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STEP 1 High Yield > Neuro Shit > Flashcards

Neuro Shit Flashcards

1
Q

Sturge-Weber Syndrome

Encephalotrigeminal Angiomatosis

A

-Congenital, non- inherited (somatic) Neurocutaneous disorder
- Developmental anomaly of Neural crest derivatives
-Activating Mutation GNAQ gene
- Small vessel dz:
Portwine Stain on face in V1 V2 distribution
Ipsilateral leptomeningeal angioma -> seizures
intellectual disability
Episcleral Hemangioma with ^IOP–> glaucoma early

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2
Q

Tuberous Sclerosis

A

HAMARTOMAS

Hamartomas in CNS and Skin- autosomal dominant
Angiofibromas, 
Mitral regurgitation, 
Ash leaf skin spot (hypo or hyper pigmented)
Rhabdomyomas cardiac
Tuberous sclerosis
dOminant
Mental Retardation
Angiomyolipomas (bilateral Renal)
Seizures
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3
Q

Neurofibromatosis - 1 (NF-1)

A

Mutated Tumor Supressor Gene on Chromosome 17
- neurofibrimin a negative reulator of RAS

Autosomal dominant
Cafe au-lait spots ,
lisch nodules (pigmented iris hamartomas)
cutaneous neurofibromas (big nasty ones on back)
optic gliomas
pheochromocytoma (also MEN 2a/2b, VHL)

Neurofibromas are from neural crest.

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4
Q

Neurofibramatosis- 2 (NF-2)

A

bilateral acoustic schwannomas, juvenile cataracts, meningiomas, ependymomas,

NF2 (tumor supressor gene) = chromosome 22 = 2 (bilateral) acoustic schwannomas.

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5
Q

Von- Hippel Lindau Dz

A

Mutation on Chromosome 3 - Autosomal Dominant

Cerebellar Hemangioblastomas,
Hereditary Bilateral Renal Cell Carcinomas (clear cell)
Cavernous hemangiomas and Retinal Hemangiomas
Pheochromocytoma

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6
Q

Cingulate (Subflcine) Herniation

A

Herniation under Falx cerebri

Can compress - Anterior Cerebral Artery
- lower leg weakness and sensory loss

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7
Q

Central (Transtentorial) herniation

A
  • Caudal Displacement of brain stem
  • Rupture of paramedian basilar artery branches (DURET HEMORRHAGE)
    Early compresses- becoming ischemic and necrosis-
    later causes bleeding when ICP is corrected
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8
Q

Uncal Herniation

A

Uncus= medial temporal lobe

  • Compresses ipsilateral CNIII (blown pupil - down and out)
  • Ipsilateral PCA - (contralateral homonymous hemianopsia with macular sparing)
  • Contralateral Crus cerebri at the Kernohan notch (anterior cerebral peduncle on the other side)- giving you ipsilateral paresis “false localization”
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9
Q

Glioblastoma Multiforme

Grade IV astrocytoma

A

Common Highly malignant Primary
Found in the cerebral hemispheres-
Butterfly Glioma crosses corpus callosum

GFAP +
Features:
-Pseudopallisading (border areas of necrosis) pleomorphic cells
- Central areas of Necrosis and Hemorrhage

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10
Q

Meningioma

A

Primary brain tumor - near surface of brain and parasagittal

Arises from arachnoid cells - extra axial (external to parenchyma) with dural attachments

Present with seizures and focal signs.

Features:
Spindle cells concentrically arranged in a whorled pattern - psammoma bodies

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11
Q

Hemangioblastoma

A

Usually cerebellar- associated with VHL Dz when found with retinal hemangiomas.

Can produce eythropoietin and 2ndary polycythemia

Features:
closely arranged thin walled cappilaries minimal parenchyma

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12
Q

Schwannoma

A

Classically at the Cerebellopontine angle, often localized to CN VIII
Bilateral vestibular acoustic schwannomas are associated with NF-2 (Chr. 22) Tumor suppressor gene

S100+

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13
Q

Oligodendroglioma

A

Fried egg cells- slow growing - frontal lobes

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14
Q

Pilocytic Astrocytoma (low grade)

A

-Childhood primary tumor-
-well circumscribed, in posterior fossa in children
GFAP +
- Rosenthal Fibers- eosinophilic corkscrew fibers

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15
Q

Medulloblastoma

A
  • Childhood primary brain tumor
  • Highly malignant cerebellar tumor
  • Neuroectodermal tumor (small blue cell)
  • compress 4th ventricle causing hydrocephalus
  • Drop Metastasis to spinal cord

Homer-wright rosettes, small blue cells

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16
Q

Ependymoma

A

Ependymal cell tumor- by 4th ventricle- can cause hydrocephalus
Perivascular rosettes

Basal ciliary bodies near the nucleus.

17
Q

Craniopharyngioma

A

Most common childhood tumor, supratentorial, compresses pituitary and causes growth retardation
derived from oral/neuroectoderm Rathkes pouch
-Cholesterol crystals found in motor oil fluid

18
Q

Pinealoma

A

Tumor of pineal that can cause Perinauds syndrome- Vertical gaze palsy, with obstructive hydrocephalus. (cerebral aqueduct compression).
precocious puberty in males (Beta - HCG production)

19
Q

Alzheimers Dz

A

Elderly and Down Syndrome (APP is on chromosome 21)

Associated with:
Apo E2- Decreased risk for sporadic form
Apo E4- increased risk for sporadic form

APP, presenilin-1 and presenilin-2 - familial early onset

Features:

  • Decrease in ACh
  • Cortical Atrophy, narrow gyri and wide sulci
  • Senile plaques- extracellular Beta amyloid may cause amyloid angiopathy- intracranial hemorrhage.
  • Neurofibrillary tangles- intracellular hyperphosphorylated tau protein (microtubule stabilizing protein).
20
Q

Fronto-temporal Dementia

A

Early changes in personality and behavior (rather than memory). Beahavior and Aphasia (primary progressive variant)

May have associated movement disorders like parkinson or ALS like.

Hyperphosphorylated tau (ROUND not Tangles) and ubiquitinated TDP43.

21
Q

Lewy Body Dementia

A

Initially dementia and visual hallucinations
followed by parkinsons features
rapid onset dementia and symptoms.
Intracellular Lewy Bodies ( alpha synnuclein) primarily in the cortex NOT the substantia like parkinsons.

22
Q

Vascular Dementia

A

Multiple microinfarcts and chronic ischemia
Step wise decline in cognitive ability with late onset memory impairments.

Hippocampus and pyramidal neurons firs to go

23
Q

Multiple Sclerosis

A

Autoimmune inflammation and demyelination of CNS
Autoimmune against oligodendrocytes

Present with optic neuritis ( marcus gunn pupil), 
INO
hemiparesis
hemisensory
bladder/bowel dysfunction

Charcot Triad:
Scanning speech
Nystagmus
Intention tremor

Increased IgG level and myelin basic protein in CSF with oligoclonal bands.
Periventricular plaques, ( oligodendrocyte loss with reactive gliosis)

Tx: Natalizumab and Glatriamer and Interferon- Beta

24
Q

Acute Disseminated (postinfectious) encephalomyelitis

A

Multifocal periventricular inflammation, and demyelination after infection or vaccination.

Rapdily progressive neuro symptoms

25
Q

Acute Inflammatory Demyelinating Polyradiculopathy

A

most common type:
Guillan Barre Syndrome:
-autoimmune condition that destroys schwann cells
-inflammation, demyelination of peripheral nerves
- Symmetric ascending weakness/paralysis

CSF- Increased protein and normal cell count (albuminocytologic dissociation)
Increased protein may cause papilledema

Associated with Campylobacter and Viral infections –autoimmune attack due to molecular mimicry

26
Q

Charcot Marie Tooth

A

Hereditary motor and sensory neuropathy,
-defective production of proteins involved in the structure and function of peripheral nerves/myelin
- Autosomal dominant
- Lower extremity deformities
Pes Cavus
Hammer toes
Weakness and sensory deficits

27
Q

Krabbe Disease

A

Autosomal Recessive Lysosomal Storage disease

  • Accumulation of galactocerebroside - due to a deficiency in galactocerebrosidase enzme
  • Destroys myelin
Findings- 
peripheral neuropathy
developmental delay
optic atrohy
GLOBOID CELLS
28
Q

Metachromatic Leukodystrophy

A

Autosomal Recessive Lysosomal storage disease-

Accumulation of Sulfatides
Deficiency in Aryl-sulfatase A

impaired production and increased destruction

Central and Peripheral Demyelination
with ataxia and dementia

29
Q

Progressive Multifocal Leukoencephalopathy

A

Demyelination of CNS
Destruction of Oliogodendrocytes
Seen in AIDS pts due to reactivation of JC virus.

increased risk with Natalizumab and Rituximab

30
Q

Adrenoleukodystrophy

A

X-Linked genetic disorder
disrupts metabolims of VLC fatty acids
excessive build up in nervous system, adrenal glands and testes.

Addition of CoA to VLCFA– decreased ability to break down.

31
Q

Friedrich Ataxia

A

Autosomal recessive
Trinucleotide repeat of GAA on Chromosome 9

Defective Frataxin (iron binding protein) impaired mitochondrial function

Degeneration of multiple spinal cord tracts

  • -Posterior and Anterior spinocerebellar tracts- falls
  • -Lateral corticospinal tracts- weakness
  • -Dorsal Columns- ataxia

Pes cavus, Hammer toes, diabetes mellitus, hypertrophic cardiomyopathy, KYPHOSCOLIOSIS.
ATAXIC GAAIT

STEP 1 High Yield (4 decks)

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