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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
Q

Diffuse lew Body Disease

A

Early dementia; prominent visual hallucinations; extreme sensitivity to EPS of neuroleptics

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26
Q

Vascular parkinsonism

A

“Lower-Half” Parkinsonism

Rigidity in the legs and marked gait impairment

27
Q

Multiple System Atrophy

A

Early and prominent features of autonomic dysfunction; evidence of corticospinal tract dysfunction; cerebellar signs; stimulus-sensitive myoclonus; vocal cord paresis

28
Q

Stiff-Person Syndrome

A

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
Q

Best antispasticity agents

A

Benzos and Baclofen

30
Q

anti-GAD antibodies (glutamic acid decarboxylase) is associated with…

A

Stiff-Person syndrome

31
Q

Chronic axial muscle rigidity and stiffness with superimposed painful muscle spasms

A

Stiff-person syndrome

32
Q

Resting Tremor

A

Present when body part is not moving

33
Q

Postural Tremor

A

Emerges during sustained maintenance of a posture

34
Q

Action Tremor

A

Appears during a voluntary movement

35
Q

Intention Tremor

A

Action tremor that increases as target is approached

36
Q

Essential Tremor

A

Condition in which postural tremor is the only symptom

37
Q

Essential Tremor findings

A

Begins at any age, develops insidiously, and progresses gradually
Always bilateral
Improves with a little Et-OH
Tx with primidone and propranolol

38
Q

Causes of resting tremor

A

PD and other parkinsonian syndromes

39
Q

Causes of Postural tremor

A

Essential tremor, physiological tremor, drugs (beta-agonists, theophylline), alcohol

40
Q

Causes of Action Tremor

A

Cerebellum and cerebellar outflow tract dysfunction (e.g. infarction, MS, tumor, Wilson disease, drugs)

41
Q

Chorea definition

A

Involuntary, abrupt, and irregular movements that flow as if randomly from one body part to another.

42
Q

Chorea findings

A

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
Q

Causes of Chorea

A

Huntington’s, poststreptococcal infection, systemic lupus erythematosus, thyrotoxicosis, and pregnancy

44
Q

Ballism

A

Large-amplitude and poorly patterned flinging or flailing movements of a limb that are frequently unilateral (hemiballismus)

45
Q

Cause of Ballism

A

Contralateral lesion in the caudate, putamen, or subthalamic nucleus. Usually stroke. Hyperglycemia may cause hemiballismus.

46
Q

Ballism tx

A

Dopamine-depleting and blocking agents. When severe, contralateral thalamotomy or pallidotomy may be beneficial.

47
Q

Dystonia

A

Sustained muscle contraction leading to repetitive twisting movements or abnormal postures.

48
Q

Geste antagoniste

A

Gently touching the affected body parts of dystonia can diminish it.

49
Q

Dystonia progression

A

Starts off being triggered by motion, severe dystonia is present at rest. Worsened by emotional stress, improved by relaxation and sleep.

50
Q

Causes of Dystonia

A

Wilson’s, degenerative diseases (including PD, HD, MSA), and other CNS disorders (anoxia, trauma, stroke, MS, drug-induced).

51
Q

Idiopathic Torsion Dystonia

A

Familial condition that may manifest as torticollis, writer’s cramp, blepharospasm, or spasmodic dysphonia.

52
Q

Myoclonus

A

Sudden lightning-like movement produced by abrupt and brief muscle contraction (positive myoclonus) or inhibition (negative myoclonus or asterixis).

53
Q

Causes of Myoclonus

A
  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
Q

Tx for Myoclonus

A

Clonazepam is the most effective. Valproate may be used.

55
Q

Tics

A

Abrupt, stereotyped, coordinated movements or vocalizations.

56
Q

PANDAS spelled out

A

Pediatric autoimmune neurologic disorders associated with streptococcal infection

57
Q

PANDAS is…

A

Children develop exacerbation of tics, OCD, or both following group A beta-hemolytic strep infection.

58
Q

Wilson Disease

A

Aut. rec.
Copper metabolism dysfunction (impaired conjugation of copper to ceruloplasmin)
Liver dysfunction and Neuropsychiatric symptoms

59
Q

Wilson Disease Neurologic manifestations

A

Tremor, ataxia, dysarthria, dyskinesia, parkinsonism, cognitive dysfunction and mood and personality issues.

60
Q

Wilson disease corneal manifestation

A

Kayser-Fleischer ring (limbic region of the cornea)

61
Q

Wilson dz diagnosis

A

24-hour urinary copper excretion is most sensitive

Elevated serum copper, low ceruloplasmin

62
Q

Wilson dz Tx

A

D-penicillamine chelation

New therapy is Trientine + zinc, less toxic

63
Q

Paroxysmal Dyskinesias

A

Recurrent attacks of hyperkinesis with preserved consciousness.

64
Q

Hyperkinetic and hypokinetic movement disorders as well as cognitive, personality, and mood distrubances

A

Wilson’s

x Neurology 200 (25 decks)

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