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x Neurology 200 > Chapter 16: Movement Disorders > Flashcards

Chapter 16: Movement Disorders Flashcards

1
Q

Idiopathic Parkinson Disease is a…

A

Chronic degenerative disorder with characteristic clinical findings, response to L-dopa replacement therapy, and pathologic changes in the brain.

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2
Q

Parkinsonism vs. PD

A

Has some characteristics of PD but differs in other ways

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3
Q

Genes responsible

A

alpha-synuclein, parkin, DJ-1, PINK1, UCH-l1, and LRRK2

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4
Q

Progressive death of what neurons

A

Substantia nigra pars compacta

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5
Q

Pathologic hallmark of PD

A

Lewy Body

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6
Q

4 Cardinal clinical features of idiopathic PD

A

Tremor
Rigidity
Bradykinesia
Postural Instability

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7
Q

PD Tremor

A 
Slow (3-5 Hz)
Most prominent when limb is at rest
Distal arm>leg
Pill rolling
May affects lips, chin, and tongue
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8
Q

PD Rigidity

A

Distal limbs>axial muscles

Lead pipe or Cogwheel

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9
Q

Bradykinesia and Bradyphrenia

A

Slow movement and thought

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10
Q

Postural instability

A

Impaired postural reflexes responsible for falls

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11
Q

PD and Dementia

A

25-30% of patients get dementia

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12
Q

PD Mainstay of Treatment

A

L-Dopa replacement
Given together with carbidopa (prevents peripheral conversion of L-dopa to dopamine; reduces peripheral dopaminergic side effects)

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13
Q

Other DA agonists

A

Ropinirole: may or may not reduce DOPA-induced dyskinesias

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14
Q

Toxic dopamine effects in the periphery

A

N/V, HTN

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15
Q

Treating tremor-predominant disease

A

Anticholinergics or amantadine

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16
Q

Tx of bradykinesia, rigidity, and gait disturbance

A

Amantadine

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17
Q

Which MAOI

A

Selegeline, MAO-B inhibitor

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18
Q

Deep brain stimulation and Lesioning

A

Motor thalamus, internal segment of the globus pallidus, or the subthalamic nucleus

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19
Q

Catechol-O-methyl transferase (COMT) inhibitors

A

Decrease L-DOPA removal

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20
Q

Neuroleptic Malignant Syndrome

A

Muscular rigidity, fever, autonomic lability, altered level of consciousness, elevated creatine kinase level, and leukocytosis

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21
Q

Akathisia

A

Dysphoric state of needing to always be moving around.

Anticholinergics and beta blockers

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22
Q

Parkinsonian Syndromes

A 
Progressive Supranuclear Palsy
Corticobasal Ganglionic Degeneration
Diffuse Lewy Body Disease
Vascular Parkinsonism
Multiple System Atrophy
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23
Q

Progressive Supranuclear Palsy

A

Supranuclear ophthalmoplegia, with limitation of vertical>horizontal gaze; axial rigidity and neck extension; early falls due to impaired postural reflexes, neck extension, inability to look down

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24
Q

Corticobasal ganglionic degeneration

A

Apraxia, alien-limb phenomenon and cortical sensory impairment; severe unilateral rigidity; stimulus-sensitive myoclonus

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25
Diffuse lew Body Disease
Early dementia; prominent visual hallucinations; extreme sensitivity to EPS of neuroleptics
26
Vascular parkinsonism
"Lower-Half" Parkinsonism | Rigidity in the legs and marked gait impairment
27
Multiple System Atrophy
Early and prominent features of autonomic dysfunction; evidence of corticospinal tract dysfunction; cerebellar signs; stimulus-sensitive myoclonus; vocal cord paresis
28
Stiff-Person Syndrome
Fluctuating and progressive muscle rigidity with spasms. Autoimmune or paraneoplastic process Axial and trunk muscles with spread to proximal limb muscles Lumbar Hyperlordosis "Tin man" gait Paroxysmal painful muscle spasms
29
Best antispasticity agents
Benzos and Baclofen
30
anti-GAD antibodies (glutamic acid decarboxylase) is associated with...
Stiff-Person syndrome
31
Chronic axial muscle rigidity and stiffness with superimposed painful muscle spasms
Stiff-person syndrome
32
Resting Tremor
Present when body part is not moving
33
Postural Tremor
Emerges during sustained maintenance of a posture
34
Action Tremor
Appears during a voluntary movement
35
Intention Tremor
Action tremor that increases as target is approached
36
Essential Tremor
Condition in which postural tremor is the only symptom
37
Essential Tremor findings
Begins at any age, develops insidiously, and progresses gradually Always bilateral Improves with a little Et-OH Tx with primidone and propranolol
38
Causes of resting tremor
PD and other parkinsonian syndromes
39
Causes of Postural tremor
Essential tremor, physiological tremor, drugs (beta-agonists, theophylline), alcohol
40
Causes of Action Tremor
Cerebellum and cerebellar outflow tract dysfunction (e.g. infarction, MS, tumor, Wilson disease, drugs)
41
Chorea definition
Involuntary, abrupt, and irregular movements that flow as if randomly from one body part to another.
42
Chorea findings
Patients are often unaware of even severe chorea, clumsiness or incoordination early on, involvement of bulbar muscles may cause dysarthria and dysphagia, motor impersistence (can't sustain contraction); can't maintain tight handgrip or keep tongue protruded (milk-maid grip and serpentine tongue, respectively)
43
Causes of Chorea
Huntington's, poststreptococcal infection, systemic lupus erythematosus, thyrotoxicosis, and pregnancy
44
Ballism
Large-amplitude and poorly patterned flinging or flailing movements of a limb that are frequently unilateral (hemiballismus)
45
Cause of Ballism
Contralateral lesion in the caudate, putamen, or subthalamic nucleus. Usually stroke. Hyperglycemia may cause hemiballismus.
46
Ballism tx
Dopamine-depleting and blocking agents. When severe, contralateral thalamotomy or pallidotomy may be beneficial.
47
Dystonia
Sustained muscle contraction leading to repetitive twisting movements or abnormal postures.
48
Geste antagoniste
Gently touching the affected body parts of dystonia can diminish it.
49
Dystonia progression
Starts off being triggered by motion, severe dystonia is present at rest. Worsened by emotional stress, improved by relaxation and sleep.
50
Causes of Dystonia
Wilson's, degenerative diseases (including PD, HD, MSA), and other CNS disorders (anoxia, trauma, stroke, MS, drug-induced).
51
Idiopathic Torsion Dystonia
Familial condition that may manifest as torticollis, writer's cramp, blepharospasm, or spasmodic dysphonia.
52
Myoclonus
Sudden lightning-like movement produced by abrupt and brief muscle contraction (positive myoclonus) or inhibition (negative myoclonus or asterixis).
53
Causes of Myoclonus
1. Physiologic: Hypnic jerks; anxiety and exercise induced; hiccups 2. Essential: nonphysiologic that occurs in isolation without other symptoms. Familial or sporadic. Improves with alcohol. 3. Epileptic: Generalized epilepsies, myoclonic epilepsis 4. Symptomatic: metabolic encephalopathy (uremia, liver failure, hypercapnia); Wilson disease, Creutzfeldt-Jakob disease, Hypoxic brain injury
54
Tx for Myoclonus
Clonazepam is the most effective. Valproate may be used.
55
Tics
Abrupt, stereotyped, coordinated movements or vocalizations.
56
PANDAS spelled out
Pediatric autoimmune neurologic disorders associated with streptococcal infection
57
PANDAS is...
Children develop exacerbation of tics, OCD, or both following group A beta-hemolytic strep infection.
58
Wilson Disease
Aut. rec. Copper metabolism dysfunction (impaired conjugation of copper to ceruloplasmin) Liver dysfunction and Neuropsychiatric symptoms
59
Wilson Disease Neurologic manifestations
Tremor, ataxia, dysarthria, dyskinesia, parkinsonism, cognitive dysfunction and mood and personality issues.
60
Wilson disease corneal manifestation
Kayser-Fleischer ring (limbic region of the cornea)
61
Wilson dz diagnosis
24-hour urinary copper excretion is most sensitive | Elevated serum copper, low ceruloplasmin
62
Wilson dz Tx
D-penicillamine chelation | New therapy is Trientine + zinc, less toxic
63
Paroxysmal Dyskinesias
Recurrent attacks of hyperkinesis with preserved consciousness.
64
Hyperkinetic and hypokinetic movement disorders as well as cognitive, personality, and mood distrubances
Wilson's

x Neurology 200 (25 decks)

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