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Neurology 2 > Clin Med - Movement Disorders > Flashcards

Clin Med - Movement Disorders Flashcards

1
Q

Tourette’s characteristics

  • definition
  • exacerbation
  • attenuation
  • sleep
A
  • abrupt, brief, repetitive movement of sound (tics) preceded by a premonitory sensation or “urge
  • Exacerbation: stress, excitement, suggestion and suppression
  • Attenuation: physical activity and distraction
  • Sleep: Tics may persist
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2
Q

Tourette’s incidence

A
  • MC movement disorder in children (onset median age 6)
  • male > female
  • 50% are tic free by age 18
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3
Q

Tourette’s DSM-5 criteria

A
  • Multiple motor AND vocal tics
  • Greater than 1 year in duration
  • Started prior to age 18
  • Not a medication side effect
  • Rule out other medical conditions (seizure disorder, Huntington’s Disease, psychogenic – behavior problems)
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4
Q

Tourette’s motor tics

-focal

A
  • Eye blinking: most common
  • Facial grimacing
  • Jaw movement
  • Head bobbing
  • Shoulder shrugging
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5
Q

Tourette’s motor tics

-complex

A
  • Hopping
  • Jumping
  • Clapping or snapping fingers
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6
Q

Tourette’s vocal tics

A
  • Sniffing – MC
  • Throat clearing
  • Grunting
  • Snorting
  • Echolalia: repeating others (MC of the true vocal tics)
  • Coprolalia: inappropriate language (rare)
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7
Q

Tourette’s co-morbidities

A
  • 85% will have some comorbidity psych disorder.
  • 57% have more than 2 psychiatric disorders

-Most common: ADHD (50%) and OCD (25-50%)

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8
Q

Tourette’s and bi-polar disorder

A

MC in patients having tics lasting past age 18.

-more common in patients treated with antidepressants

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9
Q

Tourette’s neuroleptics

-typical antipsychotics (1st gen)

A
  • Haloperidol (Haldol)
  • pimozide (Orap)
  • fluphenazine (Prolixin)
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10
Q

Tourette’s neuroleptics

-atypical antipsychotics

A
  • Risperidine (Risperdol)
  • Aripiprazole (Abilify)
  • Olanzapine (Zyprexa)
  • Quetiapine (Seroquel)
  • Ziprasidone (Geodon)
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11
Q

Other meds for Tourette’s

A
  • Alpha Agonist [Clonidine (Catapres), Guanfacine (Tenex)]
  • Antiepileptics [Toparimate (Topamax), Levetracetam (Keppra)]
  • Antidepressents: SSRI, SNRI
  • Stimulants: Ritalin, Adderall etc.
  • THC: Controversial
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12
Q

Botox for Tourette’s

A
  • effective for focal and phonic tics
  • 93% improved
  • 50% tic free
  • side effects: ptosis, neck weakness, hypophonia (80%)
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13
Q

Comprehensive Behavioral Intervention for Tic

A
  • habit reversal training
  • self awareness
  • competing response
  • most effective in older adolescents without ADHD
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14
Q

Deep brain stimulation for Tourette’s

A
  • Not FDA approved

- Current lesion targets: thalamus and internal capsule of the globus pallidus

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15
Q

Essential tremor

-definition

A

-involuntary, rhythmic, oscillating movement of the head, upper extremity, or voice

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16
Q

Essential tremor

-etiology

A
  • Genetic relation in an Autosomal Dominant pattern (doesn’t have to follow this pattern but it’s MC)
  • no gene has been identified
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17
Q

HPI for essential tremor

A
  • Is there a family history (50% with +FH)
  • Age of onset (Median age: 45)
  • Location (hands, head, and voice)
  • Current medications
  • Progression (unilateral and slow)
  • Exacerbation: stress, fatigue, caffeine, postural, kinetic
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18
Q

Essential tremor

-characteristics

A
  • MC tremor
  • worsens with stress, fatigue, anxiety, movement
  • interfere with writing, drinking, eating, working, social
  • improved with modest ETOH consumption
  • absent with sleep
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19
Q

Essential tremor exam

A
  • Tremor noted with action
  • Arms in extension and in flexion with fingers in front of the face without touching
  • Vocalization of “e” and “ah”
  • Handwriting skills
  • Fine motor tasks
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20
Q

Occupational therapy for mild essential tremor

A
  • vocational changes
  • biofeedback and relaxation techniques
  • avoidance of stimulants (like caffeine)
  • mild ETOH consumption during stress situations (lasts up to 3 hrs)
  • weight objects (wrist weights, writing, and utensils)
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21
Q

Essential tremor pharm - primidone

A

*first line: primidone (Mysoline)

  • side effects: sedation and flu-like
  • tolerance can occur
  • caution: debilitated, OSA not on CPAP, impaired hepatic and renal function
  • contraindications: porphyria, severe respiratory depression, pregnant or breast feeding
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22
Q

Essential tremor pharm - propanolol

A

*first line: propanolol (inderal) PRN or chronic

PRN: 20-80mg 30 minutes-1 hr prior to an event

  • side effects: fatigue, bradycardia, decreased exercise tolerance, bronchospasm, constipation
  • contraindications: heart failure, conduction disorders, asthma, diabetes
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23
Q

Essential tremor pharm - second line or combo

-benzo

A

Benzodiazapines

  • Alprazolam (Xanax)
  • Clonazepam (Klonopin)
  • Lorazepam (Ativan): works best with anxiety induced tremor or in combination
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24
Q

Essential tremor pharm - second line or combo

-anticonvulsants

A
  • Topiramate (Topamax): requires high doses and not tolerated well
  • Gabapentin (Neurontin): minimal efficacy
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25
Q

Essential tremor pharm

-botox

A

Botox works well for head and vocal tremors, doesn’t work for upper extremity tremors.

26
Q

Essential tremor

-surgery

A

Stereotactic/Gamma Knife Thalamotomy (unilateral)

  • ventralis intermedius (VIM) nucleus of the thalamus
  • functional improvement 78-100%
  • recurrence 20-30%

Deep brain stimulation

  • VIM of the thalamus
  • reduce risk of dysarthria d/t ability to control
27
Q

Huntington’s Disease

-genetics

A
  • Autosomal Dominant
  • Chromosome 4p16.3
  • Gene codes for the protein Huntingtin
  • Mutation results in the addition of CAG (glutamine) repeats
  • Normal <27 repeats
  • > 40 repeats = Huntington’s Disease
28
Q

Huntington’s disease

-CAG repeats

A
  • normal: <27
  • 50% chance of disease if 37 repeats
  • 100% chance of disease if >40 repeats
  • childhood onset if >60 repeats (Westphal variant – death early)
29
Q

Huntington’s disease

-pathophysiology

A

mHTT protein is responsible for cell loss and gliosis of the striatum (basal ganglia) and thalamus resulting in severe atrophy and loss of the inhibitory pathway (dopamine).

30
Q

Huntington’s disease

-incidence

A
  • 7-10 per 100,000 in the USA
  • age of onset: 4th decade (usually between 35-39)
  • death: usually 15-20 years from onset

Westphal variant (> 60 repeats of CAG):

  • onset < 20 years old
  • more severe with rapid progression
  • death: 10-15 years from onset (by age 30-35)
31
Q

Huntington’s disease

-triad of symptoms

A
  1. behavioral
    - Depression (40%), Anxiety/OCD, Irritability, Impulsivity
    - Increased rates of divorce, job loss, imprisonment, and suicide
  2. cognitive
    - executive function (attention, concentration, planning)
    - memory deficit (progresses to dementia)
  3. motor
    - clumsiness, dropping items, wide base gait, slurred speech
    - CHOREA: Involuntary, irregular, spasmodic movement, “dance like”
    - progresses to rigidity, dyskinesia and immobility
32
Q

Huntington’s disease

-physical exam

A
  • Generalized flexion/extension movements
  • Unable to perform rapid alternating movements
  • Difficulty staying on task
  • Limited eye movement on exam
  • Unable to withstand a position over time
  • Dysphagia
  • Choke on food and water
  • Awkward, clumsy, drop objects
33
Q

Huntington’s disease

-referrals

A
  • genetic counseling
  • neurology
  • psychology/psychiatry
  • social services
34
Q

Huntington’s disease

-treatments

A
  • no cure
  • only symptomatic/complex and require continuous monitoring and adjusting
  • behavioral treatment (try to minimize meds)
35
Q

Huntington’s disease

-cognitive treatment

A

Cholinesterase inhibitors and NMDA receptor antagonist have not provided any known improvement in cognitive deficit

36
Q

Huntington’s disease

-motor treatment

A
  • Tetrabenzine (Xenazine): FDA approved for HD [Black Box Warning: increased risk of depression and suicide]
  • Haloperidol (Haldol)
  • Risperidone (Risperdal)
  • Quetiapine (seroquel)
37
Q

Define Parkinson’s disease

A

Progressive neurodegenerative condition resulting from the death of the dopamine-containing cells of the substantia nigra

38
Q

What other conditions are often mistaken for/are similar to Parkinson’s?

A
  • Shy-drager syndrome
  • Progressive supranuclear palsy
  • Lewy body dementia
  • Normal pressure hydrocephalus
  • Essential tremor
  • Huntington’s disease
  • Wilson’s disease
  • Enecephalopathy
  • Drug-induced (neuroleptics)
39
Q

Which neuroleptics can cause Parkinson-isms?

A
  • antipsychotics
  • phenergan
  • metaclopramide
40
Q

Parkinson’s Incidence

A
  • Progressive neurodegenerative disorder
  • 160 per 100,00 prevalence
  • Mean age of onset over 60 years old
  • M>F
41
Q

Parkinson’s diagnosis criteria

A

Bradykinesia with at least one of the following:

  • rigidity
  • resting tremor
  • postural instability

With at least 3 supportive criteria:

  • Unilateral onset
  • Progressive signs and symptoms
  • Persistent asymmetry of affected side
  • Response to levodopa
  • Levodopa dyskinesia
  • Clinical course for more than 10 years
42
Q

Primary vs. Secondary Classification

A

Primary = Parkinson’s disease

Secondary:

  • Drug induced: anti-psychotics, anti-emetics, dopamine depleting (reserpine, tetrabenzaprine), meperidine
  • Toxins- MPTP
  • Multi-infarct into substantia nigra or deep brain tissues
  • Dementia-puglistica: post traumatic
43
Q

Parkinson’s early symptoms

A
  • Fatigue
  • Sleep disorder: insomnia
  • Dystonia: foot cramps mostly at night
  • Restless Leg Syndrome
  • Depression/Anxiety
  • Minor cognitive deficit: mostly slowness
  • Loss of smell
44
Q

Parkinson’s

-cardinal features

A
  • Slow ocular movements
  • Positive Doll’s eye
  • Myerson’s sign: glabellar tap = blinking
  • Masked facies
  • Finger tapping
  • Pronation/supination
  • Finger to nose
  • Sit to stand
  • Gait-limited arm swing*
  • Block turning*
  • Micrographia*
  • Hypophonia*

*occur in later stages

45
Q

Parkinson’s resting tremor

A
  • “Pin rolling”
  • Attenuates with movement
  • Low frequency
  • Absent in 25% of patients
46
Q

Parkinson’s rigidity on exam

A
  • axial (head and neck ROM decreased
  • distal: upper extremity (elbow ROM decreased in flexion/extension and wrist “cogwheel” rigidity)

*exacerbated by distraction

47
Q

Parkinson’s postural instability on exam

A
  • pull test positive: retropulsion
  • forward flexion on gait*
  • shuffling gait, fenestrated gait*

*later in disease progression

48
Q

What is the pull test (retropulsion) for Parkinson’s?

A
  • it is a cerebellar test
  • you pull someone back towards you and as you pull them back their instinct should be to correct that and come forward
  • but these patients don’t have that stability, so they’ll come back with you
49
Q

Parkinson’s exam

-cognition

A

in later stages, patients develop dementia.

*important to do mini mental exam at every important so you can track progression

50
Q

Parkinson’s imaging

A
  • typically not needed
  • if under 55 or other neuro finding, you can get CT or MRI

-DaT scans: SPECT scan looking at dopamine in the brain

51
Q

Treatment for Parkinson’s

A
  • physical therapy
  • speech therapy
  • medications
  • surgery
52
Q

Anticholinergics - early stages < 60

A
  • Trihexyphenol (Artane): therapeutic 6-10mg/day
  • Benzotropine (Cogentin): max 6mg/day

Side effects: memory loss, psychosis, orthostasis, constipation, dry mouth

53
Q

Anticholinergics - early stages > 60

A

Amantadine (Symmetrel): max 400mg/day

Less side effects

54
Q

Parkinson’s tx

-dopamine

A

Carbidopa/Levodopa (Sinemet)

  • Levodopa able to cross the BB barrier*
  • Causes nausea
  • Addition of carbidopa improves nausea
  • Side effects: dyskinesia
55
Q

When should you start dopamine meds for Parkinson’s? Why?

A

Don’t start initially until symptoms have become refractory and debilitating or significant instability issues.

Once you start, you continuously titrate up until you reach max dose.

56
Q

Parkinson’s tx

-dopamine agonist

A
  • Pramipexole (Mirapex)
  • Ropinirole (Requip)
  • Bromocriptine (Parlodel)

Adjuvant to Sinemet

57
Q

Parkinson’s tx

-COMT inhibitors

A
  • Entacapone (Comtan)

- Tolcapone (Tasmar)

58
Q

Parkinson’s tx

-MAO-B inhibitors

A
  • Rasagiline (Azilect)

- Selegiline (Zelapar)

59
Q

Parkinson’s tx for psychosis

A

Quetiapine (Seroquel)

60
Q

Surgery for Parkinson’s

A

Thalamotomy/Pallidotomy: unilateral

  • impaired language learning: dominant
  • impaired visuospatial ability: non dominant

Deep Brain Stimulator: bilateral
-STN vs globus pallidus

61
Q

What do you call a pig that does karate?

A

Pork chop

:)

Neurology 2 (21 decks)

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