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5.7 Movement disorders

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gojurufu's version from 2017-08-31 02:31

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major circuit of basal gangliacortex --> striatum --> GPi --> thalamus (VA/VL) --> cortex
definition of tremoran involuntary movement characterized by rhythmic oscillations of a body part produced by contractions of reciprocally innervated antagonist muscle groups
an involuntary movement characterized by rhythmic oscillations of a body part produced by contractions of reciprocally innervated antagonist muscle groupstremor
type of tremor seen in Parkinson diseaseresting tremor (3-7 Hz)
type of tremor seen in essential tremors and drug-induced tremorspostural/sustention tremor (6-11 Hz)
type of tremor seen in cerebellar diseaseintention tremor (3-7 Hz)
involuntary movements - slow writhing movementsathetosis
involuntary movements - sudden brief, irregular jerks associated with damage to striatumchorea
abrupt, high amplitude violent choreic movements (subthalamic)hemiballismus
co-contraction of agonist and antagonist muscle groups causing intermittent or persistent abnormal posturedystonia
sudden twitch-like muscular contractionsmyoclonus
arrhythmic lapse of sustained postureasterixus (liver flap)
rapid brief stereotyped jerks most often in face and head/neck, especially in childhoodtics
Four symptoms of Parkinson - TRAPTremor - Rigidity - Akinesia/hypokinesia - Postural instability
gait abnormalities, festination, retropulsionpostural instability (TRAP symptoms of Parkinson)
slowness of voluntary movement, reduction of automatic movementsakinesia/ hypokinesia (TRAP symptoms of Parkinson)
increased tone (lead pipe)rigidity (TRAP symptoms of Parkinson)
Parkinsonian tremor is common onset aspill-rolling tremor
Parkinsonian tremor is common onset as pill-rolling tremor and then progresses to supination-pronation tremor
what percentage of patients with idiopathic Parkinson have a tremor70%
decreased facial expression seen in Parkinsonhypomimia
pathology of Parkinsonloss of pigmentation and dopaminergic neruons in substantia nigra (and GP, putamen, locus ceruleus, dorsal motor nucleus of vagus)
eosinophilic intraneuronal inclusion bodies within CNSLewy bodies
pathophysiology of Parkinsondegeneration of SN-C dopaminergic neurons, disturbs the balance between inhibitory DA and excitatory Ach influence on GABA neuron in striatum
most common cause of tremoressential tremor
age of onset of essential tremor mean 5th decade
familial essential tremor pattern of inheritanceautosomal dominant
senile essential tremor age of onsetgreater than 65 years
are CNS lesions associated with essential tremors?no
what are the primary features of essential tremors?primarily distal upper extremities, wrist extension-flexion, finger abduction-adduction
when is essential tremor elicited bestwith arms in sustention (sustained posture)
what other TRAP symptoms are associated with essential tremornone - just tremor
what is tardive dyskinesiahyperkinetic movement - usually choreic
what might one cause of tardive dyskinesia be?prolonged treatment with neuroleptic medication
what type of tardive dyskinesias are most common?orofacial dyskinesias (buccolinguomasticatory syndrome) - tongue protrusions, lip-smacking, chewing
etiology of tardive dyskinesiadopamine receptor supersensitivity vs dopamine overactivity due to hypercholinergic state
neurodegenerative disease with loss of GABA neurons in striatumHuntington disease
genetic defect in Huntington diseaseAD, chromosome 4, abnormal CAG repeat number
pathology of Huntington diseaseatrophy of head of caudate
age of onset of Huntington diseaseextremely variable
symptoms of Huntington diseasepersonality changes, dementia, chorea, ataxia, death
Wilson disease is also known as hepatolenticular degeneration
autosomal recessive disorder of copper metabolismWilson disease
genetic defect associated with Wilson diseasechromosome 13 - gene ATP7B - membrane associated copper binding ATPase
what is the problem in Wilson diseasedefective copper binding ATPase causes defective binding of Cu to its transport protein, ceruplasmin, and reduction of biliary Cu excretion
Wilson disease can result in toxic deposition of copper wherebrain, liver, kidney
what is the age of presentation of Wilson diseaseteens
what are symptoms of Wilson diseasehemolytic anemia, renal tubular acidosis, hepatic dysfunction - cirrhosis; neurologic - wingbeating proximal tremor, chorea, rigidity, dystonia, facial grimacing (risus sardonicus), dysarthria, ataxia
wingbeating proximal tremor is a symptom of wilson disease
hepatic dysfunction - cirrhosis; neurologic - wingbeating proximal tremor, chorea, rigidity, dystonia, facial grimacing (risus sardonicus), dysarthria, ataxia; hemolytic anemia, renal tubular acidosis are symptoms ofwilson disease
pathology of wilson diseasedeposition of copper and neuronal loss especially in putamen and globus pallidus
corneal cooper deposition best seen with a slit lampKayser-Fleischer rings
Kayser-Fleischer ringscorneal copper deposition best seen with a slit lamp
how is wilson disease diagnosedlow serum copper levels, low serum ceruloplasmin levels, high urine copper excretion
how is wilson disease treatedreduction of dietary copper, chelation with penicillamine, trientine, zinc blocks dietary absorption, screen relatives
eye blinks, eye rolling, shoulder shrugs, lower facial twitching, rapid extension/flexion of limbssimple motor tics
sniffs, grunts, squeaks, shoutssimple vocal tics
phrases out of context, echolalia (repeating others' words), coprolalia (obscene words)complex vocal tics
tics can be primary disorders, or secondary to numerous conditions includingencephalitis, head trauma, drugs, toxins, Wilson disease
both multiple motor and one or more vocal tics present at some time, occur many times a day for at least 1 year, onset before age 21, no other medical condition to explain ticTourette syndrome
pathology of Tourrete syndromeabnormalities of corticostriatothalamocortical circuitry; unwanted movements are not suppressed
treatment of Tourrete syndromedopamine receptor agonists
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