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Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
Cns degeneration, demyelination and tumors
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Cns degeneration, demyelination and tumors

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  • 1.
    • Demyelinating Diseases
    • Selective loss of myelin
    • a) MS is demyelinating, whereas
    • b) necrosis, etc. is not demyelinating
    • Leukodystrophies reflect inherited
    • disturbances in formation and
    • preservation of myelin
    • a) metachromatic leukodystrophy
    • i) most common leukodystrophy
    • ii) autosomal recessive disorder of myelin metabolism
    www.freelivedoctor.com
  • 2. - characterized by  of a cerebroside (galactosyl sulfatide) in white matter of brain and PNS iii) predominates in infancy iv) lethal within several years b) Krabbe disease i) fatal, early months of life ii) deficiency of galactocerbroside ß- galactosidase iii) autosomal recessive www.freelivedoctor.com
  • 3. iv) almost complete loss of oligodendroglia and myelin v) presence of gobloid cells found around blood vessels c) adrenoleukodystrophy (ALD) i) x-linked inherited disorder of adrenal cortex and demyelination of nervous system ii) children 3-10 yrs iii)  levels of very long chain FA in tissues and body fluids www.freelivedoctor.com
  • 4. iv) enzyme mutation - impairs capacity to degrade VLCFA’s v) more severe demyelination in cortical white matter - parieto-occipital regions d) Alexander disease i) rare neurological disease ii) infants and children iii) loss of myelin in brain iv)  accumulation of irregular, extracellular fibers (Rosenthal fibers) www.freelivedoctor.com
  • 5. v) psychomotor retardation, progressive dementia, paralysis  death vi) mutation of gene encoding GFAP  Rosenthal fibers  vii) deposited around blood vessels Multiple Sclerosis (MS) a) chronic demyelinating disease b) most common chronic CNS disease of young adults in USA www.freelivedoctor.com
  • 6. c) affects sensory and motor functions d) acquired disease, mean age ~ 30yrs i) women 2:1 e) etiology remains obscure i) genetic predisposition f) colder climates  risk g) immune factors i) chronic MS  perivascular lymphocytes, macrophages and CD4+ and CD8+ T cells www.freelivedoctor.com
  • 7. h) infectious agents i) wide variety of viruses - mumps, rubella, herpes simplex and measels (via vaccination) I) demyelinated plaque is hallmark of MS i) usually in white matter ii) preference for optic nerves, chiasm www.freelivedoctor.com
  • 8. iii) neurons are spared, while axons degenerate when next to plaque! iv) MS has “focal” areas of injury v) demyelination is complete when in presence of plaque vi) old MS plaque exhibit gliosis (“scar”) impairs structural integrity of axons www.freelivedoctor.com
  • 9. J) clinical i) onset 30-40 yrs ii) PNS are uniformly spared iii) usually begins with symptoms in optic nerves, brainstem or spinal cord (loss of vision in one eye usually presenting complaint) iv) lesions in spinal cord  leg weakness or numbness www.freelivedoctor.com
  • 10.
    • v) disease usually follows chronic relapsing and remitting course  develop permanent lesions
    • vi) death usually from respiratory paralysis or UTI while in terminal coma
    • vii) survival 20-30 yrs following initial symptoms
    • MS variants
    • a) neuromyelitis optica (Devic disease)
    • i) Asians
    www.freelivedoctor.com
  • 11. ii) present as bilateral optic neuritis and spinal cord involvement iii) lesions similar to MS but more destructive - grey matter involvement b) Acute MS (Marburg form) i) young individuals ii) fulminant course over several months www.freelivedoctor.com
  • 12.
    • Acute disseminated encephalomyelitis
    • a) follow either
    • i) viral infection or
    • ii) viral immunization
    • b) symptoms develop 1-2 weeks following (i or ii above)
    • c) clinical
    • i) headache
    • ii) lethargy or
    • iii) coma
    • iv) these occur rather than focal findings
    www.freelivedoctor.com
  • 13.
    • v) symptoms progress rapidly  fatal in 20% and remaining cases  complete recovery
    • Acute necrotizing hemorrhagic
    • encephalomyelitis
    • a) fulminant syndrome of CNS demyelination
    • b) usually preceded by upper respiratory infection
    • i) mycoplasma pneumoniae
    • ii) most times of indeterminate cause
    www.freelivedoctor.com
  • 14.
    • iii) highly fatal
    • Central pontine myelinolysis
    • a) loss of myelin
    • b) preservation of neurons and axons
    • c) believed to be caused by rapid correction of hyponatremia
    • i) also to extreme hyperosmolarity or
    • ii) other metabolic imbalances
    • d) clinical
    • i) rapidly developing quadriplegia
    • ii) lesion in basis pontis
    www.freelivedoctor.com
  • 15.
    • iii) can occur during setting of:
    • - alcoholism
    • - severe electrolyte/osmolar imbalances
    • - orthopic liver transplantation
    • Marchiafava – Bignami disease
    • a) rare disorder of myelin
    • i) corpus callosum and
    • ii) anterior commissure
    www.freelivedoctor.com
  • 16.
    • Degenerative Diseases
    • Parkinson Disease
    • a) Common movement disorder
    • b) Characterized by loss of neurons (substantia nigra)
    • i) accumulation of Lewy bodies
    • c) Tremors at rest
    • d) Muscle rigidity
    • e) Expressionless
    www.freelivedoctor.com
  • 17.
    • Epidemiology
    • a) 6-8 decades
    • b) more than 2% in North America develop disease
    • c) men more than women
    • d) most cases are sporadic
    • i) missense mutations cause rare autosomal dominant e) most are idiopathic, exceptions
    • i) induced following viral encephalitis
    • - Von Economo encephalitis
    www.freelivedoctor.com
  • 18. ii) toxin intake - MPTP (1-methyl-4-phenyl- 1,2,3,6-tetrahydropyridine) f) substantia nigra relays information to basal ganglia through Dopaminergic synapses i) aging  dopamine ii) exaggerated in PD iii) Lewy bodies are filamentous aggregates seen in substantia nigra - also other areas www.freelivedoctor.com
  • 19. iv) oxidative stress (of catecholamines) during melanin formation injures neurons in substantia nigra g) loss of pigmentation in substantia nigra and locus ceruleus and formation of inclusion bodies (Lewy bodies) h) clinical: i) slowness of all voluntary movement and muscle rigidity - disappears with use www.freelivedoctor.com
  • 20. www.freelivedoctor.com
  • 21. ii) coarse tremor of distal extremities - at rest iii) face is expressionless (mask-like) - reduced rate of swallowing (leading to drooling) iv)  incidence of depression/dementia (~10- 15%) www.freelivedoctor.com
  • 22.
    • v) early PD tx with L-dopa
    • - after several years becomes ineffective
    • vi) neural transplantation (dopaminergic) into striatum
    • vii) deep brain stimulation can provide relief of motor symptoms of PD
    • Multiple system atrophy
    • a) rare disorder
    • b) mimics PD
    www.freelivedoctor.com
  • 23. c) less severe changes in substantia nigra and locus ceruleus d) associated with Shy-Drager disease and olivopontocerebellar atrophy (i.e., these are known as multiple system atrophy) i) patients usually have symptoms of both diseases e) 2 principle symptoms i) PD www.freelivedoctor.com
  • 24.
    • ii) Autonomic dysfunction
      • orthostatic hypotension
    • f) when present as isolated lesion 
    • i) Shy-Drager
    • ii) Striatal degeneration
    • iii) Presentation of isolated ataxic disorder with cerebellar dysfunction  olivopontocerebellar atrophy
    www.freelivedoctor.com
  • 25.
    • Amyotrophic lateral sclerosis (ALS)
    • a) leads to profound weakness and death
    • b) affects motor neurons of brain and spinal cord
    • c) worldwide disease
    • i) 1:100,000
    • d) peaks in incidence in 5th decade
    • e) ~ 2:1 incidence in men
    • f) Guam, Papua new guinea and parts of Japan
    www.freelivedoctor.com
  • 26.
    • i) Chomoro people in Guam
      • disease is rich in tau NFT
    • now classified as “neurodegerative taupathies
    • g) familial cases
    • i) autosomal dominant (gene 21q)
      • ii) ~ 5 % of all cases
      • iii) missense mutation that codes for SOD1
    • iv) disease not due to SOD activity
    www.freelivedoctor.com
  • 27.
    • h) affects motor neurons (3 locations)
    • i) anterior horn cells of cord
    • ii) motor nuclei of brainstem
      • - hypoglossal nuclei
    • iii) upper motor neurons of cerebral cortex
    • iv) loss of large motor neurons accompanied by mild gliosis
    • - may cause inclusions  sphenoids
    www.freelivedoctor.com
  • 28.
    • v) loss of pyramidal “Betz” cells in motor cortex
    • vi) loss of myelinated fibers in lateral corticospinal tracts
    • vii) anterior nerve roots are atrophic and affected muscles are pale and shrunken
    • I) clinical
      • i) begins as weakness and wasting of muscles
      • - hand (often with painful cramps)
    www.freelivedoctor.com
  • 29.
      • - irregular rapid contractions of muscles that do not move limbs (fasciculations)
      • - progressive disease
      • - speech unintelligent
      • - respiratory weakness
      • - intellectual capacity is preserved
    • - clinical course usually does not extend beyond 10 years
    www.freelivedoctor.com
  • 30.
    • Huntinton disease
    • a) inherited autosomal dominant disease
    • b) progressive movement disorders and dementia
    • c) degeneration of striatal neurons
    • d) movement disorder chorea
    • i) jerky
    • ii) hyperkinetic
    • iii) sometimes dystonic movements
    • iv) affecting all parts of body
    www.freelivedoctor.com
  • 31. e) progressive i) course ~ 15 years f) HD gene  4p16.3 encodes a protein i) huntingtin ii) repeat mutation (“trinucleotide” repeat disorder) iii) greater the # of repeats  earlier onset of disease www.freelivedoctor.com
  • 32.
    • g) clinical
    • i) 4-5th decade at onset
    • ii) motor symptoms usually precede cognitive disorders (in ~ 50% of patients)
    • iii) movement disorders are chorioform
      • - jerky, involuntary movement of all parts of body
      • -  risk of suicide (genetic screening)
    www.freelivedoctor.com
  • 33. www.freelivedoctor.com
  • 34.
    • Spinocebellar ataxias
    • loss of neurons and neural tracts in
    • cerebellum, brainstem and spinal cord
    • a) ataxia
    • b) intention tremor
    • c) rigidity
    • d) tremor
    • e) loss of deep tendon reflexes and
    • f) vibration sense and
    • g) pain
    www.freelivedoctor.com
  • 35.
    • Friedreich ataxia
    • a) most common inherited ataxia
    • b) autosomal recessive
    • c) onset of symptoms less than 25 years
    • d) hallmark is
    • i) combined ataxia of both upper and lower limbs
    • ii) Systemic abnormalities of skeletal system
      • - scoliosis
      • - pes cavus
    www.freelivedoctor.com
  • 36.
      • - hypertrophic cardiomyopathy which commonly causes death
        • - diabetes mellitus
      • e) genetic defect
      • i) chromosome 9
      • ii) lack of “frataxin” production
      • iii) triplet expansion (GAA repeat expansion)
      • - confirms diagnosis
    www.freelivedoctor.com
  • 37.
    • DEGENERATIVE DISEASES (AD)
    • (Alzheimer Disease)
    • principle cause of “so-called” senility
    • worldwide disease
    • most common cause of dementia in aged
    • a) more than half of all cases
    • age prevalence
    • a) before age 65 years  1-2 %
    • b) after 85 years  ~ 10%
    • c) women 2:1
    • d) most cases are sporatic
    • i) familial variant is recognized
    www.freelivedoctor.com
  • 38.
    • e) 2 associations
    • i) amyloid  -protein (A  )
      • -  deposition in neuritic plaques of AD
    • - plaques in cerebral cortex
    • ii) NFT
    www.freelivedoctor.com
  • 39. Alzheimer Disease 1.- amyloid β -protein (A β ) a) evidence points to  in neuritic plaques of A β i) located in cerebral cortex ii) linked to intellectual function iii) constant feature of AD b) neurons and glial cells also accumulate A β in walls of cerebral blood vessels www.freelivedoctor.com
  • 40. 2.- Neurofibrillary tangles (NFT) a) microtubule-associated protein i) abnormal helical form which is termed “tau” b) in AD phosphorylation of tau in certain areas of brain form NFT c) mutations of tau gene on chromosome 17 causes familial dementia and parkinsonism www.freelivedoctor.com
  • 41. www.freelivedoctor.com
  • 42. www.freelivedoctor.com
  • 43. www.freelivedoctor.com
  • 44.
    • d) most cases of AD are associated with lots of LEWY bodies
    • e) genetic association
    • Pathology of AD:
    • a) during course of AD
    • i) neurons are lost
    • ii) gliosis occurs
    • iii) gyri narrow
    • iv) sulci narrow
    • v) cortical atrophy
    • - bilateral and symmetrical
    www.freelivedoctor.com
  • 45.
    • b) microscopic findings
    • i) senile plaques
    • ii) NFT
    • iii) neuron loss
    • iv) Lewy bodies and granulovacuolar degeneration
    • Clinical:
    • a) patients usually present with:
    • i) gradual loss of memory and
    • ii) cognitive function
    • iii) difficulty with language
    • iv) changes in behavior
    www.freelivedoctor.com
  • 46.
    • b) AD is progressive
    • i) previously intelligent and productive persons
    • - become demented
    • - mute
    • - incontinent
    • - bed ridden
    • - bronchopneumonia usually cause of death
    • Pick disease
    • a) loss of function
    • b) dementia
    www.freelivedoctor.com
  • 47. c) difficult to distinguish from AD d) most cases are sporadic e) occurs in mid adult life i) progress to death in 3-10 yrs f) cortical atrophy i) initially unilateral - bilateral with progression ii) localized to frontotemporal g) severe atrophy i) gyri reduced to thin edge - “knife-blade” atrophy www.freelivedoctor.com
  • 48.
    • h) inclusions contain tau and argentophilic and are referred to as “Pick bodies”
    • i) densely aggregated straight filaments
    • TUMORS
    • annual incidence 10-17 per 100,000
    • a) 1-2 per 100,000 for intraspinal
    • 50-75% are primary tumors
    • a) remainder are metastatic
    www.freelivedoctor.com
  • 49.
    • rarely metastatic outside of CNS
    • classes:
    • a) gliomas
    • b) neuronal tumors
    • c) poorly differentiated
    • d) meningiomas
    • 1.- GLIOMAS (astrocytomas, oligodendrogliomas, ependymomas)
    www.freelivedoctor.com
  • 50. a) Astrocytoma i) fibrillary astrocytoma ii) glioblastoma iii) pilocytic astrocytoma iv) pleomorphic xanthoastrocytoma v) all these have histological characteristics, distribution, age and clinical course vi) mean survival time is ~ 5 yrs www.freelivedoctor.com
  • 51.
    • Fibrillary astrocytoma
    • i) ~ 80 % of adult primary tumors
    • ii) found in cerebral hemispheres
    • iii) 4-6 th decade may occur in cerebellum, brainstem, spinal cord
    • iv) most common presenting sign is
    • - seizures
    • - headache
    • - focal neurological deficits
    • v) grading predicts prognosis
    • - WHO classification
    • - grades 1-4
    www.freelivedoctor.com
  • 52. - low grade astrocytoma show inactivation of tumor suppressor gene p53 - high grade astrocytoma show  inactivation of p53 as well as RB gene, p16/CDKNZA gene and tumor suppressor gene on chromosome 19q B) Glioblastoma i) prognosis very poor - 8 to 10 months following Dx ii) 2 distinct clinical histories www.freelivedoctor.com
  • 53. 1. - short, rapidly progressive, arising without preexisting low grade tumor - typically in older patients (primary glioblastoma 2. - younger patients - previously diagnosed low grade astrocytoma (secondary astrocytoma) - p53 mutations www.freelivedoctor.com
  • 54. C) Pilocytic astrocytoma i) young adults ii) relatively benign iii) mainly cerebellum - may occur in floor of 3 rd ventricle, optic nerves, and occasionally in cerebral hemispheres iv) often cystic looking v) grow slowly vi) WHO grade 1 vii) rare p53 mutations www.freelivedoctor.com
  • 55.
    • Oligodendroglioma
    • a) 5-10 % of gliomas
    • b) most common in 4 th and 5 th decades
    • i) may have had many years of complaints
    • - seizures
    • c) lesions found most often in cerebral hemispheres
    • i) mainly white matter
    www.freelivedoctor.com
  • 56.
    • d) most common genetic defect
    • i) involves chromosome 1  and 19q
    • e) clinical:
    • i) better prognosis re: astrocytomas
    • ii) average survival 5 to 10 years
    • Ependymomas
    • a) arise next to ependyma-lined ventricular system
    • i) also central canal of cord
    www.freelivedoctor.com
  • 57. ii) first 2 decades of life - near 4 th ventricle - 5-10 % of primary tumors in this age group iii) in adults spinal cord most common location b) clinical i) posterior fossa ependymoma - often with hydrocephalus, secondary to obstruction, rather than invasion ii) poor prognosis www.freelivedoctor.com
  • 58. - CSF dissemination is common - average survival ~ 4 years iii) several other tumors occur - lining of ventricles - other cells that form wall of ventricles  choroid plexus (rare) iv) benign low grade tumor - except the rare choroid plexus carcinoma www.freelivedoctor.com
  • 59.
    • Subependymomas
    • a) solid
    • i) sometimes calcified
    • b) slow growing nodules
    • i) attached to ventricular lining
    • ii) protrude into ventricles
    • c) usually asymptomatic
    • i) may cause hydrocephalus
    • d) most often found in lateral and 4 th ventricles
    • i) difficult to remove
    • ii) have distinct histology
    www.freelivedoctor.com
  • 60.
    • Choroid plexus papillomas
    • a) occur anywhere along choroid plexus
    • b) most common in children
    • i) lateral ventricles
    • ii) 4 th ventricle in adults
    • c) usually present with hydrocephalus
    • Colloid cyst of 3 rd ventricle
    • a) non-neoplastic lesion
    • b) young adults
    • c) attached to roof of 3 rd ventricle
    www.freelivedoctor.com
  • 61.
    • i) causes noncommunicating
    • hydrocephalus
    • - may be rapidly fatal
    • d) headache (sometimes positional)
    • important symptom
    • NEURONAL TUMORS
    • Several types contain mature appearing
    • neurons (ganglion cells)
    • a) gangliocytoma
    • i) comprised only of ganglion cells
    www.freelivedoctor.com
  • 62. b) more commonly exist as admixture i) with glioma neoplasm - lesion termed “ganglioglioma” - usually presents with seizures c) most slow growing i) glioma part may progress rapidly d) dysembryoplastic neuroepithelial tumor i) low grade, distinct tumor www.freelivedoctor.com
  • 63.
    • ii) childhood
    • - presents with seizures
    • iii) slow growth
    • - good prognosis after Tx
    • iv) located
    • - superficial temporal lobe
    • Tumors with only neuronal elements
    • a) cerebral neuroblastoma
    • i) rare
    • ii) children
    • iii) hemispheres
    www.freelivedoctor.com
  • 64.
    • iv) rapid and aggressive growth
    • b) central neurocytoma
    • i) low grade
    • ii) lateral and 3 rd ventricle
    • POORLY DIFFERENTIATED NEOPLASMS
    • most common is medullablastoma
    • a) ~ 20% of brain tumors in children
    • b) exclusive to the cerebellum
    • c) largely undifferentiated
    • i) glial and neuronal markers
    • occasionally
    www.freelivedoctor.com
  • 65.
    • d) clinical
    • i) highly malignant
    • ii) very radiosensitive
    • iii) prognosis also depends on
    • amount of tumor resected
    • iv) total resection plus radiation
    • - 5 year survival ~ 75 %
    • Atypical teratoid/rhabdoid tumors
    • a) highly malignant
    • b) young children
    • c) posterior fossa
    • d) presence of rhabdoid cells is Dx
    www.freelivedoctor.com
  • 66.
    • e) clinical
    • i) occur prior to 5 years of age
    • ii) death within a year following
    • diagnosis
    • OTHER PARENCHYMAL TUMORS
    • primary CNS lymphomas
    • a) ~ 2 % of extra nodal lymphomas
    • i) ~ 1 % of intracranial tumors
    • b) most common CNS neoplasm in
    • immunosuppressed patients
    www.freelivedoctor.com
  • 67. c) in nonimmunosuppressed i) occurs after age 60 d) often presents at multiple sites e) extra CNS involvement is rare i) denotes late stage ii) NHL arising outside CNS rarely invades brain parenchyma f) majority are B-cell origin i) in immunosuppressed patients all neoplasms appear to contain EBV genome www.freelivedoctor.com
  • 68.
    • g) very aggressive
    • i) poor response to treatment
    • compared to peripheral
    • lymphomas
    • Germ cell tumors
    • a) primary brain germ cell tumor
    • i) most commonly occur along midline
    • - pineal
    • - suprasellar
    • b) young (90% in first 2 decades)
    www.freelivedoctor.com
  • 69. c) teratomas i) most common tumor that - presents as congenital tumor d) in pineal region i) male predominance ii) not seen in suprasellar region as male predominance e) unlike lymphomas i) CNS germ cell tumors not uncommon www.freelivedoctor.com
  • 70.
    • ii) similar classification to seminoma in testis
    • - termed “germinoma”
    • pineal parenchymal tumors
    • a) arise from pineocytes
    • b) differentiation
    • i) well – pinocytoma
    • ii) undifferentiated (high grade)
    • - pineoblastoma
    • - highly aggressive
    • - more common in children
    • - in pts. with retiniblastoma
    www.freelivedoctor.com
  • 71.
    • c) Gliomas also found in pineal region
    • meningioma
    • a) benign
    • b) occur in adults
    • c) usually attached to dura
    • d) clinical
    • i) slow growing
    • ii) uncommon in children
    • iii) small female preponderance
    • - 3:2
    • - 10:1 with spinal meningioma
    www.freelivedoctor.com
  • 72. www.freelivedoctor.com
  • 73.
    • metastatic tumors
    • a) mostly carcinomas
    • i) 25-50 % of hospitalized patients
    • b) sites (accounts for 80% of all metastatic tumors)
    • i) lung
    • ii) breast
    • iii) skin (i.e., melanoma)
    • iv) kidney
    • v) GI
    www.freelivedoctor.com
  • 74.
    • c) meninges frequent site of metastatic tumors
    • d) present as mass lesion
    • paraneoplastic syndromes
    • a) major underlying mechanisms
    • i) systemic development of immune response against tumor antigen
    • b) may be T-cell mediated neuronal injury in some settings
    www.freelivedoctor.com
  • 75. www.freelivedoctor.com
  • 76. BOARD QUESTIONS www.freelivedoctor.com
  • 77. Which of the following disorders affecting myelin is most likely to be found in a 30-year-old woman? (A) multiple sclerosis (B) Krabbe disease (C) Alexander disease (D) metachromatic leukodystrophy www.freelivedoctor.com
  • 78. A disease characterized by widespread patches of demyelination with less prominent axis cylinder destruction and glial overgrowth is (A) syphilis (B) poliomyelitis (C) multiple sclerosis (D) pernicious anemia (E) amyotrophic lateral sclerosis www.freelivedoctor.com
  • 79. Reactive astrocytes surrounding eosinophilic fibers radiating from a central core which stains for amyloid is characteristic of (A) Alzheimer disease (B) amyotrophic lateral sclerosis (C) olivopontocerebellar atrophy (D) Parkinson disease (E) Wilson disease www.freelivedoctor.com
  • 80. Lewy bodies are most commonly encountered in (A) idiopathic Parkinsonism (B) post-encephalitic Parkinsonism (C) rabies (D) Tay-Sachs disease (E) herpes simplex encephalitis www.freelivedoctor.com
  • 81. The most radiosensitive primary intracranial neoplasm is (A) ependymoma (B) glioblastoma (C) medulloblastoma (D) oligodendroglioma www.freelivedoctor.com
  • 82. A child presents with nausea and vomiting, a recent onset of ataxia, and a posterior fossa tumor. The most likely diagnosis is (A) craniopharyngioma (B) medulloblastoma (C) meningioma (D) neuroblastoma (E) pinealoma www.freelivedoctor.com

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