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45 Cards in this Set
- Front
- Back
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What are the 2 groups into which Parkinsons's syndromes can be divided? Which is more common?
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1. Symptomatic
2. Idiopathic - most common |
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What is symptomatic parkinsonism caused by?
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-Infections
-Cerebral arteriosclerosis -CO intoxication -Drug OD -Pesticides |
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What are the 3 hallmark microscopic features seen in idiopathic Parkinson's?
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-Degeneration/loss of neurons in the basal ganglia - Subst. nigra
-Loss of melanin from subst. nigra neurons, macrophages eat -Lewy bodies in neurons |
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What are the 3 hallmark gross findings in Parkinson's disease?
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-Atrophy of Substantia nigra
-Loss of melanin from Sub. nigra -Degeneration of caudate and lenticular nuclei dt atrophy of SN |
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What is the disease called if Lewy bodies are seen in many cortical neurons but no other signs of Alzheimer's are present?
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Diffuse Lewy Body disease
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What will a patient show clinically if they have diffuse Lewy body disease?
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-Dementia
-Parkinson symptoms |
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What are the clinical manifestations of Parkinson's?
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-Tremor
-Rigidity -Akinesia (decreased movement) -Truncal instability |
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What is the pathophysiologic cause of symptoms in Parkinsons?
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Distrubed regulation of input to the Thalamus from the Cerebellum and Basal Ganglia
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How is the thalamic input regulation disturbed?
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By a decrease in Dopamine due to the neuronal degeneration and loss in the Substantia nigra.
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What are 3 possible methods of improving neurological dysfunction in Parkinson's?
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1. Give L-Dopa (precursor to DA)
2. Transplant adrenals 3. Give fetal mesencephalic tissue to basal ganglia |
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What is the cause of the neuron degeneration in Substantia nigra in Parkinson's?
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Unknown
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What is the neuron degeneration in Substantia nigra in Parkinson's likely due to?
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Free radicals and mitochondrial dysfunction
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What genes associated with lewy bodies and Ubiquitin have been found to be abnormal in some familial forms of parkinson's?
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a-synuclein - lewy body gene
parkin - ubiquitin |
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What are 3 diseases that are associated with Parkinson's syndrome clinically?
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-Shy-Drager syndrome
-Progressive supranuclear palsy -Striatonigral degeneration |
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What are the clinical manifestations of Shy-Drager syndrome?
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-Idiopathic Parkinson symptoms
plus -Postural hypotension due to neuron loss in the ANS |
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What are 3 clinical symptoms seen in patients w/ Progressive supranuclear palsy (in addition to the Parkinson symptoms)
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-Dementia
-Brainstem nuclei involvement -Vertical gaze palsy |
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How does Striatonigral degeneration compare to Parkinson's?
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-Same clinical symptoms
-Opposite nuclei involvement - Caudate/Putamen show primary atrophy, Subs. nigra is 2ndary. |
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How is Huntington's chorea transmitted?
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Autosomal dominant
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What is the genetic defect in Huntington's chorea?
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Increased triplet CAG repeats
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Where is the genetic locus of the CAG repeats on the Huntington's gene?
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Chromosome 4 p16.3
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What protein is decreased in patients with the genetic defect on Ch 4p16.3?
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Huntingtin
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What is the loss of Huntingtin protein associated with?
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Degeneration of GABA-containing neurons
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What are the 3 clinical symptoms of Huntington's due to the loss of GABA neurons?
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1. Progressive chorea
2. Mental retardation 3. Psychosis |
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In what 2 areas of the brain are loss of neurons and gliosis seen in Huntington's?
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-Caudate/putamen
-Cortex |
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What are the 3 main microscopic findings in Huntington's chorea in the Caudate and putamen?
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-Loss of small/med neurons
-Decreased GABA -Gliosis |
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What are the gross findings seen in Huntington's?
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Atrophy of the caudate, putamen, and cortex.
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What 2 interrelated degenerative diseases are located in the cerebellum and brainstem?
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-Pontocerebellar degeneration
-Olivopontocerebellar atrophy |
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What are the manifestations of Pontocerebellar and Olivopontocerebellar degeneration?
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Progressive cerebellar ataxia
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What 3 areas of the cerebellum and brainstem undergo primary degeneration in these diseases?
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-cerebellar cortex
-pontine nuclei -brachium pontis |
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What area undergoes secondary degeneration in the diseases of pontocerebellar degeneration?
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The olives
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What cell layer is lost in Olivopontocerebellar degeneration?
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The purkinje cell layer
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What are the 2 degenerative diseases of the spinal cord?
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-ALS
-Friederich's ataxia |
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What are the 2 most common disease manifestations of ALS?
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-Progressive weakness
-Respiratory failure |
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What is the primary microscopic change in ALS?
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Loss of motor neurons and gliosis in the spinal cord, brainstem, and cortex.
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What changes secondarily to the loss of motor neurons in the cortex?
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The corticospinal tracts in the spinal cord degenerate.
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What are the 3 major gross findings in ALS?
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Atrophy of the
-spinal cord -peripheral nerves -muscles! |
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What is the cause of neuron loss in ALS?
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Unknown
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What is the defective gene in familial ALS associated with?
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Superoxide dismutase
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What is Friedreich's Ataxia?
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An autosomal recessive type of Spinocerebellar ataxia
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What is Spinocerebellar ataxia?
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A group of >30 rare familial diseases with clinical manifestations of ataxia and other anomalies
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What are the 4 clinical manifestations associated with Friederich's ataxia?
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-Clubfeet
-Kyphoscoliosis -Congenital heart disease -Diabetes |
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Where is the loss of neurons in Friederich's ataxia? What is the result of this?
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-Clarke's column
-Substantia gelatinosa So the spinocerebellar tracts degenerate |
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What 2 other tracts are involved in Friederich's ataxia?
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-Corticospinal tracts
-Posterior columns |
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What is the manifestation of corticospinal tract involvement?
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Increased deep tendon reflexes
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What are the 2 manifestations of posterior column involvement?
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-Decreased deep tendon reflexes
-Loss of proprioceptive sensation |
