68. dementia and neurodegenerative disorders, demyelinating diseases, prion disease Flashcards
68-1. degenerative disease and dementias
subtypes of degenerative disorder (5)
alzheimer disease pick disease parkinson disease huntington disease amyotrophic lateral sclerosis
68-1. degenerative disease and dementias
clinical feature of alzheimer disease (5)
slow onset memory loss progressive disorientation loss of learned motor skill and language change in behavior and personality patient become mute and bedridden
68-1. degenerative disease and dementias
pathogenesis of alzheimer disease
accumulation of beta amyloid
beta amyloid is produced by amyloid precursor protein (APP), undergoes beta cleavage
68-1. degenerative disease and dementias
related gene of alzheimer disease
apolipoprotein E4
68-1. degenerative disease and dementias
gross morphology of alzheimer disease
cerebral atrophy : narrow gyri, widening sulci, dilation of ventricle
68-1. degenerative disease and dementias
characteristics of pick disease
degeneration of frontal and temporal cortex
behavioral and language symptoms arise early
68-1. degenerative disease and dementias
microscopic morphology of pick disease
round aggregate of tau protein (pick body) in neuron of cortex
68-1. degenerative disease and dementias
affecting region of parkinson disease
dopaminergic neuron of substantia nigra
68-1. degenerative disease and dementias
clinical features of parkinson disease (4)
tremor
rigidity
akinesia/bradykinesia
postural instability
68-1. degenerative disease and dementias
microscopic morphology of parkinson disease (2)
loss of pigmented neuron in substantia nigra
lewy body formation on affected neuron
68-1. degenerative disease and dementias
affected neuron of huntington disease
GABAergic neuron in caudate nucleus of basal ganglia
68-1. degenerative disease and dementias
genetic predisposition of huntington disease
autosomal dominant disorder (chromosome 4), expanded trinucleotide repeats (CAG) in huntington gene
68-1. degenerative disease and dementias
clinical features of huntington disease (3)
onset on 40s
early onset with motor symptom (twitching)
progression to forgetfulness and depression
68-1. degenerative disease and dementias
characteristics of amyotrophic lateral sclerosis (ALS)
degeneration of motor neurons in the ventral horn of spinal cord
68-1. degenerative disease and dementias
etiology of amyotrophic lateral sclerosis
defect on chromosome 21, SOD1 gene
68-1. degenerative disease and dementias
clinical features of amyotrohpic lateral sclerosis (4)
onset on 50s
early symptoms include asymmetric weakness of hand
progression with muscle strength and mass diminish with involuntary contract
eventually involve respiratory muscle, cause respiratory failure and recurrent infection
68-2. prion disease
name of prion disease
spongiform encephalopathy : degenerative disease due to prion protein
68-2. prion disease
types of spongiform encephalopathy (2)
Creutzfeldt Jakob disease (CJD)
variant Creutzfeldt Jakob disease (vCJD)
68-2. prion disease
pathogenesis of spongiform encephalopathy
conversion of normal prion (PrPc) to beta pleated conformation (PrPsc)
- conversion can be sporadic, familial and transmitted
68-2. prion disease
characteristics of CJD
rapidly progressive dementia associated with ataxia
68-2. prion disease
characteristics of vCJD
slow progression of dementia, after exposure to bovine spongiform encephalopathy
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
subtypes of primary disease of myelin (2)
demyelinating disease : conditions that damage to myelin
dysmyelinating disease : malformed and defect myelin
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
subtypes of demyelinating disease (4)
multiple sclerosis
progressive multifocal leukoencephalopathy
acute disseminated encephalomyelitis
central pontine myelinolysis
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
etiology of multiple sclerosis
autoimmune destruction of myelin (HLA-DR2)
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
epidemiology of multiple sclerosis
predominant in 20-40 years old women
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
clinical features of multiple sclerosis
relapsing, remitting symptoms of visual impairment on one eye ataxia motor and sensory impairment change in cognitive function
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
etiology of progressive multifocal leukoencephalopathy
JC virus infection
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
clinicals of progressive multifocal leukoencephalopathy
rapidly progressive visual loss, weakness, dementia
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
etiology of acute disseminated encephalomyelitis (3)
bacterial infection
viral infection
vaccine for measles, mumps, rubella
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
pathomechanism of acute disseminated encephalomyelitis
cross reaction of pathogen antigen with myelin antigen
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
clinicals of acute disseminated encephalomyelitis
develop 1-2 weeks after infection
rapidly progress symptoms (headache, lethargy, coma)
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
etiology central pontine myelinolysis
rapid correction of hyponatermia
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
clinical signs of central pontine myelinolysis
acute bilateral paralysis
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
etiology of leukodystrophies
inherited mutation of enzymes
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
metabolic disorders affect to brain (3)
hypoglycemia
hyperglycemia
hepatic encephalopathy
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
brain change related to hypoglycemia (2)
injury on hippocampus
spared purkinje cell of cerebellum
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
cause of hyperglycemia
DM
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
consequences of hyperglycemia (3)
confusion
stupor
coma
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
pathomechanism of hepatic encephalopathy
liver fails to clear ammonia, cause change in brain function
68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain
types of neurotoxic agents (7)
metal (arsenic, mercury) industrial chemicals methanol ethanol environmental pollutant chemotherapeutic agent ionizing radiation
