Movement Disorders Flashcards
How would Parkison’s Disease be defined
A chronic, idiopathic, progressive neurodegenerative movement disorder where symptoms continue to worsen over time
Describe the etiology of Parkinson’s
- loss of dopaminergic neurons in the substantia nigra (excessive inhibition of the thalamus leads to reduced cortical motor activation)
- loss of melanin from the pars compacta
+/- the presence of lewy bodies
What are the cardinal signs of Parkinson’s
- resting tremor
- cogwheel rigidity
- bradykinesia
- postural instability
other syx include: intrinsic muscle issues, stiffness, voice fluctuations, instability, micrographia, stooped/flexed posture, propulsion/retropulsion
How do symptoms of Parkinson’s typically start
unilateral tremor
What are some characteristics of Parkinson’s that may be seen on physical exam
- pill rolling, resting tremor
- bradykinesia, gait impairment
- postural reflexes impaired
- ask about onset of symptoms and progression
What is the difference between Parkinsonism and Parkinson’s Disease
Parkinsonism = 2 or 3/4 cardinal signs
Parkinson’s Disease = 4/4 cardinal signs
What diagnostic testing may be done in Parkinson’s disease
- no specific biomarker
- Lewy bodies may be seen on MRI
- olfactory testing
Describe the treatment of Parkinson’s Disease
- provide symptom relief
- levodopa-carbidopa
- Entacapone (COMT Inhibitors)
- MAO-B inhibitors
- Anticholinergics
What are some hyperkinetic disorders
- essential tremor
- dystonia
- Huntington’s Disease
- Wilson’s Disease
- Tardive Dyskinesia
- Restless leg syndrome
Etiology of essential tremor
- sporadic or familial (autosomal dominant)
- most common movement disorder
- onset childhood to elderly (usually 45)
What is the clinical presentation of essential tremor
-postural tremor with/without kinetic component
- tremor only
- bilateral and symmetric, usually upper extremity
- normal muscle tone
What may improve essential tremor symptoms
alcohol, only for 30-60 mins
What is the major differential for essential tremor
Wilson’s disease
What is the first line treatment for essential tremor
beta blockers: propranolol
refer to neuro if contraindicated for benzos
Define tremor
involuntary rhythmic oscillating movement of a body part secondary to intermittent muscle contraction
Describe the handwriting effects of essential tremor
gets bigger, shaky, no resting tremor
Describe the pathophys of dystonia
primarily unknown
- basal ganglion dysfunction
- excitability of motor cortex
Describe the 3 types of dystonia
- primary
- focal (MC)
- secondary (drugs, neuro disorders)
Describe the major types of focal dystonia
- blepharospasm
- oromandibular dystonia
- spasmodic dysphonia
- cervical dystonia
- limb dystonias
describe the clinical presentation of dystonia
worse with emotional/physical stress or fatigue, decreases with sleep and rest
Describe the diagnostic process for dystonia
- rule out parkinson’s and Wilson’s
Describe the management of dystonia
- levodopa first line
- muscle relaxers, pumps, botox, baclofen, DBS
Describe the etiology of Huntington’s
progressive neurodegenerative disorder of the CNS - atrophy of the caudate nucleus head, putamen, cerebral cortex
- autosomal dominant
Is Parkinson’s direct or indirect pathway?
direct
Huntington’s - direct or indirect pathway?
indirect
Tourette Syndrome - direct or indirect?
Indirect pathway
Wilson’s Disease - direct or indirect pathway
Direct pathway
Describe the common symptoms of Huntington’s
- chorea
- rigidity
- psychiatric
- progressive dementia
- seizures
Diagnostics for Huntington’s
- clinical
- MRI, genetic testing
treatment of huntingtons
symptom releif
- anti-choreic (tetrabenazine
- psychiatric treatment
survival of huntingtons
10-30 years
- death commonly due to pulmonary issues, sepsis
Etiology of Wilson’s disease
- autosomal recessive
- dysfunction of copper metabolism
Symptoms of Wilsons
- copper rings in cornea
- speech disorder, extrapyramidal syx
Diagnosis of wilson’s
slit lamp (copper rings in cornea), ceruloplasmin levels
treatment of wilson’s
chelating agents to bind and remove copper
Tardive dyskinesia etiology
commonly severe and secondary to antipsychotic drugs
months to years
symptoms of tardive dyskinesia
- extrapyramidal symptoms: restlessness, dystonia, parkinsonism, chorea
etiology of restless leg syndrome
- limbs associated with sleep complaint
- W>M
symptoms of restless leg
involuntary forceful dorsiflexion of foot, sleep disturbance, fatigue, repetitive periodic jerking
pins and needles, internal itch, creeping crawling
diagnostics for restless leg
- CBC with diff
- iron panel
- BUN
- Blood gluc
- TSH
- Vitamin B12
- folate
treatment of restless leg
First line: dopaminergic agents (ropinirole)
- clonidine
- iron supplementation
- education and sleep hygeine
What condition is this
huntington’s
what might this disease be characterized by
tardive dyskinesia
Which diseases of the basal ganglia affect the direct pathway
- parkinson’s disease
- Wilson’s disease
- Essential tremor
- bradykinesia/akinesia
Which diseases of the basal ganglia affect the indirect pathway
- Huntington’s disease
- Hemiballism
- Tics
- Dystonia
- Myoclonus
- Tardive dyskinesia
