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NRISE 2018 Flashcards

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1
Q

Multiple System atrophy

A

Blue Putamen

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2
Q

Effect of dystrophinopathies on sarcoglycans

A

Decreased

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3
Q

Dematiaceous Fungi (Chromomycoses)

A

Pigmented with melanin
Cladophialophora- neurotropic yeast (chromoblastomycosis)
Phaeohyphomyocosis- hyphae

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4
Q

Acute Disseminated Encephalomyelitis (ADEM)

A

Clinical: Acute, monophasic, post-URI, vaccine, M. pneumoniae, or Campylobacter
RX: steroids and IVIG
Path: Peri-venous macrophages and T-cells in white matter, perivenous zones of demyelination

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5
Q

Petechiae in hypothalamus, mammilary bodies and periaqueductal gray matter

A

Wernicke encephelopathy

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6
Q

Choroidal/Uveal melanoma poor prognostic features

A

Epitheloid morphology, mitosis, lymhpocytes, large nucleoli, tumor size, extraoccular extension, ciliary body location > choroid > iris

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7
Q

Variant CJD MRI

A

bilateral, symmetric, pulvinar/posterior thalamus, T2 hyperintenstiy

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8
Q

Sporadic CJD MRI

A

bilateral, symmetric, head of caudate and putamen, T2 hyperintensity

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9
Q

Holoprosencephaly

A

Failure of ventricular cleavage and rotation

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10
Q

Sudden fatal obstructive hydrocephalus

A

Third ventricle colloid cyst

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11
Q

Perinaud’s syndrome

A

Caused by pineal region tumors. Paralysis of upward gaze, Pseudo-Argyll Robertson pupils, convergance retraction nystagmus, eyelid retraction, conjugate down gaze

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12
Q

Wallenberg syndrome

A

Lateral medullary infracts, vertebral artery/PICA occlusion; Most common brain stem stroke
Ipsilateral cerebellars signs and cranial nerve findings
Contralateral loss of pain/temp
Hiccups

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13
Q

ALS

A

corticospinal tract degeneration

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14
Q

Anterograde amnesia

A

Cannot form new memories. Most commonly Alzheimer disease, but any bilateral disruptionof the circuit of Papez

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15
Q

Kernicterus

A

Most common cause is hemolytic disease of the newborn

Criggler Najjar: autosomal recessive inability to conjugate bilirubin

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16
Q

Traumatic/diffuse axonal injury

A

rapid acceleration-deceleration of the head

Petechiae of the parasagita WM, corpus callosum, dorsolateral brainstem

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17
Q

Menke’s desease

A

autosomal recessive, ATP7A, copper transport protein, kinky steely hair.
Copper cannot get out of enterocytes
Low serum copper and ceruloplasmin

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18
Q

Wilson’s disease

A

autosomal recessive, ATP7B, Kayser Fleischer rings, basal ganglia lesions
Copper cant be excreted from hepatocytes
Low serum copper and ceruloplasmin

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19
Q

Encephalomalacia

A

Old damage, typically ischemic

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20
Q

Cystic leukomalacia

A

periventricular WM atrophy w/wo cysts

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21
Q

Status Marmoratus

A

Firm, white basal ganglia from hypermyelination of glial scars, myelinated astrocyte processes

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22
Q

Ulegyria

A

loss of gray matter only in deeper parts of sulci, mkaing them look like mushrooms in cross-section

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23
Q

Porencephaly

A

Total focal loss of cortex and WM, making a hole from surface to ventricle, can be a developmental issue

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24
Q

Familial amyloidotic polyneuropathy

A

Mutations in transthyretin is most common causing sensorimotor neuropathy that starts peripherally and involves autonomic functions
Other forms invovle apolipoprotein A1 or gelsolin and have more sensory neruopathy

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25
Pick bodies
Lots in dentate fascia granule neurons and cortical layers II and III (Tau)
26
Palatal myoclonus
Inferior olive lesions, symmetric jerky movements
27
Cerebral edema peaks at what days post infarct
2-5 days
28
Subdural hematomas frequently develop what as they organize?
Extramedullary hematopoiesis
29
Oligodenodrocyte coiled bodies
similar morphology regardless of the tauopathy | composed of 4Rtau, non-specific, little, round blobbies
30
Zebra bodies
Mucopolysacchroidoses Fabry dz, Hurler dz, and other lysosomal storage disorders
31
Fingerprint bodies
Neuronal ceroid lipofuscinosis
32
Mesial temporal sclerosis/Hipocampal sclerosis
Secondary to long standing epilepsy | Loss of neurons, spreading of dentate neurons, gliosis, corpora amylacea, Worst in CA1. Volume loss/atrophy on MRI
33
Multiple System Atrophy
Sickle and flame shaped a-synuclein glial cytoplasmic inclusions
34
Area Postrema
Nausea and vomiting center, linked to chemical trigger zone for bloodborne toxins, no blood brain barrier (no tight junctions)
35
Nucleus of Solitary Tract
Major autonomic nucleus connected to vagus nerve; coordinates nausea and vomiting with area postrema
36
Clear cell Ependymoma
More aggresive than usual ependymomas, not infiltrative, look like oligos
37
Ependymoma EM
Zipper like intraluminal intercellular junctions and intraluminal microvilli
38
Coat's Disease
exudative retinits or retinal telangiectasis, cholesterol crystals and lipid laden macrophages in the retina and subretinal space due to breakdown of blood-retinal barrier in the endothelial cells and leakage of blood products. Retinal detachment. Clinically mimics retinal detachment
39
Parkinson plus syndromes
Multiple system atrophy (MSA) Progressive suprnuclear palsy (PSP) Corticobasal degeneration (CBD)
40
Multiple system atrophy
wet, wacky and wobbly (like normal pressure hydrocephalus) plus parkinsonism
41
Progressive Supranuclear palsy
Vertical gaze palsy and other oculomotor problems, ataxia, emotional outbursts
42
Corticobasal degeneration
movement disorders and problems with cortical processing, "alien hand syndrome", apraxia and aphasia
43
Popcorn or berry circumscribed lesion with dark halo on T2 and bright halo on T1 and SWI
Cavernous malformation, halo is due to hemosideran
44
Spinal cord AVM causes a rapidly progressing meylopathy
Foix-Alajouanine Syndrome - mechanism unknown, can be fatal
45
Congenital myopathy
Nemaline rods forming little dark haystacks in the myofibers
46
Nemaline rods forming little dark haystacks in the myofibers
Congenital myopathy
47
Foix-Alajouanine Syndrome
Spinal cord AVM causes rapidly progressing myelopathy, can be fatal, mechanism unknown
48
Uncrossed corticospinal/pyramidal tracts, right motor strip controls the right side
Trisomy 18/Edwards Syndrome
49
Medulloepithelioma of the eye
Kids, arise in the ciliary body, can be benign or malignant, can have heterologouse elements (teratoid medulloepithelioma)
50
Neo/isocortex layers
I: Molecular layer- dendrites II: External granular layer- input from neighboring cortex III: External pyramidal- projects to neighboring cortex IV: Internal granular- receives input from thalamus V: Internal pyramidal- projects to distant structures VI: Fusiform/multiform- projects to claustum
51
Molecular layer
I: dendrites
52
External granular layer
II: receives input from neighboring cortex, usually the next gyrus over
53
External pyramidal layer
III: Projects to neighboring cortex
54
Internal granular layer
IV: Receives input from thalamus
55
Internal pyramidal layer
V: Projects to distant structures- spinal cord, striatum, brain stem
56
Fusiform/Multiform
VI: Projects to claustrum, wasted space
57
Gross appearance of demyelinating plaques
Patches of gray matter within white matter
58
Trans-synaptic degeneration
Recieveing neurons die from loss of input signal after the projecting neurons are cut. This is different from Wallerian degeneration
59
Wallerian degeneration
Distal portion of the axon dies after being transected
60
Balloned Neurons
Tau Positive, Frequent in corticobasal degeneration but also found in progressive supranuclear palsy (PSP) and other neurodegen dx (Alzheimer's)
61
Topography of the visual cortex
Retinotopic, Left VC correlates to left half of retina (right half of the visual field). Retina is upside down and backward of the visula fields
62
Chiari I malformation
Cerebellar tonsils protrude through the foramen magnum, usually asymptomatic. Good
63
Chiari II/ Arnold-Chiari Malformation
Type 1 (protrusion of cerebellar tonsils through the foramen magnum) + myelomeningocele, can have syringomyelia of cervical cord. BAD
64
Chiari III.
Type II (protrusion of cerebellar tonsils + myelomeningocele), and occipital encephalocele. WORST
65
Chiari IV
Agenesis or hypoplasia of the cerebellum. Incompatible with life
66
Hypothalamic Hamartoma Symptomatology
Gelastic seizures (random laughing fits that are not connected to any stimulus) and sometimes precocious puberty and/or obesity
67
Random laughing fits and percocious puberty and/or obesity
Hypothalamic Hamartomas- Gelastic seizures
68
Cysticercosis cause
Taenia solium, fecal oral transmission of tapeworm eggs from someone with an intestinal tapeworm, not eating undercooked pork
69
Ecchordosis Physaliphora
Notochordal remnant on the anterior brainstem, usually in front of the basilar artery on the pons, usually pea sized, bubbly (like a chordoma) but not growing
70
Notochordal remnant on the anterior brainstem
Ecchordosis physaliphora- bubbly usually pea sized on the pons in front of the basilar artery

CNS (17 decks)

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