability, festination, & truncal rigidity. Lewy bodies visualized. Also with involuntary acceleration is seen in what condition? (3x)

Answer 9

PARKINSON’S DISEASE

Question 10

35 y/o pt w/ 2 yr hx of cognitive deterioration, difficulty at work, and irritability. Exam: restless w/ slow, writhing movements in most muscle groups and frequent blinking. Pt’s father and paternal grandpa had similar sx and died in their 50s. Dx: (2x)

Answer 10

HUNTINGTON’S DISEASE

Question 11

Pt w/ depression, 3 yr hx of change in personality, irritability, impulsive outbursts, & eccentric or inappropriate social interactions. He subsequently lost his job & is now withdrawn & fidgety. Pt has increased eye blinking, marked tongue impersistence, mild bradykinesia, akinesia & mild hyperreflexia w/o clonus. The pt’s dad died of severe dementia at 55. Dx? (2x)

Answer 11

HUNTINGTON’S DISEASE

Question 12

Typical of Idiopathic Parkinson’s disease, rather than another Parkinsonian syndrome: (2x)

Answer 12

ASYMMETRICAL ONSET AND PROGRESSION OF MOTOR SYMPTOMS.

Question 13

Typical of Idiopathic Parkinson’s disease, rather than another Parkinsonian syndrome: (2x)

Answer 13

ASYMMETRICAL ONSET AND PROGRESSION OF MOTOR SYMPTOMS.

Question 14

First-line treatment for restless leg syndrome (2x)

Answer 14

PRAMIPEXOLE

Question 15

Multifocal myoclonus in a comatose patient indicates: (2x)

Answer 15

METABOLIC ENCEPHALOPATHY

Question 16

Most effective tx for “writer’s cramp” (focal dystonia assoc w/ writing) (2x)

Answer 16

BOTULINUM TOXIN

Question 17

Essential tremor improves with

Answer 17

REACHING FOR OBJECTS

Question 18

Most prevalent movement d/o in those over 70 y/o?

Answer 18

ESSENTIAL TREMOR

Question 19

6 y/o w/ 4 wk intense eye-blinking and lip pursing. Wax and wane, increase with stress. Family Hx of tic d/o. First step:

Answer 19

EXPLAIN TO FAMILY MAY BE TRANSIENT

Question 20

Dz w/ trinucleotide repeat expansion

Answer 20

HUNTINGTON’S

Question 21

Treatment for Huntington’s disease:

Answer 21

HIGH POTENCY ANTIPSYCHOTICS

Question 22

25 yo pt w several year cognitive decline, dysarthria, dysphagia, and slow movements. Has hand tremor which increases in amplitude on arm extension. Also has hepatic disease of unknown etiology. Ocular exam reveals golden brown ring around cornea. Can confirm diagnosis w blood test for?

Answer 22

CERULOPLASMIN

Question 23

For which patient would ordering a serum ceruloplasmin be indicated?

Answer 23

A YOUNG ADULT MALE WITH NEW ONSET EMOTIONAL LABILITY AND MOVEMENT DISORDER

Question 24

Huntington’s disease etiology is classified as a polymorphism due to what property?

Answer 24

THE REGION HAS MANY ALLELES DIFFERING IN THE NUMBER OF GAC REPEATS

Question 25

68 y/o pt w/ hx of schizophrenia since early adulthood and maintained for yrs on thioridazine. On exam: pt's tongue frequently retracts on its longitudinal axis and moves in lateral directions. No other findings. When tongue movements are pointed out, pt reports being unaware of making them. What recommendation for best Tx:

Answer 25

GRADUALLY D/C THIORIDAZINE AND START LOW DOSE RISPERIDONE

Question 26

What is the treatment of acute dystonia?

Answer 26

BENZTROPINE

Question 27

What symptoms are most commonly associated with Tourette's syndrome?

Answer 27

OBSESSIONS AND COMPULSIONS

Question 28

Pathologic findings in brain of Tourette's?

Answer 28

NO ABNORMALITY

Question 29

One of the earliest sx of Tourette's:

Answer 29

EYE-BLINKING AND HEAD JERKING

Question 30

Tic severity begins to decrease during which age period?

Answer 30

ADOLESCENCE

Question 31

Common cause of acute cerebellar ataxia in adults:

Answer 31

INTOXICATION WITH ANTIEPILEPTICS

Question 32

Initial approach for child with new-onset tic disorder

Answer 32

CLINICAL MONITORING

Question 33

Comorbid condition w/ Tourette's in kids:

Answer 33

ADHD

Question 34

Motor dysfunction in Parkinson's associated with:

Answer 34

INCREASED ACTIVITY IN SUBTHALAMIC NUCLEUS AND PARS INTERNA OF GLOBUS PALLIDUS

Question 35

Characteristics of Parkinson's tremor

Answer 35

BEING INHIBITED WITH VOLITIONAL MOVEMENT

Question 36

80 yo male evaluated for gait imbalance and falls. wife explains that he kicks and screams while sleeping, he reports dreams of being chased. Pt likely has/will develop?

Answer 36

PARKINSON'S DISEASE

Question 37

67 y/o recently retired pt with Parkinsons, no other psych hx, has taken Pramipexole for several years without cognitive decline. Pt has since started gambling excessively. Pt does not drink or use illicit substances. What is the most likely cause?

Answer 37

USE OF DOPAMINERGIC AGENTS

Question 38

Most appropriate initial tx for idiopathic Parkinson dz in an 81 y/o pt

Answer 38

CARBIDOPA/LEVODOPA

Question 39

66 y/o c/o frequent falls, mild axial and L UE rigidity, mild slowness of finger tapping, hand opening, and wrist opposition (all worse on left), normal posture, slow gait with short steps, does not swing left arm, slow rising from chair. What is the dx?

Answer 39

PARKINSON'S DISEASE

Question 40

Picture showing substantia nigra changes, what neurological disease would be expected?

Answer 40

PARKINSON'S DISEASE

Question 41

Clinical syndrome that is most commonly comorbid with Parkinson's disease:

Answer 41

DEPRESSION

Question 42

Pt w/ Parkinson's disease experiences visual hallucinations on levodopa/carbidopa therapy. Which med would be the most appropriate intervention:

Answer 42

QUETIAPINE

Question 43

Pt has severe postural tremor of upper extremities and dystonic posturing of the hands. Pt voice is mildly dysarthric. Eyes have a golden brown limbic ring. Deep tendon reflexes are markedly increased. Elevated LFT's. Dx?

Answer 43

WILSON'S DISEASE

Question 44

t c/o inability to sit still and describes feeling an inner tension. Exam notes pt is constantly shifting body and legs. Paced hall continuously. Received a course of neuroleptics until recently. Dx?

Answer 44

AKATHISIA

Question 45

The single most consistently documented and significant risk factor in the epidemiology of tardive dyskinesia is?

Answer 45

ADVANCED AGE

Question 46

Risk factor for TD

Answer 46

PRESENCE OF MOOD DISORDER

Question 47

TD in 63 y/o w/ end stage renal failure. Culprit:

Answer 47

METOCLOPRAMIDE

Question 48

Which gender has a higher risk for tardive dyskinesia (TD)?

Answer 48

FEMALE

Question 49

75 y/o with hx of extensive tobacco smoking presents with subacute progressive ataxic d/o over several months. MRI brain is unremarkable. CSF shows mild pleocytosis. Found positive for anti-Yo antibody. What test should be ordered next?

Answer 49

CT CHEST

Question 50

Adult onset opsoclonus-myoclonus-ataxia is associated with which form of cancer?

Answer 50

BREAST CARCINOMA

Question 51

Next test to order after anti-Yo antibody found in serum in 72 year old patient with subacute onset of progressive ataxia of gait and limbs

Answer 51

CT of chest (also CT of abdomen and pelvis)