Neuro Flashcards
Sturge-Weber syndrome
Congenital, non-inherited, inactivation of GNAQ gene, development issue with NC derivatives; affects capillary (port-wine stain), ipsi leptomeningeal angioma (seizures/epilepsy/intellectual disability), episcleral hemangioma (glaucoma)
Tuberous sclerosis
Hamartomas in CNS/skin, ash-leaf spots, rhabdomyoma/MR, mental retardation/seizures, renal angiomyolipoma
Neurofibromatosis type 1
Mutated NF1 tumor suppressor gene (neurofibromin, a negative regulator of RAS) on c17; skin tumors (from NC cells), cafe-au-lait spots, lische nodules, optic gliomas
von Hippel-Lindau disase
Hemangioblastomas (high vascularity with hyperchromatic nuclei) in retina, brain stem, cerebellum, spine; angiomatosis (e.g. cavernous hemangiomas), b/l RCC, pheo
GBM
Cerebral hemisphere, crosses corpus callosum, astrocytes stain for GFAP; histo: “pseudopalisading” pleomorphic tumor cells
Meningioma
Arachnoid cells, extra-axial, and may have dural “tail;” histo: spindle cells concentrically arranged in a whorled pattern with psammoma bodies (laminated calcification)
Hemangioblastoma
Cerebellar; a.w. von Hippel-Lindau syndrome if found with retinal angioma; produces EPO - polycythemia; histo: closely arranged, thin-walled capillaries with minimal intervening parenchyma
Schwannoma
Cerebellopontine angle, often CNVIII, S-100. If b/l vestibular, suspect NF-2
Oligodendroglioma
Frontal lobes; chicken-wire capillary pattern with oligodendrocytes with fried-egg cells
Pilocytic astrocytoma
Posterior fossa, well-circumscribed, GFAP-positive, benign; histo: rosenthal fibers (eosinophilic, corkscrew fibers), cystic
Medulloblastoma
Cerebellar, highly malignant; can compress 4th ventricle and cause hydrocephalus, can “drop metastasis” into spinal cord; histo: homer-wright rosettes, small blue cells,
Ependymoma
4th ventricle, ependymal cell, cause hydrocephalus, poor prognosis; histo: characteristic perivascular rosettes, rod-shaped blepharoplasts (basal cilary bodies) near nucleus
Craniopharyngioma
Supratentorial, derived from Rathke pouch remnants, may be confused with pituitary adenoma; histo: calcifications
Cingulate herniation
Under falx cerebri; compress ACA
Transtentorial herniation
Caudal displacement of brain step, rupture of paramedian basilar artery branches, duret hemorrhages, usually fatal
Uncal herniation
Medial temporarl lobe, compresses ipsi CN III, ipsi PCA, contra crus cerebri (ipsi paresis)
Cerebellar tonsillar herniation
Compression of brain stem, coma and death
Ventral posterolateral nucleus (VPL)
spinothalamic tract (P/T), medial lemniscus (position/propprioception); primary somatosensory cortex
Ventral posteromedial nucleus (VPM)
Trigeminal, taste pathways; primary sensory cortext
Lateral geniculate body
Opitc nerve; visual cortex (calcarine sulcus)
Medial geniculate body
Superior olivary nucleus/inferior colliculus of the pons; auditory cortex of the temporal lobe
Which sense does not have a relay nucleus in the thalamus
Smell
Three DA systems, function, and related disease
Mesolimbic-mesocortical/regulates behavior/schizophrenia; nigrostriatal/coordination of voluntary movements/ Parkinsonism; tuberinfundibular/prolactin secretion/hyperproactinemia
GABA receptors types/location
GABAa: brain, ion channel; GABAb: brain, g-protein; GABAc: retina, ion channel
BZD, barbs, ETOH moa
Bind to different GABAa receptor and facilitate inhibitory action in the CNS
Craniopharyngioma
Calcified cystic mass in suprasellar region with yellow, viscous, cholesterol-filled fluid; histo: cysts lined by stratified squamous epithelium with keratin pearls; derived from Rathke’s pouch (ectoderm that develops into anterior part of the pituitary gland); pw HA, growth failure, bitemporal hemianopia
Beta-endorphin precursor
POMC, a polypeptide precusor that also produces ACTH and MSH
S-ANS usual setup and deviants
2-neuron unit that synapse on noradrenergic receptors on target organs (viscera); the innervation of adrenal glands (no 2nd neuron) and sweat glands (both neurons use ACh)
Alzheimer disease
Neurofibrillary tangles and A-beta-amyloid plaques
Neurofibromatosis Type 1
Single-gene AD mutation of NF1 gene on c17; pw cafe-au-lai spots, multiple neurofibromas, lisch nodules (pigmented asymptomatic hamartomas of iris)
Bulimia nervosa vs anorexia
Binge-eating and either restrictive or purging compensatory behaviors; different from anorexia in that pt maintains regular body weight (>17.5 BMI) and does not have amenorrhea
Neural structures and neurons: NE, ACh, DA
nucleus ceruleus (dorsal pons), nucleus basalis of Meynert, substantia nigra
PICA
Common in pregnancy, often with ice, a.w. Fe deficiency
Thalamic stroke
Total sensory loss on contralateral side; proprioception deficit may lead to ataxia
Lacunar infarcts moa, RF
Small vessel lipohyalinosis and athersclerosis involving the pentrating vessels of the deep brain structures (BG, posterior limb of internal capsule, pons, cerebellum); uncontrolled HTN and DM
Central pontine myelinolysis presentation
Spastic quadriplegia and pseudobulbar palsy
Huntington moa, presentation, histo
AD increased CAG repeats in HD gene on c4; NMDA receptors bind glutamate and die; loss of GABA neurons in caudate nucleus and putamen; progressive dementia, aggressive behavior, choreiform
Acoustic neuromas
Sensineural hearing loss, tinnitus, facial muscle paralysis, loss of corneal reflex indicate CN 5, 7, 8 involvement; located at cerebellopontine angle
Germ cell tumors
Pineal and suprasellar regions, can cause obstructive hydrocephalus (2/2 aqueductal stenosis) and parinaud syndrome (paralysis of vertical gaze)
MCA stroke
Hemiparesis (arm>leg), and if on dominant hemisphere, Broca’s aphasia
ACA stroke
Hemiparesis (leg>arm), urinary incontinence, primitive reflexes
AICA stroke
lateral inferior pontine syndrome, which is contra loss of P/T, ipsi CN 5, 7, horner’s syndrome and cerebellar syndrome
PICA stroke
Lateral medullary syndrome (Wallenberg), which is contra P/T loss, ipsi CN 5, 8, 10, 11, and horner’s syndrome
Malignant hyperthermia
Occurs in certain genetically-susceptible individuals after inhalation anesthetics or succinylcholine. Treated with dantrolene, which blocks ryanodine receptors and prevent release of Ca into SK fibers
Diabetic neuropathy 2 moa
1) hyalinization of nerve arterioles, narrowing of lumen, ischemic injury; 2) accumulation of sorbitol and fructose in nerve axons
GBS
Segmental demyelination of peripheral nerves and endoneural inflammatory infiltrate; pw ascending flaccid paralysis (esp lungs, CN 7), areflexia, albumino-cytologic disassociation
CN4 palsy
Vertical diplopia
Werdnig-Hoffman syndrome
Anterior horn cell damage, LMN lesions; flaccid weakness, areflexia, muscle atrophy, fasciculation (“floppy child” syndrome)
Thiamine deficiency
Key coenzyme in pyruvate dehydrogenase; giving glucose without thiamine will exacerate deficiency and Wernicke encephalopathy; affect the mammillary body
Inhaled anesthetics SE
Increased cerebral blood flow and ICP; other SE include myocardial depression, hypotension, respiratory depression, and decreased renal function
Cerebral amyloid angiography
Beta-amyloid deposited into arterial wall, predisposing to rupture; related to age, unrelated to systemic amyloidosis; presents with recurrent hemorrhages in cerebral areas. Smaller, less severe than HTN hemorrhages (which tend to be in BG)
Kinesin and dynein
Microtubular transport proteins; anterograde, retrograde, respectively; Dynein also functions in ciliary and flagellar movement
B12 deficiency
Anemia with subacute, combined degeneration of the posterior and lateral spinal columns; diagnosis with increased methylmalonic acid
Old infact
Cystic cavity surrounded by wall of astrocyte processes (gliosis)
Opiod SE that do not develop tolerance
Constipation and miosis
Morphine tolerance MOA
Phosphorylation of opioid receptors, increased AC activity or increased NO activity; glutamate activation of NMDA receptors enhance morphine tolerance; NMDA receptors like ketamine block the actions of glutamate and can decreased tolerance
5-HT syndrome
Neuromuscular excitation (hyperreflexia, clonus, myoclonus, rigidity, tremor), ANS stimulation (hyperthermia, taachycardia, diaphoresis, vomiting/diarrhea), AMS (agitation, confusion); can occur with single OD or with combined MAO-I; precursor is tryptophan; can treat with cyproheptadine (antihistamine with anti-serotonergic properties)
Clozapine
Acts on D4 receptors (unlike tradiational DA agaonists, which act on D2 receptors); SE: life-threatening agranulocytosis
MS
Acute: histologically, demyelination with relative preservation of axons, accumulation of lipid-laden macrophages, astrocytosis, infiltration by lymphocytes and mononuclear cells; chronic: loss of axons, neurons, and oligodendrocytes
Primary CNS lymphoma
Diffuse large B-cell lymphoma is m/c, cells positive for B-cell markers CD-20 and CD-79a; histo: dense, cellular aggregates of uniform, atypical lymphoid cells
Phenytoin moa
Inhibits neuronal high-frequency firing by reducing Na-channels ability to recover from inactivation
Cholernergic agonist effect
GI smooth muscle (N/V, ab cramps, diarrhea), cardiac (bradycardia, negative chronotropy, ionotropy, hypotension), periphery (vasodilation/hypotension, excess sweating/salivation, lacrimation), lungs (bronchoconstriction and increased bronchial secretion)
Bethanechol
Cholinergic agaonist to stimulate peristalsis in post-operative ileus and treat non-obstructive urinary retention 2/2 atonic bladder
Carbachol, pilocarpine
Lower IOP in glaucoma (2/2 cholinergic associated miosis moving iris further from cornea and increase anterior chamber angle)
Puffer fish poisoning moa
Tetrodotoxin binds to VG Na channels in nerve and cardiac tissue to prevent Na influx and depolarization; same MOA as saxitoxin produced by dinoflagelletes in red tide
Neuron injury: 12-48 hours
“Red neurons” with eosinophilic cytoplasm, pykonic nuclei, loss of Nissl substance
Neuron injury: 24-72 hours
Necrosis and PMN
3-5 days
MO infiltration and phagocytosis
1-2 weeks
Reactive gliosis and vascular proliferation (macroscopically, liquefactive necrosis with well-demarcated soft area, 1 week - 1 month)
> 2 weeks
Glial scare (macroscopically, cystic area surrounding by gliosis, >1 month)
Ventromedial
Stimulated by leptin, mediates satiety; destruction leads to hyperphagia
Lateral
Inhibited by leptin, mediates hunger; destruction leads to anorexia
Anterior
Mediates cooling/heat dissipation via P-ANS; destruction leads to hyperthermia
Posterior
Mediates heating/heat conservation via S-ANS; destruction leads to hypothermia
Arcuate
Secretion of DA, GnRH, gonadotropin
Paraventricular
ADH, CRH, oxytocin, TRH hormone
Supraoptic
ADH, oxytocin
Suprahiasmatic
Circadian rhythm regulation and pineal gland function
AMS
Mutation in copper-zinc superoxide dismutase (SOD-1); macro: thin anterior roots, mild atrophy of the precentral gyrus; micro: degeneration of lateral corticospinal tracts, loss of neurons in the anterior horn, loss of CN, denervation atrophy of muscles;
AChase inhibitors like organophosphate compounds
Prevent breakdown of ACh in NMJ, leads to muscarinic overstimulation; depolarizing blockade (muscle weakness, fasciculations, paralysis), miosis, bradycardia, increased lacrimation/salivation
Atropine/scopalamine
Competive antagonists of muscarinic ACh receptors, leads to ACh poisoning (cutaneous vasodilation, anhidrosis, hyperthermia, mydriasis, delirium)
Botulism
Nicotinic (descending paralysis, starting with diplopia, dysphagia, difficulty speaking) and muscarinic blockade (e.g. dry mouth); enters nerve through endocytosis and binds to ACh vesicles, preventing their release
Thiopental and cause of rapid plasma decay
Redistribution
Hypocretin-1 and hypocretin-2 function, deficiency results in?
Neuropeptides produced in lateral hypothalamus that promote wakefulness and inhibito REM sleep-related phenomena; deficiency results in nacrolepsy with cataplexy (CSF= undetectable levels of hypocretin-1)
Cataplexy
Conscious, brief episodes of b/l muscle tone loss precipitated by laughter/joking; or spontaneous, abnormal facial movements without emotional triggers
Drugs that increase levodopa in the CNS
DOPA decarboxylase inhibitor (L-Dopa), COMT inhibitors like entacapone (periphery)/tolcapone (periphery and central);
Stapedius muscle
Innervated by CN7; paralysis results in hyperacusis
Alzheimer MRI finding
Hippocampus atrophy, diffuse brain atrophy
Dividing point between anterior 2/3 and posterior 1/3 of tongue
Terminal sulcus and foramen cecum
Fragile X
X-linked, pw MR, dysmoprhic facial features (large jaw, protruding ears), macroorchidism
Klinefelter syndrome
47 XXY, pw tall stature, gynecomastia, small, firm testes, infertlity