15. Clinical Approach to Movement Disorders

Question 1

What type of movement disorder is aka Akinetic Rigid Syndromes, which is MC parkinsons disease but encompasses idiopathic parkinson disease, postencephalitic, toxin induced (MC= carbon monoxide), drug induced (neuroleptics- haldol/prochlorperazine) and MPTP (meperidine) induce parkinsons symptoms?

Answer 1

Bradykinetic Disorders (lewy body dementia is a type)

Question 2

What disease is caused by depletion of dopamine in the nigrostriatal system, disrupting the balance of dopamine and acetylcholine, asso w Cardinal findings= tremor (resting unilateral/pill rolling), rigidity (inc resistance to movement/cogwheel rigidity), and bradykinesia (slowness of movement-cant initiate movement)?

Answer 2

Idiopathic Parkinson’s Disease

Sx if Rx dont work= deep brain stimulation MC*

Question 3

other common features of Idiopathic Parkinson’s Disease is postural instability, hypophonia, hypomimia (masked facies), decreased frequency of eye blink, myerson’s sign (tap on forehead leads to sustained blink), gait disturbance (festinating), dyautonomia (othostatic hypotension**MC, incontinence, impotence and anhydrosis) and many will develop?

Answer 3

Dementia

Question 4

What akinetic rigid syndrome is assoc with bradykinesia and rigidity along with loss of voluntary control of eye movements (vertical gaze)- such that involuntary is intact (dr moving head) but voluntary when following finger- typically cannot look DOWN**?

Answer 4

Progressive Supranuclear Palsy PSP

Question 5

What akinetic rigid syndrome is associated with bradykinesia and rigidity along with *pronounced autonomic dysfunction?

Answer 5

Multiple System Atrophy MSA (shy-drager syndrome)

Question 6

What akinetic rigid syndrome is characterized by both cortical and basal ganglionic dysfunction, bradykinesia, rigidity, and may also see cortical sensory loss, apraxia, myoclonus or aphasia?

Answer 6

Cortical Basal (ganglionic) degeneration CBD

No treatment exists for akinetic rigid syndromes besides anti-parkinson tx - which do not work-but still try

Question 7

Hyperkinestic movement disorders are associated with chorea (irregular brief dancelike movements), athetosis (writhing, sinuous movements), dystonia (muscle contractions that produce twisting and repetitive movements), ballism (large amplitude, flinging movement from proximal extremity) and tic which is what?

Answer 7

breif rapid repetitive, purposeless stereotyped action

Question 8

What disease has gradual onset and progression of chorea and dementia by inherited trinucleotide repeat gene defect on chr 4, AD inhereitance, sx start ages 30-50 with lifespan of 15yrs after onset sx, genetic testing for dx, no known cure?

Answer 8

Huntington’s Disease

**Psych symptoms first

Question 9

What type of chorea occurs mainly in children and adolescents as a complication of a previous infection with group A hemolytic strep, may be a form of arteritis, characterized by unilat choreiform movements which can be confused for restlessness/fidgeting, also see behavior changes- tx=bedrest and antibiotics?

Answer 9

Syndenham’s Chorea

Question 10

What type of dystonia is characterized by dystonic movements and postures without other signs, may be inherited as AD, AR, or Xlinked recessive disorder, whose onset may be in childhood or later in life but remains throughout life?

Answer 10

Idiopathic Torsion Dystonia

Question 11

Idiopathic Torsion Dystonia is associated with torticollis, blepharospasm (closure of eyelid), oromandibular dystonia (spasm muscle around mouth), arm may be help hyperpronated with wrist flexed and fingers extended, leg may be help in extension with pronation and?

Answer 11

inversion of the foot

Question 12

Idiopathic Torsion Dystonia is treated with Carbamazepine, anticholinergics, benzodiazepines, neuroleptic drugs, baclofen, and what drug which is tried FIRST?

Answer 12

low doses of Levodopa

Question 13

What type of dystonia has dystonia confined to a FOCAL AREA, associated with ONLY ONE OF THE FOLLOWING: blepharospasm, oromandibular dystonia, spasmodic torticollis, writers cramp, used same meds as idiopathic torsion dystonia but BOTOX** is treatment of choice to weaken muscles in this focal disease?

Answer 13

Focal Torsion Dystonia

EMG needle guidance to see most active muscle for injection with BOTOX

Question 14

What is an AR disorder of copper metabolism that produces neurologic and hepatic dysfunction localized to chr13 (ATP7B gene), decreased binding of copper to ceruloplasmin leading to large amount of free copper deposited into tissues- presents in childhood or young adult life?

Answer 14

Wilson’s Disease

Question 15

Wilson’s Disease has both bradykinetic and hyperkinetic** (imp) features, including resting or postural tremor, choreiform movements, rigidity, bradykinesia, dyarthria, dysphagia, ataxia, personality/behavioral changes, dementia and psychosis or?

Answer 15

hallucinations

Normal 19y/o with reptilian faces and went from A+ student to doing coke in the bathroom during study hall

Question 16

Wilson’s Disease is diagnosed based on exam findings which include increased amounts of copper excretion in 24 hr urine collection, decreased serum ceruloplasmin level**, and kayser fleischer ring on eye exam, (copper deposited in Basal ganglia)….. The treatment for wilsons include restriction of dietary copper and what drug?

Answer 16

Penicillamine (copper chelating agent)

fatal if not caught- VVVV responsive to tx

Question 17

What disorders have single motor ticks that include blinking, coughing, throat clearing and often occur as a benign entity?

Answer 17

Tic Disorders

Question 18

What tic syndrome is a chronic multiple motor and vocal tic disease onset before 21, most cases are sporadic, MC in males, vocal tics vary in presentation, can involve barks, hisses, grunts, throat clearing, and coughing?

Answer 18

Gilles De La Tourette’s Syndrome

Question 19

With Gilles De La Tourette’s Syndrome you may also see coprolalia (vulgar/obscene speech), echolalia (parroting speech of others), echoproxia (imitations of others movements), palillalia (repetition of words or phrases), tx with Haloperidol, phenothiazines or most commonly?

Answer 19

clonidine

Question 20

What type of tremor presents as postural or kinetic tremor of both** hands and may involve head or voice, can begin in early adulthood but often later in life, progresses slowly over decades, does result in disability- but social embarrassment is common, EtOH decreases*** the tremor?

Answer 20

Essential Tremor (Benign Familial Tremor)

not at rest only when moving

Question 21

Essential Tremor (Benign Familial Tremor) can be treated with beta blockers (propranolol), primidone, benzodiazepines, topiramate or if none of that works what surgery can be done?

Answer 21

Deep brain stimulation DBS