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My Pathology > CNS > Flashcards

CNS Flashcards

1
Q

Pseudotumor cerebri

A
  • Increased intracranial pressure due to decreased CSF reabsorption
  • Papilledema, headache, vision change
  • Risk factors: hypothyroidism, Cushing’s, OCPs, isotretinoin, tamoxifen
  • Flattening of posterior globe on MRI
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2
Q

Subfalcine cerebral herniation

A
  • Cingulate gyrus herniates under falx cerebri
  • Confusion and drowsiness; possible contralateral LE weakness and urinary incontinence w/ ACA compression
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3
Q

Uncal cerebral herniation

A
  • Medial temporal lobe herniates thru tentorium cerebelli
  • Midbrain compression (Duret hemorrhages)
  • CN 3 compression (down-and-out eye deviation), pupil dilation
  • PCA compression (occipital lobe infarct, visual disturbance)
  • Hemiparesis ipsilateral to mass
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4
Q

Tonsillar cerebral herniation

A
  • Cerebellar tonsils herniate into foramen magnum (“coning”)
  • Cardiorespiratory arrest
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5
Q

Hydrocephalus

A
  • Increased CSF volume —> ventricular enlargement
  • Noncommunicating hydrocephalus may be caused by pineal gland tumor or aqueduct of Sylvius (b/w 3rd and 4th ventricles) blockage
  • Communicating hydrocephalus: can result from dural venous sinus thrombosis
  • Increased head circumference in newborns
  • Hydrocephalus ex vacuo: ventricular enlargement w/ Alzheimer’s-related cerebral atrophy
  • Normal pressure hydrocephalus: wide-based gait, urinary incontinence, dementia
  • Causes: prior trauma/surgery, prior subarachnoid hemorrhage
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6
Q

Arnold-Chiari malformation

A
  • Caudal extension of medulla and cerebellar vermis thru foramen magnum
  • Noncommunicating hydrocephalus
  • Platybasia (flattened base of skull)
  • Associated w/ meningomyelocele, syringomyelia
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7
Q

Dandy-Walker malformation

A
  • Partial or complete absence of cerebellar vermis
  • Cystic dilation of 4th ventricle ‘
  • Noncommunicating hydrocephalus
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8
Q

Syringomyelia

A
  • Sx starting in 20s-30s
  • Syrinx (fluid-filled cavity) w/in cervical spinal cord expands and causes degeneration of spinal tracts
  • Associated w/ Arnold-Chiari malformation
  • Disruption of spinothalamic tracts (pain and temp loss in “cape-like” distribution)
  • Disruption of anterior horn cells (atrophy of intrinsic muscles of hand)
  • Disruption of ANS
  • Charcot joint
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9
Q

Neurofibromatosis

A
  • Autosomal dominant
  • Mutation on chr 17 (neurofibromin) or 22 (merlin), both tumor suppressors
  • Both types: café-au-lait spots, pigmented cutaneous/subQ neurofibromas (except palms and soles)
  • Type 1: Lisch nodules (pigmented hamartoma of iris), axillary/inguinal freckling, pigmented plexiform neurofibromas, pheochromocytoma, Wilms tumor (both w/ HTN), CML
  • Type 2: bilateral acoustic neuromas, meningiomas, spinal schwannomas, juvenile cataracts
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10
Q

Tuberous sclerosis

A
  • Autosomal dominant
  • Mental retardation, seizures in infancy
  • Angiofibromas on face, shagreen patches/ash leaf spots (Wood lamp), nail fibromas
  • Neuroectoderm abnormalities —> “tubers” in cerebral CTX
  • Subependymal giant cell astrocytomas (“candlestick drippings” in ventricles), angiomyolipoma of kidney, rhabdomyoma of heart
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11
Q

Sturge-Weber syndrome

A
  • Vascular malformation of face (trigeminal nerve distribution)
  • Ipsilateral AV malformation in meninges
  • Unilateral “port wine stain” on face
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12
Q

Acute epidural hematoma

A
  • Middle meningeal artery bleed creates blood-filled space b/w bone and dura
  • Temporoparietal bone Fx
  • May have period of lucidity before neuro degeneration
  • Often fatal
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13
Q

Subdural hematoma

A
  • Bridging veins b/w brain and dural sinus bleed b/w dura and arachnoid membrane
  • Slowly enlarging blood clot covers brain
  • Sx may emerge over months: HA, contralateral weakness, seizures, etc.
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14
Q

Atherosclerotic stroke

A
  • Majority preceded by TIA
  • Pale infarct (no reperfusion)
  • Edema, loss of gray-white differentiation, myelin breakdown
  • 3-10 day: liquefactive necrosis —> cystic change
  • MCA: contralateral hemiparesis and sensory loss in face and upper extremity; expressive aphasia; visual field defect
  • ACA: contralateral hemiparesis and sensory loss in lower extremity
  • Vertebrobasilar arterial system: deafness, vertigo, ataxia, ipsilateral sensory loss in face, contralateral in trunk and limbs
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15
Q

Embolic stroke

A
  • Most emboli from L heart (mural thrombus, A fib)
  • MCA
  • Hemorrhagic infarct (reperfusion)
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16
Q

Intracerebral hemorrhage

A
  • HTN
  • Abrupt Sx onset
  • Lenticulostriate branches develop Charcot-Bouchard microaneurysms that rupture
  • Basal ganglia/thalamus, pons, cerebellum
  • Slit hemorrhages (resorbed blood from small vessels)
  • Lobar ICH: white matter hemorrhage suggestive of amyloid angiopathy, vascular malformation, coagulopathy, or bleeding from tumor
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17
Q

Subarachnoid hemorrhage

A
  • Berry aneurysm or AV malformation
  • Thunderclap headache w/ nuchal rigidity
  • Risk factors: epithelial stressors
  • Communicating branches, most often anterior + ACA
  • Blood covers surface of brain
  • Xanthochromia (bilirubin makes CSF yellow)
  • In survivors, rebleeding is frequent
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18
Q

Lacunar infarct

A
  • Small cystic infarcts of parenchymal arteries or arterioles
  • Risk factors: HTN, atherosclerosis
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19
Q

Meningitis

A
  • Inflammation of pia mater
  • Usu. hematogenous spread
  • Seizures, focal neuro deficits, CN palsies, hearing loss, hydrocephalus
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20
Q

Multiple sclerosis

A
  • Females 20-40 y/o
  • HLA-DR2 (or others) and microbial triggers
  • Type 2 and 4 hypersensitivity: TH1, TH17, CD8 T cells, and Abs vs myelin sheath and oligodendrocytes
  • Episodic course
  • Often begins with visual complains
  • Paresthesias; loss of pain, temp, vibratory sense
  • Spasticity; increased DTRs; muscle weakness; urinary incontinence
  • Optic neuritis (blurry/lost vision); cerebellar ataxia; “drunken” speech; intention tremor; nystagmus; ophthalmoplegia
  • Increased CSF WBCs, protein
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21
Q

Arbovirus

A
  • Encephalitis
  • Mosquitos all over US
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22
Q

Coxsackievirus

A
  • Meningitis
  • Enterovirus
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23
Q

CMV

A
  • Encephalitis
  • Neonates (vertical transmission) or AIDS patients
  • Eosinophilic inclusions
24
Q

HSV1

A
  • Meningitis and encephalitis
  • Hemorrhagic temporal lobe necrosis
25
Q

HIV

A
  • Encephalitis
  • Slowness of thought and bradykinesis
  • Microglial cells fuse to form multinucleated cells
26
Q

Lymphocytic choriomeningitis

A
  • Meningitis and encephalitis
  • Mouse transmission
27
Q

Poliovirus

A
  • Encephalitis and spinal cord inflammation
  • Destroys upper and lower motor neurons
  • Muscle paralysis
28
Q

Rabies virus

A
  • ACh receptor; moves to CNS, then saliva
  • Incubation period 10-90 days
  • Prodrome: fever, paresthesias at wound site
  • Hydrophobia
  • Encephalitis: seizures, coma, death
  • Eosinophilic inclusions called Negri bodies
29
Q

Progressive multifocal leukoencephalopathy

A
  • Slow encephalitis
  • AIDS patients
  • Papovavirus (JC virus)
  • Scattered demyelination foci
  • “Ground-glass” inclusions in oligodendrocytes
30
Q

Subacute sclerosing panencephalitis

A
  • Slow, fatal encephalitis
  • Rubeola (measles) virus
  • Inclusions in neurons and oligodendrocytes
31
Q

Bacteria that cause neonatal meningitis

A
  • Group B strep (Gram+ coccus)
  • E. coli (Gram- rod)
  • Listeria monocytogenes (Gram+ rod with tumbling motility; cheese and hot dogs)
32
Q

Bacteria that cause meningitis

A
  • Neisseria meningitidis (Gram- diplococcus); children; may show petechial rash
  • Strep pneumoniae (Gram+ diplococcus); high incidence w/ basilar skull Fx
  • Mycobacterium tuberculosis (complication of primary tuberculosis); involves base of brain; tuberculomas w/ caseous necrosis; may also cause Pott disease (in spine)
  • T. pallidum (Gram- spirochete); multiple small infarcts; may also cause tabes dorsalis (loss of joint position sense and fine touch); late recurrence may cause dementia with “wind-blown appearance” of cortical neurons and “rod cells” (iron-encrusted microglia)
33
Q

Fungi that cause meningitis

A
  • Cryptococcus neoformans (immunocompromised; budding yeasts visible w/ India ink)
  • Mucor species (frontal lobe abscess; diabetic ketoacidosis)
  • Coccidiodes immitis (Southwest US; refractile endosporulating spherule)
34
Q

Parasites that cause CNS infections

A
  • Naegleria fowleri (amoeba from freshwater lakes; frontal lobes)
  • Trypanosoma brucei (hemoflagellate from tsetse fly; Winterbottom sign [post cerv lymph]; somnolence, cyclical fever)
  • Taenia solium (helminth; pigs; eggs invade brain)
  • Toxoplasma gondii (sporozoan; AIDS pts; ring-enhancing lesions on CT; congenital —> basal ganglia calcification)
  • Acanthamoeba: granulomatous encephalitis; usu. fatal
  • P. falciparum: diffuse swelling, microinfarcts w/ gliosis (Dürck granulomata) or many small hemorrhages
35
Q

Central pontine myelinolysis

A
  • Alcoholics w/ hyponatremia
  • Rapid salt correction CAUSES demyelination in basilar pons
36
Q

Adrenoleukodystrophy

A
  • Boys 3-10 yrs old, progresses to coma
  • XLR
  • Deficiency in beta-oxidation of FAs
  • Cortex-sparing loss of brain myelin, adrenal insufficiency
37
Q

Krabbe disease

A
  • Autosomal recessive lysosomal storage disease
  • Deficiency in galactocerebroside beta-galactocerebroside; accumulation of galactocerebroside
  • Small brain w/ widespread myelin loss
  • Large, multinucleated, histiocytic globoid cells
  • Death w/in 1-2 yrs
38
Q

Friedreich ataxia

A
  • Autosomal recessive, Sx appear before age 25
  • Frataxin deficiency (iron transport into mitochondria)
  • Degeneration of dorsal root ganglia, posterior columns, sensory peripheral neurons
  • Hypertrophic cardiomyopathy
  • Gait ataxia, DTR loss, vibratory/proprioception loss, BLE weakness
39
Q

Astrocytoma

A
  • Diffuse tumors
  • Adults: frontal lobe, children: cerebellum
  • Diffuse pontine astrocytoma: lethal pontine tumor in young children
  • Pilocytic astrocytoma: does not infiltrate; well-circumscribed; Rosenthal fibers
  • Pleomorphic xanthoastrocytoma: Hx of seizures; BRAF mutation; very pleomorphic but low mitotic activity
  • Subependymal giant cell astrocytoma: associated w/ tuberous sclerosis; low-grade tumor in lateral ventricle causing hydrocephalus
40
Q

Glioblastoma

A
  • Astrocytoma
  • Hemorrhagic tumor
  • Ring-enhancing areas of necrosis and cystic degradation
  • Commonly cross corpus callosum (“butterfly” glioblastoma)
41
Q

Meningioma

A
  • Benign tumor of meningothelial cell of arachnoid membrane
  • Female predominance (progesterone receptors)
  • Parasagittal MC location; may appear in dural venous sinuses
  • Risk factors: NF2, Hx of radiation
  • Well-circumscribed, firm, tan tumor that may infiltrate bone
  • Swirling mass of meningothelial cells encompass psammoma (calcified) bodies
  • Common cause of new-onset focal seizures
42
Q

Ependymoma

A
  • Benign tumor of ependymal cells, usually in children and young adults
  • Children: 4th ventricle, may produce hydrocephalus
  • Adults: cauda equina
  • Well-circumscribed with (pseudo-)rosettes
  • Myxopapillary ependymoma: low SC of adults
  • Subependymoma: adults, usu. asymptomatic but may block foramen of Monro or 4th ventricle outlet
43
Q

Medulloblastoma

A
  • Malignant small cell tumor of children; occasionally adults 20-45 y/o
  • Cerebellum; often seeds neuraxis and invades 4th ventricle
  • Desmoplastic/nodular variants: common in adults or infants, resembles lymph nodes; SHH gene
  • Anaplastic/large cell variants: Wnt gene
44
Q

Oligodendroglioma

A
  • Benign tumor of oligodendrocytes
  • Frontal lobe tumor that frequently calcifies
  • Highly infiltrative but responsive to therapy
  • Chromosome 1:19 translocation
  • “Fried egg” appearance of cells, blood vessels in “chicken wire” pattern
  • Cells cluster around neuron cell bodies or blood vessels
45
Q

CNS lymphoma

A
  • Majority are metastatic high-grade B-cell non-Hodgkin lymphomas
  • Often AIDS and/or EBV-associated
46
Q

Schwannoma

A
  • Benign tumor of Schwann cells
  • Acoustic neuroma: CN 8; cerebellopontine angle; NF2 association; may impinge on CN 5
47
Q

Metachromatic leukodystrophy

A
  • Accumulation of cerebroside in white matter and peripheral nerves
  • Rusty-brown coloring with violet or blue stains
  • Disease of infancy, lethal w/in several years
48
Q

Tay-Sachs disease

A
  • Hexosaminidase A deficiency w/ accumulation of ganglioside
  • Cherry-red spot in macula, lipid droplets distent neurons
  • Delayed motor development by 6 mos., death w/in 2 yrs
49
Q

Subacute combined degeneration

A
  • B12 deficiency (pernicious anemia)
  • Degeneration of thoracic posterior spinal cord
  • Burning in soles of feet, other paresthesias
  • Limb weakness, defective postural sensibility, incoordination
50
Q

Huntington’s disease

A
  • Involuntary movements, deteriorating cognitive fxn, emotional disturbances
  • Frontal CTX and caudate/putamen atrophy, lateral ventricle enlargement
51
Q

Holoprosencephaly

A
  • Telencephalon (CTX and basal ganglia) fails to divide
  • Single large ventricle
  • Mildest form: arrhinencephaly (failure of olfactory nerves)
52
Q

Choroid plexus papilloma

A
  • Children: lateral ventricle, adults: 4th ventricle
  • May disseminate via CSF
53
Q

Atypical teratoid/rhabdoid tumor (ATRT)

A
  • Malignant tumor of early childhood
  • Differentiation along multiple different cell lines
  • Often posterior fossa (cerebellum, brainstem)
  • Inactivation of INI-1
54
Q

Craniopharyngioma

A
  • Often children
  • Sella turcica region, causing endocrine and visual disturbances
  • Heterogeneous tumor
  • Adamantinomatous type: sheets of squamous epithelium with palisading, “stellate reticulum,” “wet keratin,” possible Rosenthal fibers
  • Papillary: exclusively squamous epithelium
55
Q

Germinoma

A
  • Often involve pineal gland
  • Large malignant cells + small reactive lymphocytes, may have granulomatous response
56
Q

Hemangioblastoma

A
  • Mainly cerebellum, can be seen in SC and brainstem
  • Risk factors: von Hippel-Lindau disease
  • Cystic w/ enhancing mural nodules

My Pathology (12 decks)

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