Movement Disorders Flashcards

1
Q

Generally, movement disorders are classified as what?

A

1. Bradykinetic

2. Hyperkinetic

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2
Q

Bradykinetic disorders (aka __________) ALL have what 2 features?

A
  1. Akinetic Rigid Syndromes
  2. SLOW and STIFF
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3
Q

What is the MC Bradykinetic Disorder (Akinetic Rigid Disorder)?

A

Parkinsonism, encompasses many disorders

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4
Q

What are the MCC of Parkinsonism?

A
  1. Idiopathic Parkisons Disease
  2. Von Economo Encephalitis (post-encephalitc)
  3. Toxin-induced (CO*, CO2, Manganese)
  4. Drug-induced (Neuroleptics* (Haloperidol or prochlorperazine) or Metoclopramide)).
  5. MPTP (meperidine analogue)
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5
Q

What is the cause of idiopathic Parkisinons Disease?

A

Depletion of DA in the nigrostriatal symptom, disrupting the balance of DA and ACh (MORE ACh)

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6
Q

What kind of tremor do patients with Parkinson’s Disease initially present with?

A
  • UNILATERAL resting, pill-rolling tremor (does not occur with movement)
  • Rabbit tremor = mouth or chin tremor.
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7
Q

3 Cardinal Signs of Idiopathic Parkinsonism

A

*TRAPS = slow, stiff and shaky

  1. T = tremor (unilateral, pill rolling tremor at rest)
  2. R = rigidity
  3. A = akinesia and bradykinesia
  4. P = postural instability
  5. S = shufflng gait, that festinates (starts slow => goes fast)
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8
Q

Other features of Parkisonism

A
  1. Hypophonia (quiet)
  2. Hypomimia = masked facies/reptilian stare => ⬇︎ blinking
  3. Dysautonomia (orthostatic hypotension)
  4. Many develop dementia
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9
Q

What sign can indicate Parkinsonism?

A

Meyers sign = repetive tapping on the forehead => sustained blink.

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10
Q

Although findings are similar to PD, what distinguishes Progressive Supranuclear Palsy (PSP)?

A
  1. Loss of VOLUNTARY control of eye movement, especially vertical gaze (can still involuntarily look vertically)
    • Bradykinesia & Rigidity
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11
Q

Although findings are similar to PD, what distinguishes Multiple Systems Atrophy (MSA)?

A
  1. PRONOUNCED autonomic dysfunction
    • bradykinesia + rigidity
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12
Q

Although findings are similar to PD, what distinguishes Lewy Body Dementia?

A
  • Visual hallucinations of animals and children that often do NOT bother patient.
    • bradykinesia & rigidity
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13
Q

Although findings are similar to PD, what distinguishes Cortical Basal Degeneration (CBD)?

A
  • Cortical and ganglionic dysfunction
  • May see cortical sensory loss, apraxia, myoclonus or aphasia
    • bradykinesia + rigidity
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14
Q

What should be avoided in patients with LB dementia?

A

NEUROLEPTICS => death

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15
Q

As a general rule, do anti-parkinson meds work on the other bradykinetic disorders/akinetic rigid syndromes (PSP, MSA, CBD)?

A

NO and no other useful treatment exists.

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16
Q

Treatment for Parkinsons

A
  1. DA AGO
  2. Levodopa
  3. COMT-inhibitors
  4. Anticholinergics
  5. MAO-B inhibitors
  6. Amandatine
  7. Surgery = MC = deep brain stimulation
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17
Q

MC surgery to treat Parkisons

A

DBS (Deep brain stimulation)

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18
Q

What are hyperkinetic movements?

A
  1. Chorea
  2. Athetosis
  3. Dystonia
  4. Ballism
  5. Tics
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19
Q

Sustained muscle contraction that produce twisting, repetitive movements and abnormal postures

A

Dystonia

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20
Q

Irregular, brief dance-like movements that pt tries to make it look purposeful

A

Chorea

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21
Q

Writhing (snake-like), sinous movements (often occur in combo w/ chorea)

A

Athetosis

22
Q

Large, amplitude flinging movements, usually from proximal parts of extremity (shoulder or hip)

A

Ballism

23
Q

Brief, rapid, repetitive, seemingly purposeless stereoptyped action that may involve single or multiple muscle groups.

A

Tics

24
Q

What conditions commonly exhibit Chorea?

A
  1. Huntingtons Disease
  2. Syndenham’s Chorea
25
Q

What is Syndenhams Chorea?

A
  • A complication of Group A Hemolytic Strep in children and adolescents (most likely a form of arteritis) that causes unilateral chorea (when mild, confused for restlessness or fidgeting) + behavioral changes.
26
Q

Child gets sick and when he comes back to school, his teacher notices he is restless, fidgeting and change in his behavior, causing him to get in trouble alot.

Diagnosis?

A

Syndenhams Chorea = due to GAHS infection

27
Q

Treatment for Syndenhams Chorea?

A
  1. Bedrest
  2. ABX
28
Q

What is Huntingtons Disease?

A
  • AD disorder of inherited CAG repeat that causes gradual onset and progression of chorea + dementia
29
Q

Inheritance of Huntingtons

A
  • AD; offspring of affected person have 50% of getting disease
30
Q
  • When do symptoms usually begin in Huntingtons?
  • Life span?
  • Diagnosis?
  • Cure?
A
  • 30-50 YO
  • 15 year =average lifespan after onset of sx
  • Dx = genetic testing; but ethical issues
  • No prevention or cure
31
Q

What makes Huntingtons disease harder?

A
  • Often, patient has psychiatric illness (parania/psychosis) before sx = have no when when sx hit.
  • Thus, to find out if you have it via genetic testing => require FAMILY counseling.
32
Q

What condiitons commonly exhibit Dystonia?

A
  1. Idiopathic torsion dystonia
  2. Focal torsion dystonia
33
Q

What is the difference between

Idiopathic Torsion Dystonia (ITD) and Focal Torsion Dystonia (FTD)?

A
  • ITD = many dystonic movements and postures + no other signs
  • FTD = dystonia in ONE/focal area
34
Q

How is Idiopathic Torsion Dystonia inherited and when is the onset?

A
  1. AD, AR, X-linked recessive
  2. Childhood or later, but NEVER GOES AWAY.
35
Q

What are types of dystonic movements?

A
  1. Torticollis (cervical dystonia) = neck twisted to one side
  2. Blepharospasm = spontaneously and involuntarily force close eyelids
  3. Oromandibular dystonia = muscles around mouth spasm.
36
Q

What positions MC occur in a dystonic arm and leg?

A
  • Arm = hyperpronated, flexed wrist, extended fingers
  • Leg = extended, pronated and foot is inverted
37
Q

Treatment for Idiopathic Torsion Dystonia;

Which should be used 1st bc its and easy fix and little SE?

A
  1. Low doses levodopa* = use 1st
  2. Anticholinergics
  3. Benzodiazepines
  4. Neuroleptic Drugs
  5. Baclofen
  6. Carbamazepine
38
Q

In Focal Torsion Dystonia; patient will have blepharospasm OR oromandibular dystonia OR spasmodic Torticollis. What other symptom can they have?

A

Writers cramp: dystonic posture of hand and forearm that happens AS SOON at patients get it in to position

39
Q

What is the treatment of choice for someone with Focal Torsion Dystonia?

A

BOTOX = weaken muscles

40
Q

Which disease have both bradykinetic and hyperkinetic movements?

A

Wilson’s Disease

41
Q

Wilson’s disease is a disorder of ________ metabolism that produces ______ and _______ dysfunction

A

Wilson disease is a disorder of copper metabolism that produces neurologic and HEPATIC dysfunction.

  • ⬇︎binding of copper to ceruloplasm => ⬆︎free copper => deposits in tissue.
42
Q

Who is most often affected by Wilson’s Disease?

A

Children and young adults

43
Q

Clinical features of Wilsons

A
  1. Hyperkinetic/bradykinetic movments
  2. Personality and behavior changes
  3. Dementia
  4. Psychosis and hallucinations
44
Q

Diagnosis of Wilsons Disease

A
  1. Based on Hx & Exam
    • ⬆︎ amounts of copper excretion in 24 hr. urine collection
    • ⬇︎ serum ceruloplasmin level
    • Kayser-Fleischer Ring on eye exam
45
Q

Treatment of Wilsons

A
  • Very fatal if you dont catch it. But if you can => curable.
    1. Penicillamine (copper chelating agent)
    2. ⬇︎ copper in diet
46
Q
  1. Single motor tics => ______
  2. Gilles De La Tourette’s syndrome?
A
  1. Benign
  2. Chronic multiple motor and vocal tics with onset before 21YO.
47
Q

– Gilles De La Tourette’s syndrome is MC in __ and you can also see what symptoms?

A
  • Males
  • Other symptoms:
  1. Coprolalia (Vulgar or obscene speech)
  2. Echolalia (Parroting speech of others)
  3. Echoproxia (Imitation of others’ movements)
  4. Palillalia (Repetition of words or phrases)
48
Q

Treatment of Gilles De La Tourette’s Syndrome

A
  1. Clonidine
  2. Haloperidol
  3. Phenothiazines
49
Q

What is the clinical presentation of Essential Tremor (aka Benign Familial Tremor)?

How does it progress?

A
  • Postural or kinetic tremor (occurs when moving), NOT at rest of bilateral hands and may involve head or voice
  • Can begin in early adulthood, and usually progresses slowly (years to decades)
50
Q

Does essential tremor (benign familial tremor) cause significant disability?

A

No, but does cause embarrasment

51
Q

____ often temporarily ⬇︎ tremor in essential tremor (benign familial tremor)

A

Alcohol (ETOH)

52
Q

Treatment for Benign Familial Tremor (Essential Tremor)

A
  1. Deep brain stimulation (DBS)
  2. Beta Blockers (e.g. propranolol)
  3. Primidone
  4. Benzos
  5. Topiramate