Neuro Path Flashcards by Poopy McPoopson

Decreased cognitive ability, memory, or fxn with intact consciousness

Dementia

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Alzheimer’s risk is significantly increased with what disease? Why?

Down’s Syndrome

APP (21) mutation is on Chromosome 21 –> trisomy 21 increased risk of mutation

Down’s usually get Alz. by 40’s

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Early Onset Alzheimer’s mutations

APP–Chromosome 21

Prensilin-1 (chromosome 14)

Prensilin-2 (chromosome 1)

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Late onset Alzheimer’s Mutations

ApoE4–Chromosome 19

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ApoE2

Chromosome 19

Protective against Alzheimer’s

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Alzheimer’s Pathophys

Widespread cortical atrophy
Decreased ACh
Neurofibrillary Tangles
Senile Plaques–Aß Amyloid deposition

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Neurofibrillary tangles

Intracellular, hyperphosphorylated tau proteins agregates

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Spericle tau protein aggregates
Frontotemporal atrophy
Dementia
Aphasia
Parkinsonian Sx
Personality ∆

Pick’s Disease

frontotemporal dementia

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Parkinsonism
Dementia
Hallucinations

Lewy-Body Dementia

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Lewy-Body Dementia defect

Alpha-Synuclein Defect

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Rapidly progressive dementia
Starle myoclonus
Spongiform Cx

CJD

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CJD Pathophys

Prions

PrPc –> PrPsc sheet
ß-pleated sheet resistant to proteases

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Scanning Speech
Intention Tremor
Internuclear ophthalmoplegia
Nystagumus

Multiple Sclerosis

Charcot’s Triad

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Multiple Sclerosis HLA associated

HLA-DR2

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Dx of MS

T2 MRI–oligodendrocyte loss with reactive gliosis (white spots)

Oligoclonal Bands

Increased IgG in CSF

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MS Tx

ß-INF
Immunosupression
Natalizumab

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Autoimmune demyleination and inflammation of CNS

Optic neuritis
MLF syndrome
hemiparesis
hemisensory Sx
bladder/bowel incontinence

Multiple Sclerosis

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Autoimmune destruction of Schwann Cells
inflammation and demyelination of peripheral nn and mtor fibers

Ascending mm. weakness/paralysis
Facial paralysis
autonomic dysfunction

1 varient of Guillain-Barre Syndrome

Acute inflammatory demyelinating plyradiculopathy

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Acute inflammatory demyelinating polyradiculopathy labs

increased CSF protein
Normal cell count

increased protein –> papilledema

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Infections associated with Guillain-Barre Syndrome

CMV

Campylobacter jejuni

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Pathophys of Guillain-Barre Syndrome

Autoimmune attack of peripheral myelin

MOLECULAR MIMICRY

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Guillain-Barre Syndrome Tx

Plasmapheresis
IV Ig

Respiratory support until recovery

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Progressive multifocal leukoencephalopathy

Demyelination of CNS due to oligodendrocyte destroyed

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Progressive multifocal leukoencephalopathy associated with

JC virus
HIV

“Junk, cerebrum”

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Multifocal perivenular inflammation and demyelination after infxn or vaccine

Acute Disseminated (postinfectious encephalomyelitis) VZV, measles Rabies, smallpox vaccine

Metachromatic Leukodystrophy

AD lysosomal storage disease arylsulfatase A deficiency Buildup of sulfatides --> impaired production of myelin

Hereditary motor and sensory neuropathy | defective production of proteins for fxn of peripheral nn. andmyelin sheath

Charcot-Marie-Tooth Disease

Krabbe's Disease

AR lysosomal storage disease Deficiency of galactocerebrosidase Galactocerebroside destroys myelin sheath

Seizure with impaired consciousness

Complex Partial (focal)

Seizure with intact consciousness

Simple Partial (focal)

Recurrent seizures

Epilepsy

status epilepticus

seizure for > 30 minutes or recurrent seizures without regaining consciousness

Causes of seizures in Kids

``` Genetic infection trauma congenital metabolic ```

Causes of seizures in adults

Tumors Trauma stroke Infection

Causes of seizures in elderly

``` stroke tumor trauma metabolic infection ```

Seizure that effects 1 part of the brain only

Partial (focal) seizure

3 Hz No postictal confusion blank stare

Absence Seizure

Quick repetitive jerks (seizure)

Myoclonic

Alternating stiffness and movement seizure

tonic clonic seizure

Stiffening seizure

Tonic

Drop seizure

Atonic

``` Unilateral HA 15 minutes to 3 hrs repetitive brief HA male Excruciating periorbital pain with lacrimation horner's Syndrome ```

Cluster HA

Cluster HA Tx

O2 | Sumatriptan

Bilateral HA > 30 min (4-6 hrs) steady pain No photophobia

Tension HA

``` Unilateral 4-72 hrs pulsating pain nausea photophobia phonophobia ```

Migraine HA

Migraine HA Tx

Abortive Tx: Triptans Prophylactic: propranolol, topiramate

Pathophys of migraine HA

CN V irritation, meninges, or blood vessels Substance P, CGRP, or vasoactive peptides

Worst HA of my life!!!!!!!!!!!!!!!!!!!

Subarachnoid Hemorrhage

DDx Cluster HA from trigeminal neuralgia

Trig--CN V distribution < 1 minute Cluster-- > 15 min

Spinning sensation while stationary

Vertigo

Peripheral Vertigo

Inner ear etiology: semicircular canal debris, vestibular nerve infxn, Meniere's disease Delayed positional testing with HORAZONTAL nystagmus

Central Vertigo

Brain Stem or cerebellar lesion Directional change of nystagmus skew deviation diplopia dysmetria immediate positional testing with RANDOM nytagmus that ∆ direction

Port Wine stain (nevus flammeus) Ipsilateral leptomeningeal angiomas Pheochromocytoma

Sturge-Weber Syndrome

Sturge-Weber can cause

glaucoma seizures hemiparesis mental retardation

Tuberous Sclerosis HAMARTOMAS mnemonic

``` hamartomas in CNS Adenoma Sebaceum Mitral regurg Ash-leaf spot Cardiac Rhabdomyoma Autosomal Dominant Mental Retardation renal angiomyolipoma Seizures ```

``` Cafe-au-lait spots Lisch Nodules (iris) neurofibromas in skin optic glioma pheochromocytoma ```

Neurofibromatosis Type I | von REcklinghausen's Disease

Neurofibromatosis Type I mutation

NF1 Gene on Chromosome 17

Cavenous hemangioma on skin, mucosa, organs Bilateral RCC hemangioblastoma in retina, brainstem, cerebellum Pheochromocytoma

von Hippel-Lindau Disease

von Hippel-Lindau Disease mutation

VHL on chromosome 3

Pseudopalisading pleomorphic tumor Border central areas of necrosis and hemorrhage Dies < 1 yr. Crosses corpus callosum Cerebral hemispheres

Glioblastoma multiforme

#1 type of Glioblastoma

Astrocytoma GFAP+

``` Mass in convexities of hemispheres or parasagittal region Whorled Pattern, psammomma bodies arises from arachnoid cells No Sx dural attachment Benign external to brain parenchyma ```

Meningioma

Mass on CN VIII at cerebropontine angle | S-100+

Schwannoma (acoustic schwannoma)

Schwannoma Tx

resection | sterotactic radiosurgery

Meningioma Tx

resectable

#1 1º tumor in adults

Glioblastoma Multiforme

Chicken wire capillary pattern Fried egg appearing cells Calcified Frontal Lobe Mass

Oligodendroglioma

Most common pituitary adenoma

Prolactinoma bitemporal hemianopia hyper/hypo pituitarism

Adult 1º CNS tumors in order

``` Glioblastoma multiforme meningioma schwannoma oligodendroglioma pituitary adenoma ```

Childhoon 1º tumors in order

``` Pilocytic astrocytoma Medulloblastoma Ependymoma Hemangioblastoma Craniopharyngioma ```

CNS tumors with calcifications

Craniopharyngioma | Oligodenroglioma

Well circumscribed mass in posterior fossa of child GFAP+ Benign

Pilocytic astrocytoma (#1 kids CNS 1º)

Highly malignant cerebellar tumar kids primitive neuroectodermal tumor compresses 4th ventricle --> hydrocephalus Mets down spine

Medulloblastoma

Histo buzz for Pilocytic astrocytoma

Rosental Fibers Eosinophilic corkscrew fibers

Gross appearance of pilocytic astrocytoma

Cystic and solid

Medulloblastoma histo buzz

Homer-Wright Rosettes small blue cells

medulloblastoma Tx

Radiation (radiosensitive tumor)

Tumors that cause hydrocephalus due to 4th ventricle compression

Medulloblastoma | Ependymoma

Gross appearance of medulloblastoma

solid mass

Ependymoma

Tumor of ependymal cells 4th ventricle most common site hydrocephalus poor prognosis

Ependymoma histo Buzz

perivascular pseudorosettes rod shaped blepharoplasts near nucleus--basal ciliary bodes

``` Benign childhood tumor bitemporal hemianopsia confused with pituitary adenoma calcifications Supratentoral ```

Craniopharyngioma

Craniopharyngioma derrived from

Rathke's pouch remnant pharyngeal ectoderm

Herniation that compresses ACA

Cingulate (subfalcine) herniation under falx cerebri

Herniation of medial temporal lobe

Uncal

Coma and death due to compression of brain stem Herniation through foramen magnum

Cerebellar tonsillar herniation