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35 Cards in this Set
- Front
- Back
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Extrapyramidal diseases
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Parkinson's
Huntington's Diffuse Lewy body Progressive supranuclear palsy Cortical-basal ganglionic degeneration Multiple system atrophy Thalamic dementias Wilson's Hallervorden-Spatz Fahr's |
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Cerebellar diseases
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Olivopontocerebellar atrophy
Friedrich's ataxia Spinocerebellar degenerations |
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Motor Neuron Diseases
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Motor neuron diease with dementia
Amyotrophic lateral sclerosis Parkinsonism dementia complex |
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Cardinal signs of Parkinson's
(requires 2 of 3) |
Bradykinesia
Rigidity Tremor (primarily at rest) |
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Other signs of Parkinson's
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Masked face, hypovolemic speech, swallowing difficulty, micrographia, flexed posture, shuffling gait, start hesitancy and freezing.
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resting tremor of PD
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70% of patients
pill rolling tremor in hands can involve lips, chin, jaw, legs |
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Bradykinesia
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80-90% of pt
most disabling sx of PD |
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Rigidity of PD
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>90% of pt
cogwheeling (fluctuating) or lead pipe (continuous) |
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Postural instability of PD
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indicative of advanced stage PD
Frequent cause of falls |
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Psych disorders of PD
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depression in up to 40%
Anxiety in 30% |
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Cognitive disorders of PD
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mild cognitive impairment
dementia in 15-40% of pts |
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Striatal dopamine deficiency syndrome of PD
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<50% DA loss is asymptomatic
70% DA loss for symptom manifestation -severity of DA loss best correlates with bradykinesia in PD |
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Risk factors of PD
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definite: old age
highly likely:MZ co-twin with early onset PD probable: positive family history Possible: herbicides, pesticides, heavy metals, proximity to industry, rural residence, well water, repeated head trauma |
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Protective factor for PD
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smoking
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DDx: hereditary d/o associated with parkinsonism
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wilson's
huntington's dentatorubro-pallidoluysian atrophy machado joseph disease |
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secondary parkinsonism
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drug induced
toxin induced metabolic structural lesions hydrocephalus infections |
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Drug induced PD
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crucial to r/o b/c most are reversible
- Antipsychotics: haldol, chlorpromazine, thioridizine, perphenazine, risperdal, zyprexa - antiemtics: metoclopramide, prochlorperazine |
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Toxin induced PD
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MPTP
carbon monoxide manganese cyanide |
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Structural lesions causing PD
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acute or subacute onset
other signs: hemiparesis, hyperreflexia, aphasia, sensory loss, seizures - brain tumors - infectious mass - aneurysm |
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Rx classes in PD
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- DA agents (Levadopa, Doapine agonists)
- COMT inhibitors - MAO-B inhibitors - Anticholinergics - Amantadine |
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Surgical Tx
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- Ablative (thalamotomy, pallidotomy)
- Electrical Stimulation (VIM thalamus, globus pallidus internus) - Transplant |
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Characteristics of Lewy Body Disease
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- Dementia
- Attentional impairment - Visuospatial difficulties - Fluctuations of cognitive functioning - Persistent, well-formed visual hallucinations - Parkinsonism |
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Neuropathology and tx of LBD
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- Lewy bodies in the cortical regions
- neuritic plaques - more frontal lobe atrophy than AD - Tx: Levodopa, cholinesterase inhibitor - neuroleptic sensitivity |
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Characteristics of Progressive Supranuclear Palsy
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- gait instability with falls
- axial rigidity - bradykinesia - supranuclear gaze abn. Down gaze prob - dysarthria - dementia with frontal lobe sx` |
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Neuropathology and tx of PSP
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- neuronal loss associated with gliosis and NFTs w/ straight filaments in substantia nigra, basal forebrain, subthalmic nucleus, pallidum and superior colliculus
Tx: levodopa not effective |
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Characteristics of Cortico-basal ganglionic degeneration
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- parkinsonism, often asymmetric
- Limb dystonia - postural or action tremor - focal reflex myoclonus - apraxia - cortical sensory loss - alien limb phenomena - choreiform dyskinesias - dementia frontal with depression, delusions - dysarthria - hyperreflexia |
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Wilson's Disease/hepatolenticular degeneration
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- onset in 2nd or 3rd decade
- defective copper metabolism - Autosomal recessive - excessive copper deposition in the liver, orneas, and basal ganglia - kayser fleischer rings - Psych sx: antisocial and sexually disinhibited behavior - tx: D Penicillamine |
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Huntington's dz
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- hereditary progressive neurodegenerative d/o
- emotional, behavioral and psych abnormalities - loss of previously acquired intellectual functioning - movement abnormalities |
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initial signs of Huntington's
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- slight personality changes
- forgetfulness - clumsiness - gradual devel of random, brief, fidgeting movement of fingers or toes - piano playing hands |
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neurobehavioral changes of HD
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- tend to develop gradually over time
- may become apparent before or concurrent with the motor manifestations of HD - initial sx may be personality changes - anxiety/depression/ AVH |
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Cognitive sx of HD
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- progressive dementia or grdual impairment of mental processes involved in comprehension, reasoning, judgment, and memory
- early signs of forgetfulness and difficulty maintaining focus and attention - impaired prob solving - diminished memory retrieval - impaired judgment and impulse control - diminished language skills with disorganized speech - impaired ability to plan, initiate, or perform certain purposeful movements (apraxia) |
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Motor sx of HD
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- gait is unsteady, disjointed, or lurching. "dance like"
- clumsy fine motor movements - postural instability - inability to sustain certain vol movements - poor control of tounge and diaphragm - dysphagia - dysarthria - strained, hoarse, or inappropriately loud voice. - oculomotor abnormalities (abn saccades) - rigidity and bradykinesia |
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Genetics of HD
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- gene is IT15, located on chrom 4 (4p16.3)
- encodes production of protein (Huntingtin) found in neurons throughout the brain. - expanded CAG seq result in production of abn Huntingtin protein - genetic anticipation - CAG repeats more unstable when inherited |
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Brain pathology in HD
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cortical atrophy
- basal ganglia atrophy - caudate atrophy concavity (bat wing ventricles) - selective loss of specialized cells results in dec inhibition of the thalamus. Thalamus increases its output to certain regions of the brain's cerebral cortex |
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Tx of HD
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- antipsychotics (haldol)
- benzodizepines suppress choreic movements - Tetrabenazine for chorea in ppl with HD |
