1B4Ul - Dementia And Degenerative Disorder

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degeneration of the cortex (brain) leads to

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dementia

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degeneration of the brain stem and BASAL GANGLIA leads to

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Movement disorder

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Alzheimer disease is the M.C. cause of

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Dementia

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how Dementia is formed

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Amyloid Precursor Protein (neuron receptor) is broken down to A-Beta-Amyloid, that accumulates and causes dementia

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Dementia

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is memory loss + cognitive dysfunction without loss of consciousness

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Alzheimer clinical presentation

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slow memory loss (takes years)
progressive disorientation
loss of learned motor skills and language
behavior and personality change

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focal neurological deficit is not seen in

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early stages of Alzheimers

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Alzheimer sporadic forms the major risk factor is

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Age

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in Alzheimer sporadic forms the risk is increased with

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increased Epsilon-4 allele for Apolipoprotein-E (APOE) **

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Epsilon-4 allele increases the of Amyloid precursor protein to

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A-Beta-Amyloid

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in Alzheimer sporadic forms the risk is decreased with

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Epsilon-2 allele for Apolipaprotein-E

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Alzheimer --> early --> familial form genetic mutation of

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presenilin-1

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Alzheimer --> early --> familial form increased risk with

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down-syndrome **

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Amyloid precursor protein is present on

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chromosome 21

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most cases of Alzheimer is

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sporadic

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Alzheimer hallmark is

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diffused cerebral atrophy

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Alzheimer- changes in brain are

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narrowing of gyri and widening of sulci

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Hydrocephalus EX vacuo **

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dilation of ventricles due to atrophy of brain mass

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Alzheimer pts. develop

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neuritic plaque

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Neuritic Plaque has

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A-Beta-Amyloid (extracellular)

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Neuritic Plaque entraps

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neuritic processes

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in cerebral amyloid angiopathy

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A-Beta-Amyloid deposits around blood vessels within the brain

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cerebral amyloid angiopathy leads to the

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weakens the blood vessels --> hemorrhage

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Alzheimer is associated with

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neurofibrillary tangles

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within the neurofibrillary tangles there is a protein called

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TAU (hyperphosphorylated)

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TAU is

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micro-tubule associated protein - helps to organize micro-tubules

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In Alzheimer TAU protein gets

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hyperphosphorylated - can function properly

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Diagnosis of Alzheimer is

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clinical and pathological (autopsy)

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Vascular dementia is a

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problem with blood vessels - multifocal infarction and injury

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Vascular dementia is due to

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HTN, atherosclerosis, vasculitis

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Vascular dementia is the second

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M.C. cause of dementia in elderly

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Vascular dementia is also asso. with

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moderate global cerebral ischemia

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Pick disease

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Degenerative disease of FRONTAL (behavior) and TEMPORAL (language) cortex

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Pick disease microscopic findings

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round aggregates of tau protein in neurons of cortex

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MPTP can result into

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Parkinson Disease

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Parkinson Disease is common disorder related to

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aging

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key finding within the neurons of sabstantia nigra

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round esosinphilic inclusions (lewy body)

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lewy body is composed of (hall mark of PD)

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Alpha-synuclein **

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dementia is seen

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in late stages of Parkinson disease

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early dementia with Parkinson like features is asso. with

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lewy body dementia (cortical lewy body)

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Parkinson Disease

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degenerative disease of basal ganglia

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Huntington disease*

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degenerative disease of basal ganglia*

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Huntington disease

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degeneration of GABAergic neurons in caudate nucleus of basal ganglia --> loss of inhibition of neuron firing

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GABA is a

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inhibitory NT

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Huntington disease is a autosomal

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Dominant

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Huntington disease due to

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expanded trinucleotide repeat (CAG) in huntingtin gene

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Huntington disease show

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anticipation pattern. child anticipates to get the disease earlier than his father

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Anticipation (Huntington disease ) is due to

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further expansion occurs during spermatogenesis

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Chorea (Huntington disease )

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rapid involuntary contraction of muscles

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Athetosis

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slow involuntary movement of fingers

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Huntington disease can lead to

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dementia and depression

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common cause of death in Huntington disease is

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Suicide

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Normal pressure Hydrocephalus is possible cause of

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dementia in adults

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Normal pressure Hydrocephalus triads is

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urinary incontinence, gait instability, and dementia (wet, wacky, wobbly)

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Rx of Normal pressure Hydrocephalus is

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LP improves symptoms, shunts from ventricles into peritoneum (VP shunt)

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stretching of chorona radiata leads to

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Normal pressure hyrocephalus triads (wet, wacky, wobbly)

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Spongiform encephalopathy

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degenerative ds. due to accumulation of prion protein

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pathologic protein in Spongiform encephalopathy is

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Beta pleated conformation

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Beta pleated conformation (protein) in Spongiform encephalopathy converts normal protein into

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additional Beta-pleated (pathologic) proteins

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Beta pleated conformation (protein)

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is not degradable

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accumulation of Beta pleated conformation (protein) in Spongiform encephalopathy leads to

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neuron and glial cell damage

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hallmark for Spongiform encephalopathy is

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intracellular vacuoles

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how do we get Beta pleated conformation (protein) in Spongiform encephalopathy

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Sporadic
inherited
transmitted

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M.C. form of Spongiform encephalopathy is

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CJD (usually sporadic)

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CJD hallmark is

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rapid dementia (weeks to months) - pts. dying < 1

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CJD is also asso. with

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ataxia and startle myoclonus (contraction of muscles with minimal stimuli)

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in CJD, EEG shows

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spike wave complexes

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variant CJD occurs in

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young pts.

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variant CJD is due to

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exposure to bovine spongiform encephalopathy (mad cow disease) - meat

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variant CJD presents as

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familial fetal insomnia

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familial fetal insomnia

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inherited form of prion disease,

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familial fetal insomnia characterized by

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insomnia and exaggerated startle response