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211

Entacapone

A Catechol-O-methyl-transferase (COMT) inhibitor that helps increase the bioavailability of levodopa by inhibition PERIPHERAL methylation. Tolcapone does both peripheral and central but associated with hepatotoxicity.

212

Carbamazepine mech and SE's?

Blocks voltage-gated Na+ channels in cortical neurons. Bone marrow suppression, hepatotoxic, SIADH.

213

Two major mechs of diabetic neuropathy?

Glycoslyation leads to thickness, hyalinization, and narrowing of arterial walls -> ischemic damage. Accumulating glucose as sorbital -> inc. cell osmolarity -> osmotic dmg to axons and Schwann cells. Retina, renal papilla, and scwhann cells have DEC. activity of sorbitol dehydrogenase, which prevents clearance of sorbitol into fructose.

214

Genetics affecting course of Alzheimers?

Early onset associated with APP (21), presenilin-1 (14), and preseinili-2 (1). Late onset with ApoE4 (19). ApoE2 (19) appears to be protective

215

Senile plaques vs. neurofibrillary tangles

Senile plaques are extracellular Beta-amyloid core. amyloid-Beta comes from cleavage of amyloid precursor protein (APP). Neurofibrillary tangles are INTRAcellular, hyperphosphorylated TAU protein = insoluble. Tangles correlate to degree of dementia.

216

Frontotemporal dementia vs. Lewy body dementia

Personality and parkinsonian vs. Visual hallucination -> parkinsonian. Pick bodies (spherical tau protein aggregates) + fonrtotemporal atrophy vs. alpha-synuclein defect.

217

Acute inflammatory demyelinating polyradiculopathy

Most common Guillan-Barre variant. Autoimmune condition associated w/ infection (Campy and CMV) -> destruction of Schwann cells leading to a simmering ascending muscle weakness/paralysis starting from lower extremities. Autonomic findings! Tx - respiratory support, plasmapheresis, IVIG. LP shows increased protein with NORMAL cell count and increased protein.

218

PML

Progressive multifocal leukoencephalopathy. Destruction of OLIGO's associated with JC. Found in AID's and inc. risk with natalizumab (MS drug). Usu. fatal.

219

ADEM

Acute disseminated encephalomyelitis. After infection (measles, VZV) or vaccination (rabies, smallpox), multifocal perivenular inflammation and demyelination

220

Metachromatic leukodystrophy

AR, arylsulfatase A deficiency -> buildup of sulfatides -> impaired myelin sheet production -> central and peripheral demyelintation with ATAXIA and dementia. Many-colored Arya becomes ataxic in the sulfur pits.

221

Charcot-Marie-Tooth

Progressive hereditary nerve disorder 2/2 defective protein production for structure or function of peripheral nerves or myelin. Typically AD, scoliosis, high or flat arches

222

Krabbe disease

Galactocerebrosidase (Gaucher is glucocerebrosidase) -> buildup of galactocerebroside and pyschosine -> myelin destruction -> peripheral neuropathy, dvpt delay, optic atrophy, globoid cells. Krabs are delayed.

223

Adrenoleukodystrophy

X-linked! Metabolism of VLCFA's -> buildup in nervous, adrenals (crisis), testes. ADRENO! luekodystrophy.

224

Status epilepticus

Continuous seizure for > 30 minutes or recurrent sz without regaining consciousness between sz. Emergency

225

Myoclonic vs. tonic-clonic vs. tonic vs. atonic sz.

Myoclonic are quick, repetitive jerks while tonic-clonic are grand mal alternating stiffening and movement. Tonic sz = stiffening. Atonic sz = "drop" sz.

226

Etios of sz for children?

Genetic, infection (febrile), trauma, congenital, metabolic

227

Cluster vs. Tension vs. Migraine HA's regarding DURATION

Cluster - 15min-3 hrs repetitive. Tension HA > 30 min and constant. Migraines are 4-72 hours.

228

Cluster HA vs. trigeminal neuraglia

TN produces repetitive shooting pain in distribution of CN V typically lasting < 1 min. Cluster HA pain is > 15 min.

229

Tx for cluster headaches?

Inhaled oxygen and sumatriptan vs. analgesics or amitriptyline for chronic pain

230

Peripheral vs. central vertigo

Peripheral is more common (inner ear etio). Positional testing leads to a delayed horizontal nystagmus. Central is brain stem or cerebellar with directional change of nystagmus, dipolopia, dymetria, immediate nystagmus with positional testing

231

Sturge-Weber

Sporadic, port-wine Stain, Tram track, Unilateral, Retardation, Glaucoma, GNAQ, Epilepsy. (SSTURGGE). Activating mut -> dvpt anomaly of neural crest derivatives. Port-winte in V1/V2 distribution. Ispsi leptomeningeal angioma -> sz/epilepsy. Congenital NON-inherited. GNAQ sporadic.

232

Tuberous sclerosis

HAMARTOMAS = Hamartomas in CNS/skin, Angiofibromas, MR, Ash-leaf spots, cardiac Rhabdomyoma, TS, autosomal dOminant, Mental retardation, renal Angiomyolipomas, Sz, Shagreen patches. Increased subependymal astrocytomas.

233

NF-1

Cafe-au-lait, Lisch nodules, NF's in skin, optic gliomas, pheochromocytomas. NF1 is a tumor suppressor gene (Ras regulator) on chromosome 17.

234

VHL

Cavernous hemangiomas in skin, mucosa, organs, b/l RCC, hemangioblastoma in retina, brainstem, cerebeullum, and pheochromocytomas. AD. VHL is tumor suppressor on chromsome 3 -> constitutive HIF (transcription factor) expression -> angiogenic growth factors

235

Gliobastoma multiforme

Hemispheres, "Butterfly glioma," Astrocytes stain'd for GFAP, pseudopalisading pleomorphic tumor cells with central areas of necrosis

236

Meningioma

Arachnoid cells, EXTRA-exial. Dural-tail. Often asymptomatic with sz or focal neuro. Path w/ spindle cells concentrically and Psammoma bodies

237

Hemangioblastoma

VHL associated when w/ retinal angiomas. Cerebellar. Often produce EPO => secondary polycythemia. Path - closely arranged, thin-walled caps with minimal parenchyma

238

Schwannomas

Cerebellopontine angle. Schwann cell. S100 POS. B/l acoustic schwannomas found in NF-2.

239

Oligodendoglioma

Rarer, slower growing. Frontal lobe. Path - fried egg cells (oligo's) and chicken-wire capillary pattern. Often calcified.

240

Pilocytic astrocytoma

In children, often found in posterior fossa. Well-circumscribed. Benign w/ good prognosis. Path - GFAP POS, Rosenthal fibers (corkscrew, eosinophilic), cystic on gross.