LECOM CNS Path Ch 28
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| Gradual HA, R pupil dialated and awkward movements and PCA area ischema | transtentorial herniation
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| 3 causes of microcephaly | Chromosomal, FAS, HIV-1
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| Large midline non-neoplastic cyst where vermis should be | Dandy Walker Malformation
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| Hydrocephalus, Lumbar meningiomyelocele, small posterior fossa, cerebellum extended through the foramen magnum | Chiari II malformation
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| 10-20yo, isolated loss of pain and temperature in upper extremity | syringomylia
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| Premature baby, chalky yellow plaques, white and grey involvement with large cystic lesioins | multicystic encephalopathy
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| Old traumatic lesions on brain appear | depressed, retracted, yellow/brown aka plaque jaune
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| PP post MVA. No papilledema, no decerebrate posturing , no spontaneous movements. Unconcious. Head CT: no intracranial hemmorhage or edema and no skull fractures. Remains in persistent vegetative state. Lesion? | Diffuse axonal injury. followed by axonal swelling with silver impregnation
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| A pt hits head hard, comes to ER talks to doc, becomes unconscious then becomes comatose - which type of hematoma? | Epidural - talk and die
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| Old person falls yesterday, then headache and confusion. What is the Dx what would CT scan show? | Subdural hematoma- tearing of the bridging veins Crescent-shaped hemorrhage that crosses suture lines w/ preserved gyri
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| What could cause an 80 year old to go in and out of consciousness who has blood in subdural space? | Tear of bridging vein - subdural hematoma
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| Area most susceptible to global ischemia- shows sickle shaped area of necrosis | ACA-PCA watershed area
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| Widespread white matter hemorrhages most likely due to | bone marrow emboli
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| 55 y.o. man suddenly loses conciousness. Examination: Bilateral Papilledema. No spontaneous movements. What underlying condition caused this? | Systemic Hypertension
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| Brain with intra-parenchyma bleed in cerebral cortex. Vessels are open on cross section. Cause? | CAA
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| CAA where is the bleed? What stain for vessels? What mutation? | leptomeningeal or cortical arterioles; Congo Red amyloid; e2 or e4 allele
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| 79 y.o. woman was driving her automobile when she had a sudden, severe headache. She was taken to the ER, where she remained comatose and died 6 hours later. What was the cause? | Rupture of Berry Aneurysm. Aneurysm in the circle of Willis and extensive subarachnoid hemmorhage.
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| 27yo w/ Hx of seizures living in 3rd world country. Comes to USA. Area of high blood flow on MRA. Gliosis and evidence of recent and remote hemorrhage. conglomerate of various-sized tortous vessels surrounded by gliosis. What is this caused from? | AV malformation.
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| Most common cause of meningitis in 20 year old male? How would brain appear histologically? | Neisseria meningitidis (6-60 yrs of age) -- purulent exudate on the cerebral convexities indicitve of a bacterial infection
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| 5 y.o. boy has meningitis. What would be seen on gram stain? | Gram-positive cocci. Most common organism is Streptococcus Pneumoniae, which is gram-positve coccus.
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| 90yo immunocompetent Pt with bacterial meningitis. 2 most common causes | Strep Pneumo and Listeria
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| Arachnoid fibrosis common in what meningitis? | Strep Pneumo
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| Basal purulent exudate indicative of what infection? | H. influenza meningitis
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| Dense purulent cover over cerebral convexaties with meningitis is indicative of | pneumococcal infx
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| CSF high opening pressure; increased WBC; increased protein; normal glucose Dx, most likely cause and predisposing condition | (multiple)Brain abscess, strep & staph, (acute bacterial endocarditis)
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| Pt c/o HA, vomiting, then massive cerebral ischemia. Autopsy: sub-arachnoid basal gelatinous exudate, CSF:monocytosis and TONS of protein, obliterave endarteritis in sub-arachnoid space. Acid-fast + Cause? | Tuberculosis meningoencephalitis
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| Pt presents with delusions of grandeur-> sever dementia, Inflammatory lesions in frontal lobe, Loss of neurons, proliferative rod cells, gliosis, iron deposit (Prussian blue) Dx and cause? | Neurosyphalis Paretic type
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| Pt at neurologist with Locomotive ataxia; loss of pain sensation and DTRs Histo: Dorsal root degeneration- loss of axons and myelin, atrophy of dorsal column | Neuro syphilis Tabes Dorsalis
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| Seizures, stupor, ocular palsies, asymmetrical reflexes with microglial nodules centered around necrosis and neuronophagia Cause? | Arthopod borne encephalitis
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| Polio like syndrome | West Nile encephalitis
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| Child with mood changes, and memory loss. Necrotic, hemorrhagic lesions in inferior, medial temporal lobe. Cause | HSV-1
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| BUZZ: Cowdry Type A intranuclear inclusion in glia and neuron | HSV-1
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| Neo-nate with microcephaly, periventricular necrosis and calcification Dx | CMV
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| AIDS patient autopsy: paraventricular cytomegalic cells with cytoplasmic and nuclear inclusion bodies. With hemorrhagic necrosis Dx | CMV
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| BUZZ: Negri body in Purkinji cell | Rabies
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| Cytoplasmic round/oval eosinophilic inclusions in pyramidal neurons of the hippocampus or Purkinji cells in the cerebellum | Rabies
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| Malaise, headache, fever, animal bite mark with paresthesias around it | rabies
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| Encephalitis with microglial nodules and multinucleated giant cells | HIV
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| AIDS patient presents with a demyelinating condition that resulted due to a latend virus. What is it? | JC papovavirus. Patient has progressive multifocal leukoencephalopahty (PML)
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| Multifocal lesions of hemisphereic/cerebellar liped laiden macrophages, peripheral enlarged oligodendrocyte nuclei w/ glassy amphophilic nuclear inclusions | PML w/ JC virus
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| HIV patient Gelatinous exudate in CSF with “soap bubble” destruction of basal ganglia and perivascular lesions | Cryptococcus
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| Person with following progression : ataxia, autonomic disturbances, stupor, coma. With neuronal loss in AV and DM nuclei. | FFI
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| Pt presents w/ paresthesias in arm, difficulty walking and then the problems will go away. Then he has problems seeing and writing. What would you find in CSF? how else does this disease present? how else do you diagnos? | MS - oligoclonal bands of IgG
SIN - scanning speech, Intention tremor, incontinence, internuclear opthalmoplegia, nystagmus
MRI is gold standard but diagnostic is made on Oligoclonal bands (also periventricular plaques seen)
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| Homologous perivenular lesions of myelin loss, axon preservation, lipid laiden macrophages. Child after viral Infx, died after HA and coma | ADEM
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| Perivenular necrosis of grey and white matter, small artery destruction, hemorrhage, demylination Child post respiratory illness. Death | ANHE
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| Alcoholic with hyponatremia is corrected too quickly. Results in .. | Central Pontine Myelinolysis
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| What is most important factor in the development of alzheimers? What are some histological findings? | Inheritance of the e4 allele oat the ApoE4 gene - numerous plaques, amyloid in peripheral cerebral arteries, tangles,Tau protein
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| Early onset personality change, and language disturbances. Wafer thin gyri, lobar atrophy Dx and sites | Pick Disease, fronto/temporal , altered Tau protein
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| Truncal ridgidity, disequilibrium, nuchal dystonia, trouble moving eyes. GP, STn, SN, dentate nucleus. 4R tau protiens | Progressive Supranuclear Palsy
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| Tau positive threads in grey and white matter, tufted astrocytes, coiled bodies, and cortical atrophy in the elderly | Corticobasilar Degeneration
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| SNpc degeneration with Lewy bodies a-synuclein + | Alzheimers
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| AD like Sxs, with dementia, hallucinations, with lewy bodies scattered throughout the brain | Dementia with Lewy Bodies
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| Choreaic movements, striatal degeneration | Huntingtons Disease
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| Huntington’s genetics | CAG repeat on chromosome 4
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| Which disease causes loss of UMN leading to progressive muscular weakness from grouped atrophy of skeletal muscle fibers and loss of anterior horn cells -- what is the defect? | ALS or Lou Gerigs disease - defect in superoxide dismutase 1 (SOD1) - commonly presents as fasiculations
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| 55 y.o. who had been healthy all his life now has progressive, symmetric muscular weakness. What is the condition? | Amyotropic Lateral Sclerosis aka ALS aka Lou Gehrig's Disease.
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| Krabbe Disease deficiency and buzz word | galactocerebroside B-galactosidase – Globoid Cell
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| Childhood motor changes- dead in 5-10 years-vaculolated macrophages w/ crystalloid structures | Metachromatic leukodystrophy
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| White matter dysfunction. Tissue has “tigroid” look | Pelizaeus-Merzbacher Disease
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| Alcoholic, Focus of hemorrhage and necrosis in the mammillary bodies and 3rd/4th ventricle walls What deficiency? | B1
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| What is most common histological finding in someone w/ CJD? | Spongiform encephalopathy
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| Which vitamin deficiency is seen in chronic alcoholics? What is the name of this dementia | Thiamine, B1 - Wernicke - confusion, opthalmoplegia, and ataxia (wernicke's encephalopathy.)
Korsakoff if personality change and memory problems
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| A person w/ diabetes melliitus is most likely prone to which type of dementia? | Vascular dementia - stepwise loss of cognitive function
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| Atrophy and loss of granule cells in cerebellum w/ loss of Perkinji cells and Bergman Gliosis. Truncal ataxia, nystagums Dx | Ethanol toxicity
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| Which pathologic findings will a brain present with in Parkinson's? | Loss of pigmented neurons in substantia nigra, Lewy bodies (composed of a-synuclein
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| Which neoplasm is likely to present as a mass attached to the dura of the brain w/ occasional psammoma bodies? what are psammoma bodies? | Meingiomas -spindle cells concentrically arranged in a whorled pattern w/ laminated caclifications
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| If a pt has hyponatrium and it is corrected to quickly what is the negative outcome? | Demyelination in the bais pontis - can lead to death
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| What are the two most common neoplasms in children, which location of brain? How would the better prognosis present? | Posterior fossa - the astrocytoma - would stain postiive for GFAP(better prognosis) and
medulloblastoma - pseudoroseette (homer-wright - or small blue cells, cause hydrocehphalus
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| What would you find in CNS of a pt w/ huntingtons? | Atrophy and gliosis of the caudate nuclei
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| A child presents w/ progressive worsening of movement and mental ability and dies, myelin loss is noted.. What could cause this? | What is a common opportunistic infection that affects the CNS in pt's w/ aids?
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| 5yo complained of headache for the past week. Ataxic Gait. sudden onset of vomitting, becomes comatose. Afebrile. Head CT: 4-cm mass in cerebellar vermis and dilation of cerebral ventricles. CSF: small cells with dark blue nuclei and scant cytoplasm. | Medulloblastoma.
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| 49 y.o. woman has had several headaches for 2 days. Afebrile and normotensive. Fundoscopy: Papilledema on the right. One day later, patient has right pupillary dilation and impaired ocular movement. Then becomes Obtunded What's the condition? | Transtentorial medial temporal herniation.
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| PP Primary Brain Tumor that on microscopy looks like spindle cells concentrically arranged in a whorled pattern (psmammoma bodies: laminated calcifications). What is it? | Meningioma.
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| A previously healthy 16 y.o. boy has complained of headaches for the past 9 months. Enlargement of 4th Ventricle. What is it? | Ependymoma.
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| Pt. has mass in brain. Stereotactic biopsy of the mass shows areas of necrosis surrounded by nuclear pseudopalisading. The neoplastic cells within the mass are hyperchromatic. Neoplasm? | Glioblastoma Multiforme.
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| HIV Patient has Pneumocystis Jiroveci. Brain MRI: diffuse cerebral atrophy, and no focal lesions are identified. What would be seen in brain? | White matter microglial nodules with multinucleate cells. Patient has AIDS dementia complex late in the course of his HIV infection.
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| 4yo developed clumsiness and difficulty ambulating over 6 months developed CHF from hypertrophic cardiomyopathy. She also had hyperglycemia. Autopsy: increased perinuclear iron deposition within cardiac myocytes. Which genetic abnormalities with TNRE? | GAA repeats in the frataxin gene. Patient has Friedrich's ataxia The frataxin gene encodes for a protein involved in iron regulation in cells.
Friedreich is Fratastic (frataxin): he's your favorite frat brother, always stumbling, staggering, and falling.
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| 63yo increasing irritability over 3 years. Then marked memory loss. Autopsy: Frontal cortex extensive neoronal loss on microscopy and some remaining neurons show intracytoplasmic, faintly eosinophilic, rounded inclusions that stain IHC for tau protein. | Pick Disease aka Frontotemporal Dementia. Has clinical features similar to Alzhemier's disease, but initially it causes less memory loss and more behavorial changes.
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| 20 y.o. mentally retarded woman saw physician because of flank pain for 1 week. Patches of leathery (shagreen patches) and hypopigmented (ash-leaf patches) skin were scattered over her body. What is it? | Tuberous Sclerosis is one of the phakomoatoses (or "neurocutaneous syndromes").
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| 40 y.o. man, who rarely has headaches, now experiences headaches for the past 6 months. Dies. Microscopy: mass consists of cells with round nuclei with clear cytoplasm. What's the prognosis? | Oligodendroglioma.
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| PP with a primary brain tumor that has characteristic perivascular pseudorosettes and rod-shaped blepharoplast (basal ciliary bodies) found in nucleus. What is it? | Ependymoma. The tumors arise in the ventricles (usually 4th ventricles)
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| PP w/ cell study: large cells with large nuclei and scant cytoplasm that mark CD19, but no GFAP or cytokeratin. What is it? | Diffuse Large B-cell Lymphoma. Most common adult Non-Hogkin Lymphoma
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| 60 y.o. woman with Parkinsons. IHC stain for what type of protein is positive? | alpha-synuclein.
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| PP with cerebellar tonsillar herniation through foramen magnum with aqueductal stenosis and hydrocephaly. Brain MRI would show what malformation? | Arnold-Chiari Type II malformation. Often presents with syringomyelia, thoraco-lumbar myelomenigocele.
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| Infant born at 36 weeks gestation is suffering from cyclopia. The infant died 1 hour after birth. What did the baby have and what condition is this seen in.? | Holoprosencephaly. Trisomy 13 (Patau's) M.C. but can be seen in maternal DM and fetal alcohol syndome as well
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| 86 y.o. man. Histological Exam of the brain: numerous neocortical neuritic plaques and neurofibirllary tangles. What is seen histologically in plaques and what is seen histologically in tangles? | Plaques: Aggregation of AB peptide. Tangles: tau protein Alzheimer's disease.
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| PP with brain tumor in cerebral hemisphere. "Pseudopalisading" Pleomorphic cells positive for GFAP. Neoplasm? | Glioblastoma Multiforme. Most common primary brain tumor
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