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52 Cards in this Set
- Front
- Back
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CNS component at risk for: brain contusion
Why? |
orbital and temporal regions
Abnormal inner skull surfaces |
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CNS component at risk for: ischemia
Why? |
CA1 hippocampus, Purkinje cells in cerebellum
Lots of glutamate receptors --> excitotoxicity |
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CNS component at risk for: CO intoxication
Why? |
Globus pallidus, substantia nigra
High conc. of Fe-containing substances with high affinity for CO |
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CNS component at risk for: methanol intoxication
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Putamen
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CNS component at risk for: chronic ethanol use
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Anterior cerebellar vermis
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CNS component at risk for: Neurodegenerative disorder
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Groups of neurons, glia
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CNS component at risk for: Leukodystrophy
Why? |
Myelin
Deficiency in myelin-producing/maintaining enzymes |
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CNS component at risk for: MS
Why? |
Myelin
Autoimmune attack |
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In a neurodegenrative disorder, are all cells malfunctioning?
Progression of neurodegenerative disorders? Definitive Diagnosis? |
No, selectively vulnerable cells
insidious onset and progression brain biopsy |
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What symptoms characterize Alzheimer's?
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Dementia - general deterioration in cognition (memory, language, concentration, etc)
Insidious onset |
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Gross:
-narrow gyri + wide sulci -dilated ventricles (hydrocephalus ex vacuo) What do these conditions show and when are they seen? |
Cortical atrophy
seen often in alzheimer's |
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Microscopically:
Neurfibrillary tangles Senile plaques What condition |
Alzheimer's
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What are neurofibrillary tangles?
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Hyperphosphorylated tau aggregate (cytoskeletal protein) in cytoplasm of neurons --> destabilization of neuronal cytoskeleton
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What are 2 components of senile plaques and where are they found?
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Neurites = dystropic neurons (+ tau filaments) in neuropil of cortex and in senile plaques
Beta-amyloid = peptide cleaved from APP, accumulates in brain parenchyma or cerebral blood vessels (amyloid angiopathy) |
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How do tau neurofibrillary tangles and b-amyloid destroy the brain?
one place especially |
neuron dysfunction and death converges to produce a loss of synapses
Loss of cholinergic synapse in basal forebrain nuclei |
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What are 2 sites in which neurofibrillary tangles and senile plaques are most abundant?
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Medial temporal lobe
neocortex |
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Progression of accumulation of neurofibrillary tangles and senile plaques:
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1) enterorhinal cortex
2) other limbic structures 3) neocortex |
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Most important risk factor for alzheimer's?
Genetic factors in Alzheimer's -what % -what mutations -inherited factors |
Age
10% AD is familial Autosomal dominant Mutations in presenilin1 or 2, APP Inheritance of epsilon4 alleles of apolipoproteinE (chromosome 19) |
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60 yo patientpresents with any combo of:
Resting tremor rigidity bradykinesia bradyphrenia shuffling gait stooped posture masked facies difficulty arising from chair diagnosis? Course? |
dx: Idiopathic Parkinson
Course: 25% get dementia, patients high risk of falling |
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Macro: substantia nigra becomes pale
Micro: round, eosinophilic cytoplasmic inclusions surrounded by pale halo Condition? |
classic idiopathic parkinson
Those are Lewy bodies in the cytoplasm! |
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What protein is found heavily in Lewy bodies?
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a-cynuclein
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Pathogenesis of parkinson
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Lose neurons in substantia nigra which normally innervate the basal ganglia (coordination of movement center)
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Given a patient with parkinsonism, what is one strategy for treatment
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Increase domapinergic transmission to increase substantia nigra signaling to the basal ganglia
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5 genes implicated in parkinson
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1. a synuclein gene
2. PRKN 3. DJ1 4. PINK1 5. LRRK2 |
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Environmental factors implicated in parkinson
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MPTP
Pesticides/herbicides heavy metals solvents |
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Lewy neurites (a-synuclein rich neurons in gray matter of neocortex) + parkinson symptoms + dementia
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Dementia with lewy bodies
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Dementia (more often before age 65) + language or behavioral mainfestations +/- parkinson
condition? pathology? |
Frontotemporal degeneration
Loss of neurons in frontal or temporal lobes, high tau, glial pathology common |
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genetic causes of FTD degenerations
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Mutations in Tau gene
40% have positive family history, autosomal dominant |
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Gross: 'knife edge' atrophy of fronto-temporal gyri
condition? Symptoms? |
Pick disease (FTD)
dementia +language/behavioral + before age 65 usually |
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micro: basophilic, sharply circumscribed fibrillar cytoplasmic inclusions in dentate fascia of hippocampus
What are these? What do they contain? |
Pick bodies, contain Tau
Pick disease |
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Disorder in which myelin is biochemically abnormal
Disorder in which myelin comes under autoimmune or toxic/infectious attack (what is an example) |
leukodystrophy
myelinoclastic disorders (MS) |
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Major clinical manifestations of demyelinating disorders?
pathogenesis? |
Clinical = primarily motor
Loss of myelin (usually axons are spared) --> permanent loss of saltatory conduction in CNS (regeneration limited) |
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What is the pattern of demyelination in leukodystrophies?
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Widespread, confluent demyelination
Myelin associated with U-fibers, connecting adjacent gyri, is spared |
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age of onset: infancy, early childhood
Inheritance: aut. recessive Enzyme deficiency: galactocerebroside-b-galatosidase Leukodystrophy? |
Krabbe disease
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age of onset: infancy or childhood/adolescence
Inheritance: x-linked recessive Enzyme deficiency: ABCD1 Leukodystrophy? |
Adrenoleukodystrophy
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age of onset: adulthood
Inheritance: x-linked recessive Enzyme deficiency: ABCD1 Leukodystrophy? |
adrenomyeloneuropathy
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age of onset: infancy, childhood/adolescence, or adulthood
Inheritance: aut. recessive Enzyme deficiency: arylsulfatase A Leukodystrophy? |
Metachromatic leukodystrophy
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MS
age gender place |
young to middle aged
2:1 female higher latitutdes |
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episodic attacks of demyelination over time and space
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MS
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gross: plaques of demyelination, asymmetric
Micro: foci of demyelination around venules |
MS
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Microscopic difference between active and later stages of MS myelin attack?
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active: plaques contain lymphocytes and histiocytes
Later: plaques become gliotic (more astrocytes), decreased density of myelin, sparing of axons |
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LP results:
Myelin basic protein + CSF IgG oligoclonal bands of Ig What condition |
MS
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What imaging study can be used to identify white matter bands in MS?
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MRI
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Etiology of MS
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Autoimmunity: CD4 T cells attack myelin antigens (MBP)
Demyelination by macrophages |
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Genetic factor involved in MS
Risk among first degree relatives of getting MS |
MHC HLA-DR2 haplotype
15x |
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Pathogenesis of parainfectious demyelinating disorders?
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following flu or illness, autoimmune attack on patient's myelin by antibodies that are directed at antigens on the infectious agent
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Age at which Alzheimer's usually onsets?
Earliest age? |
60
30 |
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Alzheimer's prognosis
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Usual death in 8-10 years secondary to complicating illness (pneumonia, UTI etc.)
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Lobe of the brain that controls
a. language, personality b. memory c. perception, orientation |
a. Frontal
b. Temporal c. Parietal |
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What is hydrocephalus ex vacuo?
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Enlargement of CSF spaces secondary to loss of parenchyma in brain atrophy
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What is the Lewy body variant of alzheimer's disease?
How often is it seen? |
Patients with histologically proven AD also have clinical/pathological (Lewy Bodies) features of PD
40% of AD patients |
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What is diffuse Lewy Body Disease?
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dementia + a-synuclein pathology in brain stem limbic regions, cerebral cortex
No Alzheimer's pathology |
