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25 Cards in this Set

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Alzheimer's Disease
most commonc cause of dementia in the eldery. Associated with senile plaques (B-amyloid core) and neurofibrillary tangles (abnormally phosphorylated tau)
Familial Alzheimers Dz
10% incidence

associated with genes on chromosomes 1, 14, 19 (APOE4 allele), and 21 (p-App gene)
What is Picks Dz?
associated with Pick bodies (intracytoplasmic inclusion bodies) and is specific for the frontal and temporal lobes
What is Huntington's disease?
dz of basal ganglia and brain stem

autosomal dominant inheritance

characterized by chorea and dementia; due to atrophy of the caudate nucleus (loss of GABAergic neurons)
What is the inheritance pattern of Huntington's dz?
autosomal dominant

Chromosome 4 - expansion of CAG repeats

CAG - Caudate loses ACh and GABA
What is Parkinson's Disease?
dz associated with Lewy's bodies and depigmentation of the substantia nigra (loss of dopaminergic neurons)

Rare cases have been linked to MPTP a contaminant in illicit street drugs
What are the symptoms of Parkinson's disease?
TRAP

Tremor at rest
cogwheel Rigidity
Akinesia
Postural instability

you are trapped in your body
Amyotrophic Lateral Sclerosis (ALS)
Degeneration of spinocerebellar motorneurons

associated with BOTH LMN and UMN

comonly known as Lou Gehrig's disease
Werdnig-Hoffmann disease
Degeneration of spinocerebellar motorneurons

presents at birth as a "floppy baby"; tongue fasciculations
Polio
Degeneration of spinocerebellar motorneurons

presents with LMN signs
Epidural Hematoma
rupture of middle meningeal artery, often 2ndary to fracture of temporal bone

Lucid interval; CT shows "biconcave disk" not crossing suture lines
Subdural hematoma
Rupture of bridging veins; venous bleeding (less pressure) with delayed onset of symptoms. Seen in elderly individuals, alcoholics, and blunt trauma.

CT shows crescent shaped hemorrhage that crosses suture lines
Subarachnoid hemorrrhage
Rupture of an aneurysm (usually a Berry aneurysm) or an AVM. Patienst complain of the "worst headache of my life." Bloody or xanthochromic spinal tap.
Parenchymal Hematoma
Caused by hypertension, amyloid angiopathy, Diabetes mellitus, and tumor.
Berry Aneurysms

What are the most common...
1. site of occurence?
2. complication?
occur at the bifurcations of the circle of willis. associated with adult polycystic kidney disease, Ehlers-Danlos syndrome, and Marfan's Syndrome

MC site is the bifructaion of the anterior communicating artery

MC complication is rupture
what is Multiple sclerosis(MS)?
demyelinating dz characterized by periventricular plaques, preservation of axons, loss of oligodendricytes, reactive astrocytic gliosis; increased protien/IgG in the CSF

increased prevelance with distance from the equator; most often affects women in their 20s and 30s
What is the presentation and course of MS?
many patients have relapsing-remitting course.

Pts can present with optic neuritis (sudden loss of vision), MLF syndrome (intranuclear opthalmoplegia),hemiparesis, hemisensory symptoms, or bladder/bowel incontinence
What is the classic triad of MS?
SIN

Scanning speech
Intention tremor
Nystagmus
Progressive Multifocal Leukoencephalopathy (PML)
associated with JC virus and seen in 2-4% of AIDS patients, is a reactivation of a latent viral infection
Postinfectious encephalomyelitis, Metachromatic leukodystrophy and Guillian-Barre syndrome are all examples of what type of disease?
Demyelinating/dysmyelinating diseases
What is Guillian Barres Syndrome (Acute Idiopathic polyneuritis)?
inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory effect less severe than motor), causing symmetric ascending muscle weakness beginning in distal lower extremities. Facial dysplegia occurs in 50% of cases.

Autonomic function may be severly affected (cardiac irregularities, HT, or hypotension)

associated with infections, inoculations, and stress, but no definitive link to pathogens
What are the findings assoc with Guillian Barre Syndrome?
elevated CSF protein with normal cell count (albumino-cytologic dissociation); elevated protien --> papilledema
What is poliomyelitis?
infection caused by the poliovirus, which is transmitted by the fecal-oral route. Replicates in the oropharynx and small intestine before spreading through the bloodstream to the CNS, where it leads to the destruction of cells in the anterior horn of the soinal cord, leading in turn to LMN destruction.
What are the symptoms of poliomyelitis and LMN lesions?
malaise, headahce, fever, nausea, abdominal pain, sore throat

LMN lesions - muscle weakness and atrophy, fasciculations, fibrillation, and hyporeflexia
What are the findings associated with Poliomyelitis?
CSF with lymphocytic pleocytosis with slight elevation of protein

virus recovered from stool or throat