Movement Disorders Flashcards
(46 cards)
Essential Tremor
- Most common movement disorder in adults
- The etiology is unknown, but speculated to have a genetic component and
follows an autosomal dominant model, seen in ~50-70% of cases - Prevalence is equal in men and women. Tends to be slightly higher in caucasians
than people of African descent
Essential Tremor clinical findings
- Essential tremor is activated by voluntary or
resisted movement (ie arms held against gravity). - Frequency is usually moderate to high (6-12 Hz)
- Symptoms often worsen with anxiety, excitement, fatigue, and temperature
extremes - Not usually exacerbated by caffeine
- It is typically relieved temporarily by small amounts of Alcohol
- Not present during sleep
Commonly affected areas for essential tremor:
– Most often affects the hands/arms
– Head - can be vertical or horizontal
– Voice
– Less commonly the face/trunk
* Legs are almost never affected.
Natural Course of essential tremor
- Usually worsens gradually over time, with severity increasing as well as new
areas of the body being affected. - Disability for ES can be significant, often affecting writing, using tools, and eating
or drinking. - Rarely, can be accompanied by gait changes, mild cognitive impairment, and
resting tremor
Essential Tremor diagnosis
- Diagnosis is clinical, based on symptoms and
ruling out other causes
– Labs - CBC, CMP, Thyroid
– Avoid MRI unless focal or sudden changes - Use this table to as a guide
- Differential Diagnosis:
- Wilson Disease
- Electrolyte Abnormalities
- Medication/drug induced
- Psychogenic tremor
Essential Tremor treatment
- Treatment is symptomatic, not curative
- When the tremor is affecting function or causing difficulty with ADLs:
– Propranolol
– Primidone - Alcohol is not generally recommended as treatment, but small amounts may
help is social situations (like ½ glass of wine). - Second line includes gabapentin, topiramate, and benzodiazepines
- Thalamic stimulator or subdural motor cortex stimulator can be placed
Huntington Disease
- An Incurable progressive disorder
- Sometimes called Huntington’s Disease. Named after George Huntington, the
physician who described it as “hereditary chorea” in 1872 - Characteristic features include:
– Involuntary movements
– Dementia
– Behavioral changes
Huntington Disease epidemiology
*Mean age at onset ranging
from 35-44 years * Varies from 2 years to
older than 80 years
*Juvenile HD (Westphal
variant), defined as
having an age of onset of
younger than 20 years
Huntington Disease pathophysiology
– Neostriatum
– Marked neuronal loss also is seen in deep layers of the
cerebral cortex
– Other regions, including the globus pallidus, thalamus,
subthalamic nucleus, substantia nigra, and
cerebellum, show varying degrees of atrophy
*Autosomal dominant inheritance
*CAG nucleotide repeat
Huntington Disease clinical presentation
*Chorea
* Fidgetiness
* Uncontrollable flailing of the
extremities
* Less prominent in juvenile HD
*Gradually, chorea is replaced by
dystonia and parkinsonian features
*Dysarthria and dysphagia *Abnormal eye movements *Tics and myoclonus *Cognitive decline and dementia
Huntington Disease diagnosis
*Genetic testing
*CT or MRI or PET
* Bicaudate diameter
* Cerebral atrophy
Huntington Disease treatment
*Neurology referral
*Severe chorea
* Clonazepam or diazepam
* Valproic acid
* Dopamine-depleting agents, such as reserpine or tetrabenazine
* Neuroleptics
*Patients who have predominant features of bradykinesia and rigidity may
benefit from treatment with levodopa or dopamine agonists
*SSRIs for depression
*Antipsychotic medications may be necessary
Huntington Disease prognosis
– Death usually occurs 10-25 years after diagnosis
* average of 19 years
– Pneumonia and CVD most common cause
Parkinson’s Disease (PD)
- PD, or Parkinsonism, or Parkinson disease, or Parkinson’s disease
– Slowly progressive disorder
– Degenerative disorder
– Bradykinesia - slow and decreased movement
– Resting tremors
– Postural and gait instability
Parkinson’s Disease (PD) pathophysiology
- Lewy body formation - Synuclein
protein build up in neural cells
– usually in the nigrostriatal system - Rare cases where there are no Lewy
bodies, but there is a mutation of
PARK 2 gene - Dopaminergic cells degenerate in the
substantia nigra
– results in depletion of dopamine
and results in motor
manifestations of PD
Parkinson’s Disease (PD) clinical findings
- Insidious onset and asymmetric
- Resting tremor of one hand is often the 1st symptom
- Slow and coarse tremor
- Worse at rest, Lessening during movement
- Absent at sleep
- Tremor increases with emotional stress and fatigue
- Watch the wrist and fingers - Pill rolling
- Jaw and tongue are commonly affected (Not often the voice)
- Tremor may be less prominent as disease progresses
- Increased rigidity - increased resistance to passive movement
- Bradykinesia - slow movements, hypokinesia and akinesia can also be seen
- Postural instability - Can result in falls
- Difficulty initiation of gait
- Difficulty in maneuvering
– Sense of instability while walking - Parkinsonian shuffle
– Quick short, almost sliding gait - Facial expressions are flat and fixed
- Micrographia (Small writing)
- Soft voice
- Clumsiness
Non-motor symptoms of parkinsons disease
– Decreased sense of smell
– Disorder of REM
– Daytime sleepiness
– Forgetfulness
– Excessive salivation
– Urinary urgency
Physical exam tips for assessing parkinson’s disease
- Resting tremor
– assess with pt sitting relaxed with hands in the lap
– ask pt to count backward from 10 to 1 - Assess tremor with outstretched hands
– look for a postural tremor - Finger-to-nose test to assess for kinetic tremor
- Rigidity is tested by looking for cogwheeling, flex and extend at wrist and elbows
- Observe facial expression and blink rate
Parkinson’s Disease diagnosis
– PD is a clinical diagnosis based on motor symptoms
* Unilateral resting tremor
* Bradykinesia
* Rigidity
* Finger-to-nose test: tremor disappears or drastically reduces
* Postural instability
– Shuffle gait, hunched shoulders
Parkinson’s Disease treatment
- Goals: control signs and symptoms for as long as possible while minimizing
adverse effects - Medications
– Amantadine
– Sinemet
– Combination of: Levodopa and Carbidopa
Amantadine use in parkinson’s disease
– For mild cases without disability
– MOA is unclear, thought to potentiate CNS dopaminergic responses
– Side effects are rare
Sinemet use in parkinson’s disease
– Combination of: Levodopa and Carbidopa
– Levodopa
* Provides the greatest antiparkinsonian benefit for motor signs and
symptoms, with the fewest adverse effects in the short term
* Long-term use is associated with the development of motor
fluctuations (“wearing-off”) and dyskinesias
– Carbidopa
* Inhibits the decarboxylation of levodopa to dopamine in the systemic
circulation, allowing for greater levodopa distribution into the central
nervous system
Monoamine Oxidase (MAO) - B Inhibitors (Selegiline) use in parkinson’s disease
- Inhibits the degeneration of dopamine and therefore helps decrease
fluctuation in dopamine concentration
Dopamine Agonists (ropinirole, pramipexole) use in parkinson’s disease
- Help to mitigate the effects of long-term levodopa therapy
- Act directly on dopamine receptors to stimulate dopamine response.
- Used in pts that are on Levodopa and develop dyskinesias
- 15% increase in adverse events such as somnolence, sudden-onset sleep,
hallucinations, edema, and impulse control disorders
