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RITE > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

Vitamin E deficiency

A

can cause a spinocerebellar ataxia

  • hyporeflexia, ataxia, decreased proprioception/vibratory sense, distal musc weakness
  • can cause night blindness (nyctalopia)
  • limitation in upward gaze
  • truncal & limb ataxia
  • can be seen in small bowel resection
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2
Q

Manganese toxicity

A

can cause Parkinsonism

seen in pts on prolonged TPN with chronic liver disease or Welders

  • diminished motor skills, psych disturb, tremor, incoordination
  • manganese deposition in the basal ganglia
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3
Q

MPTP

(1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)

A
  • is metabolized into the toxic cation 1-methyl-4-phenylpyridinium (MPP+) by the enzyme MAO-B.
  • MPP+ kills primarily dopamine-producing neurons in the pars compacta of the substantia nigra, causing permanent symptoms of Parkinson’s disease
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4
Q

Which of the following may help distinguish Parkinson Disease from other parkinsonian syndromes?

a) MRI
b) PET
c) smell testing
d) autonomic study
e) trial of levodopa

A

e) trial of levodopa

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5
Q

Describe the clinical features of Parkinson Disease

A

Resting Tremor 4-6 Hz

Bradykinesia

Rigidity

Asymmetric Onset

Atypical early signs: bulbar symptoms, dementia, postural instability, autonomic dysfunction

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6
Q

Which of the following side effects is more likely with levodopa than dopamine agonists?

a) orthostatic hypotension
b) impulse control disorder
c) hallucinations
d) dyskinesias
e) peripheral edema

A

d) dyskinesias

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7
Q

A patient presents with a 6 month history of difficulty with movements. Exam reveals symmetric rigidity, bradykinesia, and postural tremor. Which of the following medications may be implicated?

a) sumatriptan
b) venlafaxine
c) metaclopramide
d) carbemazepine
e) rosuvastatin

A

c) metaclopramide

Drug-induced Parkinsonism
-Antipsychotics
Risperidone > ziprasidone > olanzapine > quetiapine > clozapine (1% risk of myelosuppression)
-Antiemetics
-Reserpine, tetrabenazine
-Weaker associations with SSRIs, lithium, phenytoin, valproate.

Clinical: symmetric vs PD, tremor is postural > resting

Reversible but may take up to 6 months.

Other side effects include tardive dyskinesia and dystonic reaction.

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8
Q

A 64 year old woman presents with a 1 year history of parkinsonism. MRI shows T1 hyperintensity in the globus pallidus, striatum, and midbrain. Which of the following toxic exposures is a potential etiology?

a) iron
b) manganese
c) arsenic
d) lead
e) reality television

A

b) manganese

seen in pts on prolonged TPN with chronic liver disease or Welders

  • diminished motor skills, psych disturb, tremor, incoordination
  • manganese deposition in the basal ganglia
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9
Q

A patient presents to your office with a 2-year history of tremor that interferes with writing and eating. He notes that it improves after he drinks an appletini. There is positive family history for a similar tremor. Exam reveals a symmetric postural and action tremor, otherwise normal. What is a potential treatment for this condition?

a) primidone
b) propranolol
c) gabapentin
d) topiramate
e) deep brain stimulator
f) all of the above

A

f) all of the above

Deep brain stimulator should be placed in thalamus

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10
Q

You are asked to see an exciting hospital consult on a patient with a 3 month history of tremor. Which of the patient’s long-standing medications may be implicated in tremor?

a) amiodarone
b) metoprolol
c) verapamil
d) digoxin
e) Lamotrigine

What are some other side effects of this drug?

A

a) amiodarone

Ataxia, peripheral neuropathy, pulmonary fibrosis, thyroid dysfunction

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11
Q

A 57 year old man presents with a 1 year history of gait instability and slowed movements. Exam shows vertical gaze paresis, retrocollis, bradykinesia, rigidity, and postural instability. Diagnosis?

a) PD
b) DLB
c) PSP
d) CBD
e) FTD

What are the associated findings pictured?

A

c) PSP

Tufted astrocytes

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12
Q

A patient presents with a 2 year history of declining cognition and visual hallucinations plus 6 months of difficulty with movement. Exam reveals symmetric rigidity, bradykinesia, and postural tremor. Which medication, if given to this patient, is most likely to cause severe akinesia or dystonia?

a) haloperidol
b) levodopa
c) rivastigmine
d) quetiapine
e) temozolamide

A

Lewy Body Dementia

a) haloperidol - neuroleptic sensitivity

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13
Q

Patient presents with declining cognition and biopsy pictured below. What is the diagnosis?

What are the clinical features?

What are potential treatment options?

A

Lewy Body Dementia

Dementia with 2/3 of parkinsonism, fluctations, visual hallucinations

Suggestive features include REM behavior disorder and neuroleptic sensitivity

PDD if parkinsonism precedes dementia by >1 year

Treatment includes levodopa, cholinesterase inhibitors, atypical antipsychotics, SSRIs

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14
Q

You are asked to see an exciting hospital consult on a post-op patient for new onset multifocal myoclonus. Which of the patient’s following medications may be implicated in myoclonus?

a) potassium
b) omeprazole
c) ondansetron
d) meperidine
e) oxygen by nasal cannula

A

d) meperidine

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15
Q

What medications may be useful to treat posthypoxic myoclonus (Lance-Adams syndrome)?

A

Levetiracetam, valproate, clonazepam

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16
Q
  1. What is the protein for DYT-1?
  2. What is the protein for dopa-responsive dystonia (Segawa syndrome)?
A
  1. Protein torsin A
  2. GTP cyclohydrolase 1, enzyme used in the production of tetrahydrobiopterin, subsequently needed for tyrosine hydroxylase to produce dopamine/norepinephrine. May also see episodic hyperthemia.
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17
Q

How does botulinum toxin work in cervical dystonia?

A

A – SNAP-25, B – synaptobrevin, C – syntaxin (& SNAP-25)

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18
Q

Which of the following may predispose a patient to restless legs syndrome?

a) iron deficiency
b) pregnancy
c) peripheral neuropathy
d) renal failure
e) all of the above

What are treatment options?
What medications can worsen RLS?

A

e) all of the above

Urge to move the legs, worse at night, worse when inactive, relieved with moving

Associated with age and sleep disorders

Always check ferritin (<50 micrograms / liter abnormal)

Treatment – dopamine agonists, levodopa, clonidine, opioids, benzos, anticonvulsants

Most serotonin reuptake inhibitors can worsen RLS

19
Q

Describe periodic limb movements of sleep (PLMS)

A

4 consecutive movements, 0.5 to 5 seconds long, and between 5 and 90 seconds apart on sleep study.

Treat like RLS.

20
Q

Describe Narcolepsy

What is treatment?

A

sleep paralysis, cataplexy, decreased REM latency, hypnogogic/pompic hallucinations.

Treat with stimulants.

21
Q

What is disease pictured?

Clinical Features?

Genetics?

A

Huntington Disease

Onset third or fourth decade

Dementia, personality change

Involuntary movements

Autosomal dominant

Huntingtin – CAG repeat on chromosome 4 – mitochondrial iron chaperone

22
Q

Describe clinical features of Friedreich Ataxia

What is mode of inheritance?

What gene?

A

Rapidly progressive gait then limb ataxia before age 20

Areflexia with extensor plantor response

Loss of vibratory and propioceptive sensation

Cardiomyopathy, atrophy, weakness, diabetes, deafness

Autosomal recessive

Frataxin – GAA repeat on chromosome 9 – mitochondrial iron chaperone

23
Q

Describe Episodic Ataxia Type 2

  1. Clinical Features?
  2. Mode of Inheritance?
  3. Gene?
  4. What other disease is associated with this gene?
  5. Treatment?
A
  1. Minutes-hours of limb ataxia, dysarthria, gait instability
  2. Autosomal dominant
  3. CACNA1A (calcium channel 1a), chromosome 19p13
  4. Familial hemiplegic migraine
  5. Acetazolamide
24
Q

Which of the following antibodies is most strongly implicated in paraneoplastic cerebellar degeneration?

a) P/Q VGCC
b) AChR
c) MuSK
d) anti-Yo
e) CRMP-5

A

d) anti-Yo

25
Q

Which of the following is associated with obsessive compulsive disorder?

a) autism
b) neurofibromatosis type 2
c) Refsum Syndrome
d) Rett Syndrome
e) Tourette Syndrome

What are potential treatment options?

A

e) Tourette Syndrome

Typical antipsychotics, clonidine

26
Q

A 6-year old girl presents to your office. She had met developmental milestones up to a year, but than regressed. You observe hand wringing, bruxism, and intermittent hyperventilation. Diagnosis?

a) autism
b) neurofibromatosis type 2
c) Refsum Syndrome
d) Rett Syndrome
e) Tourette Syndrome

A

d) Rett Syndrome

27
Q

Describe Neuroacanthocytosis

  1. Clinical Features?
  2. Genetics
  3. Age of onset?
  4. Findings on autopsy
A
  1. Chorea/dystonia/tics esp. orofacial dystonia and dyskinesias

50% with seizures

Elevated CK with myopathy or axonal neuropathy

Acanthocytes (RBCs with uneven thorny projections) also seen in abetalipoproteinemia and McLeod syndrome (older onset, X-linked recessive)

  1. Autosomal recessive (linked to CHAC gene chromosome 9)
  2. Onset in 30s, progressive cognitive and MD
  3. Autopsy – atrophy of basal ganglia
28
Q

A 7-month old boy has clusters of head drops with flexor spasms of the trunk. He has met all developmental milestones thus far. EEG is normal. Diagnosis?

a) Infantile spasms
b) Benign myoclonus of infancy
c) Tourette’s syndrome
d) Benign occipital epilepsy of childhood
e) Lafora body disease

A

b) Benign myoclonus of infancy

29
Q

What is the disease pictured?

A

MSA-C Olivopontocerebellar Degeneration

30
Q

What is the disease pictured?

A

Spinocerebellar Ataxia Type 6

31
Q

What is the disease pictured?

A

Wilson’s disease

-bright basal ganglia and thalamus

32
Q

What is the disease pictured?

A

Fahr’s Disease (idiopathic calcification of basal ganglia and cerebellum)

  • calcium dark on T2 imaging
33
Q

What is the disease pictured?

A

Carbon monoxide poisoning
Necrosis of globus pallidus

34
Q

What is the disease pictured?

A

Creutzfeldt-Jakob Disease (CJD)

35
Q

What is the disease pictured?

A

Panthothenate Kinase-Associated Neurodegeneration (PKAN)
formerly known as Hallvorden-Spatz

“Eye of the Tiger”

36
Q

What is the disease pictured?

A

Panthothenate Kinase-Associated Neurodegeneration (PKAN)
formerly known as Hallvorden-Spatz

“Eye of the Tiger”

37
Q

What is the disease pictured?

A

Alcoholic Cerebellar Degeneration

38
Q

What is the disease pictured?

A

Alcoholic Cerebellar Degeneration

39
Q

What is pictured? What disease is it associated with? What other pathologic feature would you see?

A

Globoid neurofibrillary tangle

Seen in PSP

Also see Tufted Astrocytes

Clinical Features:
Falls, postural instability, retrocollis, bradykinesia, pseudobulbar palsy, dementia

40
Q

What condition is this?

What structures are affected?

A

Wilson’s Disease

Putamen and Thalami

41
Q

What metabolic abnormality would you expect in this patient and what lab finding would be elevated?

A

Leigh’s Disease

Metabolic Acidosis with elevated Lactate

Affects BG and Brainstem

42
Q

What is the disease pictured?

What structures are affected?

A

Fahr’s Disease

= idiopathic calcification of the BG & cerebellum

Calcifications are hypointense on T2

43
Q

What structures are affected in the following?

1) Wilson’s Disease
2) Leigh’s Disease
3) Fahr’s Disease
4) Anoxic Injury

A

1) Putamen & Thalami
2) BG & Brainstem
3) BG & Cerebellum
4) Globus Pallidus

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