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PNS, CNS Flashcards

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1
Q

MC inherited peripheral neuropathy

A

Charcot Marie Tooth
MC type: CMT1, 2nd decade, distal demyelinating
CMT2A: early, childhood, axonal neuropathy

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2
Q

MC chronic acquired peripheral neuropathy

A

Chronic inflammatory demyelinating poly (radiculo) neuropathy
symmetric mixed sensorimotor polyneuropathy >/2 mos
recurrent demyelination and remyelination with Schwann cell prolif

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3
Q

acute symmetric ascending demyelinating polyradiculoneuropathy
T cell mediated
ascending paralysis and areflexia

A

Guillan Barre syndrome
CSF: Albuminocytologic dissociation

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4
Q

MCC of PN

A

DM

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5
Q

Neuromuscular disease
+ weakness with exertion
EOM muscle group

A

Myasthenia gravis
HS type II
Assoc: Thymoma, thymic hyperplasia
post synaptic ACh receptor autoantibodies

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6
Q

Neuromuscular disease
- muscle weakness upon exertion
Extremities
assoc: neuroendocrine Ca of the lungs

A

Lambert eaton myasthenic syndrome (LEMS)
autoantibodies against Presynaptic Calcium channels

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7
Q

MC inflammatory myopathy

A

Juvenile - Dermatomyositis
Adult - usually paraneoplastic

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8
Q

Skin changes seen in Dermatomyositis

A

Heliotrophe rash - periorbital lilac discoloration
Gottron papules - dusky rash patches over knuckles, knees and elbows

pattern of atrophy: perifascicular

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9
Q

antibodies in Dermatomyositis for gottron and heliotrope rash

A

anti-Mi2

P155/P140 - paraneoplastic, juvenile form

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10
Q

total absence of Dystrophin X on chromosome
pelvic girdle weakness
pseudohypertrophy of lower leg muscles
Gower sign
- Dystrophin IHC

A

Duchene muscular dystrophy

Becker - milder, late onset, reduced dystrophin on IHC

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11
Q

Cell dense areas with spindle cells arranged in fascicles with nuclear palisading

A

Antoni A

Verocay bodies - Cell-free areas due to nuclear palisading

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12
Q

Cell poor areas with prominent MYXOID stroma

A

Antoni B

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13
Q

mutation: NF2 (Merlin) (Ch22) inactivation
Biphasic appearance: Antoni A and Antoni B
Verocay Bodies
+ S100

A

Schwannoma

+S100 - positive in neural crest cell derived cells (other: melanocytes)

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14
Q

mutation: NF1 (Ch17) inactivation
+ S100, spindle cells, + CD34
plexiform: Bag of worms appearance

A

Neurifibroma
“shredded carrots appearance”

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15
Q

Components of neurofibromatosis

A

Bilateral 8th nerve
Schwannoma
multiple meningioma
ependymoma

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16
Q

Most important pathologic marker of CNS injury

A

Gliosis

acute neuronal injury: Red neuron
subacute and chronic neuronal injury: Degeneration

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17
Q

Herniating part in Subfalcine

A

Cingulate
herniation thru under flax cerebri
compressed: anterior cerebral artery

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18
Q

compressed structure in Transtentorial herniation

A

CN III
Posterior Cerebral artery
CL cerebral peduncle

herniating part: medial aspect of temporal lobe
herniating thru: tentorium cerebelli

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19
Q

linear or flame-shaped lesion hemorrhagic lesion in brainstem and pons

A

Duret hemorrhages

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20
Q

tonsillar herniate through
Compressed sturcture

A

Foramen magnum
herniating part: cerebellar tonsils\
Compressed: Brainstem – cardio and respi depression

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21
Q

MC CNS malformation

A

Neural Tube defects
RF: folate deficiency

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22
Q

MC neural tube defect

A

Spinal Dysraphism/Spina bifida

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23
Q

extension of the spinal cord and meninges through the vertebral column defect

Study These Flashcards
A

Myelomeningocele

meningocele: meninges only

24
Q

extrusion of malformed brain tissue thru a midline cranial defects

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A

Encephalocele
Loc: Occiput

25
Incomplete separation of the cerebral hemispheres across the midline
Holoprosencephaly assoc. Trisomy 13 Cyclopia, Arrhinencephaly - absence of olfactory cranial nerves
26
Enlarged posterior fossa (absence of hypoplasia of cerebellar vermis) Cystic dilatation of the 4th ventricle
Dandy-walker malformation
27
MC site of contusion
Frontal lobe
28
Small cavitary infarcts assoc with hypertension
Lacunar Infarcts Common site: Putamen
29
Neurons most SENSITIVE to ischemia
Pyramidal layer of hippocampus Pyramidal cells of cerebral cortex Cerebellar Purkinje cells
30
Infarcts in areas that lie at borders of arterial territories
Borderzone (watershed) Infarcts MC area: ACA-MCA border zone
31
MC type of intracranial aneurysm
Saccular aneurysm MC location: ACA- ACoA junction
32
Most clinically significant vascular malformation
Arteriovenous Malformation 10-30yrs, Males MC site: Posterior branches of MCA Large BV separated by gliotic tissue + shunting
33
Distended loosely organized back to back vascular channels without intervening brain tissue
Cavernous Malformation MC site: Cerebellum - shunting
34
MCC of Acute aseptic Meningitis
Enterovirus 80% Viral: Lymphocytic Chemical - neutrophil N sugar, Inc Protein, - culture
35
MC agents in Brain Abscess
Staphylococci Strepcocci
36
MC Demyelinating disorder
Multiple sclerosis
37
AI demyelinating disorder Cell mediated CD4+ Unilateral involvement of the optic nerve (optic neuritis)
Multiple Sclerosis CSF: moderate pleocytosis, mildly elevated protein, IgG, Oligoclonal IgG bands
38
MC prion disease
Creutzfeldt-Jakob disease Progressive dementia, Startle myoclonus, 7th decade, Kuru plaques Prion - rapidly progressive neurodegenerative disease
39
MCC of dementia in older adults
Alzheimer disease AB (forms plaques and tangles), Tau aggregates (stress response) Chromosome 21, 19 (ApoE) terminal event: pneumonia
40
Parkinson TRIAD
Tremor Rigidity Bradykinesia
41
Loss of Dopaminergic neurons in Substantia Nigra Dementia with Lewy Bodies Pallor of substantia nigra and locus coeruleus
Parkinson Disease Dx HM: Lewy body (a-synuclein)
42
Loss of striatal neurons that dampen motor output mutation in Glucocerebrosidase CAG trinucleotide repeat Cortical atrophy Huntingtin aggregates
Huntington Disease
43
AR, progressive ataxia, cardiomyopathy GAA trinucleotide repeat expansion (Ch9)
Friedreich Ataxia
44
Weakness and atrophy SOD1 (Ch21) mutation Bunina bodies: PAS+ Corticospinal tract degeneration
Amytrophic lateral sclerosis - loss of Upper motor neuron and Lower motor Neuron
45
most susceptible neuron in Hypoglycemia state
Dorsomedial nucleus of the thalamus
46
MC primary tumor
Gliomas: Astrocytoma, Oligodendroglioma, Ependymoma
47
High grade infiltrating astrocytoma Necrosis: Palisading Microvascular prolif: Glomeruloid
Glioblastoma Necrosis: Palisading Microvascular prolif: Glomeruloid
48
WHO IV/IV
Glioblastoma Necrosis: Palisading Microvascular prolif: Glomeruloid
49
WHO I/IV
Pilocytic astrocytoma localized low grade glioma -- found in children mural nodule Rosenthal fibers
50
WHO II/IV Fried egg appearance of cells
Oligoendroglioma mutation in IDH1 and concurrent 1p19q codeletion Chicken wire vascular network Calcifications Perineuronal Satellitosis in cortical tumors
51
WHO II/IV Perivascular rossette
Ependymoma first 2 decades of life: at 4th ventricle adult: spinal cord
52
MC embryonal neoplasm
Medulloblastoa WHO IV/IV afflicts children Homer-wright rosette small round blue cells inc mitosis
53
MC CNS neoplasm in Immnocompromised individuals
primary CNS Lymphoma MC subtype: DLBCL in immunocompromised: +EBV
54
NF2 deletion Ch22q12 Dural tail I/IV: Meningothelial, Psammoma bodies
Menigioma
55
Cytogenic Origin of Meningioma
Meningothelial cells of arachnoid
56
AD, loss of function TSC1 (Ch9) and TSC2 (Ch16) Hamartomatous cortical tubers CT: Melted candled
Tuberous Sclerosis Complex

Pathology (18 decks)

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