Glossary of Block VIII, Week I
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- the cytoplasmic granularity found in neurons is called?
- Nissl substance
- what type of stains are used for the best visualizations of neurons?
- silver-based stains
("Beil" stain, Bodian stain)
- T/F: neurons have no regenerative capacity
- under what conditions would a neuron undergo chromatolysis?
- when the axon is severed
(chromatolysis = swelling of cell body)
- a neurological rabies infection would have what microscopic neuronal manifestation?
- inclusion bodies
- describe the microscopic characteristics of ischemic change in a neuron
- pyknotic nucleus
- what occurs in chromatolysis and what are the microscopic characteristics of it?
- chromatolysis - severing of axon
see an eccentric nucleus, ground glass cytoplasm
- function of astrocytes?
- transport substances from blood to CNS
- useful stains that help us visualize astrocytes? (3)
- MC reaction of astrocytes to injury?
- proliferation, cause gliosis (glial scar)
- what is the "gemistocytic change" sometimes seen in neuronal injury?
- astrocytes that are plump, round and have an abundant eosinophilic cytoplasm
- when are Alzheimer's type II astrocytes seen and what do they look like?
- seen in hepatic failure
swollen, bizarre nuclei
- when are Rosenthal fibers seen?
- in chronic gliosis
- stain used to best see myelin?
- luxol-fast blue
- reaction of oligodendrocytes to injury?
- most likely cell death with resulting myelin loss
- where are ependymal cells found?
- line the ventricular cavities and the neural tube
- function of choroid plexus?
- produces CSF by ultrafiltration
- changes seen in the ependymal lining when there is underlying astrocytic proliferation going on?
- ependymal granulations
- describe the ependymal cell
- ciliated cuboidal epithelial cell
- function of microglial cells?
- specialized CNS macrophages
- what is the stimulus for transformation of microglial cells to "rod cells?"
- injury causes proliferation of microglial cells with elongation of nuclei = rod cells
- what reaction does a microglial cell have to a virally infected neuron?
- attacks it
- how does a microglial cell become a foamy macrophage?
- it scavenges lipid in infarcted and demyelinated areas
- what stain is used to best visualize lipid-laden microglial cells?
- oil-red-O stain
(orange is the lipid scavenged by the macrophages)
- what are the four horizontal levels of a neuropathological lesion?
- 1. supratentorial
2. posterior fossa
- what are the 4 basic differentiations we should make in the clinic to evaluate a neurological problem?
- 1. anatomic level of lesion
2. focal or diffuse
3. progressive or nonprogressive
4. acute, subacute, chronic
- most acute neuropathological incidents are of what etiology?
- give an example of a focal, acute neuropathological incident
- most subacute neuropathological incidents are of what etiology?
- give an example of an acute, diffuse, neuropathological incident
- subarachnoid hemorrhage
- give an example of a focal, subacute neuropathological etiology
- give an example of a diffuse, subacute neuropathological etiology
- meningitis or encephalitis
- what is the most likely etiology of chronic, focal neuropathological symptoms?
- what is the most likely etiology of chronic, diffuse neuropathological symptoms?
- what are the proposed mechanisms behind demyelinating disease? (3)
- 1. inflammatory (immune, autoimmune)
- what type of demyelinating disease is characterized by a relapsing and remitting course?
- is MS characterized by focal or diffuse deficits?
(distinct demyelinating lesions in the CNS)
- when is the typical onset of MS?
- 3rd-4th decade
- F:M of MS?
- F:M = 2:1
- what pathways are impaired in MS?
- two types of MS?
- 1. classic (Charcot) form
- most common, classic relapsing and remitting course with gradual overall decline
2. acute (Marburg) form
- young adults, rapidly progressive and unrelenting course
- MS is most apparent in what portion of the brain and spinal cord?
- white matter
(in MS you see gray matter plaques in white matter areas)
- what is a shadow plaque?
- an older MS plaque that shows limited remyelination
- microscopic characteristics of the axons in MS?
- axons are relatively spared so they are most likely "normal" looking
- where in the brain is it common for MS plaques to appear?
- around the ventricles (esp. the lateral horns of the lateral ventricles)
- describe the borders of an MS plaque
- well circumscribed borders
- describe the immune pathogenesis of MS that Dr. Weiland gave.
- * oligodendrocyte injury mediated by cytotoxic CD4+ T-cells
* see oligoclonal immunoglobulins in the CSF
* lymphocytic infiltrates in the plaques
- is there a genetic link for MS?
which HLA is MS associated with?
- * increased risk in 1st degree relatives, increased in twins
* associated w/HLA-DR2
- geographically, where is MS more common?
- temperate zones
(risk conferred by 18 yrs of age)
- which demyelinating disease is characterized by prominent visual and spinal cord involvement and NO cerebral or cerebellar involvement?
- neuromyelitis optica (Devic's disease)
- how can acute disseminated encephalomyelitis (ADEM) be distinguished from MS?
- ADEM is a diffuse demyelinating autoimmune disease, MS is a focal demyelinating autoimmune disease
- common etiologies of ADEM?
- * often occurs after childhood viral illness (measles, rubella, VZV)
* vaccinations (rabies, mumps, rubella)
- microscopic characteristics of ADEM?
- perivascular inflammatory infiltrates with demyelination (seen in entire brain)
- prognosis of ADEM?
- variable, but most mild cases completely recover
- describe acute hemorrhagic leukoencephalitis (AHL)
- "hemorrhage with demyelination"
(a hyperacute form of ADEM with hemorrhage - often fatal within days)
- micro characteristics of acute hemorrhagic leukoencephalitis? (4)
- * perivascular demyelination
* inflammatory infiltrates
* fibrinoid necrosis
* punctate hemorrhages
- symmetric non-inflammatory demyelination of the pons is known as?
- central pontine myelinolysis
- possible etiology of central pontine myelinolysis?
- * hyperosmolality due to rapid overcorrection of hyponatremia
- clinical features of central pontine myelinolysis?
- ranges from asymptomatic to flaccid quadraplegia to stupor or coma.
- leukodystrophies are hereditary disorders of?
- myelin production or metabolism
- metachromatic leukodystrophy is a defect in what enzyme? result?
- defect in arylsulfatase A
*results in accumulation of sulfatides in oligodendrocytes
- Krabbe disease is a defiency in?
- Adrenoleukodystrophy results in?
- high levels of very long chain fatty acids
- inheritance pattern of leukodystrophies?
- inheritance pattern of neuronal storage diseases?
- Tay-Sachs is a deficiency in?
- hexosaminidase A
*result is accumulation of gangliosides
- Hurler syndrome is a deficiency in?
* result is accumulation of mucopolysaccharides
- Gaucher's disease is a deficiency in?
- Neimann-Pick syndrome is a deficiency in?
- PKU is a deficiency in?
- phenylalanine hydroxylase (get accumulation of phenylalanine=neurotoxin)
- result of excess phenylalanine?
- irreversible mental retardation, seizures
- describe cretinism
- congenital hypothyroidism
*see growth retardation, severe MR, deaf, mutism, motor spasticity/rigidity
- neurological symptoms of Wilson's disease?
- dystonia, tremor, ataxia
- what is the location of CNS injury in wilson's disease?
- basal ganglia (esp. putamen, caudate nucleus)
- define spina bifida occulta
- vertebral arch defect
- describe a meningocele
- cyst like protrusion of the meninges
- describe a meningomyelocele
- cyst like protrusion of the meninges with nerve roots and spinal cord elements. (neurological problems are common)
- describe a rachischisis
- complete failure of closure of the caudal end of the neuropore
- describe anencephaly
- failed closure of the anterior neuropore
- describe Wernicke's encephalopathy
- acute deficiency of thiamine (B1) - manifests as confusion, opthalmoplegia, altered temperature regulation. FATAL if untreated.
- describe Korsakoff's syndrome
- chronic deficiency of B1 (thiamine) - see antegrade amnesia, confabulation
- in which thiamine deficiency are petechial lesions seen in the mammillary bodies?
- cardiovascular manifestations of a thiamine deficiency?
- Beriberi heart disease
- neurological manifestations of a B12 deficiency?
(atrophy of posterior columns - position sense)
*sensory > motor deficits, dysesthesias common
- which portion of the cerebellum is commonly atrophied in chronic alcoholism?
- cerebellar vermis
- besides atrophy of the cerebellar vermis, what other CNS changes are seen in relation to chronic alcoholism?
- 1. cortical atrophy
2. hydrocephalus-ex-vacuo (ventricles look larger due to cortical atrophy)
- Parkinson's disease generally impairs voluntary or involuntary functions?
- involuntary functions
- what type of tremor is seen in PD?
(may be absent in 1/4th of all PD patients)
- avg. age of onset of PD?
- PD involves a loss of?
- DA neurons in the substantia nigra and locus ceruleus
- what are Lewy bodies composed of?
- what characterizes parkinsonism-dementia complex of Guam?
- parkinsonism w/ progressive dementia, death in 5-10 yrs
- how does progressive supranuclear palsy (PSP) differ from PD?
- PSP - no Lewy bodies, neuronal loss is in brainstem, basal ganglia, cerebellum
- some possible etiologies of parkinsonism? (4)
- 1. post encephalitic
2. drug induced
3. toxin related (MPTP)
4. repeated head trauma
- inheritance pattern of HD?
- describe the progression of HD
- early cognitive and emotional impairment
* then, extrapyramidal symptoms (choreoathetosis)
- gross pathology of a brain with HD?
- atrophy of frontal cortex, striatum
* secondary lateral ventricle enlargement
- HD involves a mutation in a gene on which chromosome?
- chromosome 4
- MC genetic mutation type seen in HD?
- trinucleotide repeat expansion
- ALS involves which neurons?
- upper and lower MOTOR neurons
- describe the clinical course of ALS
- * progressive muscular weakness
* atrophy, cramps, fasciculations
* motor speech disturbance
* intellect intact
* death usually w/i 3-5 yrs
- avg. age of onset of ALS?
- 40-60 yrs
- gender differences in ALS?
- familial ALS (5-10%) is related to a mutation in?
- superoxide dismutase 1 gene (SOD1)
- nervous system areas that atrophy in ALS?
- * cortical motor neurons
* motor nuclei of brain stem
* loss of axons in LCST
* anterior horn spinal cord neurons
* anterior spinal nerve roots
- which degenerative disease accounts for >50% of dementia
- characteristics of AD?
- progressive dementia with memory and cognitive impairment
- risk factors for AD? (3)
- 1. age
2. family history
3. female gender
- what genetic disorder does AD have a strong connection to?
- Down's syndrome (trisomy 21)
- Four etiological factors that may contribute to AD?
- 1. amyloid beta
2. amyloid precursor protein
3. Apoprotein E e4
- function of presenilins?
- create amyloid protein from amyloid precursor protein (APP)
- mutations in presenilins result in?
- most cases of familial AD
- which type of apolipoprotein has the lowest risk of AD?
- ApoE e2 or 3
- gross characteristics of AD?
- *cortical atrophy (esp. frontal-parietal, temporal)
*atrophy of gyri, widening of sulci
* hydrocephalus ex vacuo
- microscopic features of AD?
- *neurofibrillary tangles
- in AD, neurofibrillary tangles are primarily made up of what protein?
- tau (microtubule associated protein)
- in AD, what is found at the core of neuritic plaques?
- in AD, where is granulovascular degeneration found?
- in the cytoplasm of hippocampal neurons
- in AD, what are Hirano bodies?
- eosinophilic bodies by hippocampal neurons (seen alongside granulovascular degeneration)
- describe diffuse Lewy body disease
- dementia with Lewy bodies, may have accompanying akinetic rigidity syndrome (Parkinson like)
- gross characteristics of Lewy body dementia?
- not much, relatively little atrophy
- gross features of Pick's disease?
- *marked cerebral atrophy limited to frontal and temporal lobes
- what is a Pick body?
neurons with eosinophilic cytoplasmic inclusions
- which stain is used to see Pick bodies?
- silver stain
- 50% of dementia can be classified as?
- dementia in which a personality change or language disorder is seen is called?
- frontotemporal dementia
(10% of all dementias)
- what must be present in order to make the diagnosis of dementia?
- acquired memory impairment WITH impairment in other domains (cognitive or social).
- how common is dementia?
- 5-10% of community dwelling adults over the age of 65
50% of those over the age of 80.
70% of all nursing home pts.
- how can a person have cognitive impairment but not meet the definition of dementia?
- poor memory performance but NORMAL general cognition and ADLs
- what are some "reversible" causes of dementia? (7)
- 1. depression
3. alcohol use
5. chronic menigitis
7. normal pressure hydrocephalus
- describe progressive pseudodementia
- paitents describe cognitive complaints, have difficulty encoding memories.
DURATION OF DEMENTIA IS SHORTER, depression common, antidepressant trial often warranted
- List 5 medications commonly responsible for cognitive decline
- 1. pain meds
2. anticholinergics (amitryptiline, nortryptaline)
5. sedative-hypnotics (sleeping pills)
- how common is true inherited AD?
- mean survival of an AD patient?
- 8.1 years
- MC cause of death in an AD patient?
- physical manifestations of late AD?
- increased tone,
- psychiatric manifestations of AD?
- in which type of dementia are hallucinations very common?
- Lewy body dementia
- 6 risk factors for AD?
- 1. Apo-E4-Allele
2. Family history
5. head injury
6. lower intelligence/smaller head size
- two pharmacologic treatments of AD?
- 1. acetylcholinesterase inhibitors
2. NMDA antagonists (Namenda)
- what is the second leading cause of dementia?
- Vascular dementia
(single stroke, multi-infarct, subcortical arteriosclerotic encephalopathy)
- what is it called when vascular dementia is mixed with Alzheimer's pathology?
- mixed dementia
- describe the classic onset of vascular dementia
- sudden onset or stepwise deterioration
- how can vascular dementia be diagnosed?
- abnormal imaging studies
(also see focal neurological signs and stroke risk factors)
- what types of strokes cause dementia?
- lacunar strokes
strategically placed "normal" stroke
- what does a white matter change indicate?
- many things, SOMETIMES can indicate dementia and/or AD.
- what is the 3rd cause of dementia?
- subcortical arteriosclerotic encephalopathy (SAE), aka. Binswanger's disease
- where is demyelination and gliosis seen in subcortical arteriosclerotic encephalopathy?
- 3 psychological findings in SAE?
- psychomotor retardation
abula (lack of initiative)
- 5 physical findings in SAE?
- 1. pseudobulbar brainstem signs (trouble swallowing)
3. upgoing toes
4. gait disturbance
- treatment for multi-stroke dementia? (4)
*supportive, risk factor management
- avg. age of onset of frontotemporal dementias?
- EARLY <65
- three distinct pathologies of frontotemporal dementia?
- 1. Pick's disease
2. Frontal lobe degeneration
3. progressive subcortical gliosis
- what is semantic dementia?
- fluent empty speech "verbal diarrhea"
(loss of knowledge about items, trouble with naming, word comprehension. grammar and pronunciation remains intact)
- what is progressive nonfluent aphasia?
- nonfluent speech, progresses to muteness
- describe the progression of CJD
- subacute dementia with myoclonus, rapidly progressive
*80% dead in 1 yr, 50% dead w/i 5 mo.
- avg age of onset of CJD?
- 50-75 yrs
- pathogenesis of CJD?
- prion protein (PRP - normally an a-helix) changes conformation to a beta-pleated sheet (bad form: protease resistant). this form accumulates in neuronal tissue, causes neuronal death.
- 7 classical signs/symptoms of Parkinsonism?
- 1. rigidity
4. stooped posture
6. changes in handwriting
7. sleep disorders
- T/F: DBS helps parkinsonism
DBS only helps PD, not all cases of Parkinsonism
- PD makes up for what proportion of parkinsonism cases?
- 5 other causes of parkinsonism?
- 1. PSP (progressive supranuclear palsy)
2. MSA (Multi-system atrophy)
3. CBD (Corticobasal degeneration)
4. LBD (Lewy body disease)
- which two races have the lowest incidence of PD?
- voice changes seen in PD?
(soft, tremulous voice)
- what clues suggest a diagnosis OTHER than PD? (7)
- 1. lack of tremor
2. prominent/early dementia
3. autonomic dysfunction
4. early instability/falls
5. eye movement difficulties
6. lack of response to levodopa
7. early hallucinations
- how many DA neurons must be lost before motor symptoms appear in PD and a diagnosis is made?
- how long before the onset of PD does loss of the DA neurons begin?
- 20-30 yrs prior
- early symptoms of PD? (5)
- 1. anxiety, depression
2. changes in sense of smell
4. postural changes
5. sleep disorder/weight loss
- in the on-off phenomenon, what does the off phase correspond to?
- no DA stimulation
- in a PD patient, what are the leading cognitive risk factors for nursing home placement?
- delerium and dementia
- three stages of PD?
- 1. honeymoon period
2. battle area
3. late phase
- which type of PD is thought to have a better prognosis?
- tremor predominant PD
- which 2 types of PD are thought to have a prolonged course?
- unilateral disease
- gold standard treatment of PD?
- Sinemet (carbidopa/levodopa)
- MOA of carbidopa/levadopa?
- *levadopa causes peripheral side effects (toxic)
*carbidopa counteracts these peripheral side effects (MAO inhibitor, blocks levodopa breakdown into toxins in periophery)
- how can nausea be treated in the PD patient on carbidopa/levadopa?
- increase carbidopa
take with meals
- 3 dopamine agonists used to treat PD?
- 1. Mirapex
- neuroprotectant used to prevent progression of PD?
- role of Entacapone (Comptan) in PD treatment?
- extends 1/2 life of levadopa
- 3 anticholinergics used to treat PD?
- 1. Congentin
- MOA of Amantadine?
- NMDA antagonist
- in DBS for PD, where are the electrodes placed (1 of 3 locations)
- 1. globus pallidus
2. subthalamic nucleus
- 2 positive outcomes of surgery for PD?
- 1. improves time spent in "on" state
2. decreases need for meds
- risks/negative outcomes of surgery for PD?
- *risk of bleed, stroke, infx
*risk of masking dementia, dysarthria
*poor placement or equipment failure
- characteristics of Lewy Body Dementia (DLBD) when it comes to meds?
- very sensitive to meds & their adverse effects - treat gingerly
- in which sex is DLBD more common?
- MC of death in DLBD?
- aspiration pneumonia
- how long from symptoms to death in DLBD?
- 6.4 yrs
- where are the Lewy Bodies found in DLBD?
- dopaminergic neurons (diffusely located)
- treatment of DLBD?
- 1. low dose carbidopa/levadopa
2. low dose antipsychotic for hallucinations (quetiapine or clozapine)
3. AChE inhibitor for cognition, behavior problems
4. Klonopin for periodic limb movements of sleep
- hallmark symptom of progressive supranuclear palsy (PSP)?
- supranuclear opthalmoplegia
- does PSP respond to levadopa?
- avg age of onset of PSP?
- regarding PSP: on average, how long after diagnosis does death occur?
- 6 yrs
- Treatment for PSP? (3)
- 1. Sinemet - limited motor response
2. SSRIs (depression/anxiety, pseudobulbar effect)
3. AChEIs for dementia
- 3 variants of multiple system atrophy (MSA)?
- 1. Shy Drager syndrome (MSA-A)
2. striatonigral degeneration (MSA-B)
3. OPCA (MSA-C)
- characteristics of MSA-A (Shy-Drager syndrome)?
- dysautonomia (faining, cold hands, mottling, incontinence)
- characteristics of striatonigral degeneration (MSA-B)?
- Parkinsonian symptoms
- characteristics of OPCA (MSA-C)
- treatment for MSA?
- PT/Speech Therapy
early response to levadopa
fludrocortisone or midodrine for BP support
- four causes of acquired ataxias?
- 1. alcohol
- two inherited ataxias?
- 1. Friedreich's ataxia (AR)
2. Spinocerebellar ataxia (AD and sporatic)
- what is the rarest Parkinsonian condition?
- Corticobasal degeneration
- age of onset of corticobasal degeneration?
- 60's or later
- s/s of corticobasal degeneration?
- "alien limb phenomenon"
asymmetric parkinsonism and limb dystonia WITH UMN signs and late dementia
- "lower-half" parkinsonism with prominent gait disturbance, subcortical dementia, AND a history of stroke is called?
- vascular parkinsonism
- what is the cause of dementia pugulistica?
- repeated head trauma
- s/s of dementia pugilistica?
- *attentional and memory deficits
- is there any genetic group that is at higher risk of dementia pugilistica?
- ApoE4 carriers
- symptomatic, communicating, adult onset hydrocephalus is known as?
- normal pressure hydrocephalus
- what is the triad seen in normal pressure hydrocephalus?
- 1. subcortical dementia (subacute)
2. frontal "magnetic" gait disturbance
3. urinary incontinence
- what is frequently seen in normal pressure hydrocephalus?
(with bradykinesia, rigidity, limb tremors)
- causes of normal pressure hydrocephalus? (3)
- 1. remote trauma, infection
2. subarachnoid hemorrhage
- of the triad seen in normal pressure hydrocephalus, which symptom is earliest?
- gait disturbance (apraxic gait - "stuck to floor" feeling)
- MRI findings in NPH?
- 1. ventriculomegaly
2. thinning of corpus callosum
3. no CSF flow
- shunting is what is normally done to treat normal pressure hydrocephalus. when is it less effective?
- if small vessel disease is present
- success rates/results of shunting in NPH?
- 1/3 have marked improvement
1/4 have mild improvement
1/4 have moderate complications
1/4 have shunt malfunction
- describe an essential tremor
- tremor that is most promiment with intention.
*often distal in the limb
- which type of tremor, resting or essential, is responsive to alcohol?
- what am I?
hyperkinetic movements with irregular, unpredictable, brief, jerky movements that move from one part of the body to another...
- what is the difference between chorea and choreoathetosis?
- choreoathetosis is slower than the characteristically brisk chorea
- what am I?
inherited, progressive disorder manifested by chorea and other movements, behavior disorder and dementia
- inheritence pattern of HD?
- when do s/s of HD appear?
- in the 4th or 5th decade
- where is the HD gene and what happens genetically in HD?
- chromosome 4
HD is the result of an expanded CAG trinucleotide sequence
- genetic correlation between severity of the disease?
- increased number of repeats = increased severity of disease
- under which type of inheritance is the number of repeats in HD more likely to increase?
- father to child
- what type of reflexes would most likely be present in a HD patient?
- brisk reflexes
- what part of the brain atrophies in HD?
- what is the life expectancy after an HD diagnosis?
- 10-20 yrs
- MCC of death in HD?
- signifance of earlier onset HD as compared with "normal" onset?
- earlier onset has faster progression and seizures are more likely
- the condition of chronic dopamine receptor blockade is known as?
- tardive dyskinesia
- MC location of tardive dyskinesia?
- around the face
(can occur in the body as well)
- what disorder am I?
disorder of sustained muscle contraction which creates abnormal posture?
- generalized dystonia is often inherited. it is due to a mutation of?
- the DYT1 gene
- any treatment for dystonia?
SOME are dopamine responsive, others are not
- which type of dystonia often starts late in adult life?
- focal or segmental
- which sex is tics more common in?
- age of onset of tics?
- what is Tourette's syndrome?
- childhood onset of motor and vocal tics
- inheritance pattern of Tourette's syndrome (TS)?
- TS is commonly seen with what other conduct disorders? (2)
- which disorder am I?
sudden, brief, shock-like involuntary movements
- myoclonus is seen in what brain diseases/injuries? (3)
- 1. hypoxic brain injury
2. late stage neurodegenerative diseases
3. early stage CJD
- name three metabolic movement disorders with associated dementia
- 1. Wilsons disease
2. Idiopathic basal ganglia calcification
- what is Hallevorden-Spatz disease?
- early onset dementia with parkinsonism - iron deposition in basal ganglia
- idiopathic basal ganglia calcification presents as?
- dementia with parkinsonism
- inheritance pattern of Wilsons disease?
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