Movement disorders-Aversano Flashcards
What are some features of Progressive Supranuclear Palsy?
- Onset—Elderly > 65
- Appear parkinsonian with early gait disturbance, falls, freezing
- Dystonias and axial rigidity
- Vertical gaze palsy > downward gaze
- *Wide eyed unblinking stare
- Emotional lability-pseudobulbar palsy
What are the atypical parkinsonian syndromes?
- Progressive Supranuclear Palsy (PSP)
- Corticobasal Degeneration
- Multiple System Atrophy
- Striatonigral Degeneration
- Shy-Drager Syndrome
- Olivopontocerebellar - Atrophy
What are some features of MSA (multiple system atrophy)?
-Parkinsonism plus:
- Autonomic dysfunction-(orthostatic hypotension)
- Dementia
- Cerebellar signs
- more progressive than parkinsons
What is dystonia?
Involuntary sustained muscle contractions producing twisting or squeezing movement and abnormal postures
What are some features of primary dystonia?
- DYT1=Early Onset Dystonia
- Autosomal Dominant
- Ashkenazi Jewish Families
- Most severe and common form of hereditary early onset dystonia
- Avg age at onset: 12 years, most before age 26
- *Initial Sx: Crural Dystonia often with foot involvement: Twisted and plantar flexed
- Progresses over 5 years to other areas
- 50% bedridden or W/C bound
- Stabilize, often improve slightly, but never remit
- Genetic testing available
What are some features of cranial dystonia?
-Meige Syndrome: Blepharospasm then oromandibular dystonia then lingual and pharyngeal dystonia
-more common in women.
severity fluctuated day to day and disappears with sleep
-blepharospasm: Eye irritation, photophobia, increased blinking may precede involuntary blinking, repetitive contractions, squinting, or sustained closing of the eye. 12% are functionally blind. worsened by sun, stress, fatigue and watching TV
What is Oromandibular dystonia?
- Jaw opening or closing is often accompanied by lingual protrusion
- Worsened by talking, chewing, swallowing
- Sensory tricks: Press on lips or teeth, tongue to palate, finger in mouth
- 20% eating is compromised
How is cervical dystonia (spasmodic torticollis) treated?
Botulinum toxin
What are some features of Writer’s cramp?
- focal dystonia
- Task-specific or action induced dystonia
- Age at onset varies from 20-70 years
- Handwriting becomes sloppy & illegible
- Jerking writing motion (forearm and wrist contractions); paper may tear
- Squeeze pen tightly, press down hard, fingers may splay from pen
- Pt’s often learn to change hands to write
- 25% become bilateral
- May be associated with Essential tremor
- Pianist’s and Violinist Palsy
- Dart-Thrower’s Palsy
- Golfer’s Palsy (Yips)–> Cannot putt because of the jerky motion
What does the progression of dopa responsive dystonia (DYT5) look like?
- Onset: Childhood or adolescence
- *video of 2 siblings that were completely fixed with Dopamine
- Females 4x’s greater than males
- Foot dystonia in childhood
- Parkinsonism in adults–> more with age
- Diurnal fluctuations
- Dystonic limb presentations are most common
- developmental delay
- spasticity (looks like cerebral palsy)
- **profound and sustained response to levodopa (<300 mg/day)
What are some causes of secondary dystonias?
- drugs: Dopamine Antagonists, Dopamine Agonists, Antidepressants, Antihistamines, Calcium Channel Blockers, Stimulants (Cocaine), Buspirone (Buspar)
- vascular disease: Basal Ganglia infarction, Basal Ganglia hemorrhage, Arteriovenous malformation
- neoplasms (brain tumor): basal ganglia glioma, metastatic neoplasm, c-spine tumor
- other: head trauma, anoxia, meningitis, AIDS, psych
What does the presence of a hemidystonia suggest?
A focal lesion such as a tumor, infarct or abscess in the basal ganglia (opposite side)
What should be ruled out in new-onset dystonia in a young patient?
Wilson Disease
What is the most common adult onset movement disorder?
essential tremor
What are some features of essential tremor?
- Postural (static) and kinetic (active) tremor
- Onset early 20’s and late adulthood
- Improves with ETOH consumption ~ 50%
- Familial 50-70% Autosomal dominant
