movement disorders Flashcards
parkinson’s results from..
idiopathic?
reduced dopaminergic transmission at basal ganglia level
Idiopathic in ~75% of cases
Mutations in several genes may contribute
~1,000,000 affected
Peak onset 60 years of age
parkinson’s features
2 out of 3:
rest tremor
rigidity
bradykinesia
May include masked facies, decreased eye blinking, stooped posture, decreased arm swing
balance btw glutamate, ACh and dopamine
in Parkinson’s
less dopamine
ddx parkinsonism
Wilson’s
Huntington’s
Multisystem Atrophy
Multisystem Atrophy (formerly Shy Drager Syndrome)
ANS insufficiency (blood pools in legs), parkinsonism, LMN signs, cerebellar dysfunction -think tests?
Huntington’s
fam hx and dementia
tx: tetrabenazine for
dyskinesia – interferes with storage of amines
Wilson’s
don’t want to miss
Early age onset,
Kayser Fleischer rings (eyes), increased copper in tissues
parkinson’s motor features
Bradykinesia Micrographia Hypophonia Rest Tremor, 4 to 6 Hz, “Pill rolling” Rigidity, “Plastic” quality, “Cogwheel” sensation Short shuffling steps Myerson Sign
lead pipe vs cogwheel rigidity
stiff vs jerky
parkinson’s non-motor features
Depression, Anxiety Cognitive impairment Sleep disturbances Anosmia Orthostatic hypotension – Syncope
park: Neuropsychiatric Symptoms
Lewy body dementia
Depression – 50% of patients
park: patho: atrophy where?
degeneration of ?? with presence of ??
Frontal atrophy
Degeneration of dopaminergic cells with presence of Lewy bodies that have high concentration of alpha-synuclein
park tx: dopaminamimetic tx
-Dopamine agonists cross blood-brain barrier acting on dopamine receptors
-Levadopa converted to dopamine in body
Combined with carbidopa, which inhibits enzyme that breaks down levodopa to dopamine, but does NOT cross blood brain barrier
levels can go up and down
take at sp. times
park tx: selegilline
Monoamine oxidase inhibitor (MAO-I)
Added to help with tremor
part tx: Entacapone
Catechol O-methyltransferase (COMT) inhibitors
Blocks enzyme that degrades levodopa and dopamine, allows for more sustained levels of response
park tx considerations
Time of medications very important to prevent symptom return/withdrawal
Don’t give Levodopa if patient has narrow angle glaucoma, or is on MAOI within 2 weeks of stopping the drug (HTN crisis)
combo Carbidopa/Levodopa brand name
Sinemet
another tx for parkinson’s
deep brain stimulation
essential tremor
Most common movement disorder (hands, head, or voice)
May be worse with stress/emotions, sleep deprived
Autosomal dominant
Generally bilateral
essential tremor improved with
with alcohol, certain beta-blockers (propranolol)
Hemiballismus/hemichorea
Dramatic “flailing”
Violent form of chorea
Supportive care
Tx: haloperidol, propranolol, phenytoin, clonazepam, baclofen
Huntington’s
genetic inheritance?
onset?
Autosomal Dominant –
CAG mutation on Chromosome 4
genetic testing confirms diagnosis
Fatal
Onset 30-50 years of age (50% chance of your children have it)
Chorea “dance”
dystonia
tx?
Syndrome of involuntary contractions of muscles, twisting and repetitive movements
Treatment is symptomatic Anticholinergic medications (trihexyphenidyl)
If dystonia is due to Wilson’s disease ??
copper chelation therapy
Tourette Syndrome
age of onset?? how often?
Tic
Neurologic and behavioral
Symptoms before age 21
criteria: tics occur for at least 1 year, tics very in number frequency and nature over time
Tourettes tx: atypical antipsychotic
CBT (cog. behav. therapy)
Clonidine, Botox options
Risperidone, olanzapine, ziprasidone in more severe cases
Tarditive dyskenisia:
med-induced hyperkinetic disorder
Usually from exposure to central dopamine blockers
1/3 temporary, but can be perminant
Myerson sign
glabellar tap sign is a medical condition where a patient is unable to resist blinking when tapped on the glabella, the area above the nose and between the eyebrows
Neuroleptic Malignant Syndrome (NMS):
med-induced hyperkinetic disorder
Serious complication, mortality as high as 20%
Muscle rigidity
Treatment is dantrolene, cooling body
tx, problem is recognition, fam hx
