Involuntary Movement Disorders Flashcards
What is the term for movement disorder that is velocity dependent? Velocity independent?
spasticity - velocity dependent increase in muscle tone
rigidity - velocity independent increase in muscle tone
What are the 7 hyperkinetic movements?
- ballism / chorea / athetosis
- dystonia myclonus
- tics / stereotypies
- restless legs
- tremor
- akathisia
What are the not patterned, flowing hyperkinetic movements?
ballism / chorea / athetosis
What are the patterned, non-flowing hyperkinetic movements?
dystonia
myoclonus
tics / stereotypies
What are the hypokinetic movement disorders?
bradykinesia
akinesia
rigidity
(Parkinson’s Disease)
What is the function of the thalamus in terms of movement?
enhance movement → send excitatory signals to the cortex to induce movement
What are the outflow nuclei of the basal ganglia? What is their function?
- Globus Pallidus interna
- Substantia Nigra
They want to shut the thalamus down
What is the striatum & what does it do?
caudate + putamen
regulates movement (move when you want to & don’t move when you don’t want to)
What is the function of the subthalamic nucleus?
stimulates the outflow nuclei (GPi & SNr) → & encourage their inhibition of the thalamus
What is the function of the Globus Pallidus externa?
inhibits the subthalamic nucleus (preventing int from stimulating the GPi & SNr)
Briefly describe the pathway of the direct & indirect pathways. Which increases movement & which inhibits movement?
- Direct
- Striatum inhibits GPi → disinhibits the inhibition of the thalamus → movement
- Indirect
- Striatum inhibits GPe → disinhibits the inhibition of STN → STM stimulates GPi → GPi inhibits thalamus → inhibits movement
How does the Substantia negra pars compacta impact the direct & indirect pathways?
via dopamine (facilitates movement)
stimulates direct pathway
inhibits indirect pathway
At what age does Parkinson Disease typically present?
It is caused by decreased production of what neurotransmitter?
5th - 6th decade
decreased dopamine from substantia nigra pars compacts
Parkinson Disease causes what motor symptoms?
Treatment?
- T- tremor at rest, pill rolling, MCP tremor
- R- rigidity, cogwheeling at elbows
- A- akinesia/bradykinesia/hypokinesia
- slow initiation of movements, masked facies, micrographia, hypophona, hypomimia, decreased rate/amplitude finger tapping
- P- postural instability, stopped posture, slow shuffled gait, block-like turning
Treatment: respond to L-dopa
What are common non-motor symptoms seen in Parkinson disease?
-
affective disorders -
- depression, anhedonia, anxiety, impulsiveness, hallucinations
-
cognitive dysfunction -
- bradyphrenia, memory impairment, anosmia
-
visual disturbances -
- impaired color discrimination, visuospatial abnormalities
-
autonomic dysfunction -
- orthostatic hypotension
-
gastrointestinal dysfunction
- constipation
-
sexual dysfunction
- erectile dysfunction
-
sleep disturbance
- REM sleep disturbance
What are the causes of Parkinson’s?
- Idiopathic: sporadic or genetic
-
Secondary:
- drugs
- metabolic - hypothyroidism / low B12 / diabetes
- Environmental - synthetic heroin, pestacide
- Structural - stroke involving basal ganglia
- Infection - influenza encephalitis,
What are the red flags seen in atypical Parkinsonism?
- Atypical:
- early dementia, early falls, prominent ocular dysmotility, prominent dysautonomia, prominent ataxia,
- poor response to L-dopa
What are the drugs given to Parkinson patients to replace / boost dopamine?
- Replace Dopamine
- dopamine analogue: carbidopa / levodopa
- dopamine agonist: pramipexole,
- MAO-B inhibition (decrease dopamine degradation_
- irreversible - selegiline, rasagiline
- reversible - safinamide
- COMTI (prevents L-dopa breakdown - enhances carbidopa / levodopa)
- entacapone
- Amantadine (increases dopamine release / blocks reuptake)
What are the stimulants used to treat Parkinson disease?
Istradefylline: adenosine receptor agonist
What are the targets for deep brain stimulation as a treatment for Parkinson Disease?
GPi >>> STN
In addition to drugs, what is a treatment option for Parkinson Patients?
deep brain stimulation surgery
physical therapy
What are the 5 types of action tremors?
- postural
- kinetic
- task specific
- isometric
- psychogenic
What are the two types of postural action tremors? Provide examples of each.
- postural
-
enhanced physiologic:
- stress, fatigue, fever, hypoglycemia, drugs, EtOH withdrawal, hyperthyroidism,
-
Pathologic:
- essential tremor, midbrain or cerebellar pathology, muscle/nerve disease
-
enhanced physiologic:
What are the common causes of kinetic action tremors?
cerebellar disorders, stroke, MS, midbrain lesion
What are the common causes of task specific action tremors?
handwriting tremor, orthostatic
What are the common causes of isometric action tremors?
muscle contraction during sustained exertion
What are the common causes of psychogenic action tremors?
distractibility, entrainment, suggestibility
What are the age qualification of an essential tremor?
Type of tremor? Indicative of what type of brain dysfunction?
Symmetry? Intention?
before 40 or after 60
kinetic > postural of arms (wrist flexion/extension)
cerebellar / thalamo-occipital dysfunction
~30% asymmetric; ~50% intention element
Treatment for essential tremor?
better with alcohol
propranolol, primidone, gabapentin, topiramate, thalamic DBS
Cerebellar tremor is what type of tremor?
Symptoms?
essential tremor (+)
overshoot on finger to nose maneuver
several planes involved in tremor
dysarthria / scanning speech
gait ataxia / hypotonia
What are the common causes of cerebellar tremor?
cerebellar stroke, mass, B12 & vitamin E deficiency
What type of tremor is a Rubral Temor?
Symptoms?
kinetic > postural > resting (multiphase)
unilateral: severe (arm may be functionally useless) - may be present in sleep
mild dystonia or ataxia
What are the major causes of a rubral tremor?
Treatment?
- Causes
- stroke, tumor, MS
- Treatment
- levodopa
What is the term for involuntary, irregular, purposeless, non-rhythmic, abrupt, random & quick movements that flow from one body region to another?
Chorea
What is the term for large-amplitude chorea involving proximal arms, flinging & flailing movements (usually unilateral?
Ballism
What is the term for slow chorea; writing movements- similar to dystonia but not sustained, patterned or painful
athetosis
Ballism is seen in what situation?
post-stroke following hemiparesis
caused by lesion to contralateral subthalamic nucleus
Athetosis is seen in what situation?
children with cerebral palsy secondary to kernicterus
What is the most common cause of hereditary chorea?
huntington disease
Huntington Disease exhibits what type of inheritance patten? Type of mutation?
autosomal dominant
chromosome 4 ; CAG repeat → anticipation
What 3 symptom complexes are seen in Huntington Disease?
- motor
- chorea, dystonia, tics, parkinsonism
- cognitive
- dementia
- neuropsychiatric
- psychosis
What brain center is classically atrophied in Huntington disease?
caudate atrophy
What is the differential diagnosis for a patient with Chorea?
- Huntington Disease
- Metabolic
- B12 def, hypothyroidism, Sedenham chorea (post rheumatic, HIV, post encephalitic, chorea gravidarum, Lupus
- Vascular
- autoimmune
- demyelinating
What is the treatment for Huntington Chorea?
- Dopamine depletion
- tetrabenazine / deutetrabenazine (vesicular monoamine transporter 2 inhibitors → dopamine monoamine depletion)
What is the term for movements that are sustained, usually involving twisting & repetition with same muscle groups that is worsened by physical activity?
dystonia
What are the types of dystonia & what muscle groups does it commonly involve?
focal, segmental, multifocal, hemidystonic or generalized
eyes, vocal cords, neck, limbs
What is the term for cervical dystonia?
Treatment?
spasmodic torticollis
Treatment: clonazepam, trihexylphenidyl, baclofen, onabotulinumtoxin
What are the most common genetic causes of isolated dystonias?
- DYT-TOR1A
- DYT-THAP1
What diagnosis should you consider if you have a pediatric patient with dystonia? Treatmet?
Dopa-responsive dystonia : DTY5
diurnal variation (family history parkinsonism)
levodopa responsive
What is the term for sudden, brief, shock-like involuntary movements that are usually irregular but can be rhythmical? This can involve what body locations?
myoclonus
palate, eyes, limbs
What are the two types of myoclonus? Example?
- positive
- d/t additional muscle contraction
- negative
- d/t motor inhibition
- asterixis: brief flapping of outstretched arms seen in hyperammonemia or hyperuremia
Where is the electrical generator for myoclonus? Treatment?
cortical (epileptic)
subcortical
brainstem
spinal cord
What is Lance-Adams syndrome?
post anoxic generalized myoclonus; action or intention type
What are the treatments for myoclonus?
valproic acid, levetiracetam, clonazepam
What is the term for coordinated movements that repeat continually & identically that are not preceded by an irresistible urge
sterotypy
(ie. rocking in autism)
What are abnormal suppressible movements that accompany an urge or compulsion? They can be motor or phonic.
tics
What is tourette syndrome? Treatment?
motor & phonic tics onset prior to age 18
treatment: guanfacine, fluphenazine, SSRI, tetrabenazine, deep brain stimulation
What are the two medication-induced dyskinesias? Cause?
-
akathisia
- feeling of inner, restlessness that is reduced by moving - may be stereotypic
- focal (oral, genital) → withdrawal dopamine in parkinson patients
- generalized
-
tardivine dyskinesia
- rapid, repetitive, stereotypic movements involving oral, buccal & lingual areas
- lip smacking, tongue protrusion
usually caused by antipsychotics that block dopamine
What is the treatment for medication-induced dyskinesias?
benzodiazepines, benzotropine, amantadine
valbenazine & deuterabenzine (tardive dyskinesia)
What is the inheritance pattern & mutation seen in Wilson Disease?
autosomal recessive
chromosome 13; ATP7B gene → impaired biliar copper excretion
inability to incorporate copper into apoceruloplasmin to form ceruloplasmin
copper accumulates in liver & other tissues
Wilson Disease should be considered in a young patient with what symptoms?
tremors, dystonias, choreoathetosis, rigidityy associated with psychiatric disorder
What lab tests should be performed in a patient you suspect Wilson Disease? Treatment?
- Abnormal copper metabolism
- decreased serum ceruloplasmin
- increased serum copper
- increased 24 hr urinary copper
- gold standard → liver biopsy
- Treatment
- zinc, penicillamine
The provided image is indicative of what disease? What is the name of this sign?
Wilson Disease
Kayser-Fleischer ring
