Kaplan Neurology (copied + block 3 mnem’s) – Flashcards

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Neuroectoderm: Neural Crest: Mesoderm:
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CNS neurons, ependymal cells, oligodendroglia, astrocytes schwann cells, PNS neurons microglia (Mesoderm = Microglia = Macrophage)
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nissl substance
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RER in cell body and dendrite of neuron BUT NOT AXON
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Wallerian degeneration
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axon injury -> neuron degeneration distally, axonal reaction proximally (cellular swelling, dispersal of Nissl substance)
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astrocyte marker
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GFAP (FAP to the stars!)
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Schwann cell:
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myelinates only 1 PNS axon promotes AXONAL REGENERATION (increased plasticity) increased conduction velocity via saltatory conduction b/w nodes of Ranvier where there are high concentrations of Na+ channels DESTROYED IN GUILLAIN-BARRE
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free nerve ending: meissner's corpuscles: pacinian corpuscles: merkel's disks:
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- C (slow unmyelinated); A (fast myelinated), skin/epidermis/some viscera, PAIN AND TEMP, do not adapt - large myelinated, glaborous skin, POSITION/DYNAMIC FINE SENSE, adapt QUICKLY - large myelinated, deep skin/ligaments/joints, VIBRATION AND PRESSURE - large myelinated, hair follicles, POSITION SENSE/STATIC TOUCH, adapt SLOWLY
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NTs: change in disease and location(s) of synthesis - NE: - Dopamine: - 5-HT: - ACh: - GABA:
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NE: increased in anxiety, decreased in depression - locus ceruleus - (Locus Ceruleus was a Roman emperor that struggled with depression and anxiety because of a bum kNEe) Dopamine: increased in schiz/Huntington, decreased in Parkinson/depression - ventral tegmentum and SNc 5-HT: decreased in anxiety and depression - raphe nucleus (midline tegmentum) ACh: decreased in Alz/Huntington, increased in REM sleep - basal nucleus of Meynert (immediately inferior to anterior commissure) GABA: decreased in anxiety/Huntington - nucleus accumbens
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BBB components
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tight junction b/w nonfenestrated capillary endothelial cells basement membrane astrocyte processes - glucose and AAs: slow transport through transport-mediated channels - nonpolar/lipid soluble: rapid entry via diffusion (think anesthesia) fenestrated capillaries and no BBB: - area postrema (vomiting after chemo) - OLVT (senses osmolarity) - neurohypophysis (ADH release)
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Hypothalamus functions
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TAN HATS: - thirst and water balance - adenohypophysis control - neurohypophysis release - hunger - autonomic regulation - temperature regulation sexual urges
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Hypothalamic Nuclei: - lateral - VM - anterior - posterior - suprachiasmatic nucleus
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-VM - Ventro(justhada)meal nuclei -Satiety center (lesion = obesity) Lateral - I could "laterally" eat a horse (Parks quote - lateral hypothalamic nuclei) Anterior Region - It's getting hot in(terior) here!- cooling and pArasympathetic (anterior = cooling off = AC) Posterior Region - Opposite of anterior so it's also thermal regulation (lesion = inability to thermoregulate) + sympathetic system stimulation Suprachiiasmic - above optic chiasm - thus regulates circadian rhythms based on visual input
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thalamus function
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major relay for all ascending SENSORY information EXCEPT OLFACTION
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thalamus nuclei: (input, info, destination) -VA/VL -LGB / MGB - VPL - VPM - LGN - MGN
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LGB - Lateral Geniculate body / nucleus L = Light - for seeing! MGB - Medial Geniculate body / nucleus M = music- for auditory! Visual information --> lingual gyrus through temporal lobe (thus called Meyer loop) Vs. the other loop on top going to cuneus gyrus that goes through the parietal lobe VAsaL Ganglia = VL and VA (venterolateral / ventroanterior) part of thalamus (and since involves coordinating / initiating movement - cerebellum too!) VPM = Ventropostero (Very Pretty) MOUTH = sensory from face + taste VPL = Ventropostero (Very Pretty) LEGS = sensory from body + limbs Pulvinar = crushing together - think of it as combining all of sense, visual, audio MD (mediodorsal) = Medical Doctor! Need to remember a lot of stuff (involved in memory) and you drink! (damaged in wernicke-korsakoff) AN (anterior nuclear) - closest to mammillary bodies so gets its input
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Limbic system:
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cingulate gyrus, amygdala, hippocampus, fornix, mammillary bodies, septal nucleus responsible for four F's - Feeding, Fleeing, Fighting, Feeling, and Fukking
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Cerebellum
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receives CONTRALATERAL cortical input via middle cerebellar peduncle and IPSILATERAL proprioceptive information via inferior cerebellar peduncle - input nerves: climbing and mossy fibers provides stimulatory feedback to CONTRALATERAL cortex via Purkinje fibers output via deep nuclei of cerebellum --> superior cerebellar peduncle
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deep nuclei of cerebellum
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lateral to medial: dentate, emboliform, globose, fastigial (DEGF) - Don't Eat Greasy Food lateral = voluntary movement of EXTREMITIES medial = balance, truncal coordination, ataxia - injury leads to falling on IPSILATERAL side of lesion
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Basal ganglia
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important in voluntary movements and making postural adjustments striatum = caudate (cognitive) and putanem (motor)
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Basal ganglia: Direct pathway Indirect pathway
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D1, Excitatory - cortex stimulates striatum --> release of ACh --> INHIBITS GPi/SNr --> DISINHIBITION of thalamus --> increased motion D2, Inhibitory - cortex stimulates striatum --> INHIBITION of GPe --> DISINHIBITION of STh --> STIMULATION of GPi/SNr --> INHIBITION of thalamus --> decreased motion activation of GPi/SNr --> inhibition of thalamus --> inhibition of motion activation of STh --> activation of GPi/SNr inhibition of GPe --> activation of STh (usually activated --> decreased STh --> decreased motion) SNc --> activation of D1 and inhibition of D2 (increased motion) - dopamine binds to D1 and activates it, also binds to D2 but inactivates it
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Parkinson's Disease
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That's some dope parkin' son right in the black shiet! Avoid handicap 'cuz that's an alpha-"syn" Loss of dopaminergic neurons in the substantia nigra (hypokinetic disease) Alpha-synuclein Lewy bodies in affected neurons - loss of excitatory pathway and no inhibition of inhibitory pathway TRAP: Tremor (at rest), cogwheel Rigidity, Akinesia, Postural instability
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hemiballismus
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ARABIC DANCING sudden, wild flailing of 1 arm +/- leg characteristic of CONTRALATERAL subthalamic nucleus lesion (e.g. lacunar stroke) loss of inhibition of thalamus through GPi/SNr (STh usually excites GPi/SNr --> inhibition of thalamus --> decreased motion)
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Huntington's Disease
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CAG repeat in huntingtin gene - Caudate (degeneration), ACh (decrease), GABA (decrease) chorea (sudden, jerky, purposeless movements), aggression, depression, dementia Neuronal death via NMDA-R binding and glutamate toxicity atrophy of striatal nuclei CAG: caudate loses ACh and GABA
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Athetosis
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INDIAN DANCE slow, writhing movements, esp fingers basal ganglia lesions (Huntington)
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myoclonus dystonia
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- sudden, brief muscle contraction (hiccup) (Mummy with neck back since contracted) - sustained, involuntary muscle contractions (writer's cramp)
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tremor
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essential/postural tremor: ACTION tremor (worsens when holding a posture) - autosomal dominant (often with family history), often self-medicate with alcohol to decrease the tremor - treat with B-blockers resting tremor: most noticeable DISTALLY (pill-rolling, PARKINSONS) intention tremor: slow, zigzag motion with pointing towards a target - associated with CEREBELLAR DYSFUNCTION (intermediate)
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bilateral amygdala lesion
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Kluver-Bucy syndrome (hyperorality, hypersexuality, disinhibited behavior) associated with HSV-1! (temporal lobe encephalitis)
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right parietal lobe lesion
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spatial neglect syndrome (agnosia of the contralateral side of the world)
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RAS lesion
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(in midbrain) reduced levels of arousal and wakefulness = coma
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hippocampal lesion
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anterograde amnesia (inability to make new memories) -- medial temporal lobes
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paramedian pontine reticular formation lesion (PPRF) vs. frontal eye field lesion
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eyes look AWAY from side of lesion eyes look TOWARD lesion
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locked in syndrome:
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BASILAR ARTERY INJURY - CN III is spared at most
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posterior circulation is derived from the:
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subclavian: - AICA, ASA, Basilar, PICA, PCA, vertebral
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MCA stroke
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Area of lesion: motor cortex (upper limb and face) sensory cortex (upper limb and face) Wernicke's area, Broca's area (L SIDE ONLY) Symptoms: - contralateral paralysis and loss of sensation in upper limb and face - hemineglect if on R SIDE ONLY - aphasia if on L SIDE ONLY
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ACA stroke
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Area of Lesion: - motor and sensory cortex to lower limb Symptoms: contralateral paralysis and loss of sensation to lower limb
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Lateral striate artery stroke
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Area of lesion: striatum, internal capsule Symptoms: contralateral hemiparesis/hemiplegia (pure MOTOR) Common location of lacunar infarcts, SECONDARY TO HTN
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ASA stroke
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MEDIAL MEDULLARY SYNDROME area of lesion: lateral CS tract, medial lemniscus, caudal medulla (hypoglossal nerve) symptoms: - contralateral hemiparesis (lower limbs) - decreased contralateral proprioception - ipsilateral hypoglossal dysfunction (tongue deviates ipsilaterally)
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PICA stroke
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LATERAL MEDULLARY SYNDROME (Wallenberg) area of lesion: - lateral medulla = vestibular nuclei, lateral STT, spinal trigeminal nucleus, nucleus ambiguous, sympathetic fibers - inferior cerebellar peduncle symptoms: - vomiting, vertigo, nystagmus - decreased pain and temp in limbs and face - *dysphagia, hoarseness, decreased gag reflex - ipsilateral Horner's syndrome - ataxia, dysmetria * nucleus ambiguous effects are SPECIFIC TO PICA - don't PICA hoarse you can't eat (dysphagia)
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AICA stroke
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LATERAL PONTINE SYNDROME area of lesion: - lateral pons = vestibular nuclei, FACIAL nuclei, spinal trigeminal nucleus, cochlear nuclei, sympathetic fibers - middle and inferior cerebellar peducles symptoms: - vomiting, vertigo, nystagmus - *paralysis of face, decreased lacrimation, salivation, decreased taste from anterior 2/3, decreased corneal reflex - decreased pain and temp sensation on face - decreased ipsilateral hearing - ipsilateral Horner syndrome * facial nucleus effects are specific to AICA
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PCA stroke
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area of lesion: occipital cortex, visual cortex symptoms: contralateral hemianopsia with macular sparing
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AComm stroke
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visual field defects (lesions are typically aneurysms, not strokes)
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PComm stroke
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CN III palsy (eye is DOWN and OUT) lesions are typically aneurysms, not strokes
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aneurysms
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associated with ADPKD, Ehlers-Danlos syndrome, Marfans syndrome other risk factors: advanced age, HTN, smoking, race (higher in blacks)
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irreversible brain damage after ___
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5 minutes - most vulnerable areas: hippocampus, neocortex, cerebellum, watershed areas
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irreversible neuronal injury:
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red neurons (dead red's) (12-48 hours) necrosis + neutrophils (24-72 hours) macrophages (3-5 days) reactive gliosis + vascular proliferation (1-2 weeks) glial scar (>2 weeks)
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Strokes: atherosclerosis - hemorrhagic - ischemic - TIA -
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thrombi --> ischemic atroke with necrosis forming cystic cavity with reactive gliosis - may be secondary to ischemic stroke due to reperfusion - emboli block large vessels, lacunar strokes block small vessels, may be secondary to HTN - brief reversible episode of neurologic dysfunction lasting FEWER THAN 24 HOURS (focal ischemia)
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ventricles
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lateral --> 3rd through Foramen of Monro or Ventricular Foramen 3rd --> 4th through Cerebral aqueduct 4th --> subarachnoid through foramen of Luschka (Lateral) and Magendie (medial) CSF is made in choroid plexus, reabsorbed by venous sinus arachnoid granulations
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normal pressure hydrocephalus
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Wet, Wobbly, Wacky (urinary incontinence, ataxia, dementia) does NOT result in increased subarachnoid space volume
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communicating hydrocephalus
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decreased CSF absorption by arachnoid villi --> increased ICP papilledema and herniation
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non-communicating hydrocephalus
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structural block of CSF circulation within ventricular system (e.g. stenosis of aqueduct of Sylvius or cerebral aqueduct)
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hydrocephalus ex vacuo
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appearance of increased CSF in atrophy (Alz, advanced HIV, Picks) intracranial pressure is NORMAL decreased brain parenchyma
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nerves C1-C8 exit ___ all others exit ___
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intervertebral foramina ABOVE corresponding vertebrae BELOW
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Poliomyelitis and ___:
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Werdnig-Hoffmann disease LMN lesion only! (anterior horn destruction --> flaccid paralysis) - POLIOVIRUS: fecal/oral, replicates in oropharynx and SI then enters CNS and damages the anterior horn of the spinal cord Findings: CSF with lymphocytic pleocytosis with slight protein elevation and no change in glucose - W-F DISEASE: aka infantile spinal muscular atrophy, autosomal-recessive, presents at birth as "floppy baby"
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MS spinal lesion
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mostly white matter of cervical region random and asymmetric lesions due to demyelination scanning speech, intention tremor, nystagmus
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ALS
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combined UMN and LMN lesion (anterior horn and corticospinal) NO SENSORY OR COGNITIVE DEFICITS - can be caused by defect in SOD1 - presents as fasiculations and eventual atrophy - RILUZOLE: modestly lengthens survival by decreasing presynaptic glutamate release
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ASA spinal lesion
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sparing of DCs and tract of Lissauer
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Tabes dorsalis spinal lesion
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degeneration of dorsal roots and dorsal columns impaired proprioception and locomotor ataxia associated with Charcot's joints, shooting pain, Argyll Robertson pupils, absent DTRs, positive Romberg, sensory ataxia at night
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Syringomyelia
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damage to anterior white commissure of STT --> bilateral loss of pain/temp sensation usually seen with Chiari I type 1 and 2
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VitB12 neuropathy, ___, ___, and ___
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Vit E deficiency, Friedreich's ataxia, subacute combined degeneration demyelination of DCs, lateral CS tracts, spinocerebellar tracts ataxic gait, hyperreflexia, impaired position and vibration sense BOTH SENSORY AND MOTOR Friedrich's Ataxia: autosomal recessive TRINUCLEOTIDE REPEAT (GAA), encodes FRATAXIN --> impairment of mitochondrial functioning - staggering gait, frequent falls, pes cavus, hammer toes, HYPERTROPHIC CARDIOMYOPATHY - presents in childhood with SCOLIOSIS
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Horner syndrome
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Ptosis: slight drooping of eyelid, SUPERIOR TARSAL muscle Anhidrosis: absent sweating and flushing Miosis: pupillary constriction Lesion of spinal cord ABOVE T1!!
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Primitive reflexes: moro - rooting - sucking - palmar and plantar - babinski -
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usually diappear within a year of life, may reemerge following frontal lobe lesion - abduct/extend limbs when startled and then draws them together - movement of head toward one side if cheek/mouth is stroked - sucking response when roof of mouth is touched - curling of fingers/toes if palms of hands/feet are stroked - dorsiflexion of large toe
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CNs that lie medially: CN nuclei in midbrain: CN nuclei in pons: CN nuclei in medulla:
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3, 6, 12 3, 4 5, 6, 7, 8 9, 10, 11, 12
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Parinaud syndrome
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paralysis of conjugate vertical gaze due to lesion in superior colliculi (e.g. pinealoma)
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oculomotor function
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eye movement (SR, IR, MR, IO) pupillary constriction (PS: E-W nucleus, muscarinic-R) accommodation eyelid opening (levator palpebrae)
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facial nerve function
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facial movement taste from anterior 2/3 of tongue lacrimation, salivation (submandibular and sublingual) eyelid closing (orbicularis oculi) STAPEDIUS muscle in ear
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glossopharyngeal nerve function
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taste from posterior 1/3 of tongue swallowing, salivation (parotid) monitoring CAROTID BODY and sinus chemo/baroreceptors STYLOPHARNGEUS (elevates pharynx and larynx)
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vagus nerve function
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taste from epiglottic region swallowing palate elevation midline uvula, talking, coughing thoracoabdominal viscera monitoring AORTIC arch chemo/baroreceptors
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corneal reflex
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afferent: V1 (nasociliary branch: levator palpebrae) efferent: VII (temporal branch: orbicularis oculi)
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pupillary reflex
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afferent: II efferent: III
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gag reflex
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afferent: IX efferent: IX and X
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Vagal nuclei
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nucleus Soltarius: visceral Sensory information (VII, IX, X) nucleus aMbiguous: Motor innervation of pharynx, larynx, upper esophagus (IX, X, XI) dorsal motor nucleus: sends autonomic (parasympathetic) fibers to heart/lungs/upper GI
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conductive hearing loss weber test? sensorineural hearing loss weber test?
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Weber Test - WEBER it's louder in this ear or the other? Thus Rinne is the other one (mastoid bone ) - localizes to AFFECTED SIDE - localizes to UNAFFECTED SIDE
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Bell's palsy
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complete destruction of facial nucleus of branchial efferent fibers (facial nerve proper) peripheral ipsilateral facial paralysis with inability to close eye seen as complication in: - AIDS, Lyme disease, Herpes simplex, Sarcoid, Tumors, Diabetes
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UMN facial lesion LMN facial lesion
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contralateral paralysis of lower face only ipsilateral paralysis of upper AND lower face
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near vision distant vision
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ciliary muscle contracts (muscarinic receptor) --> lens relaxation ciliary muscle relaxes --> lens flattening
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retinal artery occlusion
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acute, PAINLESS monocular loss of vision pale retina and cherry red macula
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glaucoma
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impaired flow --> increased pressure --> optic disk atrophy with CUPPING open/wide angle: obstructed outflow, associated with myopia/increased age/AA race, more common, SILENT and PAINLESS closed/narrow angle: obstructed flow b/w iris and lens --> increased pressure behind iris, VERY PAINFUL/decreased vision/frontal headache - DO NOT GIVE EPI!! (B-agonist --> increased AH formation)
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risk factors for cataracts
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age, smoking, EtOH, sunlight, classic galactosemia, galactokinase deficiency, diabetes (sorbitol), trauma, infection
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Eyes: CN III damage = CN IV damage = CN VI damage =
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eye looks DOWN AND OUT, ptosis, pupillary dilation, loss of accommodation eye drifts UPWARD causing vertical diplopia (problems reading newspaper or going downstairs) medially directed eye
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pupillary light reflex
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CN II --> pretectal nucleus in midbrain --> activation of bilateral E-W nucleus --> CN III bilateral constriction via clilary gangion and pupillary constrictor muscle
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retinal detachment
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separation of neurosensory layer of retina from pigment epithelium --> degeneration of photoreceptors --> VISION LOSS flurries: no rods or cones in affected area MEDICAL EMERGENCY
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age-related macular degeneration
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causes loss of central vision (scotomas) dry: atrophic, slow, due to fat deposits, gradual decrease in vision wet: rapid, due to neovascularization
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MLF syndrome
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medial longitudinal fasciculus MEDIAL RECTUS PALSY on attempted lateral gaze (convergence is normal) nystagmus in abducting eye seen with MS!
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Alzheimer's disease
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Alzheimer's Disease - Don't want A beta with AmyloiD who's hyper with TP (imagine crazily tp'ing) If amyloid broken down with beta then it's Aβ-Amyloid, which can't be broken down and accumulates) Hyper-phosphorylated tau protein in neurofibrillary tangles (NFT) Increased Risk? Apo ε4 vs ε2 (reduced risk) 4 > 2 so higher risk! Early onset of AD? Familial examples... Presenilin 1/2 mutations - self-explanatory - have it and you go crazy Down to Drink? Down's syndrome = chromosome 21 Amyloid Precursor protein (APP) on chrom 21...makes sense Histology: - widespread cortical atrophy - decreased ACh - Senile plaques: EXTRACELLULAR B-amyloid core, may cause amyloid angiopathy --> intracranial hemorrhage (Abeta amyloid is synthesized by cleaving amyloid precursor protein) - Neurofibrillary tangles: INTRACELLULAR, abnormally phosphorylated tau protein = insoluble cytoskeletal elements (tangles correlate to degree of dementia)
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Pick's Disease
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Pick round poo's with TP (round tau proteins) and pick the frontal and temporal cortex Has round tau proteins (vs neurofibrillary tangles in AD) and affects frontal/temporal. dementia, aphasia, parkinsonian aspects, CHANGE IN PERSONALITY spares parietal lobe and posterior 2/3 of superior temporal gyrus Histology: - pick bodies (intracellular, aggregated tau protein) - frontotemporal atrophy Dx: irritability, anger, etc...
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Lewy Body Dementia
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parkinsonism with dementia and HALLUCINATIONS alpha-synuclein defect
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CJD
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rapidly progressive (weeks to months!!) dementia with MYOCLONUS (sudden, brief muscle contraction) Histology: - spongiform cortex - prions (PrPc --> PrPsc sheet which is resistant to proteases)
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other less common causes of dementia:
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Multi-infarct (2nd most common in elderly) Syphilis HIV Vit B12 deficiency Wilson's disease
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Multiple Sclerosis
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Autoimmune, demyelination of CNS Can present with optic neuritis (sudden loss of vision), MLF syndrome (MR paralysis), hemiparesis, hemisensory symptoms, bladder/bowel incontinence Relapsing and remitting! Findings: increased protein (IgG) in CSF, OLIGOCLONAL BANDS ARE DIAGNOSTIC - MRI is gold standard - periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons Treatment: B-IFN, immunosuppression, natalizumab (against alpha-4 integrin) - baclofen for muscle spasm
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Guillain-Barre syndrome
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(acute inflammatory demyelinating polyradiculopathy) Inflammation and demyelination of PERIPHERAL NERVES and MOTOR FIBERS of VENTRAL ROOTS (more motor than sensory) --> symmetric ascending muscle weakness Facial paralysis in 50% of cases, autonomic function may be severely affected Findings: increased CSF protein with normal cell count (albuminocytologic dissociation) - increased protein --> papilledema Associated with infections: autoimmune attack of peripheral myelin due to molecular mimicry (CAMPYLOBACTER JEJUNI or herpes), inoculation, stress, but no real link to pathogens Tx: resp support, plasmapheresis, IV immune globulins (to overcome anti-Ab)
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Progressive Multifocal Leukoencephalopathy
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PML demyelination of CNS due to destruction of oligodendrocytes associated with JC virus seen in 2-4% of AIDS patients (reactivation of latent viral infection) RAPIDLY PROGRESSIVE, usually fatal LOOKS A LOT LIKE MS!!!
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Acute disseminated (postinfectious) encephalomyelitis
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multifocal perivenular inflammation and demyelination after infection, e.g. chicken pox measles, or certain vaccinations, e.g. rabies, small pox
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Charcot-Marie-Tooth
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hereditary motor and sensory neuropathy group of progressive hereditary nerve disorders related to defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath - deformities in feet are common (slapping gait)
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Partial seizures
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1 area of the brain most commonly originates in the medial temporal lobe often preceded by an aura, can secondarily generalize SIMPLE PARTIAL: consciousness intact, motor/sensory/autonomic/psychic COMPLEX PARTIAL: impaired consciousness
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Generalized seizures
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diffuse neural involvement ABSENCE: petit mal, 3 Hz, no postictal confusion = blank stare MYOCLONIC: quick, repetitive jerks TONIC-CLONIC: grand mal, alternating stiffening and movement TONIC: stiffening ATONIC: "drop" seizures (falls to the floor), commonly mistaken for fainting (frequency down the list: childhood --> adulthood)
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Causes of seizures by age:
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children: genetic, infection (febrile), trauma, congenital, metabolic adults: tumors, trauma, stroke, infection elderly: stroke, tumor, trauma, metabolic, infection
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seizure medication for pregnant women? seizure medication for absent seizures?
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phenobarbital ethosuzimide
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Headache
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pain due to irritation of structures like dura, cranial nerves, or extracranial structures, NOT THE BRAIN ITSELF (no pain receptors) MIGRAINE: unilateral, more common in females, nausea/photophobia/phonophobia, +/- aura - due to irritation of CN V and release of substance P, CGRP, and vasoactive peptides - Tx: propranolol, NSAIDs, sumatriptan for ACUTE mgmt TENSION: bilateral, >30 minutes of steady pain, not aggrevated by light/noise, no aura - Tx: NSAIDs CLUSTER: unilateral, repetitive brief HAs characterized by PERIORBITAL PAIN associated with ipsilateral lacrimation, rhinorrhea, Horner's syndrome, much more common in males - Tx: inhaled oxygen and sumatriptan
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Vertigo:
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illusion of MOVEMENT, not to be confused with dizziness or lightheadedness PERIPHERAL: more common, inner ear etiology (semicircular canal debris, vestibular nerve infection, Meniere's disease) - positional testing --> delayed horizontal nystagmus CENTRAL: brain stem or cerebellar lesion (vesticular nuclei, posterior fossa tumor) - positional testing --> immediate nystagmus in any direction, may change direction
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Tuberous Sclerosis
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HAMARTOMAS - Hamartomas of the skin and CNS - Adenoma sebaceum (cutaneous angiofibromas) - Mitral regurg - Ash-leaf spots - cardiac Rhabdomyoma - Tuberous sclerosis - autOsomal dominant - Mental retardation - renal Angiomyolipomas - Seizures
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Neurofibromatosis Type 1
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von Recklinghausen's disease - Cafe-au-lait spots - Lisch nodules (pigmented iris hamartomas) - neurofibromas in skin - optic gliomas - pheochromocytoma Autosomal dominant, 100% penetrant, variable expression Mutated NF-1 gene on chromosome 17 the DOMINANT 17-yr old von Recklinghausen the 1st drank CAFE while watching his staff HAMAR the EYE with GLE which gave him much ENERGY (epinephrine)
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von Hippel-Lindau disease
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cavernous hemangiomas in skin, mucosa, organs bilateral renal cell carcinoma hemangioblastoma in retina, brain stem, cerebellum pheochromocytomas Autosomal dominant - mutated tumor suppressor VHL on chromosome 3 the DOMINANT 3-yr old VON HIPPEL was filled with ENERGY (epinephrine) as HE BLASTed the CAVERNOUS (renal) CELL
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Meniere's Disease
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abrupt, recurrent, N/V Vertigo, hearing loss, tinnitus Distention of spaces in cochlear/vestibular labyrinths
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Glioblastoma multiforme
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multiple forms = lined up caterpillars to butterfly (lesion) who GFAP's to stars Caterpillar lined up = pseudopallisading (line up) around necrosis, butterfly lesion crosses corpus callosum, and GFAP positive (int. filament in astrocytes) ADULT most common primary brain tumor can cross corpus callosum stain astrocytes for GFAP!! "pseudopalisading" pleomorphic tumor cells border central areas of NECROSIS and HEMORRHAGE
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Meningioma
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Men, your psammoma's a whor(l)e! Psamomma bodies (calcified like sand) and whorled pattern on histology Affects women more than men since tumor expresses estrogen receptor (involved in growth) ADULT 2nd most common primary brain tumor, most common benign neoplasm most often occurs in convexities of hemispheres and parasagittal region -- pushing pattern without infiltration arises from arachnoid cells external to brain RESECTABLE Spindle cells concentrically arranged in a whorled pattern, PSAMMOMA BODIES
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Schwannoma
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Can no longer sing my schwannoma 100 times (S-100 positive) Frequently affects CN 8 @ cerebellopontine angle (lose hearing + tinnitus) ADULT 3rd most common primary often localized to CN VII = acoustic schwannoma Resectable Usually found at cerebellopontine angle S-100 positive!! Bilateral schwannoma found in neurofibromatosis type 2
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Ol"eggo"dendroglioma
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obv. in white matter (usually frontal) ADULT relatively rare, slow growing most often in FRONTAL LOBE chicken-wire capillary pattern "FRIED-EGG CELLS", round nuclei with clear cytoplasm (perinuclear halos) Often calcified
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Pilo"cyst"tic (low-grade) astrocytoma
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CHILDREN usually well-circumscribed in kids, most often found in posterior fossa, may be supratentorial GFAP positive (like GM) Benign, good prognosis ROSENTHAL FIBERS -- eosinophilic, corkscrew fibers Cystic + solid
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Medulloblastoma
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Baby homer "write" rossettes with blueberries (small, round blue cells) while sitting on little brain CHILDREN highly malignant cerebellar tumor a form of primitive neuroectodermal tumor (PNET) can compress 4th ventricle causing hydrocephalus most commonly found on cerebellar vermis ROSETTES or PERIVASCULAR PSEUDOROSETTES solid (gross), small blue cells (histo) RADIOSENSITIVE
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Ependymoma
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Perivascular pseudorosette on biopsy (flowers, so also kids) thus infratentorial so hydrocephalus too CHILDREN most commonly found in 4th ventricle Poor Prognosis
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Craniopharyngioma
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from Rathke's pouch (breakup name and makes sense) - location = bitemporal hemianopsia too benign childhood tumor, confused with pit adenoma most common form of childhood supratentorial tumor calcification is common (tooth enamel-like)
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Uncal herniation clinical signs: - ipsilateral dilated pupil/ptosis - contralateral homonymous hemianopia - ipsilateral paresis - duret hemorrhages/paramedial artery rupture
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- stretching of CN III - compression of ipsilateral PCA - compression of contralateral crus cerebri - caudal displacement of brain stem
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ring enhancing lesion: uniformly enhancing lesion: heterogeneusly enhancing lesion:
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- mets (lung>breast>melanoma>kidney>GI), abscesses, TOXO, primary CNS lymphoma (associated with AIDS, EBV, usually B cell) - metastatic lymphoma (usually B-cell non-Hodgkin), meningioma, mets (but these are usually ring enhancing) - GM
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Anesthetics: general principles
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CNS drugs must be lipid soluble or be actively transported drugs with decreased solubility in blood = rapid induction and recovery drugs with increased solubility in lipids = increased potency = 1/MAC - MAC = minimal alveolar conc at which 50% of the population is anesthetized (varies with age) - e.g. N2O has low blood and lipid solubility = fast induction and low potency vs. Halothane has high lipid and blood solubility = high potency and slow induction
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Organ and MOA and anesthetic: Lung Blood Tissue (brain)
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- increased rate and depth of ventilation = increased gas tension - increased blood solubility = increased blood/gas partition coefficient = increased solubility = increased gas required to saturate the blood = slower onset of induction - AV conc gradient increases = increased solubility = increased gas required to saturate the tissue = slower onset of action
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Parkinson's Disease Treatment Strategy
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(due to loss of dopaminergic neurons and excess cholinergic activity) - agonist dopamine receptors: bromocriptine (ergot) or pramipexole/ropinirole (non-ergot which is preferred) - increase dopamine: amantadine (may increase dopamine release, also used as an antiviral against influenza A and rubella, toxicity is ataxia) and L-dopa/carbidopa (converted to dopamine in CNS) - prevent dopamine breakdown: selegiline (selective MAO type B inhibitor), entacopone/talcopone (COMT inhibitors that prevent L-dopa degradation) - curb excess cholinergic activity: benztropine (antimuscarinic, improves tremor and rigidity but has LITTLE EFFECT ON BRADYKINESIA) BALSA: bromocriptine, amantadine, L-dopa, selegiline, antimuscarinic
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Huntington Treatment
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disease: increased dopamine, decreased GABA and ACh Reserpine and tetrabenazine --> amine depleting Haloperidol --> dopamine receptor antagonist
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Size of pain fibers
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Size of pain fibers goes in alphabetical order: Aalpha (largest/fastest) > Abeta / Agamma > Adelta (sharp pain) > B / C (smallest / slowest) Innervation? Think logically, what needs to go fast --> muscle spindle afferents / golgi tendons (thus Aalpha) Pain can be slower so it's C (dull pain) / Adelta (sharp pain) C = glove get punched (dull pain), δ (delta = bomb - SHARP pain)
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Arnold Chiari Malformation Type II
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Arnold (Schwarz) - Chiari malformation Type II is a muscle man that pushes your tonsils through the foramen magnum!
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Dandy Walker Malformation
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Dandy Walker - Err'thing fine and dandy since we just walking around the fourth ventricle Enormous dilation of 4th ventricle (agenesis of cerebellar vermis - sometimes since lateral / medial foramen didn't open)
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Hematoma vs. Hemorrhage
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Hemorrhage just means blood loss; it can be external or internal. Hematoma is an enclosed hemorrhage into a tissue space, whether subdural, epidural, or into a joint capsule. Hope that helps.
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