Neuro Path Flashcards
Decreased cognitive ability, memory, or fxn with intact consciousness
Dementia
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
Early Onset Alzheimer’s mutations
APP–Chromosome 21
Prensilin-1 (chromosome 14)
Prensilin-2 (chromosome 1)
Late onset Alzheimer’s Mutations
ApoE4–Chromosome 19
ApoE2
Chromosome 19
Protective against Alzheimer’s
Alzheimer’s Pathophys
Widespread cortical atrophy
Decreased ACh
Neurofibrillary Tangles
Senile Plaques–Aß Amyloid deposition
Neurofibrillary tangles
Intracellular, hyperphosphorylated tau proteins agregates
Spericle tau protein aggregates Frontotemporal atrophy Dementia Aphasia Parkinsonian Sx Personality ∆
Pick’s Disease
frontotemporal dementia
Parkinsonism
Dementia
Hallucinations
Lewy-Body Dementia
Lewy-Body Dementia defect
Alpha-Synuclein Defect
Rapidly progressive dementia
Starle myoclonus
Spongiform Cx
CJD
CJD Pathophys
Prions
PrPc –> PrPsc sheet
ß-pleated sheet resistant to proteases
Scanning Speech
Intention Tremor
Internuclear ophthalmoplegia
Nystagumus
Multiple Sclerosis
Charcot’s Triad
Multiple Sclerosis HLA associated
HLA-DR2
Dx of MS
T2 MRI–oligodendrocyte loss with reactive gliosis (white spots)
Oligoclonal Bands
Increased IgG in CSF
MS Tx
ß-INF
Immunosupression
Natalizumab
Autoimmune demyleination and inflammation of CNS
Optic neuritis MLF syndrome hemiparesis hemisensory Sx bladder/bowel incontinence
Multiple Sclerosis
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
Acute inflammatory demyelinating polyradiculopathy labs
increased CSF protein
Normal cell count
increased protein –> papilledema
Infections associated with Guillain-Barre Syndrome
CMV
Campylobacter jejuni
Pathophys of Guillain-Barre Syndrome
Autoimmune attack of peripheral myelin
MOLECULAR MIMICRY
Guillain-Barre Syndrome Tx
Plasmapheresis
IV Ig
Respiratory support until recovery
Progressive multifocal leukoencephalopathy
Demyelination of CNS due to oligodendrocyte destroyed
Progressive multifocal leukoencephalopathy associated with
JC virus
HIV
“Junk, cerebrum”
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
