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Neuro II > Involuntary Movement Disorders > Flashcards

Involuntary Movement Disorders Flashcards

1
Q

What is the term for movement disorder that is velocity dependent? Velocity independent?

A

spasticity - velocity dependent increase in muscle tone
rigidity - velocity independent increase in muscle tone

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2
Q

What are the 7 hyperkinetic movements?

A
  • ballism / chorea / athetosis
  • dystonia myclonus
  • tics / stereotypies
  • restless legs
  • tremor
  • akathisia
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3
Q

What are the not patterned, flowing hyperkinetic movements?

A

ballism / chorea / athetosis

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4
Q

What are the patterned, non-flowing hyperkinetic movements?

A

dystonia

myoclonus

tics / stereotypies

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5
Q

What are the hypokinetic movement disorders?

A

bradykinesia

akinesia

rigidity

(Parkinson’s Disease)

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6
Q

What is the function of the thalamus in terms of movement?

A

enhance movement → send excitatory signals to the cortex to induce movement

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7
Q

What are the outflow nuclei of the basal ganglia? What is their function?

A
  • Globus Pallidus interna
  • Substantia Nigra

They want to shut the thalamus down

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8
Q

What is the striatum & what does it do?

A

caudate + putamen

regulates movement (move when you want to & don’t move when you don’t want to)

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9
Q

What is the function of the subthalamic nucleus?

A

stimulates the outflow nuclei (GPi & SNr) → & encourage their inhibition of the thalamus

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10
Q

What is the function of the Globus Pallidus externa?

A

inhibits the subthalamic nucleus (preventing int from stimulating the GPi & SNr)

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11
Q

Briefly describe the pathway of the direct & indirect pathways. Which increases movement & which inhibits movement?

A
  • Direct
    • Striatum inhibits GPi → disinhibits the inhibition of the thalamus → movement
  • Indirect
    • Striatum inhibits GPe → disinhibits the inhibition of STN → STM stimulates GPi → GPi inhibits thalamus → inhibits movement
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12
Q

How does the Substantia negra pars compacta impact the direct & indirect pathways?

A

via dopamine (facilitates movement)

stimulates direct pathway

inhibits indirect pathway

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13
Q

At what age does Parkinson Disease typically present?

It is caused by decreased production of what neurotransmitter?

A

5th - 6th decade

decreased dopamine from substantia nigra pars compacts

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14
Q

Parkinson Disease causes what motor symptoms?

Treatment?

A
  • T- tremor at rest, pill rolling, MCP tremor
  • R- rigidity, cogwheeling at elbows
  • A- akinesia/bradykinesia/hypokinesia
    • slow initiation of movements, masked facies, micrographia, hypophona, hypomimia, decreased rate/amplitude finger tapping
  • P- postural instability, stopped posture, slow shuffled gait, block-like turning

Treatment: respond to L-dopa

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15
Q

What are common non-motor symptoms seen in Parkinson disease?

A
  • affective disorders -
    • depression, anhedonia, anxiety, impulsiveness, hallucinations
  • cognitive dysfunction -
    • bradyphrenia, memory impairment, anosmia
  • visual disturbances -
    • impaired color discrimination, visuospatial abnormalities
  • autonomic dysfunction -
    • orthostatic hypotension
  • gastrointestinal dysfunction
    • constipation
  • sexual dysfunction
    • erectile dysfunction
  • sleep disturbance
    • REM sleep disturbance
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16
Q

What are the causes of Parkinson’s?

A
  • Idiopathic: sporadic or genetic
  • Secondary:
    • drugs
    • metabolic - hypothyroidism / low B12 / diabetes
    • Environmental - synthetic heroin, pestacide
    • Structural - stroke involving basal ganglia
    • Infection - influenza encephalitis,
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17
Q

What are the red flags seen in atypical Parkinsonism?

A
  • Atypical:
    • early dementia, early falls, prominent ocular dysmotility, prominent dysautonomia, prominent ataxia,
    • poor response to L-dopa
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18
Q

What are the drugs given to Parkinson patients to replace / boost dopamine?

A
  • Replace Dopamine
    • dopamine analogue: carbidopa / levodopa
    • dopamine agonist: pramipexole,
    • MAO-B inhibition (decrease dopamine degradation_
      • irreversible - selegiline, rasagiline
      • reversible - safinamide
    • COMTI (prevents L-dopa breakdown - enhances carbidopa / levodopa)
      • entacapone
    • Amantadine (increases dopamine release / blocks reuptake)
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19
Q

What are the stimulants used to treat Parkinson disease?

A

Istradefylline: adenosine receptor agonist

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20
Q

What are the targets for deep brain stimulation as a treatment for Parkinson Disease?

A

GPi >>> STN

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21
Q

In addition to drugs, what is a treatment option for Parkinson Patients?

A

deep brain stimulation surgery

physical therapy

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22
Q

What are the 5 types of action tremors?

A
  • postural
  • kinetic
  • task specific
  • isometric
  • psychogenic
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23
Q

What are the two types of postural action tremors? Provide examples of each.

A
  • postural
    • enhanced physiologic:
      • stress, fatigue, fever, hypoglycemia, drugs, EtOH withdrawal, hyperthyroidism,
    • Pathologic:
      • essential tremor, midbrain or cerebellar pathology, muscle/nerve disease
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24
Q

What are the common causes of kinetic action tremors?

A

cerebellar disorders, stroke, MS, midbrain lesion

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25
Q

What are the common causes of task specific action tremors?

A

handwriting tremor, orthostatic

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26
Q

What are the common causes of isometric action tremors?

A

muscle contraction during sustained exertion

27
Q

What are the common causes of psychogenic action tremors?

A

distractibility, entrainment, suggestibility

28
Q

What are the age qualification of an essential tremor?

Type of tremor? Indicative of what type of brain dysfunction?

Symmetry? Intention?

A

before 40 or after 60

kinetic > postural of arms (wrist flexion/extension)

cerebellar / thalamo-occipital dysfunction

~30% asymmetric; ~50% intention element

29
Q

Treatment for essential tremor?

A

better with alcohol

propranolol, primidone, gabapentin, topiramate, thalamic DBS

30
Q

Cerebellar tremor is what type of tremor?

Symptoms?

A

essential tremor (+)

overshoot on finger to nose maneuver

several planes involved in tremor

dysarthria / scanning speech

gait ataxia / hypotonia

31
Q

What are the common causes of cerebellar tremor?

A

cerebellar stroke, mass, B12 & vitamin E deficiency

32
Q

What type of tremor is a Rubral Temor?

Symptoms?

A

kinetic > postural > resting (multiphase)

unilateral: severe (arm may be functionally useless) - may be present in sleep

mild dystonia or ataxia

33
Q

What are the major causes of a rubral tremor?

Treatment?

A
  • Causes
    • stroke, tumor, MS
  • Treatment
    • levodopa
34
Q

What is the term for involuntary, irregular, purposeless, non-rhythmic, abrupt, random & quick movements that flow from one body region to another?

A

Chorea

35
Q

What is the term for large-amplitude chorea involving proximal arms, flinging & flailing movements (usually unilateral?

A

Ballism

36
Q

What is the term for slow chorea; writing movements- similar to dystonia but not sustained, patterned or painful

A

athetosis

37
Q

Ballism is seen in what situation?

A

post-stroke following hemiparesis

caused by lesion to contralateral subthalamic nucleus

38
Q

Athetosis is seen in what situation?

A

children with cerebral palsy secondary to kernicterus

39
Q

What is the most common cause of hereditary chorea?

A

huntington disease

40
Q

Huntington Disease exhibits what type of inheritance patten? Type of mutation?

A

autosomal dominant

chromosome 4 ; CAG repeat → anticipation

41
Q

What 3 symptom complexes are seen in Huntington Disease?

A
  • motor
    • chorea, dystonia, tics, parkinsonism
  • cognitive
    • dementia
  • neuropsychiatric
    • psychosis
42
Q

What brain center is classically atrophied in Huntington disease?

A

caudate atrophy

43
Q

What is the differential diagnosis for a patient with Chorea?

A
  • Huntington Disease
  • Metabolic
    • B12 def, hypothyroidism, Sedenham chorea (post rheumatic, HIV, post encephalitic, chorea gravidarum, Lupus
  • Vascular
  • autoimmune
  • demyelinating
44
Q

What is the treatment for Huntington Chorea?

A
  • Dopamine depletion
    • tetrabenazine / deutetrabenazine (vesicular monoamine transporter 2 inhibitors → dopamine monoamine depletion)
45
Q

What is the term for movements that are sustained, usually involving twisting & repetition with same muscle groups that is worsened by physical activity?

A

dystonia

46
Q

What are the types of dystonia & what muscle groups does it commonly involve?

A

focal, segmental, multifocal, hemidystonic or generalized

eyes, vocal cords, neck, limbs

47
Q

What is the term for cervical dystonia?

Treatment?

A

spasmodic torticollis

Treatment: clonazepam, trihexylphenidyl, baclofen, onabotulinumtoxin

48
Q

What are the most common genetic causes of isolated dystonias?

A
  • DYT-TOR1A
  • DYT-THAP1
49
Q

What diagnosis should you consider if you have a pediatric patient with dystonia? Treatmet?

A

Dopa-responsive dystonia : DTY5

diurnal variation (family history parkinsonism)

levodopa responsive

50
Q

What is the term for sudden, brief, shock-like involuntary movements that are usually irregular but can be rhythmical? This can involve what body locations?

A

myoclonus

palate, eyes, limbs

51
Q

What are the two types of myoclonus? Example?

A
  • positive
    • d/t additional muscle contraction
  • negative
    • d/t motor inhibition
    • asterixis: brief flapping of outstretched arms seen in hyperammonemia or hyperuremia
52
Q

Where is the electrical generator for myoclonus? Treatment?

A

cortical (epileptic)

subcortical

brainstem

spinal cord

53
Q

What is Lance-Adams syndrome?

A

post anoxic generalized myoclonus; action or intention type

54
Q

What are the treatments for myoclonus?

A

valproic acid, levetiracetam, clonazepam

55
Q

What is the term for coordinated movements that repeat continually & identically that are not preceded by an irresistible urge

A

sterotypy

(ie. rocking in autism)

56
Q

What are abnormal suppressible movements that accompany an urge or compulsion? They can be motor or phonic.

A

tics

57
Q

What is tourette syndrome? Treatment?

A

motor & phonic tics onset prior to age 18

treatment: guanfacine, fluphenazine, SSRI, tetrabenazine, deep brain stimulation

58
Q

What are the two medication-induced dyskinesias? Cause?

A
  • akathisia
    • feeling of inner, restlessness that is reduced by moving - may be stereotypic
    • focal (oral, genital) → withdrawal dopamine in parkinson patients
    • generalized
  • tardivine dyskinesia
    • rapid, repetitive, stereotypic movements involving oral, buccal & lingual areas
    • lip smacking, tongue protrusion

usually caused by antipsychotics that block dopamine

59
Q

What is the treatment for medication-induced dyskinesias?

A

benzodiazepines, benzotropine, amantadine

valbenazine & deuterabenzine (tardive dyskinesia)

60
Q

What is the inheritance pattern & mutation seen in Wilson Disease?

A

autosomal recessive

chromosome 13; ATP7B gene → impaired biliar copper excretion

inability to incorporate copper into apoceruloplasmin to form ceruloplasmin

copper accumulates in liver & other tissues

61
Q

Wilson Disease should be considered in a young patient with what symptoms?

A

tremors, dystonias, choreoathetosis, rigidityy associated with psychiatric disorder

62
Q

What lab tests should be performed in a patient you suspect Wilson Disease? Treatment?

A
  • Abnormal copper metabolism
    • decreased serum ceruloplasmin
    • increased serum copper
    • increased 24 hr urinary copper
  • gold standard → liver biopsy
  • Treatment
    • zinc, penicillamine
63
Q

The provided image is indicative of what disease? What is the name of this sign?

A

Wilson Disease

Kayser-Fleischer ring

Neuro II (30 decks)

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