Movement Disorders Flashcards
What presents with unilateral resting tremor, cogwheel rigidity, bradykinesia, and hypomimia?
Parkinson’s disease
What symptoms can present years before classic PD symptoms appear?
REM sleep behavior disorder, constipation, and anosmia
Psychiatric co-morbidity in PD
Major depression
Diagnosis criteria for PD with dementia (Parkinsons +)
PD for at least 1 year before the onset of dementia symptoms
Gene mutation present in 10% of familial PD and 5% of sporadic PD
LRRK2 mutations (autosomal dominant)
Most common in North African Arabs
Parkinson’s pathologic findings
Alpha-synuclein inclusions/Lewy bodies primarily within the substantia nigra and locus coeruleus
How are dopamine transporter (DAT-SPECT) scans used for PD?
Measures the availability of striatal dopamine
How are PET scans for 11F or 11C dopa used for PD?
Measure dopa decarboxylase activity while PET scans for 11C DTBZ assess vesicular monoamine transporter-2 activity
α-synucleinopathies
Parkinson disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB)
Marked clinically by parkinsonism and pathologically by deposition of α-
Gold standard for PD diagnosis
Post-mortem pathology
Atrophy of dopaminergic cells in the substantia nigra and accumulation of α-synuclein in Lewy bodies and neurites in the brain
What drugs can cause drug induced parkinsonism?
Antipsychotics
* chlorpromazine, prochlorperazine, risperidone, clozapine, etc.
Antiemetics (metoclopramide)
Lithium
SSRIs
Valproic acid
Phenytoin
Is drug induced parkinsonism always resolved with medication resolution?
Usually but not always
Symptoms of restless leg syndrome
Uncomfortable sensation in the legs and irresistible urge to move legs, worse at night/periods of rest and relieved by movement
What are the tauopathies?
Progressive Supranuclear Palsy
Corticobasal Degeneration
Frontotemporal with Parkinson linked to chromosome 17
Symptoms of PSP
Parkinsonism (asymmetric limb rigidity)
Behavioral changes
Early falls
Vertical gaze palsy
Impaired smooth oculomotor pursuit
PSP imaging findings
Atrophy of the midbrain tegmentum, corpus callosum, and anterior cingulate gyrus
Known as hummingbird or penguin sign
PSP pathology
Globose neurofibrillary tangles in the brainstem and ganglia. Tufted astrocytes
What drugs can cause chorea (aka tardive dyskinesia?
Dopamine blocking agents - metoclopramide
Treatment for tardive dyskinesia:
Vesicular monoamine transporter 2 (VMAT2) inhibitors: valbenazine and deutetrabenazine
Characteristics of physiologic tremor
Low frequency, high amplitude tremor that involves the hands: no other associated disorder
Can be worsened by sympathetic activity: drinking coffee, SSRIs
What medication increase physiologic tremor
Adrenergic agonists
Lithium
Tricyclics
Levodopa
Nicotine
Xanthines
Antidepressants
Corticosteroids
VPA
Bromides
Frequency of a PD tremor:
4-6 Hz, low amplitude
Characteristics of spinocerebellar ataxia type 1:
Starts around 30-40 yo
Progressive cerebellar ataxia
Dysarthria
Bulbar dysfunction
Mode of inheritance for spinocerebellar ataxias:
Autosomal dominant
Characteristics of spinocerebellar ataxia type 7:
Similar to type 1 with seizures, myoclonus and retinal degeneration
Presents in teenagers/young adults
Dentatorubral pallidoluysian atrophy (DRPLA) is caused by:
CAG trinucleotide repeat expansion of the polyglutamine receptor on atrophin-1 gene on chromosome 12p
MRI findings for Dentatorubral pallidoluysian atrophy (DRPLA)
Cerebellar and brainstem atrophy, calcification of the basal ganglia and leukodystrophic changes
What are the pathologic characteristics of corticobasal degeneration?
Widespread neurodegeneration and deposits of hyperphosphoralated tau in neurons, glia, astrocytic plaques and corticobasal inclusions
Classic presentation of corticobasilar degeneration:
Asymmetric rigidity
Dystonia
Ideomotor apraxia
Neuroimaging findings for corticobasal degeneration:
Asymmetric frontoparietal atrophy
