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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
Parkinsonism Management (5)
- PT - Speech therapy - Aids to daily living - Devices to amplify voice - High frequency thalamic stimulation
26
Huntington's Dz Characteristics
- Characterized by chorea and dementia - Inherited, autosomal dominant dz - huntington gene is at 4p16.3 - Onset 30-50 - Fatal within 15-20yrs
27
Initial S/S of Huntington's
- Abnormal movement or intellectual changes - Early mental changes are behavioral: irritability, antisocial behavior, moody - progress to more obvious dementia
28
Huntington's Dyskinesia
- 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
Imaging for Huntington's Dz
- CT/MRI show cerebral and caudate nucleus atrophy | - PET shows decreased metabolic rate in corpus striatum
30
Huntington's Dz Differential Diagnosis (8)
- Stroke - SLE - Paraneoplastic Syndrome - HIV - Various medications - Sydenham chorea (group A strep) - Huntington's dz like disorder - Dentatorubral-pallidoluysian atrophy
31
Huntington's tx
- Symptomatic, no cure - Underactivity of GABA and Ach neurons - Overactivity of dopaminergic neurons
32
Huntington's Medications (5)
- Tetrabenazine - Phenothiazines - Haloperidol - Quetiapine - Amantadine
33
Childhood Onset Idiopathic Torsion Dystonia
- FmHx - Symptoms start in the legs - Progress to disabling severe dystonia
34
Adult Onset Idiopathic Torsion Dystonia
- No FmHx - Symptoms start in the arms or trunk - Severe disability is not usual
35
Diagnosis of Idiopathic Torsion Dystonia
- 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
Tx of Idiopathic Torsion Dystonia (5)
- 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
Focal Torsion Dystonia
- Atypical or attenuated form of ITD if there is FmHx | - Focal manifestation of adult on set if no FmHx of ITD
38
Focal Torsion Dystonia (4)
- Spastic torticolis - Blepharospasms - Oromandibular dystonia - Writer's cramp
39
Spastic torticolis
- Twisting the head to one side - Onset 25-50 - Some resolve in a year, if not it persists for life
40
Blepharospasms
- Spontaneous, forced closure of the eyelids
41
Oromandibular Dystonia (4)
- Involuntary contraction of muscles about the mouth - Opening and closing of the mouth - Roving or protrusion of the tongue - Retraction of the platysma
42
Writer's Cramp
- Dystonic contraction of the hand and forearm when the hand is used
43
Focal Torsion Dystonia Tx
- 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
Myoclonus
- 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
Myoclonus Tx (3)
- Responds well to valproic acid, benzodiazepines, piracetam - Anoxic brain injury: oxitriptan - Segmental myoclonus: could be d/t spinal cord lesion, get an MRI
46
Wilson's Dz Characteristics (5)
- 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
Wilson's Dz Physical Signs
- Serum ceruloplasmin (plasma copper carrying capacity) is low 40mg/dL - liver biopsy may show hepatitis or cirrhosis - Kayser-Fleischer ring is pathognomonic
48
Wilson's Dz Neurological Presentation (5)
- 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
Wilson's Dz Tx (4)
- 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
What drugs can cause chorea? (9)
- Levodopa - Bromocriptine - Anticholinergics - Phenytoin - Carbamazepine - Lithium - Amphetamines - OC - Chorea resolves with withdrawal of medication
51
What drugs cause dystonia? (4)
- Levodopa - Bromocriptine - Lithium - Carbamazepine
52
What drugs cause Parkinsonism? (2)
- Reserpine | - Tetrabenaine
53
What drugs can cause a postural tremor? (8)
- Epinephrine - Isoproterenol - Theophylline * *Caffeine - Lithium * *Thyroid - TCAs - Valproic acid
54
Restless Leg Syndrome
- 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
What can cause restless leg syndrome?
- Idiopathic - Pregnancy - Iron deficiency anemia - Peripheral neuropathy (esp. uremic or diabetic)
56
Restless Leg Syndrome Tx (6)
- Pramipexole - Ropinirole - Benzos - Gabapentin - Levodopa - Opioids
57
Tourette's Syndrome Characteristics (4)
- 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
Initial presentation of Tourette's
- 80% motor - 20% phonic - Motor tic usually happen around the head and shoulders (sniffing, blinking, frowning, shoulder shrugging, head thrusting)
59
Phonic Tics
- Grunts - Barks - Hisses - Throat clearing - Coughs
60
Coprolalia Tourette's
Swearing
61
Echolalia Tourette's
Imitates everything you say
62
Echopraxia Tourette's
Imitates everything you do
63
Other Types of Tourette's
- Self mutilating: nail biting, hair pulling, lip biting | - OCD is commonly associated
64
Tourette's PE (5)
- 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
Tourette's Diagnosis
- 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
Tourette's Tx (7)
- Haloperidol - Clonazepam - Clonidine - Fluphenazine - Pimozide - Bilateral high frequency deep brain stimulation - Botox
67
If someone presents with a movement disorder and there is a concern for seizure, what test is indicated? (2)
- MRI | - EEG
68
If someone presents with myoclonus in a focal pattern, what test is indicated?
MRI of the spine

Neurology (12 decks)

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