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)

22
Q

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

23
Q

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

24
Q

What conditions commonly exhibit Chorea?

A
  1. Huntingtons Disease
  2. Syndenham’s Chorea
25
What is **Syndenhams Chorea?**
* 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
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. ## Footnote **Diagnosis?**
**Syndenhams Chorea** = due to GAHS infection
27
Treatment for **Syndenhams Chorea?**
1. **Bedrest** 2. **ABX**
28
What is **Huntingtons Disease?**
* **AD disorder** of inherited CAG repeat that causes _gradual onset_ and _progression_ of _chorea_ + _dementia_
29
Inheritance of **Huntingtons**
* **AD**; offspring of affected person have **50%** of getting disease
30
* When do symptoms usually begin in **Huntingtons**? * Life span? * Diagnosis? * Cure?
* **30-50 YO** * **15 year** =average lifespan after onset of sx * Dx = **genetic testing;** but ethical issues * **No** prevention or cure
31
What makes **Huntingtons disease** harder?
* 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
What condiitons commonly exhibit **Dystonia**?
1. **Idiopathic torsion dystonia** 2. **Focal torsion dystonia**
33
What is the difference between **Idiopathic Torsion Dystonia (ITD)** and **Focal Torsion Dystonia (FTD)?**
* **_ITD_** = **many** dystonic movements and postures + no other signs * **_FTD_** = dystonia in **ONE/focal area**
34
How is **Idiopathic Torsion Dystonia** _inherited_ and when is the _onset_?
1. **AD, AR, X-linked recessive** 2. **Childhood** or later, but **NEVER GOES AWAY.**
35
What are types of **dystonic movements?**
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
What positions MC occur in a **_dystonic_** arm and leg?
* **Arm** = hyperpronated, flexed wrist, extended fingers * **Leg** = extended, pronated and foot is inverted
37
**_Treatment for Idiopathic Torsion Dystonia;_** Which should be _used 1st_ bc its and easy fix and little SE?
1. **Low doses levodopa\* = use 1st** 2. Anticholinergics 3. Benzodiazepines 4. Neuroleptic Drugs 5. Baclofen 6. Carbamazepine
38
In **_Focal Torsion Dystonia_**; patient will have blepharospasm **OR** oromandibular dystonia **OR** spasmodic Torticollis. What other symptom can they have?
**Writers cramp:** dystonic posture of hand and forearm that happens AS SOON at patients get it in to position
39
What is the _treatment of choice_ for someone with **Focal Torsion Dystonia?**
**BOTOX** = weaken muscles
40
Which disease have both **bradykinetic** and **hyperkinetic** movements?
**Wilson's Disease**
41
**Wilson's disease** is a disorder of ________ metabolism that produces ______ and _______ dysfunction
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
**Who** is most often affected by **Wilson's Disease?**
**Children** and **young adults**
43
Clinical features of **Wilsons**
1. Hyperkinetic/bradykinetic movments 2. Personality and behavior changes 3. Dementia 4. Psychosis and hallucinations
44
Diagnosis of **Wilsons Disease**
1. **Based on Hx & Exam** * ⬆︎ amounts of copper excretion in 24 hr. urine collection * ⬇︎ serum ceruloplasmin level * Kayser-Fleischer Ring on eye exam
45
**Treatment** of Wilsons
* Very fatal if you dont catch it. But if you can =\> curable. 1. Penicillamine (copper chelating agent) 2. ⬇︎ copper in diet
46
1. Single motor tics =\> \_\_\_\_\_\_ 2. Gilles De La Tourette’s syndrome?
1. Benign 2. Chronic multiple **motor** and **vocal** tics with onset **before 21YO.**
47
**– Gilles De La Tourette’s syndrome** is MC in __ and you can also see what symptoms?
* 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
Treatment of Gilles De La Tourette’s Syndrome
1. **Clonidine** 2. **Haloperidol** 3. **Phenothiazines**
49
What is the clinical presentation of **Essential Tremor** (aka Benign Familial Tremor)? How does it progress?
- 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
Does **essential tremor** (benign familial tremor) cause significant disability?
**No**, but does cause **embarrasment**
51
\_\_\_\_ often temporarily ⬇︎ tremor in **essential tremor (benign familial tremor)**
**Alcohol (ETOH)**
52
**Treatment** for Benign Familial Tremor (Essential Tremor)
1. **Deep brain stimulation (DBS)** 2. Beta Blockers (e.g. propranolol) 3. Primidone 4. Benzos 5. Topiramate