Movement Disorders Flashcards
Parkinson’s Disease
Results from destruction of dopamine producing neurons in the substantia nigra in the midbrain. symptoms after 80% are gone
TRAP: Tremor ( resting, goes away during sleep), Rigidity, Akinesia (bradykinesia), Postural instability. Symptoms are asymmetric.
- other symptoms include depression, apathy, masked facial expression, loss of smell, dysphagia/dysarthria; urinary dysfunction/constipation, sleep disturbances ( REM sleep), micrographia is often the first symptom.
Hallmark of PD are lewy bodies ( alpha synuclein)
What addition test can be used to aid in the diagnosis of Parkinson’s disease?
DaTscan: contrast agent used with single proton emission computed tomography ( SPECT) for detecting dopamine transporters (DaT) in suspected parkinsonian syndromes
Progressive Supranuclear palsy
- Tauopathy
- p/w Parkinsonian symptoms marked by vertical gaze palsies ( reptilian wide eyed stare cannot look down) which lead to falls early in disease. Alteration in mood and behavior including depression and apathy as well as progressive mild dementia. Doesn’t respond to Parkinson’s meds.
- Atrophy of the midbrain can be seen on MRI ( hummingbird sign)
Medication-induced parkinsonism
Caused by high potency typical antipsychotic (haloperinol) and antiemetics (reglan, zofran)
Multisystem atrophy
Broken up into Multisystem atrophy-cerebellar type and multisystem atrophy parkinsonian type include:
- Lack of response to meds used to treat Parkinsons, wide-based gait ( parkinsons gait is narrow),
- eye movement abnormalities,
- symmetrical onset of symptoms, rapid onset ( within 5 yrs),
- early dementia early onset falling,
- Prominent autonomic dysfunction.
Tx for parkinson’s
- Levadopa/carbodopa (sinemet): carbodopa prevents conversion of levadopa to dopamine but can still be converted in the CNS, can cause paranoia and hallucinations ( due to pro dopaminergic action) Seroquel or clozaril ( antipsychotic) can be used to treat psychotic symptoms.
- Dopamine agonists: Ropinirole (requip) or Pampiprexole ( mirapex) Dopamine agonists have a potential side effect of impulse control disorders such as pathological gambling or hypersexuality. These are contraindicated in elderly pts due to psych symptoms
Selegiline ( MAO inhibitors), Artane ( anticholinergics) are also used
Tx of orthostatic hypotension in these patients ( dizziness with standing)
- Fludrocortisone ( mineralcorticoid increases blood pressure) Midodrine ( alpha agonist) can be used but can cause supine htn
Target for deep brain stimulation in parkinson’s disease?
Subthalamic nucleus ( lesions here cause hemiballism: wild, flinging movement of contralateral extremities). - Deep brain stimulation is reserved for ppl with advanced cases who have on-off response to meds. Dyskinesias and tremors are responsive to this therapy.
Parkinsonism, plus autonomic dysfunction ( orthostatic hypotention, constipation, bladder dysfunction, sweating dysfunction.) hot cross bun sign points to what disease?
Multisystem atrophy.
- PSP: lack of vertical gaze
- Lewy Body dementia: rapid progression of dementia, hallucinations and hypersensitivity to antipsychotics
- Medication-induced parkinsonism: exposure to antipsychotics/antiemetics
- Vascular Parkinsons: sudden onset or step wise deterioration
- Wilson’s disease: parkinsonism + hepatic failure
- MSA- cerebellar type: dysarthria, ataxia, lack of tremor, b/l onset of symptoms
- MSA- parkinson type: orthostatic hypotension, b/l onset of symptoms , lack of tremor
Essential tremor: b/l onset, action tremor family hx.
Huntington’s Dementia
Autosomal dominant, trinucleoide ( CAG) on chromosome 4 characterized by psychosis, dementia, and choreaform movements.
- Shows anticipation: each generation presents earlier
- Atrophy of the caudate can be seen on MRI
- Tx: Tetrabenazine ( dopamine depleting agent), or high potency typical antipsychotics ( haldol)
- poor prognosis
Chorea gravidarum
- Chorea of pregnancy
When young women present with chorea much check pregnancy test.
Sydenham’s Chorea
Post group A strep infection leading to chorea in children.
Wilson’s Disease
- autosomal recessive disorder of copper accumulation leading to damage of brain, eyes ( keyser-flecher rings),
- CNS symptoms can be any movement disorder and ataxia. Classically is a wing-flapping tremor, 1/2 of pts with have psych symptoms or mood.
- Dx; measure of copper in blood and urine + ceruloplasmin which is main copper carrying protein is decreased.
CO2 poisoning manifestation
- Residual parkinsonism plus signal abnormality in the globus pallidus
Spastic torticollis
Painful spasm of the neck muscles that forces the head to rotate and tilt forward, backwards or sideways. Imagining is usually normal and tx is botulinim toxin