Movement Disorders - Cohen Flashcards
parkinsons disease
4 main features
1 - resting tremor
2 - bradykinesia - slow movement - initiating movement
3 - dysequilibrium - instability of gait and posture
4 - rigidity - later in illness
diagnosis - should have at least 3 of 4
tremor
regular oscillatory movement
usually always present
poor control agonist/antagonist muscles
worse with fatigue, stress, drugs, age
chorea
dance like movement
myoclonus
brief jerk
athetosis
writhing movement
snake/worms
dystonic movement
slow uncomfortable movement
pathologic tremor
disturb normal functioning
resting tremor
parkinsons
often asymmetric
first in hand
-to head, jaw, leg
can be only feature of parkinsons
parkinsons bradykinesia
slow movement
patient like statue when standing - no fidgeting when sitting
drool - hard to swallow
can overcome - under stress - kinesis paradoxica
kinesis paradoxica
in parkinsons bradykinesia
-pt can overcome it
dysequilibrium parkinson
hard to become steady
don’t swing arms well
dangerous sign and least responsive to meds
parkinsons rigidity
later sign
-cramps - shoulders
problem when medication wears off
other symptoms of PD
sleep disorder loss semll autonomic problem cramps, pain depression small handwriting loss of power of voice - monotonous and hurried
pathology of PD
lewy bodys - substantia nigra
loss of dopaminergic neurons
loss of cells in BS, thalamus, cerebarl hemisphers, and basal ganglia
lewy bodies
alpha synuclein
eosinophilic structures in cytoplasm of neurons - especially in substantia nigra
genetics of PD
rare in twins
genes - PARK 1-11
markin protein
epidemiology of PD
age of incidence 55-59yo
older - rapid deterioration
majority men
tx of PD
best tx - L-dopa
dopamine does not cross BBB - but L-dopa does
dopamine synthesis
from tyrosine
rate limiting - tyrosine hydroxylase
carbidopa
inhibit metabolism of L - dopamine in blood
-more L-dopa to brain
sinemet
levodopa/carbidopa
tx - can wear off after 5-10 years
can have dyskinesia - too much movement
also possible psychosis
other dopaminergic drugs
bind DA receptors - especially D2
less wearing off effect
not as effective as sinemet
psychosis may occur
psych problems in PD
dementia - later in disease
depression common
psychosis - troubling, violent/aggression
50% PD patient
die within 15 years
surgery for PD
deep brain stimulation - very effective - advanced PD disease
also - small strokes in lower basal ganglia - thalamus or subthalamic nucleus
drug induced parkinsons
with drugs that block dopamine
nausea drugs - phenothiazines and butyrophenones
progressive supranuclear palsy
PSP
- severe dysequilibrium
- falls early - as is choking
NO tremors
puzzled look on face
progressive loss of extraocular movements
-cannot move eyes
often die - aspiration pneumonia
PSP treatment
levodopa works for some
but no known tx
essential tremor
4-8 Hz
symmetric - arms, head, voice, legs
action tremor or familial
“Is there when I do things.”
DDx for essential tremor
hyperthyroid
antidepressant drugs
cerebellar disease
parkinsonian features
tx of essential tremor
weight arms and wrists
alcohol helps
meds - propranolol and primidone
tics
mostly disease of kids
-quick random simple movements
usually head
may fade with time
never serious pathology
tourettes syndrome
motor and vocal tics
-start before age 21
minority use obscenities
does not fade away with adolescence
many also have OCD and depression
pathology of tourette
unknown
possible dopamine derangement
Tx of tourette
haloperidol or other D2 blockers
chorea
dance like disorder
irregular and variable movement
no clear pattern
st vitus dance
untreated group A strep infection
-syndeham chorea
delayed autoAbs for that affect brain
huntington disease
inherited disease - auto dom
typically begin 40-50yo
trinucleotide repeat chromosome 4
-CAG
movement disorder - chorea, tremor, jerk
cognitive loss - dementia
psychiatric - agitation, depression, psychosis
often suicidal
Tx of huntington
dopamine blockers
new drug - tetrabenazine
diagnosis of HD
fam hx
symptoms in middle age
genetic testing available - huntingtin protein
restless leg syndrome
cannot stop moving
sleep disorder as well
bad when get into bed
pathology of RLS
reduction of brain iron
reduced dopamine activity in BS and basal ganglia
tx - dietary iron
tyrosine hydroxylase - iron cofactor
L-dopa helps
best tx - dopamine agonists - pramipexole
