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Head and Neck Clinical Correlations > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

What are abnormal movements?

A

Involuntary contractions of voluntary muscles

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2
Q

Name the systems in the basal ganglia?

A

• Nigro-striatal dopaminergic system
• Intra-striatal cholinergic system
• GABAergic system from the striatum to the GP and SN

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3
Q

Name hyperkinetic abnormal movement disorders?

A

• Hyperkinetic
• Tremor
• Chorea
• Dystonia
• Myoclonus
• Hemiballism
• Tics
• Dyskinesias
• Akathisia
• Restless Leg Syndrome

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4
Q

Name hypokinetic movement disorders?

A

Parkinsonism

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5
Q

Describe the epidemiology of Parkinsonism?

A

• Occurs in all ethnic groups
• Prevalence is 1-2/1000
• Males = Females

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6
Q

Describe the Etiology of Parkinsonism?

A
  1. Idiopathic Parkinson Disease (most common)
  2. Encephalitis Lethargica
  3. Familial
  4. Drug or Toxin-Induced
    - Manganese and carbon disulfide.
    - MPTP
    > Meperidine analogue
    - Therapeutic drugs (Dopamine depleting agents and dopamine antagonists)
  5. Parkinson-plus Syndromes
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7
Q

Describe the pathology of Parkinsonism?

A

• Loss of pigmentation in substantia nigra
• Cell loss in globus pallidus and putamen
• Lewy bodies (alpha-synuclein) in basal ganglia

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8
Q

Describe the pathogenesis of Parkinsonism?

A

• Depletion of dopamine
•Disorder of balance of excitation and inhibition within the basal ganglia connections

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9
Q

What are the major clinical features of Parkinsonism?

A
  1. Resting tremor
  2. Rigidity
  3. Bradykinesia
  4. Gait and Postural instability
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10
Q

State additional features of Parkinsonism?

A

• Sialorrhea
• Dysphagia
• Myerson sign
• Depression
• Visual hallucinations
• Autonomic dysfunction
• Dementia
• REM sleep behavior disorder

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11
Q

What are the differential diagnoses of Parkinsonism?

A

• Depression
• Essential Tremor
• Wilson’s disease
• Huntington’s disease
• Creutzfeld-Jakob disease
• Normal Pressure Hydrocephalus
• Parkinson Plus Syndromes
> Diffuse Lewy Body disease
> Multiple System Atrophy
> Progressive Supranuclear Palsy
> Corticobasal Degeneration

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12
Q

Describe the use of anticholinergic drugs in the management of Parkinsonism?

A

• Most helpful in treating tremor and rigidity,
> Trihexyphenidyl (Artane)
> Benztropine (Cogentin)

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13
Q

Describe the use of amantadine in the management of Parkinsonism?

A

Used in mild disease
Reduces dyskinesias in advanced disease

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14
Q

Describe the use of Carbidopa/Levodopa in the management of Parkinsonism?

A

• Levodopa converted to dopamine via dopa decarboxylase
• Carbidopa inhibits dopa decarboxylase, prevents conversion of levodopa to dopamine in the periphery
• Dopamine unable to cross BBB, but levodopa does cross BBB

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15
Q

What are the side effects and complications of levodopa?

A

Side-effects of levodopa
• Nausea/vomiting, Gl upset
• Orthostatic Hypotension
• Dyskinesias
• Confusion/hallucinations

Other complications of levodopa
• Wearing-off
• On-off phenomenon

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16
Q

Describe the use of dopamine agonists in the management of Parkinsonism?

A

• Bromocriptine (Parlodel)
• Pramipexole (Mirapex)
• Ropinirole (Requip)
• Similar side-effects as levodopa, increased hallucinations, sleepiness
• Less likely to cause dyskinesias or on-off phenomenon

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17
Q

Describe the use of catechol-o-methyltransferase inhibitors?

A

• Talcapone and Entacapone
• Reduce dose requirements and decrease fluctuations
• More sustained plasma levels of levodopa

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18
Q

Describe the use of monoamine oxidase type B inhibitors in treatment of Parkinsonism?

A

• Selegiline and Rasagiline
• Inhibits metabolic breakdown of dopamine
• ?Neuroprotective

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19
Q

Describe other treatment considerations for Parkinsonism?

A
  1. Surgery
    - Thalamotomy - tremor
    - Pallidotomy - Bradykinesia
  2. Deep Brain Stimulation
    - Globus pallidus and subthalamic nucleus
  3. Cellular Therapies
  4. Protective Therapies (?)
    - MAO B inhibitors
    - Coenzyme Q10
  5. Physical Therapy/Aids for Daily Living
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20
Q

Describe symptoms of progressive supranuclear palsy?

A

Gait instability (early falls)
Supranuclear opthalmoplegia

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21
Q

Describe multiple system atrophy?

A

Autonomic dysfunction (orthostatic hypotension)
Cerebellar dysfunction

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22
Q

Describe symptoms of corticobasal degeneration?

A

Apraxia/clumsiness
Neglect
Alien hand syndrome
Myoclonus

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23
Q

Describe dementia and Lewy bodies?

A

Fluctuating dementia and psychosis
Dementia appears before or within one year of Parkinsonism

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24
Q

What is a tremor?

A

Involuntary relatively rhythmic purposeless oscillatory movements with alternating contraction of opposing groups of muscles

25
Q

How can tremors be classified by?

A

• Rate (Slow = 3-5 Hz OR Fast = 10-20 Hz)
• Location (Arms, feet, tongue, jaw, head, voice)
• Amplitude (Coarse, medium, fine)
• Rhythmicity (Rhythmic or non-rhythmic)
• Relationship to rest and movement (Rest, action, postural, intention)
• Etiology
• Underlying pathologic change

26
Q

Name the types of tremors?

A

• Physiologic
• Rest
• Postural
• Intention
• Rubral
• Psychogenic

27
Q

What causes drug induced tremors?

A

• Theophylline
• Caffeine
• Lithium
• Valproic Acid
• Tricyclic Antidepressants
• Isoproterenol
• Thyroid hormone

28
Q

Describe an essential tremor?

A

Typically a postural tremor, may worsen with goal-directed activity
• Familial in ~50%
Hands, head, voice, trunk
• Alcohol improves

29
Q

Describe the treatment for an essential tremor?

A

• Propranolol
• Primidone
• Gabapentin
• Topiramate
• Deep Brain Stimulation

30
Q

Describe an intention tremor?

A

• Localizes to Cerebellum
• Occurs with deliberate movement
• Tremor worsens as the target is approached
• Can occur in
extremities, head or entire body

31
Q

Describe chorea?

A

• Involuntary, irregular, purposeless, asymmetric, non-rhythmic movements
• Abrupt, brief, rapid, jerky, non-sustained, and explosive in character
• Worse with activity
• Usually involves distal upper extremities
• Associated with motor impersistence - “milkmaid grip” due to contractions alternating with relaxation of grip
• Associated with cell loss in striatum (caudate and putamen)

32
Q

Describe the etiology of chorea?

A

• Etiology
• Hereditary
• Huntington
• Wilson
• Sydenham Chorea
• Chorea Gravidarum
• Drug Toxicity
• Levodopa (dopaminergic drugs)
• Antipsychotics
• Lithium
• Phenytoin
• Oral contraceptives

33
Q

What medical disorders cause chorea?

A

• Etiology
• Cerebrovascular Disorders
• Medical Disorders
• Thyroid
• SLE, lupus anticoagulant
• Encephalitis
• AIDS
• Hypocalcemia, hypomagnesemia, hypernatremia
Hyper-/Hypoglycemia
• Hepatic Cirrhosis

34
Q

Describe Huntington disease?

A

• Autosomal dominant with complete penetrance
• Mutation of huntingtin gene on chromosome 4
• Expansion of CAG trinucleotide repeat
• Cell loss in cortex and striatum
• MRI shows atrophy of caudate
• Genetic testing available - Genetic counseling.

35
Q

Describe the clinical presentation of Huntington disease?

A

• Clinical
• Dementia
• Chorea,difficulty initiating saccadic eye movements
• Psychiatric
• Depression
• OCD

36
Q

What is the prognosis of Huntington disease?

A

Progressive to death within 10 to 20 years

37
Q

Describe hemiballism?

A

• Unilateral choreiform movements, more violent and rapid compared to typical
chorea
• Involves proximal muscles
• Lesion of contralateral subthalamic nucleus (usually vascular)

38
Q

Describe dystonia?

A

• Slow, bizarre, may be grotesque in type, and writhing, twisting, and turning character
• Characterized by repetitive muscle contractions that are sustained at peak
• Muscles often in constant state of hypertonicity, may be associated with severe pain
• Eventually postures become fixed by contractures and deformities develop

39
Q

Describe how dystopia is classified?

A

• According to the site of involvement, dystonia is classified as:
1)Focal - One body part involved
2)Segmental - Two or more contiguous parts involved
3) Multifocal - Two or more noncontiguous parts involved
4)Hemidystonia - One side of the body affected
5)Generalized

40
Q

Describe causes of dystonia?

A

• Many recognized hereditary dystonias
• Other causes of dystonias include:
• Parkinson’s/Parkinson-plus syndromes
• Huntington Disease
• Wilson disease
• Drugs
• Dopamine receptor antagonists
• Chronic levodopa therapy,
• AEDs
• SSRIs
• Stroke

41
Q

What is the treatment of dystonia?

A

• Treatment
• Medications frequently not helpful
• Anticholinergics
• Benzodiazepines
• AEDs
• Levodopa
• Baclofen
• Botox for focal dystonia/writer’s
cramp

42
Q

Describe myoclonus?

A

• Abrupt, brief, rapid, jerky, arrhythmic, involuntary contractions involving portions of muscles, entire muscles, or groups of
muscles
• Seen primarily in muscles of the extremities and trunk, but involvement may be diffuse and widespread
• Occur at a rate of ~ 10-15/minute, usually at irregular intervals
• May be decreased by voluntary movement and increased during stimulation
• May be focal, segmental, multifocal, or generalized
• Asterixis - Negative myoclonus

43
Q

Describe the etiology of myoclonus?

A

• Physiologic
• Essential Myoclonus
• Epileptic
• IME
• Progressive myoclonic epilepsy syndromes
• Drug-Induced
• symptomatic

44
Q

What are the drug induced causes of myoclonus?

A

• SSRIs
• TCAs
• Lithium
• Levodopa
• Opiates

45
Q

What are the symptomatic causes of myoclonus?

A

• Etiology
• Symptomatic
• CID
• SSPE
• Metabolic disorders
• Vascular lesions
• Neoplasms
• Spinal cord lesions
• Wilson disease/ degenerative disorders

46
Q

What is the treatment of myoclonus?

A

• Difficult to treat
• Valproic acid
• Benzodiazepines

47
Q

What is TICs?

A

• Brief, involuntary, rapid, recurrent, non-rhythmic movements (motor tics) or sounds (vocal tics)
• Worsened by stress, anxiety, and fatigue
• Relieved by concentrating on a task or absorbing activities such as reading
• Frequently involves face, neck and shoulders
• Irresistible urge to move before the tic, relieved with execution of the tic
• Can be voluntarily suppressed for short period, but leads to build-up of tension and rebound exacerbation

48
Q

Describe Tourette syndrome?

A

• Multiple (at least two) motor and vocal tics
• 3x more common in males
• Associated with OCD,ADHD, depression

49
Q

Describe the treatment for Tourette syndrome?

A

• Treatment
• Clonidine
• Low dose, high potency antipsychotics
• Haloperidol (Haldol)
• Dopamine agonists
• ?Botox

50
Q

What is a acute dystonia?

A

• Acute athetosis or dystonia of midline structures (torticollis, oculogyrus, opisthotonus)
• Usually occurs within hours to one week of starting a dopamine receptor antagonist treatment

51
Q

What is the treatment of acute dystonia?

A

• IM/IV Diphenhydramine (Benadryl) or Benztropine (Cogentin)

52
Q

What is tardive dyskinesia?

A

• Bizarre movements commonly of mouth, face, jaw, and tongue that consists of grimacing, pursing of the mouth and lips, and writhing movements of the tongue
• Often develop after prolonged use (months to years) of dopamine blocking agents, but may occur shortly after they are started
• May also be associated with levodopa and dopamine agonists in a dose-related fashion

53
Q

What is the treatment for tardive dyskinesia?

A

• Treatment
• Discontinue use of medication
• Switch to atypical antipsychotic
• Riserpine and tetrabenazine
• Lithium
• Baclofen
• Benzodiazepines

54
Q

Describe neuroleptic malignant syndrome?

A

• Caused by decreased dopaminergic transmission
• Associated with treatment with neuroleptics, abrupt withdrawal of dopamine
• Clinical
• Altered mental status, autonomic dysfunction, rigidity, fever
• CK markedly elevated (> 1000)
• Treatment
• Withdrawal of medication
• Reduction of body temperature
• Dantrolene and/or bromocriptine
• ECT for refractory cases

55
Q

Describe Wilson disease?

A

• Autosomal recessive
• Impairment of ceruloplasmin synthesis with excessive copper deposition
• Clinical features
• Acute/chronic hepatitis
• Movement disorders
• Chorea, tremor (wing-beating), rigidity, bradykinesia, ataxia, dystonia
• Kayser-Fleischer rings
• Psychiatric
• Psychosis, dementia, depression

56
Q

What are the lab results in Wilson disease?

A

• Labs
• Abnormal liver function
• Low serum copper and ceruloplasmin
• Increased urinary copper excretion
• High copper in liver biopsy

57
Q

Describe the treatment of Wilson disease?

A

• Reduction of dietary copper
• Penicillamine (copper-chelating agent)
• Pyridoxine to prevent anemia
• Liver transplantation

58
Q

What is restless leg syndrome?

A

• Unpleasant, creeping discomfort
• Usually legs, can involve trunk and arms
• Worse at rest (lying down or sitting), improves with activity
• Worse in evening or at night
• Associated with:
• Pregnancy
• Iron Deficiency Anemia
• Renal failure

59
Q

What is the treatment of restless leg syndrome?

A

• Dopamine agonists
• Carbidopa/levodopa
• Benzodiazepines
• Opiates
• Gabapentin
• Carbamazepine

Head and Neck Clinical Correlations (7 decks)

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