Hemategenous is most common; retrograde venous spread from face to skull sinuses, post traumatic direct implantation, iatrogenic

(LP needle), local extension; sinuses, teeth, surgical site, meningomyelocele; peripheral nerves Epidural/subdural;- bacterial.fungal infection; usually direct local spread; compression surgical emergency Abscess usually osteomyelitis Subdural space can cause empyema; usually stays but can grow and cause thrombophlebitis; fever compression of artery infarction; coma; drainage; thickened dura Meningitis Leptomineninges and CSF; usually infection but can be chemical Meningoencephalitis- spread to brain Acute pyogenic (bacterial)- systemic signs; headache, photophobia, irritability, cloudy consciousness, neck stiffness; LP increased pressure; NEUTROPHILS, HIGH PROTEIN, LOW GLUCOSE (bacterial/pmn fragments and energy consumption); Neonates- e coli; group b strep Kids through adul- neiseria Old people- strep pneumo, listeria Aseptic/viral- meningitis w.o recognizeable organisms; less fulminant; high lymphocytes. Slight protein elevate; usually enterovirus; brain swelling Tuberculous- headache, malaise, confusion, vomiting; pleiocytosis (mononuclear), high protein, regular glucose; tuberculoma, arachnoid fibrosis and hydrocephalus Jelly in subarachnoid space especially base; obliterative endarteritis; caseious necrosis, giant cells Neurosyphilis- tertiary stage;10% of untreated; meningovascular neuro (meningeal; base of brain, perivascular inflammation; cerebral gummas are mass lesions with plasma cells); paretic (infestation of brain, parenchymal damage in frontal lobe, neuron death, microglia expansion) insidious loss of function, grandeur, sever dementia; more likely with HIV Tabes dorsalis- damage to sensory nerves; locomotro ataxia, loss of pain; charcot joints; lightning pain no reflexes Neuroborreliosis- lyme disease of the brain; facial nerve palsiy, mild encephalopathy Viral broad; bacterial localized Brain abscess- bacterial infections, direct implant, local extension (mastoiditis, paranasal) hematogenous spread; endocarditis gives multiple abscesses, cyanotic congenital heart disease; bronchiectasia (pulmonary sepsis) Destructive lesions; focal deficits, ICP; pleiocytosis, high protein, normal glucose; Central liquefying necrosis, fibrous capsule, edema Viral encephalitis- parenchymal and meningeal; perivascular, cell infiltrates, nodules, neuronophagia Arbovirus- tropical; equine encephaliti, west nile virus; animals are hosts, transmitted by mosquitoes; seizures, confusions, delirum, coma, reflex asymmetry, ocular palsies; CSF neutrophils/lymphocytes, high protein

Herpes- HSV1 in children and adolescents; oral lesions sometimes; mood memory behavior changes; inferior and medial regions of temporal lobes; orbital gyri; necoritizing hemorrhagic Hsv2- adult meningitis; neonates may see disseminated; Varicella zoster- chinpox; dorsal root ganglia; erupts in a dermatome (shingles) resolves or pain can last afterwards; granulomatous arteritis and infarcts in immunocompromised CMv- fetuses, immunocompromised; periventricular necrosis (brain destruction, microcephaly, ventricular calcification) inclusion cells Polio Enterovirus; paralysis; gastroenteritis then invades nervous system damaging motor neurons; paralysis, hypotonia, hyporeflexia (LMN) paralysis of diaphragm; Postpolio syndrome- decreased muscle; 25 yrs later Rabie Encephalitis from animals; ascends peripheral nerves taking a few months; malaise headache fever; progresses to cns excitement (pain, violent motor responses, convulsisons) mania, stupor, respiratory failure constrictionof laryngeal muscles (hydrophiba) HIV Direct effect on CNS and opportunistic infections Aseptic hiv 1 meningitis- 2 weeks after seroconversion in some patients; mild lymphocytic, perivascular inflammation, myelin loss Meningoencephalitis- aids dementia complex (mental slow, memory, mood) inflammation migrogial nodules; giant cells and damage in response to cyto/chemokines Vaculoalar myelopathy- spinal cord tracts, looks like subacute degeneration but b12 is normal (no virus inside lesion) Progressive multifocal leukoencephalopathy- JC virus; poliovirus; oligodendrocytes, demyelination; immunosuppressed and clinical settings; viral reactivation; ring lesions in white matter Fungi Hematogenous, direct extensions (mucor in DKA)aspergillus (septic hemorrhagic infarction) Crypto-AIDS; kill in 2 weeks; high protein, little lymph; india ink Cerebral toxoplasmosis- protozoa; AIDS; subacute quick symptoms; ring lesions; abscesses in cortex with petechiaie Cysticerocosis- tape worm tenia solium; larvae encyst; subarachnoid space; lesions can cause seizeures; ater therapy symptoms can worsen from dead organisms Prions

EEG Electrical activity; needs to be amplified; good for seizures; depth electrography nodes inside brain Superficial readings; summed currents through extracellular spaces; waves moderated by thalamus and reticular formation Hyperventilation for three minutes; photostimulation also used; can be done while sleeping

Alpha ryhtmns- eyes closed, awake; 8-12 hz; synchronized; desynch occurs because thalmic input Alpha block- eyes open, concentrating or sensory stimulation; fast, irregular, low voltage Beta wave- low amp; frontal over 12hz Theta rhythmns 4-7 hz; temporal lobes, older or dysfunctional people Delta- 1-3hz; abnormal Epilepsy- expression of dysfunction; high voltage waves; spikes and sharp waves Absence seizures (petit mal) 30 seconds unconscious without losing tone; 3/second spike Complex partial- temporal lobe; impaired awareness; focal spikes or normal Cj disease- high voltage on flat background; burst suppression Evoked potentials- repeated stimuli to figure out whats going on in frontal cortex Visual- response to stimulus is abnormal; behind chiasm different cerebral responses will be recorded Sleep Reticular formation keeps you conscious; found in tegmentum, hypothalamus, and thalamus. Serotogenic, noradrogenic; branch out to cerebral cortex;; reticulospinal tract reflexes Responds to stimulus; regardless of type, causes arousal and focus; if depressed or destroyed sensory tracts only stimulate specific areas Consciousness- requires cortex and mesencephalic reticular Syncope- sudden and brief; coma is prolonged, unresponsive (pain may see corneal movement or limb withdrawal) Stupor and obtundation- lesser depression; reaction to stimuli unlike dementia Intracranial coma- head, stroke, infection, tumor, ICP Extracranial- vascular (shock hypotension) metabolic (DKA) intoxication Sleep- reticular regularition; pons lesions by trigeminal produce hyperalertness and less sleep; this is an active physio process; Slow wave spindle stage 1 sleep- easy arousal; next stages are deeper and eeg synchronized; stage 4 low BP, HR, RR, O2 consumption; unknown control mechanism REM- asynchronous pattern EEG; dreams, atonic, triggered by dorsal midbrain/pontine tegmentum; blocked by dopa monoaminaoxidase inhibitors; Midline raphe-pons; brings sleep; serotonin modifies reticular system; norepinephrine then follows from locus cereleus resembling awakefulness; lesions distrurb sleep Rostral reticular needed for REM, but not slow wave p-chlorophenyalanine (serotonin ibhibotor) causes wakefulness somnambulism nocturnal enuresis- sleepwalking (eyes open avoiding obstacles) and bed wetting; aroused from slow wave; hypersomnia/apnea- sleepiness and apnea common in obese middle aged men who snore; obstruction of oropharyngeal airway; trachesotomy narcolepsy- intermittent uncontrollable sleep; sudden atonic; muscle weakness when stressed; sleep paralysis (cant move between sleep and arousal); hypogognic halucinations (as you come out of or go to sleep); orexin plays role

EpilepsyRecurrent seizure; eeg under sleep deprivation; if epileptic signs on first seizure should treat Aura/altered mental status (postictal confusion) First seizure usually before 20; but bimodal (old or young) Neonatal- birth defects; young adults head trauma; older- brain tumors, vascular; withdrawal from alcohol, barbituates; cocaine use in youth; hypoglycemia, hypo calcemia, hyponatremia; infections (partial and generalized secondary tonic clonic) Pseudo seizures- change in mentation with abnormal paroxysmal electrical activity; intractable seizeres, no real treatment Encephalomalacic lesions- orgonized, perinatal asphyxia, trauma or scars Porencephaly- hypoxia in 2nd trimesters; defects in mantle and cortex, usually bilateral, sylvian fissure; gliosis Schiencephaly- bilateral severe porencephaly; congenital hemiplegia, mental delay, handicap; ischemic damage leads to defective growth Perinatal asphyxia-acute gray.white matter disease/perventricular leukomalacia (necrosis/coagulation by lateral ventricles; newborns esp preemies; cerebral palsy and other developmental problems) Periventricular cysts (unilocular, matrix bleeding, macrophages); hydrocephalus; status marmoratus (basal ganglia lesions, marbled; neuronal loss and gliosis) Cortical development malformations- neuronal migration disorder; focal cerebral cortex lesions found after death; temporal lobes; environmental, intrauterine ischemia/infection Neurons dont leave ventricles; only a few reach cortex; end up in subarachnoid; late stages are disrupted Double cortin (xlinked lissencephaly)- dcx gene, microtubule associated; females are mosaic, males lissencephaly (severe malformation of misplaced neuron layer between cortex and ventricles, lack of gyri) Macroscopic neuronal heterotropias- abnormalities of neuroblast migration; microscopic (architectural disorganization, clusters) Polymicrogyria- bumps, too many gyri, overfolding reducing sulci; psychomotor retardation and seizures; diverse dispersion focalization Srpx2 (bilateral perisylvian dysphagia dyslexia), pax6, tbr2, gpr 56 ( bilateral frontopareital, cobblestone); CMV, hypoxic ischemia Focal cortical dysplasia- dysmorphic neurons (abnormal orientation, size appearance; nissl substances (RER) clump) balloon cells (abnormal cells with thin membranes; gemistocytic astrocytes (low grade tumor, nucleus pushed to side by eosinophilic material; gfap reactivity) Giant Neurons- central nuclei Immature neurons- round, large nucleus, common Localized chronic inflammatory- unilateral neuro deficit on one hemisphere; can begin in young; might be ebv or cmv; glutamate receptor 3. AI disease Destroy caudate nucleus, thin cortex, ventricular dilation; mainly t cells; no viral, no immune complexes, no BBB problems seen Encephalopathy dd Type Clinical Path

Rasmussen

Acute viral encephalitis Russian encephalitis Subacute sclerosing pan encephalitispersonality changes

No acute; epc (localized seizure that last a while); progressive, long term survival Abrupt; no epc or viral Encephalitis, epc, ataxia, dysfunction, seasonal Rademecker Eeg (sharp slow big spikes that occur every few seconds for a whle) ; measles (before two, fine then years later symptoms appear) Late onset, rapid, fatal Variable onset, possible regression

Unilateral, inflammation, cystic degeneration Bilateral; later viral inclusions and entigens Bilateral, plasma cells; tick borne Bilateral, increased plasma, viral inclusions, white matter lesions

Prion disease Epileptic enceph

Bilateral, prion protein, spongiform Bilateral, no inflammation, cystic degeneration

Pleomorphic xanthoastrocytoma- who grade II astrocytic; favorable prognosis, children young adults, superficial brain, meningeal; multicentered, disseminated; have gfap Desmoplastic infantile astrocytoma- large superficial infant tumors; leptomenigenal; males; can also pop up in kids; who grade I; desmoplastic stroma, neuroepithelial population; neuronal component known as desmoplastic infantile ganglioma Cystic appearance, meningeal, extracerebral Fibroblast SPINLES; Idaho potatos, immature; synaptophysin Dysembrioplastic neuroepithelial tumorbeningn glioneuronal; children young adults; multinodular; drug resistant, temporal lobe, hippocampus caudate, surgery cures; well demarcated with aligned axons and floating neurons(in a pool of cytoplasm); gfap Ganglioglioma/cytoma- slow grower ( can become anaplastic and progress rapidly); differentiated; mature ganglion cells and astrocytes; long standing epilepsy; mainly kids but can strike any age; cerebrum, brain stem, cerebellum, spinal cord, optic pineal Cytoma entire lesion made up of those Sturge webber dimitri syndrome- encephalofacial angiomatosis- facial vascular nevi; cortical malformation (facial nevus- birthmarks) neveus flameus (port wine supraorbital); choroidal angiomas glaucoma, buphthalmos; calcifications Hippocampal mesial temporal0 sclerosis- cellular loss with temporal lobe epilepsy; extratemporal epileptogenic; loss of neurons with gliosis Common surgey Seizures Seizure- excessive overysynchronized discharges of cerebral neurons (tend to not have prodromal symptoms, localized sensory or motor symptoms point to contralateral origin; tonic stiffening, clonic jerking; postictal state); syncope due to reduced blood supply, vasovagal

reflexes, ortho htn, decreased CO, ischemia (lightheadedness, limpness, short, if not laying down can see some jerking) Beningn febrile convulsions- kids on first day of fever (have to rule out meningitis) FH sign of recurrence; less than 15 minutes and not focalized; most have 1-2 but quick onset in younger children is associated with recurrence; most recurrence is within 2 years; prolonged convulsions need diazepam; phenoarbitol disputed Idiopathic epilepsy- 75%; 5-25 yrs old most before 18 yrs; eeg abnormalities sign of recurrence Head truma- perinatal, skull fracture depression; intracerebral subdural hematoma; if occur within a week usually self limiting; prophylaxis in first week may help Stroke- cerebral cortex stroke can produce seiures; early seizures not indivitative of chronic problems Mass lesion, meningitis Hypoglycemia- glucose below 30, or rate of falling; hyponatremia- sodium below 120; hypoerosmolar- above 330; hypocalcemia- 4-9 with tetany; uremia- rapid change not so much absolute level; hepatic encephalopathy, porphyria- anticonvulsants make worse, use bromides (gi, psychiatric, rash side effects); withdrawal and overdose; global cerebral ischemia-brief myoclonus; hypertensive; eclampsia; hyperthermia; Orthostatic htn- faint while standing Generalized Tonic- unconcsiocus, tonic contractions in limbs 10-30 seconds; arched back; tonic respiration produces noise and cyanosis; may bite tongue Clonic-muscle contraction and relaxing (jerking) 30 seconds or longer; breathing returns; saliva, relax and then flaccid; detrusor contraction or sphinteric relaxation may see incontinence (can be seen in syncope too) Postical confusion- headache, orientation takes 15 minutes or so (longer in status epilepticus; may see Babinski sign immediately; pupil reflexs intact; todds paralysiss (unilateral hemiparesis) suggests focal brain lesion Status epilepticus- 30 minutes or no pause or return to consciousness; can cause permament damage immediate treatment Myoclonic- jerks of a limb; can precede generalized; Atonic- brief; sudden loss of muscle control, fall with loose jaw and head; injuries; no aura Absence seizures (petit mal) Genetically transmitted; childhood, usually end in adolescence; brief LOC and tone doesnt always fall; motor symptoms (blinking, turning head); might have automatisms; orientation is immediate; hyperventilation can induce; 3/s during seizure spike and waves Partial seizures Simple-begin with motor, sensory auto (pallor, flushing, sweating, piloerection, dilation, vomiting, borborygmi, incontinence, dysphasia, dj vu, hallucinations) phenomen; can be localized to small part of cortex then spread (jacksonian march); consciousness kept unless spread occurs (secondary generalization) aura- right before LOC and is remembered; can have postictal state with todd paralysis (localized) Complex-temporal lobe/psychomotor seizures; consciousness, responsiveness, memory affected; aura, epigastric sensations, emotions, dj vu and hallucinations; short

generally; involuntary motor activy (automatism; picking clothes, smacking lips, repeating words) usually in face eyes or tongue then head neck sometimes limbs 5 half lives to get stable serum levels Epilepsy in older people (tumors, cerebrovascular more of them); injuries; higher risk of death (accident and underlying cause) Developmental delay; negative academics, cognitive problems, depression; restrictions; unemployment Estrogen may be proconvulsant; reduce inhibition at gaba receptor and gaba synthesis Progesterone proconvulsant- increase inhibnition at gaba receptor; attenuates glutamate, increases gaba Lower fertility rates in epileptics; seizures increase during pregnancy; treatment should be set up before pregnancy; lots of folic acid; West syndrome- infantile spasms, mental retardation, hypsarrhythmia Lennox gastaut- heigh seizure frequency and status epilepticus; mental retardation

Pharmacology of epilepsy Blocking voltage gated channels (na or ca); enhance gaba, reduce glutamate; suppression of seiaures General side effects: N/V; sedation; ataxia; rash; hyponatremia; weight change; teratogenicity; osteoporosis Benzodiazepines- bind gaba inhibitory receptors; diazepam (oral febrile seziures)/lorazepam; myoclonic, partial and generalized sseizures; lorazepam shorter half life but stays in brain longer; diazepam rectal for prolonged seizures; others are last resort Carbamazepine- reduce abnormal signal by blocking na channels inhibiting epileptic signal; parital, secondary generalized; rigeminal neuralgia and bipolar disorder; slow erratic absorption (induces its own metabolism)hyponatremia in elderly; increases absence seizures; rash, hypotension, arrhythmia, dry mouth, sedation, aplastic anemia, steven johnson; erythro and cimetidine increase levels; increases clearance of OC; spina bifida and clefts Ethosuximide- inhibit t type channels by decreaseing presynaptic ca influx; primary generalized; absence seizures Felbamate- blocking na channels; competitor for nmda, blocks ca channels, potentiates gaba,; refractory epilepsy (lennox gastaut); hepatic failure and aplastic anemia Gabapentin-GABA analog; MOA N/A; partial seizures and postherpetic neuralgia; nonlinear kinetics because of absoprtion in gut; excreted in renal unchanged, decreased in renal failure; no pharmokinetic effects good for elderly Lacosamide-na channels; stabilizes membranes and repetitive firing; binds crmp2 role unknown; partial seizures; causes euphoria like alprazolam (controlled substance) dizziness and headache Lamotrigine- block na channels and high voltage ca channels; parital, generalized, absence, lennox gastaut, bipolar; metabolized by carba and phenytoin; rash can be serious steven johnson; good with oldies Levetiracetam- adjunct therapy partial seizures, primary generalized; MOA N/A; affinity for synaptic vesicle proteins; mostly unchanged and peed out; sleep dizzy

Oxcarbazepine- prodrug reduced to 10monohydroxy; block na channels; may affect ca channels; partial onset; less metabolite effects than carbama; general symptoms; more hyponatremia; rash and cross reactivity Phenobarbital- enhances gaba; status epilepticus Phenytoin/ fosphenytoin- blocks na channels; at high concentration blocks calcium channels; partial and generalized, status epilepticus; binds to albumin; induces metabolism; saturates at low concentrations so small increases can give drug toxicity; depress vestibular and cerebellum, nystagmus, ataxia; elderly bad; gingival hyperplasia; peripheral neuropathy; osteoporosis, hirsutism, nystagmus, ataxia, megaloblastic anemia, steven johnson; very common and cheap; cleft lips/palates Fosphenytoin- prodrug converted to phenytoin; phenytoin sodium no IM as it causes necrosis; other version IM; cerebyx Pregabalin- binds to ca channels inhibiting excitation; partial onset, neuropathy, diabetic neuropathy; postherpetic, fibromyalgia; renal elimination; drowsiness, vision, weight, edema Rufinamide- na channel blocker; lennox gastaut; food increases absorption, valproate inhibits levels; reduces efficacy of birth control, short qt Tiagababine- block GABA uptake; partial onset reduction; albumin binding; fatigue, gi; caused seizures in non epileptics Topiramate- block na channels, increase cl openings by binding gaba receptor; calcium reduced, carbonic anhydrase inhibitor; partial, primary generalized, migraine; lamo increases concentration; reduced ethinyl estradiol; drowsy, weight loss, paresthesias, memory; kidney stones; carbonic anhydrase part = glaucoma, alligohidrosis, hyperthermia Valproic acid na channel blocker, block gaba transaminase and t type ca channels; partial and primary generalized; DOC myoclonic; thrombocytopenia, weight, hepatotoxic, pancreatitis teratogenic (more so than others spina bifida) Divalproex- salt form that converts in gut Vigabatrin- irreversible inhibitor of GABA transaminase; visual field loss, only in share program; infantile spasms Zonisamide- sulfonamide derivative; block na channels and ca channels; carbonic anhydrase; partial epilepsy; kidney stones; oligohydrosis Vagal nerve stimulation- small pulse generator, battery and wire around vagus; partial seizures; alternative if drug therapy fails; patient activates when they feel seizure coming Status elipticus- IV drugs; lorazepam, diazepam, fosphenytoin, phenoarbital Pregnancy- folic acid; divalproex/barbits no good; lowest dose with control should be sought; seizure intensity and frequency can increase during pregnancy;

COMA Sleeplike state; no responses, cant be aroused; no spontaneous opening; pain can elicit some response sometimes Delirium- floridly abnormal state- disorientation, fear, irritability, misperception, hallucinations, sleep wake cycle, lucid period Less alpha more delta and theta on eeg Dementia- enduring, permanent declin, organic, no reduction in arousal

Emergency- airway/ventilation (absence of cyanosis, RR >8/min; auscultation; ABG); cervical stability; catheter and blood (drugs, bleeding, hepatic/renal function; electrolytes blood sugar); dextrose (25G by 50% 50ml ), thiamine (Wernicke encephalopathy and dextrose reduce levels), naloxone (opiate overdose) flumazenil for benzo overdoese (unless epileptic; chronic abuse, or antidepressants) Sudden origin- vascular; rapid progression after hemispheric signs intracerebral bleed; longer can be tumor, abscess, chronic subdural hematoma; confusion or delirum before hand metabolic or infection Trauma- raccoon eyes, battle sign (mastoid swelling behind ear); hemotympanum; CSF rhinorrhea otorrhea (beta 2 transferrin) Blood pressure- above 250 hypertensive encephalopathy; longstanding stroke hemorrhage; can also be due to brainstem stroke or compensation of neuro problem Temphypothermia in ethanol or sedation drugs, hypoglycemia, Wernicke, hepatic, myxedema hyperthermia- heat stroke, status elipticus, inhalants, anticholinergic, pontine hemorrhage meningitis- brudzinki sign (not present in deep coma) optic fundi- signs of hemorrhage of ICP examearly diencephalic- pupils react, reflexive; response to pain makes sense late diencephalic- pupils react, reflex, pain elicits posturing (aminalistic) midbrain- pupils are fixed and midsized; non reflexive (one eye might move to water); pain stimulation elicits random movement pons/medulla- fixed non reflexives eyes; pain may cause leg to twitch pupils thalmic compression- small fixed dilated- big and unreactive to light, compression of CN3 and dilation; anticholinergic overdose; transtentorial herniation, medial temporal lobe fixed midsize- midbrain damage pinpoint- very small, opioid overdose; focal structural lesion in pons, organophosphate or opiods; some reflex seen with magnifying glass; syphlyiis pupils asymmetrical- normal finding in some dolls head- eyes should move opposite movement; can be overcome in conscious coldwater (inhibition by vestibulocochlear)- conscious fast movement away from water; coma but intact brainstem tonic deviation toward water; bilateral causes down eyes; full horizontal movement in coma suggests metabolic causes CN3 lesions transtentorial herniation only contralateral eye moves away (ipsilateral cant adduct) Pons lesion- no response Pain responses- supraortbital ridge, sternum, nails; symmetrical response (metabolic) asymmetrical probably lesions; limb abductions never reflex Decorticate (flexion of arm, shoulder adduction, extension leg/ankle) thalamus

Decerebrate- arm extension, internal rotation, leg extension; brain dysfunction has descended to midbrain; more severe dysfunction Pontine medullary lesions no response to pain other than occasional flexion at knee Supratentorial mass lesion (superficial pushing down)- dysfunction of one cerebral hemisphere (contralateral hemiparesis, sensory, aphasia) edema causes lethargy in part due to compression of other hemisphere coma; progressive anatomical signs; Subtentorial lesions- sudden onset focal signs of brainstem dysfunction; abnormal pupillary function esp asymmetric; ventialation is variable, ataxic and gasping (fully developed supra looks same as subtentorial) Diffuse encephalopathy- no focal signs; tend to slip slowly into coma; symmetric exam; reactive pupils with impaired brainstem function HEPATIC encephalopathy- ammonia overload, should be made into urea in liver and glutamine in brain Severe liver disease, portacaval shunting; hepatic necrosis after viral hepatitis; alcoholism; not necessarily jaundice; might see GI hemorrhage or ammonia from bacteria; elevated gaba reception and benzo receptors; somnolence, delirium; tense, hyperreflexia, hemiparesis; seziures infrequent Hyperventilation, respiratory alkalosis (blowing the h away); csf yellow and extra glutamine, triphasic waves on eeg; derease protein intake, gi bleed, and colonic ph with lactulose (osmotic diarrhea), and neomycin Alcohol withdrawal- tremors and hallucinations then seizures then delirum tremens (risk of Wernicke and need thiamine) glutamate receptor up, gaba receptor down Hallucintions and tremulousness- 2days of cessation; agitation, anorexia, nausea, insomnia, tachycardia, htn; mild confusion; hallucinations in 25%; lorazepam until calm can reduce symptoms Seizures- 48 hrs usually first day; usually single and if recurrent follow soon after; lorazepam sometimes given; status epilpticus or postictal state suggest other problems and observations Delirium tremens- 3-5 days post alcohol; confusion agitation, fever, sweating, tachycardia, htn; infections, pancreatitis, cardio collapse; larazepam and electorolyte balance, sometimes beta blockers for cardio symptoms Wernickes Complication of chronic alcoholism, malnutrition, cancer, bariatric surgery; thiamine defiency; neuronal loss, demyelination, gliosis; petechial hemorrhages; shrunken mammillary bodies Triad- opthalmoplegia, ataxia and confusion Symptoms- nystagmus, cn6 palsy, gaze palsy, memory problems, absent ankle jerks, macrocytic anemia Treatment- thiamine; 100mg, with dextrose; ocular better in a day and ataxia a week; other shit a month, have to watch out for korsakoff syndrome Korsakoff Thiamine defincy preceded by Wernicke usually; bilateral degeneration of dorsomedial thalamic nuclei; amnesia during Wernicke recovery; polyneuropathy, nystagmus, gait ataxia; unable to

make new memories so short term is fucked; long term small effect; apathy no insight to disorder; confabulation; thiamine can prevent and reduce deficits but unlikely to be reversed Persistent vegetative- cerebral hypoxia, ischemia, trauma, bilateral stroke After comatose wake up but not aware. After a month its persistent vegetative; spont eye opening and sleeping; brainstem and autonomic functions; no language function or motor; rare recovery after 3 months from non trauma; 12 months for trauma; Minimally conscious state- minimal environmental awareness, responsiveness sometimes recovered Locked in- reticular formation above pons; midbrain transection leaves akintetic mute conscious individual; quadriplegic, decerebrate and spasms occasionally; voluntary eye opening and movements and normal eeg; some recovery, pneumonia often kills Brain death- irreversible cessation of all function, watch out in kiddies Unresponsive to sensory input, pain, speech Absent brainstem reflexes- pupillary, corneal, oropharyngeal reflexes gone; no caloric water or dolls head movements; fail apnea test (let co2 rise and have no ventilator effort) Irreversibility- have to know cause and pathophys; drugs, hypothermia, shock cant be cause (reversible) Persistence- six hours with flat eeg, 12 hours without eeg; 24 hours for anoxic w/o eeg

Headache Traction, displacement inflammation, spasm distension of structures in head/neck; not bone or dura Venous sinuses, meningeal arteries, base dura, cn 5, 9, 10; internal carotid, sensory thalamus; pereosteum, skin, muscles, cervical nerves, eyes , ears, teeth, pharynx Cn5- radiations of anterior and middle fossae to cn5 Cn 9/10- posterior fossa C2/3- infratentorial to dermatomes New acute types Worst headache (subarachnoid Neck stiffness- meningitis Focalized on one eye glaucoma Subarachnoid hemorrhage- spont usually ruptured cerebral arterial aneurysm; usually berry aneurysm, some are familia, polycystic, coarctation of aorta; middle aged; acute BP elvation can rupture, htn might cause formation; fusiform aneurysms (circumferential dilation of cerebral arterial trunk; atherlosclerosis, ischemia, mass effect and rupture; intracranial AVM more common in men, adolescens and teens; not as intense Infections (endocarditis, mycotic aneuryms) can cause aneurysm Elevation of intracranial pressure to the point of perfusion and decreased blood flow causing LOC; can cause retinal hemorrhages

Worst headache, N/V, neck stiffness, htn, fever, confusion, stupor; rarely focal signs (CN3 palsy is exception due to compression of ipsilateral nerve) new, sometime sentinel headache occurred before (prodromal hemorrhage); chance of rebleeding Ct shows bleed infarction; LP high pressure and bloody; supernantant is green or yellow; compare first and last tubes for blood (trauma), pleocytosis due to inflammation; if going to surgery need angiography in carotids and veretebral (multiple lesions malfomrations possible) Blood can compress brain; ischemia and arterial narrowing, hydrocephalus, seizures Reduce intracranial pressure and prevent rerupture; bed rest, sedation, analgesics, htn treatment but no hypotension as perfusions is needed; nimodipine can reduce vasspasm; anticonvulsant because htn might increase and reruptures Surgery- clip neck in alert patients; early intervention (2 days) No antiplatelets Intracerebral hemorrhage- headache vomiting, confusions, focal deficits; compression causes headache; htn, avmm tumor; basal ganglia, thalamus, cerebellum pons Cerebral ischemia- stroke; mild; usually thrombosis; ipsilateral; blood flow determines pain lovation; retinal artery embolism or posterior can be confused with migraine because of visual Carotid endartectomy- cerebral hyperperfusion syndrome- htn, focal deficits, consicouness; 2nd post op day, intense throbbing, nausea Meningitis- inflammation; throbbing, bilateral, occipital, nuchal; worse sitting upright or moving or anything that increases pressure; photophobia; slow developing Seizures-postictal with lethargy, muscle soreness LP- hx of lp and postural; N/V; relieve by laying down. Use smaller needle, caffeine may help; blood can be injected to seal Spontaneous intracranial hypotension- similar to lp; sagging brain Hypertensive encephalopathy- pheochromocytoma, orgasm, monoamine oxidase inhibitors, tyramine foods (cheese) malignant htn (above 250)severe throbbing; lethargy, hemiparesis; drugs but dont lower too much to fast Coitus- subarachnoid; but almost always benign; men; dull bilateral pain, or severe during orgasm; others have lp like; indomethacin for non aneurysm can help Ophthalmic- migraine and cluster; iritis, glaucoma; acute (extremem pain and photophobia, needs dilation); close angle glaucoma (pain in globe radiating to forehead, after middle age need to be referred for tonometry Subacute- weeks to months; beware progressive Trauma- subdural, postconcussion syndrome Neck- meningitis Focal abnormalities and weight loss- tumor Vision- giant cell arteritis, intracranial htn Medications- nitrates Giant cell arteritis- temporal arteritis; systemi vasculitis; external carotid branches; granulomatous inflammation and aortic stenosis cause ischemia; women middle aged; malaise myalgia weight los fever; polymyalgia reymatic; severe pain in scalp (lying with head on pillow or combing painful, chewing sucks); esr high; thick artery, non pulsatile,

dilated; patchy effect so several sections for biopsy; prednisone should be started to avoid blindness Ophthalmic artery- permanent blindness in 50%, often bilateral; sudden, 1 month after symptoms start Intracrnail mass- new headache in middle age or later; nonspecific, mild/moderate, dull, steady; bifrontal ipsilateral worse with increased pressure or movement; worse in morning; N/V; can get very painful very quick then subside in an hour (can see drop attacks of fainting) often third ventricular colloid cysts; ct/mri Idiopathic intracranial htn- pseudotumor cerebri; increase in ICP, headache papileedema, tinnitus; impaired csf resoprtion; women in 20s, obese; diffuse headache, visual problems, optic nerve atrophy, constricting fields; several months with self limited symptoms, but pressure can remain elevated for years; slitlike ventricles, empty sella tunica; lp can help, carbonic anhydrase inhibitors, diuretics; corticosteroids; shunting for refractory med resistant Trigeminal neuralgia- tic duoloreux; facial pain usually women; microvascular compression and demyelination; unilateral and focalized to v2/3; intense short pain; can have no sumptoms for period but permanent remission Is rare; simple light touch can trigger; carbamazepine can help (oxcarbazapemine no blood dyscrasia) lamotrigine, baclofen Glossopharyngeal neuralgia- unilateral pain; oropharynx tonsillar base auditory meatus; paroxysmal, cardiac braddyarrythmia; swallowing talking can trigger, paroxysmal; young age; lidocaine, carbamazepine Postherpetic neuralgia- cn5 v1 varicella zoster; with rash; finished in 2 months but can stay for life; after 50 or older; immunocompomised; constant severe stabbing, bnurning; decreased pinprick sensitivity and less blinking and sensitivity can damage and blind; acyclovir, corticosteroids helps with herpes but not neuralgia (use tricyclic antidepressant amitriptyline; combine with phenothiazine) topical (lidocaine, capsaicin) Hypertension- invoked, no proof; paroxysmal htn sees headache sweating (pheochromocytoma) Persisten idiopathic facial pain- constant unilateral lower facial pain; not trigeminal distribution, not paroxysmal; neuro is normal, differentiate from tumor and infection; amitriptyline/phenelzine, phenytoin Chronic- years; benign cause Migraine- common recurrent headache syndrome; unilateral, pulsatilem N/V, photo/phonophobia, lassitude(weak), prodromal symptoms and aura; women, younger patients; family history (familial hemiplegic migraine AD; CADASIL hereditary stroke); intracranial vasoconstriction and extra vasodialation; might be disturbance f neuronal activity; decreased blood flow to occipital during aura then spreads (spreading depression as well with depolarization) not exactly correlated to symptoms, severity, duration; headache due to either trigeminal neurons innervating meninges and blood vessels and inflammation sending signals through nervous system OR disturbance of central pain pathways so sensory mistooken for pain (allodynia); tyramine cheese, nitrite meats, phenylethylamine chocolates, msg, OC, nitrates, menses can trigger; analgesics, serotonin receptor agonists (triptans) ; calcitonin gene related perptide receptor antagonists (telcagepant), most cause nausea, tricyclic antidepressants for >2

a week, b blockers, anticonvulsants, ccb; medication overuse (analgesics, triptans) esp women can cause syndrome; 15 days a month for three months; stop drugs give corticosteroids Classic migraine- preceded by aura; throbbing unilateral hemicranial headaches (can be bilateral or occipital) lightheaded N/V lassitude, ataxia (basilar migraine altered consiocusness gradual onset); usually a few hours; frequent during preggers and menopause; older people can have aura then no migraine Common migraine- no aura; more common, bilateral periorbital throbbing; N/V photoboia; cervical muscle contraction; lasts for hours-days, compressing carotid temporal arteries can help Chronic/transformed migraine- episodes become chronic daily events; obesity caffeine risk factors Migraine pharm Nsaid analgesics opiates for pain Prochlomrperazine- antiemetic; phenothiazine derivative Triptans and hihydroergotamine are specific (inhibition of inflammatory neuropeptides) Triptans- suma, nara, riza, ele, almo, frova, zol; effective in70%; act on peripherl nerve serotonin receptors found on vasculature; less nausea than others (riza and zolmi are best); quick clearance and headache can recur within a day; nara and almo well tolerated, frova longest; cardiac and bp problems; zecurity sumatriptan patch by battery and processor; no preggos or heart problem Ergotamine- serotonin receptor; no preggos or heart problems Dihydroergotamine- ergotamine derivative, IV, sumatriptan but with nausea Prophylaxis-two or more a month propranolol (might also be serotonin agonist), b blockers, nadolol, dont use in asthma; tricyclic antidepressants- block serotonin reuptake; secondary amine (block NE reuptake, less side effects, metabolites of tertiary amines, nortriptyline) tertiary amines (block serotonin reuptake, severe side effects, amitriptyline); sedation, dry mouth , constipation, dizziness, blurred vision, urinary retention, arrhythmia, postural hypotension onabotulininum toxin a- block NMJ and ach anticonvulsant, ccb Cluster- brief, severe unilateral constant nonthrobbing clusters; few mins to hours; always unilateral, recur same side; during sleep waking patient up; circadian periodicity; restless pacing, no probnlems between clusters; hypothalamic activation; men in 30s with no FH; begins as burning on side of nose and behind eye; alcrimation, nasal stuffiness, horners, precipitated by alcohol or vasodilators; inhalation 100% oxygen relieves, subcutaneous sumatriptan; ccb triptans, ergot alkaloids may prevent, verapamil most common, lithium might be efectie (side effects reduced with low levels N/V, diarrhea, polyuria, renal failure, hypothyroidism, tremor, dysarythria; good for old people and hypnic) melatonin and anticonvulsants also used during cluster period; prednisone at beginning of cluster then tapered is abortive; indomethacin for chronic Tension- chronic recurrent headache with no known cause; not migraine or other type; contraction of neck and scalp probablu secondary; after 20 yrs old, non throbbing, occipital

bilateral; no N/V prodrome and feels like tight band around head; women; aspirin, NSAIDS, acetaminophen, ergotamine, hihydroergotamine; proponalol for prophylaxis not SSRI; benzo helps Icepick pain- bery brief sharp severe pain in scalp outside CN5 area; paroxysmal; electric jab in a second; flinching; common in migraine/cluster sufferers; indomethacin, gabapentin, melatonin Cervical spine disease- irritation of c2 (scalp jaw), disks radiate to shoulders Sinusitis- acute sinusitis; ethmoid spenoid behind nose; increased by bending forward; vasoconstrictor nose drops Dental disease- temporomandibular joint (preauricular facial pain, limitation of jaw, tenderness, clicking; spasm of chewing muscles, teeth grinding) infection (extraction sites, unilateral, aching/burning, relief by local anesthetic; curettage/antibiotics Auras- scintillating scotomas (flickering light), visual changes common in migrane Pulsating/throbbing- migraine/tensions Intracranial mass- dull Sharp stabbing- neuritic (trigeminal) Icepick- migraine cluster giante cell arteritis Unilateral- cluster (always) migraine (most) tension (very rare) Ocular- iritis, glaucoma, cn2 inflammation; migraine/clusters Paranasal- sinuses Occipital bands- tensions headache; meningeal Burning v1- post herpetic neuralgia Lancinating v2/3- trigeminal neuralgia (tic duolourex) Dyspnea/cv symptoms- endocarditis/brain abscess N/V- migraine and post trauma Photophobia- migraine, meningitis, subarachnoid Myalgia- tension headache, systemic viral, giant cell Ipsilateral rhinorrhea- cluster Lesions sinuses- worse awakening; increase over time (lesions) Clusters- awaken patients at same time Tensions- stress, usually end of day Coughing sneezing- raised ICP intracranial mass Postural- standing horrible, nothing when laying down; no csf

Encephalopathy Vitamins- malabsoprtion, malnutrition, no supplementation, vitamin antoagnoists Vitamin b1 (thiamine pyrophosphate)- cofactor for transketolase and glucose metabolism; malnutrition, gastritis, vomiting, IV feeding, alcoholics nutrition and effects on absorption; cerebellar atrophy (ataxia, superior vermis usually) Wernicke- thiamine complication, ocular retinal hemorrhage, ocular/gaze palsy, nystagmus; autonomic hypo/hyper tension/thermis, arrhythmia, rr failure; stupor/obtundation/coma; ataxia; pyruvate elevates and lesions (periventricular/thalamus symmetrically); petechial hemorrhages; neuronal sparing with myelin damage; macrophages with lipid, hemosiderin Korsakoff- recent memory impairment, long term memory much better, confabulation, apathy, initiative loss; appears after wsernicke recovery; dorsomedial thalmic lesions B12 cobalamine-absoprtion requires intrinsic factor from parietal cells; autoimmune gastriis can affect; gastrectomy, enteritis, malabsoption, malignancies, tapeworm (fish); degeneration of spinal cord and magaloblastic anemia; myelin loss = nervous symptoms (paresthesias, weakness, less vibration sense, Romberg, ataxia, confusion, iq loss) atrophied spinal cord, grayish discoloration, myelin degeneration in posterior columns; macrophages with lipids Hepatic encephalopathy- suddenly with fulminant hepatic failure or slow with cirrhosis/shunt; nitrogen metabolism elevates ammonia; altered mental state, delirium, seizures, coma; behavior changes, asterixis, tremor, ataxia; eeg triphais or slow waves; bilirubin can make green brain (atrophied); s100 astroglia with little chromatin Hypoglycemic-drop to below 40 and brain damage in 1 hour; insulin overdose, insulinoma, endocrine disease and edema; headaches perspiration, nervous, tremulosness, confusions, myoclonic jerks, seizures and coma; longer more damage; neurons die by excitotxicity (excess stimulation), excess ammonia, aspartate, alkalosis, diminished lactate; atrophy of cortex, less nissl bodies; nesidio blastosis Uremic- renal failure, hemodialysis; tremor, asterix, jerks, seizures, stupor; edema, cortex and cerebellum damage, subdural hematoma

Hypernatremia- diabetes insipidus, osmotic diuresis, salt ingestion; venous thrombosis, hemorrhages Hyponatremia- central pontine myelinolysis due to rapid correction; pons centered Dialysis- dementia in chronic hemodialysis due to aluminum toxicity Reyes syndrome- after viral infection and aspirin; fever vomiting, liver enlargement; encephalopathy rapid and dangerous; ammonia up, low sugar; liver failure ICP; swollen brain Hyperthermia- 40 degrees (stroke); 41 see headache, vomiting, incoordination, acute respiratory, ARF, DIC, confusions, delirum seizures; degenerationof purkinje, replacement glia have heat schock protein, cerebellar dysfunction Multifocal necrotizing leukoencephalopathy- focal pontine; immunosuppression, leukemia, cytotoxic, xrays; spongiosis focalized, calcification and necrosis Cerebral calcification- globus pallidus, ammon horn dentate nucleus calcification; fahr disease (striatopallidodentate calcification; parathyroid disorders, sporadic and inherited [parathyroid is normal] ) can be asymptomatic; presents with coreoathetosis, parkinsonism, dystonia, cerebellar ataxias, seizure, retardation, dementia Central pontine myelinolysis- malnourished alcoholics, liver disease, transplants, burn victims; rapid hyponatremia treatment; acute paraparesis, dysarthria, dysphagia, like locked in; poor prognosis; grey butterfly in pons, demyelination of fiber tracts; lipid macrophages Marchiafava-bignami- chronic alcoholics; rare; interhemispheric disconnection; demyelination of genu & corpous callosum into the centum semiovale anterior commissure Optic neuropathy- nutritional defiency is slow; myelin axonal degeneration on optic nerve blindness; alcoholics Cerebral atrophy- alcoholics will have small brains, frontal lobe, neuronal loss, cognitive decline Fetal alcohol syndrome-microcephaly, facial (thin lips, incomplete jaw) growth retardation, cerebellar dysgenesis, neuroglial heterotopia (nervous tissue in wrong places) Porphyria- metabolic enzyme defiency; accumulation of porphyrins; erythropoeitic or hepatic (neuro disease); symptoms after puberty; axonal neuropathy; chromatolysis (nissl substances disintegrated) AIP- confusions, psychosis, focal deficit motor neuropathy, no photosensitivy, regular poop; hydroxymethylbilane synthase Coproporhyria- symptoms are rare; perinatal hepatosplenomagaly, jaundice; coproporyinogen oxidase Variegate porphyria- confusion psychosis delirum; motor neuropathy; AIP with hyperpigmentation; south Africa; protoporphyrinogen oxidase ALA dehydrogenase defiency- psychosis, seizures; some photosensitivity; perinatal hepatosplenomegaly; no neuropathy; porphobilinogen synthase (aminolevulinic acid dehydratase) urine pbg normal vs aip high Pancreatic encephalopathy-vasoactive peptide, hormones, acute pancreatitis; 2-5 days later with edema, petechial hemorrhages, reactive gliosis Celiac disease- cerebellar atrophy; loss of purkinje, replacement with astrocytes Mitochondrial encephalopathies leukodystrophies Sporadic/maternally inherited Deletion/duplications of mtdna

Point mutations of rrna, trna Point mutation of protein genes nDNA encodes complex II kearns sayre syndrome- before 20 yrs old; opthalmoplegia, ptosis, pigmentary retinopathy, sensonurial hearing loss, cerebellar ataxia, myopathy, heart problems, elevated csf; white matter spongiform; neuronal degeneration, calcification, red ragged myopathy MELAS- mitochondrial encephalopathy, lactic acidosis stroke; before middle age usually kids; seizures, headaches, cramps, temporary paralysis, dementia; mtdna mutation; progressive/fatal; keep having strokes at young age; trna MERRF- myoclonus epilepsy ragged red fibers; lactic acidosis, difficulty speaking/hearing, nystagmus; trna mutation; kids Lebers- hereditary optic neuropathy; progressive central vision loss; more males and adolescents; degeneration retinal ganglion cells Leigh syndrome- early childhood; brainstem damage leads to death in 2yrs; hypotonia, vomiting, ataxia, choreoathetosis, hyperventialation, hearing sight loss; ndna, mtdna; complexes fucked up so no energy; heteroplasmy may reduce symptoms Leukodystrophy-inherited demyelinationg; lysosomal, perioxisomal, mutation in myelin; damage after normal formation; white matter is translucent and grey, small, atrophied, enlarged ventricles Metachromatic leukodystrophy- lysosomal, rare, presents at any age; arylsulfatase a defiency (sulfatide in myelin buiolds up killing oligodendroglia) or saponin b; sulfatide inclusions giving orange color Krabbe- globoid cell leuko; lysosomal, AR; infants; galactocerebrosidase defiency; accumulation in oligo and schwann cells killing cells; small atrophied brain, gelatinous, engulfed oligos form multinucleated globoid cell; onion bulb schwann cell Adrenoleukodystrophy peroxisomal; rare; xlinked; sometimes adrenal dysfunction; aldp and vlcfa defects and accumulation causes instability in myelin; AI response and destruction Canavan disease- AR; aspartocetylase defiency; large brain gelatinous, demyelination, spongy brain, ladder cristae Pelizaeus merzbacher- x linked; myelin proteolipid protein gener all fucked; white matter atrophied, tiger myelin loss, peripheral myeling ok so are u fibers Alexander sporadic with male predominance; gain of function; white matter destroyed with big cavities, huge ventricles, demyelination, Rosenthal fibers in heat shock protines, blood vessels Vanishing white matter disease childhood ataxia with central hypomyelination; defect in translation initiation with destruction of oligodendrocytes and astrocytes; dementia overain insufficiency Lorenzo had adrenoleukodystophy, needed erulic acid made with canola and rapeseed oil to prevent vlfa accumulation Tumors

Primary mainly, children common usually posterior fossa (cystic cerebellar astrocytoma, medulloblastoma, brainstem glioma), adults supratentorial (gliobastoma multiforme, meningioma, ependyoma) Slow growth and differentiation dont matter as much because infiltration can have serious effects Gliomas-tumors of parenchyma Astrocytoma- diffuse show p53 mutations, overexpression of egfr, pdgf, fgf, vegf; usually progresses as more tumor suppressors are knocked out Protoplasmic- homogenous translucent gelatinous; little cytoplasm Gemistocytic- soft, homogenous; eosinophilic cytoplasm displacing nuclei Fibrillary- firm even rubbery; 80% primary brain tumor, middle aged; cerebral hemispheres, seizures, headaches, focal neurologic defecits; astrocytoma (slow progression, 5 years), anaplastic astrocytoma (rapid clinical deterioration, and growth, mainly adults, cerebral hemispheres, no necrosis or vascularity; no cell cycle regulation), gliobastoma multiforme (poor prognosis, even with resection and chemo most die in a few months, older people, can be primary from embryonal glial or anaplastic; primary is in older people, shorter history of symptoms,more egfr; secondary is younger symptoms for months, often has p53; distorts te brain as it invades; vascular supply, giant monsterous cells, , spreads via csf as well; glioblastoma sees regions with variety pseudo palisading cells (lining up around dead space)); gfap positive, transkion is blurry with infiltration at a range of places Pilocytic- relatively benign, cystic; commonest younger; optic nerve(NF1) cerebellum and third ventricles; resection not always complete and sees cysts; hypothalamic invasion is worst; hairlike bipolar cells, gfap positive, Rosenthal fibers, no necrosis/mitosis Pleomorphic xanthoastrocytoma- young; cerebral hemispheres; cysts or mural nodule, spindle cells Pilomyxoid astrocytoma- more aggressive pilocyticp; bipolar cell in myxoid background, solid with some infiltration; children, optic chaism is common; failure to thrive, developmental delay, vomiting, less head movement, headached nausea Oligodendroma- middle aged, not as common as astro; several years of complaints; cerebrum esp white matter; better prognosis (5-10 yrs); heterozygotes chromosome 1/19 (respond to chemo and radiation); gelatinous, cysts, halo cells, calcification, interlacing capillaries; anaplastic oligodendroglioma (adults, seziures, and focalized neuro signs; gelatinous, increased mitosis, necrosis, higer grade nucles anaplasia, denser cuz of calcifications and just worse; fried eggs) Ependyoma- ependymal lines ventricles and spinal cord central canal (most common spinal; sausage); in children near 4th ventricle (solid, fibrous, rosettes and pseudorosettes) in adults in spinal cord and associated with neurofibromatosis type 2; affects csf flow; posterior fossa worst place; children worse than adults, noncerbellar lesions survive better Tancytic- spinal cord Myxopapillary- filum terminale Clear cell- oligolike, aggressive

Choroid plexus papilloma- benign; infants but not congenital; papovavirus s40; firm cauliflower; hydrocephalud; ventricles especially lateral and 4; spreads through csf Carcinoma- mitoses, pleomorphic, necrotic; invasive Neuronal Central- low grade by ventricles; round nuclei, soft tumor, young adults, birght on mri, indian filling, resection Gangliocytoma-matrix with neurons only vs ganglioglioma; synaptophysin. neurophilament Ganglioglioma- low grade astrocytoma mixed with mature neurons; slow growers but glial (astrocytes) can become anaplastic; seizures Dysembryoplastic neuroepithelial low grade, childhood; slow growth, good prognosis with resection; seizures; superficial temporal lobe, floating neurons I the pools of sugar water Desmoplastic infantile astrocytoma gangliioglioma- firm cystic tumor, infiltrate meminges Medullablastoma- children, midline of cerebellum (lateral in adults); neuronal and glial markers usually undifferentiated; malignant, prognosis is bad but radiation helps with resection to save most for a while; similar to cns primitive neuroectodermal tumor; under 3yrs high risk; survival 60% Atypical teratoid rhabdoid tumor- malignant; young children; rhabdoid multiple antigen; (ini1/hsnf5 gene mutations) supra infra and spinal cord seeding; small hyperchromatic cells, cytokeratin; less than 2 years, chemo, BMT, stem cells can help Primary central nervous system lymphoma- semi rare but common in immunocompromised due to ebv (aids and transplants); non immunocompromissed more after 60; usually large b cell lymphoma; poor chemo response, aggressive and multiple sites within brina; usually no crossing lymphoma in or out; solitary deep lesions usually; bright on ct/MRI; calcification rare Intravascular b cell lymphoma- angioendotheliomatosis proliferans systemisata; high grade extranodal non hodkin lymphoma, proliferation of neoplastic lymphocytes; stroke, meningitis, myelopathy neuropathy, rash Lymphohistiocytic Langerhans- childhood, skull lesions; handschuller (bone lesions and hypothalamus) abt letterew sewe (skin lymp viscera) also see diabetes insipudus Castlemans angiofollicular lymphoid hyperplasia; hyaline vascular (unifocal, curable) or plasma cell type (multifocal); weight loss, fever anemia, dysgammaglobulinemia; extranodal rare Rosai dorfman- sinus hisitocytosis lymphadenopathy; lymphnodapathy cervical, tonsilar enlargement, nasal obstruction, many cases have other organs Germ cell tumors- usually in midline in pineal or suprasellar; common in Japanese; younger, pineal usually male; similar to gonadal germcell; more down and klinefelter; congenital (no chromo abnormalities) noncongenital (aneuploidy, hypometilated chromo x) Germinoma- clear cytoplas, focal necrosis, no mitosis, granulomas Embryonal carcinoma- mixed tumor, lacy pattern, necrotic Yolk sac- mixed germ cell; schiller duval, Choriocarcinoma- aggressive, rare; hemorrhagic, necrotic, mitotic, hcg

Meningioma- beningn adult tumors attached to dura, females, external surface of brain or ventricles (arachnoid cells) compression can have localizing effects; 8th nerve scwanomas might be neurofibromatosis type 2 or mutation without disease; easily separated from dura, can extend into bone; trauma has link Syncytial/meningothelial- whirled clusters with visible membranes, cytoplasmic clearing Fibroblastic- elongated abundant collagen; spindles Transistion- features of both Psammomatous- lots of psammoma bodies Angiomatous- vessels Microcystic- cytoplasmic processes seperted Metatatic usually carcinoma from lung breast skin (melanoma) kidney gi; usualy very demarcated with edema Familial tumor syndromes NF1- AD;neurofibromas (which can become malignant peripheral nerve sheathtumors), optic gliomas, pigmented iris nodules (lisch) caf au lait spots; high malignant transformation rate; nf1 is tumor suppressor Whole gene deletion- large early cutaneous neurofibromas; worse cognition; big hands and feet 3bp has no cutaneous neurofibromas NF2- AD; bilateral vestibular schwannomas (younger vs sporadic, entrap nerve bundles, higher proliferation) and multiple meningiomas (aggressive); gliomas ependyomas of spinal cord, non neoplastic lesions; les common, nonsense mutations more severe; schwannonas Tuberous sclerosis- AD, hamartoma and bening neoplasms, uncontrollable seizures;cysts and tumors throughout body, angiofibromas, shagreen patches, ash leaf pathes; due to TSC1/2 involved in protein synthesis Hamartomas not organized and firm like potato Von hippel-landau- AD, hemangioblastomas in cerebellym (slow gorwing lesion with fast cysting) and eye (most patients, on fundoscopy, peripheral retina, detachment); renal cell carcinoma and pancreatic cysts throughout body; resection of ymptomatic neoplasms; VHL mutations give pheochromocytoma as well Aggressive papillary middle ear tumor- tinnitus, hearing loss, adenocarcinoma Cowden lhermitte duclos AD, hammartomas, breask, medullary, uterin cancer; mucocutaenous lesions (facial trichilemmomas, acral keratosis, papillomatous; macrocephaly Lhermitte duclos- grade one neoplasm or hamartoma; cerebellar dysfunction, dysplastic gangliocytoma (enlargement) Turcot syndrome- AD, colorectal adenomatous polyps, hnpcc, fap; malignant neuroepitheliail, brain tumor polyposis syndrome Type 1- glioblastoma hnpcc, pms mutations Type 2- crail; medulloblastoma fap Li fraumeni- AD, multiple neoplasms, tp53 mutation; sarcoma before 45, first degree relative with tumor; relative with cancer before 45 or a sarcoma Brain tumors early, astrocutyic usually

Neurological paraneoplastic syndrome- any nervous system, rare; Neuroendocrine (small cell lung) thymomas, lymph/plasma cancers; antibodies in csf/blood; cerebellar degerneration; opsoclonus myoclonus Classic- cerebellum, no spioinal cord, lambert eaton, sensory neuropathy Nonclassic- brainstem encephalitis, stiffperson, myasthenia graivs, motor neuropathy

Tremor- rhythmic oscillatory movement, related to voluntary motor; disappears during sleep; postural (physiologic, enhanced by fear/anxiety, also caused by poisoning), static/rest (parkinsonism, rigidity, hypokinesia, pill rolling), intention (dont confuse with ataxia which has no ryhtmn; superior cerebellar peduncle; thalmic stimulation/surgery) Beningn essential tremor- familial, AD, dopamine receptor; later, no legs; alcohol makes better, propranolol; gradual primidone; thalamotomy or stimulation at all times while park stops during movement Asterixis- myoclonus, metabolic encephalopathy, flapping Chorea- rapid irregular jerks, can be imposed on regular; cant maintain motion (milkmaid grip) dancing gait, not present during sleep, provoked by dopa agonists Hemiballismus- proximal, unilateral powerful chorea, cures self Dystonia /athetosis-slow fucked up, dystonia is postural, not during sleep Myoclonus- sudden rapid twitchlike; general occurs during sleep/hiccup; Segmental- lesion, metabolic Iepilepsia partialis continu) Anticonvulsants Tics- abnormal quick, worse with stress Transient- children, end soon Chronic simple- treatment not needed Persistent multiple- children; remits after adolescence Chronic multiple motor/vocal; gilles de la TouretteBradykinesia/hypokinesia- slow, lacking; parkinsonimsm, fixed face, dangling arm ; freezing Parkinsonism- common as you get older; tremor, hypokinesia, rigidity, gait; idiopathic (paralysis agitans/Parkinson disease) responds to dopa; hyposmia, constipation, anxiety, depression precedes Drug induced- phenothiazine, butyrophenones, metoclopramide, reserpine Toxins- manganese (methcathinone), c2s, mptp Vascular- infarcts, gait problems Post trauma- boxers Familial- AD a synuclein Idiopathic- misfolding aggregation of a synuclein; loss of substantia nigra, lewy bodies; loss of inhibition of gaba, too much gaba cant move; flexed posture/rigidity; hypokinesia, festinating gait Treatment- block ach or increase dopa, mmonoamine oxidase inhibitor (selegiline), comt inhibitors Encephalitis lethargica- von enonomo encephalitis, doesnt happen cuz infection is gone

Lewy body disease- dementia; 50-85, function can vary over the day, hallucinations, less tremor more rigidity and bradykinesia; postural hypotension; cognitive lasts longer in Parkinson; levodopa gives hallucinations and makes cognition worse; cholinesterase inhibniotrs Progressive supranuclear palsy- idiopathic, tauopathyneurofibrillary tangles, decreased dopa; gait, opthalmoplegia (supranuclear, no vertical gaze then horizontal), pseudobulbar palsy dystonia, men, middle to old age, rarely tremor, dopa and anticholinergics, die in 5 years Corticobasal degeneration- rare nonfamilial; middle or old age; tau deposits, simulate bradykinesia rigidity, clumsiness, limb apraxia; treatment not much; aspiration pneumonia will get ya Huntington- chorea and dementia; AD, parental descent and anticipation; cell loss in cortex, gaba cells affected first; gaba, ach, and enzymes are reduced, less glucose, dopa is fine; abnormal movement or intellectual changes with degeneration starting beforehand (dementia, chorea, rigidity akinesia in youngsters (westphal variant)); stopping dopa inhibition tetrabenazine haloperidol can help Dentatorubral pallidoluysian atrophy- AD, japan; dementia, choreoathetosis, ataxia, myoclonic; atrophin cag repeat Sydenham chorea/pandaschildren,. Group a strep, rheumatic fever or polyarthritis, common in children; arteritis; preggers recurrence (OC as well); chorea unilateral, appears like restlessness, behavioral changes, OCD; bed rest andtibiotics Pandas- pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections; ocd tics with strep Primary generalized torsion dystonia- dystonic postures and nothing else; birth is normal; AD inheritance, AR, x linked; lated childhood, NTs all fucked up; legs and progresses in children; arms and axial I nadults but not as bad; exacerbated by voluntary activity; levodopa helps Tardive dyskinesia- long term treatment with antipsychotics; dopamine sesntivity; adults faces wont stop kids arms; dyskinesia after months of use see it in facilities; drug holidays Giles de la Tourette- lifelong tics; any person, before 21, remission relapse; some family history; dopamine excess; histamine are hypothesis; vocal motor tics; separate from Wilson

Down syndrome Trisomy 21; most common liveborn; 66% spont abort Nondisjunction- sc usually highly conserved; mlh foci recomb site; interruptions here can destroy nondisjunction; extra chromosome is lost at some point isolated and lost Mitotoic- mosaicism, monosomic cell dies off and trisomic continues on; higher levels of trisomy in body Most is from mother; xxy and xyy and xmonosomy have dad Ectoderm (becomes neuroectoderm unless inhibited by bmp) primitive streak; notochord neural plate ectoderm; alar sensory dorsal; basal ventral motor; suklcus limitans Meningo- not retarded; meninmyelo Arnold chiari Forebrain midbrain hindbrain telen dien mesen meten myel

Alar medulla- cochlear/vestibular, special somatic afferent; trigeminal, solitary (taste); dorsal column inferior olivary nuclei Basal medulla- vagus, glosso, ambigus, hypoglossal Cerebellum- rhobic lips Basal- edinger westphal Arnold chiari- cerebellum through foramen magnum; type 1 tonsils (ok); type 2 (swallowing, no paint/temp on back and neck, mental retardation, death) Dandy walker- virus b2 defiency; cerebllary hypoplasia; 4th ventricle dilation, occipital meningocele, posterior cysts, retardation, high death rate Holoprosencephaly- fetal alchol, patau; no midline, single ventricle; cycloplegia, retardation Charge- 2nd month, crest cells; coloboma, heart, astresia chonae, retarded groth, genitals, ears Ntd- afp; need folic acid; age, obesity, teratogens, diabetes, obesity, Anencephaly-no upper closing of spinal cord Craniorachischisis-brain and spine open Encephalocele- brain through skull Spinal bifida meningocele-meninges stickout Myelomenin-spinal cord Myelocele- just spinal cord sticiking Megaencephaly- 2.5 sd above; isolatated finding, achrondoplasia, familial, males; metabolic, neurocutananous side effects Microencephaly- brain weight 2sds below; down, fragile x (long face, big forehead, ears and balls, gaze aversion); iugr, phenylketonuria, fetal alcohol; TORCH, Porencephaly- multiple cysts Hydranencephaly- presence huge cyst Schizencephaly- cortical cleft from ventricle to cortex Holoprosencephaly- pros doesnt cleave and telen is one ventrickle; no olfactory; patau, hedgehog Agenesis of corpus callosum Lisenechepahlly- no grooves or gyri Cavum septum pellucidum- fetal, adults Tethered- thick short terminale; lower extremity Cerebral palsy- some time of injury in unterine, delivery or perinatal period; spastic, ataxic, usually semi normal lives sometimes even improve; subdural hemorrhages(, asphyxia perinatal distress; compression ruptures veings) subarachnoid (premature) subependymal (bilateral) Little disease- spastic weak 4 limbs; prematurity, slightly retarded, necrosis calcification intrapartum asphyxia White matter necrosis- infarcts in the supratentorial periventricular; premature, sepsis Psych More men to women (women more severe) ; 1.5% lower than expected Environemtn can affect iq, not parents genes Disintegrative disorder- fine then worse Leschnyhan- self inflicted harm

Velocardiofacial- bipolar psychosis Reading-speed, accuracy processing brain interconnection; poor performance for expected; 1st Writing-can me motor, and language centers; 2nd Math- organization, reasoning; 3rd; more girls Boys disruptive, girls internalize Expressive disorder- understand cant express Phonological- impairment in creating speech sounds; decrease with age; boys; speech therapy Stuttering- reptitions, porlongationg, involuntary hesitation, blocking; 5 yrs old and last longer than 6 months; worse with anxiety; males Autistic-disinterest in social, obsession with sameness; stereotypies; impaired language, before 30 months; some genetic familial relationship Social interest but lack reciprocal understanding; normal development then regression; distant aloof Retts- females, small hands and feet, gi, seizures; no verbal skill or ambulatory; mecp2 gene usually de novo (male sperm); low ne; normal for a few months, stagnate and regress, microencephaly over time; breathing problems; hand movement ADHD- deficit in attention concentration short term memory, impulsive hyperactive (attention to detail, cant maintain, cant listen, doesnt follow instruction, cant organize, avoids concentrating activities, loses important thing, easily distracted forgetful; fidgeting, cant stat seated, runs, cant play quiet, motor powered, talks, blurts, cant turn, intrudes)

Neglect- ignore an area; agnosia perceives stimulus doesnt know what it is Executive function- ability to transform thoughts in action Dorsolateral focus, flexibility, planning, predicting, problem solving Lesion- cant concentrate or try or organize Orbitofrontal- emotional integration, reward, instincts Lesion- impulsive, emotional, personality change Split brain Dysarythia- neuro injury, poor articulation Brocas- spontaneous, nonfluent, naming impaired, problems with comprehension, cant read Wernicke- spont but fluent ,errors, weird naming, cant understand, repeat, read or wright Conduction- hesitant, understands, cant repeat or read alout