Extrapyramidal Disease Flashcards
oxidative stress
adverse effects on tissues of reactive oxygen species (free radicals)
extrapyramidal
those parts of the motor system that are not directly pyramidal; that is not the corticospinal tracts (upper motor neurons); always includes basal ganglia– often includes cerebellar system
parkinson disease
dopamine deficiency syndrome characterized by bradykinesia, poor postural responses, rigidity, tremor
parkinson disease
dopamine deficiency syndrome characterized by bradykinesia, poor postural responses, rigidity, and tremor
bradykinesia
pathological slowness of movement
delayed postural reflexes
refers to a delay in the normal righting responses that permit stance and gait
resting tremor
tremor that occurs with the hands resting in the lap or at the sides when walking or standing
cogwheel
referring to an irregular, ratchety resistance to passive movement which is superimposition of the tremor rigidity
festination
shortening of stride such that the patient takes many short steps to cover a distance; they may become propulsive if they cannot catch up with their center of gravity
on-off
sudden and unpredictable loss of effectiveness of parkinson’s medication
hypophonia
pathological loss of power in the voice
corpus striatum
caudate nucleus
lenticular nucleus- putamen, globus pallidus
subthalamic nucleus of luys
target for movement disorders because overactive in many disorders such as parkinson disease
where do you do DBS?
subthalamic nucleus of luys
substantia nigra
striatum
caudate
putamen
pars compacta
produces dopamine and axons of dopaminergic neurons that project to
striatum–>globus pallidus–>thalamus–>cortex–>back to red nucleus/brainstem/cerebellum/spinal cord
caudate
puatmen
basal ganglia functions
unconsciously; helps to correct pattern organized movements nad inhibit unwanted ones
slowest postural reactions
delayed reactions to being knocked off balance
mask facies
decreased facial expressiveness; bradykinesia of facial muscles
rigitidy + tremor
cogwheeling
differential dx of choreoathetosis
autoimmune-sydenham's (post strep) encephalitis metabolic-thyrotoxicosis, hyper and hypothyroidism, hypocalcemia, hypoglycemia drugs- neuroleptics poisioning- CO, mercury genetic disease- huntington, wilsons, other pregnancy senie, cerebrovascular perinatnal injury
huntington disease genetics
autosomal dominant with complete penetrance
epi of disease
CAG repeats- number of repeats anywhere from 35-40
mean onset huntingtons
35-40; mean duration 13 years
clinical symptoms
chorea athetosis severe and disabling dementia behavior changes (not nec in that order)
symptoms of huntington’s made worse by
levodopa
pharm treatment huntingtons
neuroleptic agents: most effective dopamine blocking properties–> haloperido, perphenazine
neurotransmitter depletors: reserpine (potentiates depression), tetrabenazine
chronic use of neuroleptics
sensitizes system–>reverse effect–>tardive dyskinesia
essential treatment genetics
sporadic
OR
autosomal dominant with incomplete penetrance
essential tremors worsen
with age
what type of tremor is an essential tremor?
postural and intentional
symmetric
postural tremor
hands shake while held in sustained position
action tremor
worsens with finger to nose testing or bringing a spoon to the mouth
suppresses essential tremor
ETOH
treatment of essential tremor
beta blockers Primidone Neurotonin, Topiramate- anti epileptic ethanol surgery-thalamotomy, DBS (ventral intermediate nucleus of thalamus) botulinum toxin
what drug do you not use for essential tremor
valproate- high tremor side effect
parkinson tremor
static tremor
usually unilateral in beginning–>progresses quickly with noticeable worsening when seen at six month intervals
parkinson vs essential area of body
park- hand/leg
essential-hand/head/voice
progress parkinson vs essential
parkinson- quickly
essential- slowly; over years
diagnosis of parkinsonism requires 2/3 of the cardinal signals
bradykinesia
rigidity
tremor
things that cause parkinsonism
genetics
other things like drugs
Parkinson DIsease
marked depigmentation of substantia nigra dopamine loss (50% asymptomatic, 70% first symptom, 90% death)
parkinson pathological hallmark
lewy body inclusions
pathology correlates best with
bradykinesia
survival with parkinsons
15 years after onset
longer in non-demented PD cases
longer with L-Dopa use
cause of death is usually
pulmonary infection/aspiration
urinary tract infection
MPTP toxicicty
reproduces all major clinicopathological elements of PD (including lewy bodies)
MPTP is in astrocytes–>converted to MPP+ by MAO-B
MPP+= toxic metabolite; free radical that destroys mito fx and kills sells by inhibiting complex 1 of resp pathway–>ATP, cell death
corner stone of parkinson’s treatment
promoting DA
antagonizing acetylcholine
carbidopa
taken with levodopa to prevent conversion of levodopa in the blood before it gets to the brain, therefore use a lot less levodopa with fewer systemic effects, such as nausea
too much DA too soon produces oxidative metabolites–>damages R
other pharm of parkinson
COMT inhibitors
MAO-B inhibitors
anticholinergics
amantadine
suppresses dyskinesias that people get as a side effect of too much dopamine
THalmotomy
treat tremor by ablating globus pallidus
palidotomy
useful for bradykinesia or rigidity but not tremor
DBS for parkinson
most effective in internal segment of gp and esp subthalamic nucleus
Tics
fleeting, purposeful actions that may be simple (appearing as a muscle twitch) or complex (repetitive behavior)
suppression
not voluntary, but tics can be suppressed for a perioid of time through force of will
Tourette syndrome
motor and repetitive coval tics-throat clearing, snorting, sniffinf
tics begin
age 5-18
what worsens tics?
psychostimulants used to treat adhd
medication for tics
neuroleptic
hemibalism leasion
contralateral subthalamic nucleus
