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MBBS 2: 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
How can tremors be classified by?
• 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
Name the types of tremors?
• Physiologic • Rest • Postural • Intention • Rubral • Psychogenic
27
What causes drug induced tremors?
• Theophylline • Caffeine • Lithium • Valproic Acid • Tricyclic Antidepressants • Isoproterenol • Thyroid hormone
28
Describe an essential tremor?
Typically a postural tremor, may worsen with goal-directed activity • Familial in ~50% Hands, head, voice, trunk • Alcohol improves
29
Describe the treatment for an essential tremor?
• Propranolol • Primidone • Gabapentin • Topiramate • Deep Brain Stimulation
30
Describe an intention tremor?
• Localizes to Cerebellum • Occurs with deliberate movement • Tremor worsens as the target is approached • Can occur in extremities, head or entire body
31
Describe chorea?
• 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
Describe the etiology of chorea?
• Etiology • Hereditary • Huntington • Wilson • Sydenham Chorea • Chorea Gravidarum • Drug Toxicity • Levodopa (dopaminergic drugs) • Antipsychotics • Lithium • Phenytoin • Oral contraceptives
33
What medical disorders cause chorea?
• Etiology • Cerebrovascular Disorders • Medical Disorders • Thyroid • SLE, lupus anticoagulant • Encephalitis • AIDS • Hypocalcemia, hypomagnesemia, hypernatremia Hyper-/Hypoglycemia • Hepatic Cirrhosis
34
Describe Huntington disease?
• 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
Describe the clinical presentation of Huntington disease?
• Clinical • Dementia • Chorea,difficulty initiating saccadic eye movements • Psychiatric • Depression • OCD
36
What is the prognosis of Huntington disease?
Progressive to death within 10 to 20 years
37
Describe hemiballism?
• Unilateral choreiform movements, more violent and rapid compared to typical chorea • Involves proximal muscles • Lesion of contralateral subthalamic nucleus (usually vascular)
38
Describe dystonia?
• 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
Describe how dystopia is classified?
• 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
Describe causes of dystonia?
• 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
What is the treatment of dystonia?
• Treatment • Medications frequently not helpful • Anticholinergics • Benzodiazepines • AEDs • Levodopa • Baclofen • Botox for focal dystonia/writer's cramp
42
Describe myoclonus?
• 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
Describe the etiology of myoclonus?
• Physiologic • Essential Myoclonus • Epileptic • IME • Progressive myoclonic epilepsy syndromes • Drug-Induced • symptomatic
44
What are the drug induced causes of myoclonus?
• SSRIs • TCAs • Lithium • Levodopa • Opiates
45
What are the symptomatic causes of myoclonus?
• Etiology • Symptomatic • CID • SSPE • Metabolic disorders • Vascular lesions • Neoplasms • Spinal cord lesions • Wilson disease/ degenerative disorders
46
What is the treatment of myoclonus?
• Difficult to treat • Valproic acid • Benzodiazepines
47
What is TICs?
• 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
Describe Tourette syndrome?
• Multiple (at least two) motor and vocal tics • 3x more common in males • Associated with OCD,ADHD, depression
49
Describe the treatment for Tourette syndrome?
• Treatment • Clonidine • Low dose, high potency antipsychotics • Haloperidol (Haldol) • Dopamine agonists • ?Botox
50
What is a acute dystonia?
• 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
What is the treatment of acute dystonia?
• IM/IV Diphenhydramine (Benadryl) or Benztropine (Cogentin)
52
What is tardive dyskinesia?
• 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
What is the treatment for tardive dyskinesia?
• Treatment • Discontinue use of medication • Switch to atypical antipsychotic • Riserpine and tetrabenazine • Lithium • Baclofen • Benzodiazepines
54
Describe neuroleptic malignant syndrome?
• 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
Describe Wilson disease?
• 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
What are the lab results in Wilson disease?
• Labs • Abnormal liver function • Low serum copper and ceruloplasmin • Increased urinary copper excretion • High copper in liver biopsy
57
Describe the treatment of Wilson disease?
• Reduction of dietary copper • Penicillamine (copper-chelating agent) • Pyridoxine to prevent anemia • Liver transplantation
58
What is restless leg syndrome?
• 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
What is the treatment of restless leg syndrome?
• Dopamine agonists • Carbidopa/levodopa • Benzodiazepines • Opiates • Gabapentin • Carbamazepine

MBBS 2: Head and Neck Clinical Correlations (7 decks)

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