Movement Disorders Flashcards

1
Q

Benign Essential Tremors (7)

A
  • Familial Tremor, FmHx common
  • Autosomal dominant
  • May temporarily improve with alcohol
  • No other abnormal findings
  • Can start at any age and is enhanced by stress
  • Postural tremor of the hands, head (or both) or voice (legs are spared)
  • Little disability (manual skills, handwriting)
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2
Q

Tx for Benign Essential Tremors

A
  • Usually unnecessary
  • Propanolol is 1st line
  • Primidone, alprazolam, clozapine, topiramate, gabapentin, mirtazapine
  • Botox (if localized)
  • If unresponsive to meds try contralateral thalamotomy or unilateral high frequency thalamic stimulation
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3
Q

Causes of Parkinsonism (6)

A
  • Idiopathic parkinson’s dz (onset 45-65, rarely familial)
  • Postencephalitic parkinsonism
  • Secondary to toxin exposure
  • Secondary to head trauma
  • Neuroleptic drugs (reversible)
  • Malignancy (rare!)
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4
Q

Idiopathic Parkinson’d Dz

A
  • Dopmine depletion d/t degeneration of the dopaminergic nigrostriatal system
  • Dopamine and Ach imbalance in corpus striatum
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5
Q

Clinical Presentation of Parkinsonism

A
  • Tremor, rigidity, bradykinesia and postural instability are hallmarks
  • Bradykinesia + tremor or rigidity
  • Often the tremor starts on one side
  • Tremor is most noticeable at rest and worsens with rapid motion of the opposite side
  • Mild decline in intellectual fxn
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6
Q

Rigidity- Parkonsonism

A
  • Increased resistance to passive movements
  • Cogwheeling (rigidity with passive movements)
  • Characteristic flexed posture
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7
Q

Bradykinesia- Parkinsonism (6)

A
  • Slow voluntary movements
  • Reduction in automatic movements (not swinging the arms while walking)
  • Difficulty getting up from a chair
  • Walking is difficult to start, but once they start it quickens. looks like they are falling forward
  • Shuffling gait, difficulty turning, difficulty stopping
  • Effective voluntary movement can be regained in an emergent situation
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8
Q

Physical Features of Parksonism (7)

A
  • Mask-like facies
  • immobile face
  • widened palpebral fissures (opening of the eye)
  • infrequent blinking
  • seborrhea on the face and scalp is common
  • mild blepharoclonus (muscle spasms of the eye)
  • tremor about the mouth
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9
Q

Signs of Parkinsonism (7)

A
  • Myerson’s sign (Pt continues to blink as you tap on their forehead, glabellar reflex)
  • Drooling
  • Soft, monotone voice
  • Dysdiadochokinesia
  • Micrographia
  • No muscle weakness
  • No alteration in DTR’s or Babinski’s reflex
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10
Q

Differential Diagnosis for Parkinsonism (7)

A
  • Slight tremor
  • Depression
  • Wilson’s dz
  • Huntington’s dz
  • Multisystem atrophy (Shy-Drager syndrome)
  • Progressive supranuclear palsy
  • Creutzfeld-Jakob dz
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11
Q

Parkinsonism Medication Goals

A
  • Tx is symptomatic
  • Increase dopamine
  • Decrease Ach
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12
Q

Amantadine

A
  • Parkinsonism medication
  • Mild symptoms, no disability
  • Mode of action is unclear
  • *Improves all the types of symptoms
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13
Q

Anticholinergic Drugs (6)

A
  • Tx for Parkinsonism
  • Help alleviate tremor and rigidity
  • Does not help bradykinesia
  • Dose is titrated up to response
  • Contraindications: BPH, narrow angle glaucoma, obstructive GI dz, elderly
  • Side effects: dry mouth, constipation, arrhythmias, confusion, increased ocular eye movements etc…
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14
Q

Levodopa (5)

A
  • Medication for Parkinsonism
  • Improves all features of Parkinsonism
  • Does not stop or progress the disease
  • Early side effects: N/V, hypotension, cardiac arrhythmias
  • Later on: dyskinesia, restlessness, confusion and behavioral changes
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15
Q

Levodopa-Induced Dyskinesia (4)

A
  • Many forms: chorea, athetosis, dystonia tremor, tics or myoclonus
  • On/off phenomenon d/t changing levodopa serum levels
  • On: dyskinesias are conspicuous but mobility is improved (high meds)
  • Off: dyskinesias are better but mobility is limited (meds low)
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16
Q

Levodopa- Carbidopa (Sinemet)

A
  • Parkinsonism medication
  • Carbidopa inhibits the breakdown of levodopa to dopamine in the periphery, does not cross the BBB
  • Decreases overall dose of levodopa
  • Lowers systemic side effects
  • Does not prevent on/off phenomenon
  • Dyskinesia, psychiatric complications may be increased
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17
Q

How do you treat Levodopa-Induced Dyskinesias?

A
  • Amantadine

- contraindicated in psychotic illness and narrow angle glaucoma

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18
Q

Dopamine Agonists

A
  • Parkinsonism medication
  • Given before or in junction with levodopa
  • Bromocriptine
  • Pramipexole
  • Ropinirole
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19
Q

Dopamine Agonist Side Effects (9)

A
  • Anorexia (bromocriptine)
  • N/V/C
  • postural hypotension
  • digital vasospasm
  • cardiac arrhythmias
  • nasal congestion
  • erythromelalgia
  • pulmonary infiltrates
  • pericardial/pleural/pulmonary fibrosis
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20
Q

Selective Monoamine Oxidase Inhibitors (MAOI)

A
  • Parkinsonism medication
  • Rasagiline/Selegiline
  • Adjunctive therapy in patients with response fluctuations to levodopa
  • Reduce metabolism of L-dopa, more sustained plasma levels, more constant dopaminergic stimulation to the brain
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21
Q

MAOI side effects

A
  • Tyramine rich foods may cause hypertension

- May slow progression?

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22
Q

COMT inhibitors

A
  • tolcapone/entacapone
  • reduce the metabolism of levodopa
  • more sustained plasma levels
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23
Q

COMT side effects

A
  • fulminant hepatic failure (tolcapone)

- Entacapone does not have hepatotoxicity

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24
Q

Atypical Antipsychotics

A
  • Used for Parkinsonism
  • Used for confusion and psychotic symptoms
  • olanzapine, quetiapine, risperdone, clozapine
  • Improves iatrogenic dyskinesias
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25
Q

Parkinsonism Management (5)

A
  • PT
  • Speech therapy
  • Aids to daily living
  • Devices to amplify voice
  • High frequency thalamic stimulation
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26
Q

Huntington’s Dz Characteristics

A
  • Characterized by chorea and dementia
  • Inherited, autosomal dominant dz
  • huntington gene is at 4p16.3
  • Onset 30-50
  • Fatal within 15-20yrs
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27
Q

Initial S/S of Huntington’s

A
  • Abnormal movement or intellectual changes
  • Early mental changes are behavioral: irritability, antisocial behavior, moody
  • progress to more obvious dementia
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28
Q

Huntington’s Dyskinesia

A
  • Starts as fidgeting or restlessness
  • Progresses to choreiform (occasional jerking or writhering) movements and dystonic posturing
  • Then progressive rigidity and akinesia rather than chorea may occur, especially in childhood cases
29
Q

Imaging for Huntington’s Dz

A
  • CT/MRI show cerebral and caudate nucleus atrophy

- PET shows decreased metabolic rate in corpus striatum

30
Q

Huntington’s Dz Differential Diagnosis (8)

A
  • Stroke
  • SLE
  • Paraneoplastic Syndrome
  • HIV
  • Various medications
  • Sydenham chorea (group A strep)
  • Huntington’s dz like disorder
  • Dentatorubral-pallidoluysian atrophy
31
Q

Huntington’s tx

A
  • Symptomatic, no cure
  • Underactivity of GABA and Ach neurons
  • Overactivity of dopaminergic neurons
32
Q

Huntington’s Medications (5)

A
  • Tetrabenazine
  • Phenothiazines
  • Haloperidol
  • Quetiapine
  • Amantadine
33
Q

Childhood Onset Idiopathic Torsion Dystonia

A
  • FmHx
  • Symptoms start in the legs
  • Progress to disabling severe dystonia
34
Q

Adult Onset Idiopathic Torsion Dystonia

A
  • No FmHx
  • Symptoms start in the arms or trunk
  • Severe disability is not usual
35
Q

Diagnosis of Idiopathic Torsion Dystonia

A
  • Diagnosis of exclusion
  • R/o birth trauma, anoxia, kernicterus
  • No mental delay with ITD
  • Other thing to consider: wilson’s dz, huntington’s dz, parkinsonism, sequela of encephalitis, neuroleptic drug therapy
36
Q

Tx of Idiopathic Torsion Dystonia (5)

A
  • Responds poorly to meds
  • 1st line therapy: levodopa, diazepam, baclofen, carbamazepine, amantidine, anticholinergics
  • 2nd line: tetrabenazine, phenothiazine, haloperidol
  • pallidal deep brain stimulation
  • thalamotomy if unilateral
37
Q

Focal Torsion Dystonia

A
  • Atypical or attenuated form of ITD if there is FmHx

- Focal manifestation of adult on set if no FmHx of ITD

38
Q

Focal Torsion Dystonia (4)

A
  • Spastic torticolis
  • Blepharospasms
  • Oromandibular dystonia
  • Writer’s cramp
39
Q

Spastic torticolis

A
  • Twisting the head to one side
  • Onset 25-50
  • Some resolve in a year, if not it persists for life
40
Q

Blepharospasms

A
  • Spontaneous, forced closure of the eyelids
41
Q

Oromandibular Dystonia (4)

A
  • Involuntary contraction of muscles about the mouth
  • Opening and closing of the mouth
  • Roving or protrusion of the tongue
  • Retraction of the platysma
42
Q

Writer’s Cramp

A
  • Dystonic contraction of the hand and forearm when the hand is used
43
Q

Focal Torsion Dystonia Tx

A
  • Medical tx usually doesn’t work
  • Trial of drugs used for ITD
  • Blepharospasms and torticolis can be treated with botox injections or nerve section
  • Writer’s cramp can be treated with botox or learn to use their other hand
44
Q

Myoclonus

A
  • General or focal jerks, seen in:
  • idiopathic epilepsy
  • Lipid storage dz
  • Ramsay-Hunt Syndrome
  • subacute sclerosing pan-encephalitis
  • Creutzfeld-Jakob Dz
  • Metabolic encephalopathies
  • Medication related
  • Anoxic brain injury
45
Q

Myoclonus Tx (3)

A
  • Responds well to valproic acid, benzodiazepines, piracetam
  • Anoxic brain injury: oxitriptan
  • Segmental myoclonus: could be d/t spinal cord lesion, get an MRI
46
Q

Wilson’s Dz Characteristics (5)

A
  • Metabolic disorder, excessive deposition of copper in the liver and brain
  • autosomal recessive
  • Presents before age 40
  • Abnormal movement and posture with or w/out signs of liver involvement
  • Psychiatric and neuropsychological symptoms are common
47
Q

Wilson’s Dz Physical Signs

A
  • Serum ceruloplasmin (plasma copper carrying capacity) is low 40mg/dL
  • liver biopsy may show hepatitis or cirrhosis
  • Kayser-Fleischer ring is pathognomonic
48
Q

Wilson’s Dz Neurological Presentation (5)

A
  • Related to basal ganglia fxn
  • Presents similarly to parkinson’s, pseudosclerosis w/tremor, ataxia, dystonic syndrome
  • dysarthria, dysphagia, incoordination and spasticity
  • Migraines, insomnia, seizures
  • behavioral/personality changes, emotional lability
49
Q

Wilson’s Dz Tx (4)

A
  • Chelating agents, penicillamine, trientine
  • Oral Zinc acetate or Zinc gluconate
  • Prognosis is good if there is no liver or brain damage
  • Family members should be screened
50
Q

What drugs can cause chorea? (9)

A
  • Levodopa
  • Bromocriptine
  • Anticholinergics
  • Phenytoin
  • Carbamazepine
  • Lithium
  • Amphetamines
  • OC
  • Chorea resolves with withdrawal of medication
51
Q

What drugs cause dystonia? (4)

A
  • Levodopa
  • Bromocriptine
  • Lithium
  • Carbamazepine
52
Q

What drugs cause Parkinsonism? (2)

A
  • Reserpine

- Tetrabenaine

53
Q

What drugs can cause a postural tremor? (8)

A
  • Epinephrine
  • Isoproterenol
  • Theophylline
  • *Caffeine
  • Lithium
  • *Thyroid
  • TCAs
  • Valproic acid
54
Q

Restless Leg Syndrome

A
  • Restlessness, curious sensory disturbances lead to an irresistible urge to move the legs
  • Disturbed nocturnal sleep and daytime sleepiness can result
  • FmHx, genetic loci have been found
55
Q

What can cause restless leg syndrome?

A
  • Idiopathic
  • Pregnancy
  • Iron deficiency anemia
  • Peripheral neuropathy (esp. uremic or diabetic)
56
Q

Restless Leg Syndrome Tx (6)

A
  • Pramipexole
  • Ropinirole
  • Benzos
  • Gabapentin
  • Levodopa
  • Opioids
57
Q

Tourette’s Syndrome Characteristics (4)

A
  • Multiple motor and phonic tics
  • Symptoms start before 21
  • Tics occur frequently for at least 1 yr
  • Tics vary in nature, number and frequency over time
58
Q

Initial presentation of Tourette’s

A
  • 80% motor
  • 20% phonic
  • Motor tic usually happen around the head and shoulders (sniffing, blinking, frowning, shoulder shrugging, head thrusting)
59
Q

Phonic Tics

A
  • Grunts
  • Barks
  • Hisses
  • Throat clearing
  • Coughs
60
Q

Coprolalia Tourette’s

A

Swearing

61
Q

Echolalia Tourette’s

A

Imitates everything you say

62
Q

Echopraxia Tourette’s

A

Imitates everything you do

63
Q

Other Types of Tourette’s

A
  • Self mutilating: nail biting, hair pulling, lip biting

- OCD is commonly associated

64
Q

Tourette’s PE (5)

A
  • No neurologic deficits other than the tics
  • Psychiatric disturbances may occur
  • EEG may show minor non-specific abnormalities
  • FmHx
  • Autosomal dominant with variable penetrance
65
Q

Tourette’s Diagnosis

A
  • Often delayed
  • Tic like character of the movements
  • absence of other neurological signs
  • Can present like Wilson’s dz so you need to rule this out
66
Q

Tourette’s Tx (7)

A
  • Haloperidol
  • Clonazepam
  • Clonidine
  • Fluphenazine
  • Pimozide
  • Bilateral high frequency deep brain stimulation
  • Botox
67
Q

If someone presents with a movement disorder and there is a concern for seizure, what test is indicated? (2)

A
  • MRI

- EEG

68
Q

If someone presents with myoclonus in a focal pattern, what test is indicated?

A

MRI of the spine