Movement Disorders Flashcards
Vitamin E deficiency
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
Manganese toxicity
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
MPTP
(1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)
- 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
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
e) trial of levodopa
Describe the clinical features of Parkinson Disease
Resting Tremor 4-6 Hz
Bradykinesia
Rigidity
Asymmetric Onset
Atypical early signs: bulbar symptoms, dementia, postural instability, autonomic dysfunction
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
d) dyskinesias
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
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.
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
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
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
f) all of the above
Deep brain stimulator should be placed in thalamus
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) amiodarone
Ataxia, peripheral neuropathy, pulmonary fibrosis, thyroid dysfunction
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?
c) PSP
Tufted astrocytes
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
Lewy Body Dementia
a) haloperidol - neuroleptic sensitivity
Patient presents with declining cognition and biopsy pictured below. What is the diagnosis?
What are the clinical features?
What are potential treatment options?
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
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
d) meperidine
What medications may be useful to treat posthypoxic myoclonus (Lance-Adams syndrome)?
Levetiracetam, valproate, clonazepam
- What is the protein for DYT-1?
- What is the protein for dopa-responsive dystonia (Segawa syndrome)?
- Protein torsin A
- GTP cyclohydrolase 1, enzyme used in the production of tetrahydrobiopterin, subsequently needed for tyrosine hydroxylase to produce dopamine/norepinephrine. May also see episodic hyperthemia.
How does botulinum toxin work in cervical dystonia?
A – SNAP-25, B – synaptobrevin, C – syntaxin (& SNAP-25)
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?
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
Describe periodic limb movements of sleep (PLMS)
4 consecutive movements, 0.5 to 5 seconds long, and between 5 and 90 seconds apart on sleep study.
Treat like RLS.
Describe Narcolepsy
What is treatment?
sleep paralysis, cataplexy, decreased REM latency, hypnogogic/pompic hallucinations.
Treat with stimulants.
What is disease pictured?
Clinical Features?
Genetics?
Huntington Disease
Onset third or fourth decade
Dementia, personality change
Involuntary movements
Autosomal dominant
Huntingtin – CAG repeat on chromosome 4 – mitochondrial iron chaperone
Describe clinical features of Friedreich Ataxia
What is mode of inheritance?
What gene?
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
Describe Episodic Ataxia Type 2
- Clinical Features?
- Mode of Inheritance?
- Gene?
- What other disease is associated with this gene?
- Treatment?
- Minutes-hours of limb ataxia, dysarthria, gait instability
- Autosomal dominant
- CACNA1A (calcium channel 1a), chromosome 19p13
- Familial hemiplegic migraine
- Acetazolamide
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
d) anti-Yo
