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273 Cards in this Set
- Front
- Back
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- Large cells w/prominent nucleoli
- Nissl substance (RER) in cell body (not axon) |
Neurons
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- Physical support, repair, K+ metabolism,
- removal of excess neurotransmitter - maintains the BBB |
Astrocytes
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Astrocyte response to injury
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Reactive gliosis
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Astrocyte marker
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GFAP
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- CNS phagocytes
- not readily discernible in Nissl stains - small irregular nuclei and relatively little cytoplasm |
Microglia
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Microglia originates from what germ layer
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Mesoderm
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Free nerve ending sense
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Pain/temperature
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Meissner's corpuscles
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Position sense, fine touch
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Pacinian corporpuscles
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Vibration, pressure
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Merkel's disk
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static touch (shapes, edges, textures) position sense
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Peripheral nerve layers
- surrounds a fascicle of nerve fibers - permeability layer - must be rejoined in microsurgery for limb |
Perineuriem
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Peripheral nerve layers
- invest single nerve fiber - inflammatory infiltrate in Guillain-Barre |
Endoneurium
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Peripheral nerve layers
- dense connective tissue that surrounds entire nerve - fascicles and blood vessels |
Epineurium
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Neurotransmitters
Type: Norepinephrine Location: ? |
Locus ceruleus
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Neurotransmitters
Type: Dopamine Location: ? |
Ventral tegmentum and SNc
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Neurotransmitters
Type: serotonin (5-HT) Location: ? |
Raphe nucleus
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Neurotransmitters
Type: ACh Location: ? |
Basal nucleus of Meynert
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Neurotransmitters
Type: GABA Location: ? |
Nucleus accumbens
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Neurotransmitters
Disease: increases in anxiety, decreases in depression Transmitter: ? |
Norepinephrine
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Neurotransmitters
Disease: increase in schizophrenia, decrease in Parkinson's and depression Transmitter: ? |
Dopamine
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Neurotransmitters
Disease: decreases in anxiety and depression Transmitter: ? |
Serotonin (5-HT)
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Neurotransmitters
Disease: decreases in Alzheimer's Huntington's, and REM sleep Transmitter: ? |
ACh
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Neurotransmitters
Disease: decreases in anxiety and Huntingtons Transmitter: ? |
GABA
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Neurotransmitter area responsible for stress and panic
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Locus ceruleus
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Neurotransmitter area responsible for reward center, pleasure, addiction
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Nucleus accumbens & septal nucleus
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The blood-brain barrier is formed by?
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- tight junctions
- basement membrane - astrocyte processes |
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Area of Hypothalamus
- makes ADH |
Supraoptic nucleus
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Area of Hypothalamus
- makes oxytocin |
Paraventricular nucleus
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Area of Hypothalamus
- controls hunger |
Lateral area
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Area of Hypothalamus
- controls satiety |
Ventrolateral area
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Destruction of the later area of the hypothalamus result in
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Weight lost (never hungry)
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Destruction of the ventrolateral area of the hypothalamus result in
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Weight gain (never satisfied)le for cool
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Area of Hypothalamus
- responsible for cooling - controlled by parasympathetic |
Anterior Hypothalamus
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Area of Hypothalamus
- responsible for heating - controlled by sympathetic |
Posterior Hypothalamus
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Anterior Pituitary gland makes?
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- FSH
- LH - ACTH - TSH - GH - Prolactin |
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Posterior Pituitary gland makes?
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- ADH
- Oxytocin |
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Thalamus
Nucleus: VPL Input: ? |
Spinothalamic and dorsal columns
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Thalamus
Nucleus: VPM Input: ? |
CN V (trigeminal)
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Thalamus
Nucleus: LGN Input: ? |
CN II (optics)
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Thalamus
Nucleus: MGN Input: ? |
inferior colliculus of the pon and Superior olive
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Thalamus
Nucleus: VPL Info received: ? |
Spinothalamic and Dorsal column
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Thalamus
Nucleus: VPM Info received: ? |
CN V (trigeminal)
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Thalamus
Nucleus: LGN Info received: ? |
CN II (optic)
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Thalamus
Nucleus: MGN Info received: ? |
inferior colliculus of pons and superior olive
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Parts of the limbic system
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- cingulate gyrus
- hippocampus - fornix - mammillary bodies - septal nucleus |
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Degenerative disorder of CNS associated w/ Lewy bodies (alpha-synuclein)
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Parkinson's Disease
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Parkinson's is depigmentation of the?
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substantia nigra pars compacta
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Sudden, wild flailing of 1 arm +/- leg
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Hemiballismus
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Hemiballismus is characterized by?
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contralateral subthalamic nucleus lesion
(lacunar stroke "Hx: of HTN") |
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Hemiballismus is loss of inhibition of?
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thalamus through globus pallidus
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In Huntington's, neuronal death is via?
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NMDA-R binding and glutamate toxicity
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Huntington's leads to atrophy of?
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striatal nuclei
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Tremor
- action tremor (worse when holding posture) - Pts often self medicate with alcohol |
Essential "familial" tremor
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Treatment for Essential "familial" tremor
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Beta-blocker
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Tremor
- most noticeable distally - seen in Parkinson's Pts |
Resting tremor
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Tremor
- slow, zigzag motion when pointing toward a target - associated w/cerebellar dysfunction |
Intention tremor
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Brain lesions
-Sign/Symptoms: Kluver-Bucy syndrome (hyperorality, hypersexuality, disinhibition) - Area of Lesion: ? |
Amygdala (bilateral)
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Brain lesions
-Sign/Symptoms: disinhibition and defects in concentration, lacks judgement - Area of Lesion: ? |
Frontal lobe
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Brain lesions
-Sign/Symptoms: Spatial neglect syndrome (left side) - Area of Lesion: ? |
Right parietal lobe
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Brain lesions
-Sign/Symptoms: reduced levels of arousal and wakefulness - Area of Lesion: ? |
Reticular activating system (midbrain)
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Brain lesions
-Sign/Symptoms: Wernicke-Korsakoff syndrome (confusion, ataxia, memory loss) - Area of Lesion: ? |
Mammillary bodies (bilateral)
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Brain lesions
-Sign/Symptoms: tremor at rest, chorea, or athetosis - Area of Lesion: ? |
Basal ganglia
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Brain lesions
-Sign/Symptoms: intention tremor, falls toward lesion - Area of Lesion: ? |
Cerebellar hemisphere
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Brain lesions
-Sign/Symptoms: contralateral hemiballismus (arm flailing) - Area of Lesion: ? |
Subthalamic nucleus
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Brain lesions
-Sign/Symptoms: Truncal ataxia, dysarthria (broken speech) - Area of Lesion: ? |
Central cerebellar vermis
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Brain lesions
-Sign/Symptoms: anterograde amnesia ( no new memories) - Area of Lesion: ? |
Hippocampus
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Brain lesions
-Sign/Symptoms: Eyes can't look toward lesion - Area of Lesion: ? |
Paramedian pontine reticular formation (PPRF)
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Brain lesions
-Sign/Symptoms: Eye look toward lesion - Area of Lesion: ? |
Frontal eye fields
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Brain lesions
-Sign/Symptoms: acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness - Area of Lesion: ? |
Central pontine myelinolysis
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Central pontine myelinolysis is caused by?
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rapid infusion of Na+
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Higher-order inability to speak
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Aphasia
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Motor inability to speak
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Dysarthria
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Loss of ability to recognize objects, persons, sounds, shapes, or smells while the specific sense is not defective
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Agnosia
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reading disorder
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Alexia
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Nonfluent aphasia w/intact comprehension
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Broca's
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Fluent aphasia w/impaired comprehension
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Wernicke's
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Nonfluent aphasia w/impaired comprehension
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Global aphasia
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Poor repetition but fluent speech, intact comprehension
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Conduction aphasia (arcuate fasciculus)
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Broca's area
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inferior frontal gyrus
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Wernicke's area
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superior temporal gyrus
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Conduction Aphasia area
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arcuate fasciculus
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Circle of Willis
-area/defect: 1. Contralateral hemiparesis (lower extremities) 2. medial lemniscus (decreased contralateral proprioception) 3. ipsilateral paralysis of hypoglossal nerve. (decrease in pain/temp) |
Anterior spinal artery (medial medullary syndrome)
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Circle of Willis
-area/defect: 1. contralateral loss of pain/temp 2. ipsilateral dysphagia, 3. hoarseness, decrease gag, vertigo, diplopia, nystagmus, vomiting, 4. ipsilateral Horner's, face pain/temp |
PICA (posterior inferior cerebellar artery)
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Circle of Willis
-area/defect: 1. ipsilateral facial paralysis, cochlear, nystagmus, 2. ipsilateral face pain/temp |
AICA (anterior inferior cerebellar artery)
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Circle of Willis
-area/defect: 1. contralateral hemianopia w/macular sparing 2. supplies occipital cortex |
Posterior cerebral artery
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Circle of Willis
-area/defect: 1. contralateral face and arm paralysis and sensory loss 2. aphasia 3. left-side neglect |
Middle cerebral artery
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Circle of Willis
-area/defect: 1. Supplies medial surface of the brain 2. leg-foot area of motor and sensory cortices |
Anterior cerebral artery
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Circle of Willis
-area/defect: 1. most commons site of willis aneurysm 2. lesions may cause visual field defects |
Anterior communicating Artery
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Circle of Willis
-area/defect: 1. causes CN III palsy 2. 2nd common site for aneurysm |
Posterior communicating artery
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Circle of Willis
-area/defect: 1. divisions of middle cerebral artery 2. supplies internal capsule, caudate, putamen, globus pallidus 3. "arteries of stroke" 4. infarct of posterior limb of the internal capsule causes pure motor hemiparesis |
Lateral striate
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Circle of Willis
-area/defect: 1. damage in severe hypotension 2. upper leg/arm weakness 3. defects in higher-order visual processing |
Watershed zone
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Watershed zones in the circle of willis
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- anterior/middle cerebral arteries
- middle/posterior cerebral arteries |
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Circle of Willis
-area/defect: 1. "locked-in syndrome" |
Basilar artery
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What is typically left unaffected in a basilar artery defect
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CN III (oculomotor)
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Epidural hematoma is most often due to the rupture of?
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Middle meningeal artery (2nd temporal bone fracture)
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Intracranial hemorrhage
- lucid interval is seen - CT shows biconvex disk - high pressure bleed - CN III palsy |
Epidural hematoma
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Intracranial hemorrhage
- rupture on the bridging veins - low pressure bleed - CT shows crescent-shaped hemorrhage - seen in elderly, alcoholics, shaken babies |
Subdural hematoma
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Intracranial hemorrhage
- rupture of an aneurysm - worst headache of their life - bloody/yellow spinal tap |
Subarachnoid hematoma
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Subarachnoid hematoma can lead to
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vasospasms (due to blood product breakdown)
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Treatment for Subarachnoid hematoma
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Calcium channel blockers
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Ischemic brain disease (timeline)
- 12-48 hours |
red neurons
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Ischemic brain disease (timeline)
- 24-72 hours |
necrosis + neutrophils
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Ischemic brain disease (timeline)
- 3-5 days |
macrophages
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Ischemic brain disease (timeline)
- 1-2 weeks |
reactive gliosis + vascular proliferation
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Ischemic brain disease (timeline)
- more than 2 weeks |
glial scar
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CSF is made by
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choroid plexus
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CSF is reabsorbed by
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arachnoid granulation
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The lateral ventricles flow to the 3rd ventricle via
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Foramen of Monro
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The 3rd ventricle flows to the 4th ventricle via
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The cerebral aqueduct
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- Expansion of ventricles distorts the fibers of the fibers of the corona radiata and leads to the clinical triad of dementia, ataxia, and urinary incontinence.
- does not result in increased subarachnoid space volume - reversible |
Normal pressure hydrocephalus
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Wet, wobbly, wacky
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Normal pressure hydrocephalus
(urinary incontinence, ataxia, dementia) |
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Normal pressure hydrocephalus leads to distortions of the fibers of?
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Corona radiata
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- Decreased CSF absorption be arachnoid villi
- leads to increase intracranial pressure - papilledema and herniation |
Communicating hydrocephalus
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A communicating hydrocephalus is caused by
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arachnoid scarring post-meningitis
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- caused by structural blockage of CSF circulation w/in the ventricular system
- ex. stenosis of the aqueduct of Sylvius |
Obstructive (noncommunicating) hydrocephalus
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- false appearance of increase CSF due to atrophy
- intracranial pressure is normal; triad is not seen |
Ex Vacuo (hydrocephalus)
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Conditions that can lead to Ex Vacuo (hydrocephalus)
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Alzheimer's disease, advanced HIV, Pick's disease
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Nerves C1-C7 exit via intervertebral foramina (above or below)
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Above
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Vertebral disk herniation usually occurs between?
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L5-S1
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where Lumbar punctures are performed
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L4-L5
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Synapse point of the Dorsal column
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Ipsilateral nucleus cuneatus or gracilis
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Decussates (cross midline) point of the Dorsal column
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Medulla
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Synapse point of the Spinothalamic tract
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Ipsilateral gray matter (in spinal cord)
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Decussates (cross midline) point of the Spinothalamic tract
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Anterior white commissure
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Decussates (cross midline) point of the Corticospinal tract
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Caudal Medulla
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Spinal cord lesions (site?)
- Poliomyelitis and Werdnig-Hoffmann disease |
Low motor only; destruction of anterior horns
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Spinal cord lesions (site?)
- Multiple sclerosis (MS) |
- mostly white matter of cervical region
- random lesions |
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Spinal cord lesions (site?)
- ALS |
both upper and lower motor neurons
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Spinal cord lesions (site?)
- Anterior spinal artery |
- spares dorsal columns & tract of Lissauer
- all else is lost |
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A complete occlusion of anterior spinal artery would lead to effect of a watershed area where?
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Upper Thoracic ASA
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Artery that supplies the anterior spinal artery below T8
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Adamkiewicz
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Spinal cord lesions (site?)
- Tabes dorsalis (3rd degree syphilis) |
Dorsal root and dorsal column
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Spinal cord lesions (site?)
- Syringomyelia |
- damage of anterior white commissure
- loss of pain/temp |
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Spinal cord lesions (site?)
- Vit B12 neuropathy & Vit E deficiency |
demyelination of:
- dorsal columns - lateral corticospinal - spinocerebellar |
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Spinal cord lesions (site?)
- Friedreich's axtaxia |
demyelination of:
- dorsal columns - lateral corticospinal - spinocerebellar |
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Defect in what causes of (ALS) amyotrophic lateral sclerosis
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superoxide dismutase 1 (SOD1)
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Treatment with what will modest lengthen the survival of a ALS Pt.
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Riluzole
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Riluzole's mechanism of action
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decreased presynaptic glutamate
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- Associated w/Charcot's joints
- absent DTRs (deep tendon reflex) - positive Romberg test |
Tabes dorsalis
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Cause of Friedreich's ataxia
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- Autosomal-recessive (FXN gene)
- trinucleotide repeat (GAA) - leads to impaired mitochondrial functioning |
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Common cause of death for a Pt w/Friedreich's ataxia
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Hypertrophic cardiomyopathy
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Seen in childhood Friedreich's ataxia
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kyphoscoliosis
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- Ptosis
- Anhidrosis - Miosis |
Horner's syndrome
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Oculosympathetic pathway that can disrupted causing Horner's Syndrome
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- Hypothalamus
- intermediolateral column (lateral horn) - Superior cervical ganglion |
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Associations these lesions leads to Horner's Syndrome
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- Pancoast tumor
- Brown-Sequard syndrome (hemisection) - late stage syringomyelia |
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Diaphragm and gallbladder pain referred to
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right shoulder via the phrenic nerve
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Appearance of primitive reflexes after 1yrs of age
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frontal lobe lesion
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Primitive reflexes
- "hang on for life" reflex; abduct/extend limbs when startled, and then draw together |
Moro reflex
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Primitive reflexes
- movement of head toward one side if cheek or mouth is stroked (nipple seeking) |
Rooting reflex
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Primitive reflexes
- sucking response when roof of mouth is touched |
Sucking reflex
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Primitive reflexes
- curling of fingers/toes if palms of hands/feet stroked |
Palmar and plantar reflex
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Primitive reflexes
- dorsiflexion of large toe and fanning of others toes w/plantar stimulation |
Babinski sign
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conjugate vertical gaze center
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Superior colliculi
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Parinaud syndrome
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paralysis of conjugate vertical gaze due to lesion in superior colliculi (ei. pinealoma)
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paralysis of conjugate vertical gaze due to
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lesion in superior colliculi (ei. pinealoma)
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lesion in superior colliculi due to
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pinealoma
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Cranial nerves
- Nerve: Olfactory - Function(s): ? |
smell (only CN w/out thalamic relay to cortex)
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Cranial nerves
- Nerve: Optic - Function(s): ? |
Sight
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Cranial nerves
- Nerve: Oculomotor - Function(s): ? |
- Eye movement (SR, IR, MR, IO)
- Pupillary constriction - accommodation - eye lid opening |
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Cranial nerves
- Nerve: Trochlear - Function(s): ? |
Eye movement (SO)
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Cranial nerves
- Nerve: Trigeminal - Function(s): ? |
- Mastication
- facial sensation |
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Cranial nerves
- Nerve: Abducens - Function(s): ? |
Eye movement (LR)
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Cranial nerves
- Nerve: Facial - Function(s): ? |
- facial movements
- taste anterior 2/3 of tongue - lacrimation, salivation - eye closing - stapedius in ear |
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Cranial nerves
- Nerve: Vestibulocochlear - Function(s): ? |
- hearing
- balance |
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Cranial nerves
- Nerve: Glossopharyngeal - Function(s): ? |
- Taste poster 1/4 of tongue
- swallowing, salivation - monitors carotid body and sinus chem & baroreceptors - stylopharyngeus |
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Cranial nerves
- Nerve: Vagus - Function(s): ? |
- taste epiglottic region
- swallowing, palate elevation, uvula - talking, coughing - monitors aortic arch chemo & barorecptors |
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Cranial nerves
- Nerve: Accessory - Function(s): ? |
- head turning
- shoulder shrugging (SCM, trapezius) |
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Cranial nerves
- Nerve: Hypoglossal - Function(s): ? |
tongue movement
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Cranial nerve reflex
- Afferent: V(1) ophthalmic - Efferent: VII - Reflex: ? |
Corneal
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Cranial nerve reflex
- Afferent: V(1) "doesn't produce tears" - Efferent: VII - Reflex: ? |
Lacrimation
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Cranial nerve reflex
- Afferent: V(3) "muscle spindle from masseter" - Efferent: V(3) "motor-masseter" - Reflex: ? |
Jaw Jerk
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Cranial nerve reflex
- Afferent: CN II - Efferent: CN III - Reflex: ? |
Pupillary
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Cranial nerve reflex
- Afferent: CN IX - Efferent: CN IX,X - Reflex: ? |
Gag
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Vagal nuclei:
- visceral Sensory information (taste, baroreceptors, gut distention) |
Nucleus Solitarius
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Vagal nuclei:
- Motor innervation of pharynx, larynx, and upper esophagus |
Nucleus aMbiguus
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Vagal nuclei:
- send autonomic (parasympathetic) fibers to heart, lungs, and upper GI |
Dorsal motor nucleus
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Cranial nerve and vessel pathways
- Cribriform plate |
CN I- olfactory
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Cranial nerve and vessel pathways
- optical canal |
CN II- optic
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Cranial nerve and vessel pathways
- Superior orbital fissure |
CN III, IV, V(1), VI
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Cranial nerve and vessel pathways
- Foramen Rotundum |
CN V(2)
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Cranial nerve and vessel pathways
- Foramen Ovale |
CN V(3)
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Cranial nerve and vessel pathways
- Foramen Spinosum |
Middle meningeal artery
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Cranial nerve and vessel pathways
- internal auditory meatus |
CN VII, VIII
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Cranial nerve and vessel pathways
- jugular foramen |
CN IIX, IX, X
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Cranial nerve and vessel pathways
- hypoglossal canal |
CN XII
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Cranial nerve and vessel pathways
- Foramen megnum |
CN XI
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Cranial nerve lesions
- CN XII lesion (LMN) |
- Tongue deviates TOWARD lesion (lick your wounds)
-Decussates (crosses before medulla and synapse on contralateral hypoglossal nucleus) |
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Cranial nerve lesions
- CN V motor lesion |
- Jaw deviates TOWARD lesion
- Bilateral cortical input to lateral pterygoid muscle |
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Cranial nerve lesions
- CN X lesion |
- Uvula deviates AWAY from lesion
- week side collapses and points away |
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Cranial nerve lesions
- CN XI lesion |
- weakness turning head to AWAY from lesion (SCM)
- shoulder droop on side of lesion (trapezius) |
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Kuh-kuh-kuh test- motor movement?
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palate elevation
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Kuh-kuh kuh test- Cranial Nerve?
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CN X- vagus
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La-la-la test- motor movement?
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tongue movement
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La-la-la test- cranial nerve?
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XII- hypoglossal
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Mi-mi-mi test- motor movement
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lip movement
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Mi-mi-mi test- cranial nerve
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VII- facial
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Produces aqueous humor
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Ciliary procress
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Absorbs aqueous humor
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Trabecular Meshwork
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Collects aqueous humor from trabecular meshwork
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Canal of Schlemm
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Uncal herniation
Sign: ipsilateral dilated pupil/ptosis Cause:? |
Stretching if CN III
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Uncal herniation
Sign: Contralateral homonymous hemianopia Cause:? |
Compression of ipsilateral posterior cerebral artey
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Uncal herniation
Sign: Ipsilateral paresis Cause:? |
Compression of contralateral crus cerebri (Kernohan's notch)
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Uncal herniation
Sign: Duret (hemorrhages paramedian artery rupture) Cause:? |
Caudal displacement of brain stem
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Primary brain tumor
- can cross corpus callosum "butterfly glioma" - most common brain tumor - Stained with GFAP - prognosis grave |
Glioblastoma multiforme
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Primary brain tumor
- "pseudopalisading" pleomorphic tumor cells - border central areas of necrosis and hemorrhage |
Glioblastoma multiforme
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Primary brain tumor
- often occurs in convexities of hemispheres and parasagittal - arises from arachnoid cell external to brain - resectable |
Meningioma
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Primary brain tumor
- spindle cells concentrically arranged in a whorled pattern - psammoma bodies (laminated calcifications) |
Meningioma
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Primary brain tumor
- localized to CN VIII - found at cerebellopontine angle - S-100 positive - resectable |
Schwannoma
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Primary brain tumor
- often in frontal lobes - chicken-wire capillary pattern - rare and slow growing |
Oligodendroglioma
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Primary brain tumor
- round nuclei w/clear cytoplasm - often calcified |
"fried egg"appearance
Oligodendroglioma |
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Primary brain tumor
- bitemporal hemianopia - due to pressure on optic chiasm |
Pituitary adenoma
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Primary brain tumor
-located in Rathke's pouch |
Pituitary adenoma
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Primary brain tumor
- often found in posterior fossa/ maybe supratentorial - GFAP positive - most common in children |
Pilocytic astrocytoma
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Primary brain tumor
- eosinophilic corkscrew fibers - cystic + solid (gross) |
(Rosenthal fibers)
Pilocytic astrocytoma |
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Primary brain tumor
- form of primitive neuroectodermal tumor (PNET) - can compress 4th ventricle - highly malignant cerebellar tumor |
Medulloblastoma
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Primary brain tumor
- rosettes or perivascular pseudorosette pattern of cells - small blue cells |
Medulloblastoma
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Primary brain tumor
- found in 4th ventricle - cause hydrocephalus - poor prognosis |
Ependymoma
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Primary brain tumor
- perivascular pseudorosette pattern of cells - rod-shaped blepharoplasts |
(basal ciliary bodies)
Ependymoma |
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Primary brain tumor
- found w/retinal angiomas - associated w/von Hippel=Lindau - can produce EPO ->polycythemia |
Hemangioblastoma
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Primary brain tumor
- foamy cells - high vascularity |
Hemangioblastoma
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Primary brain tumor
- often confused w/pituitary adenoma - can also cause bitemporal hemianopia - benign - most common child supratentorial tumor |
Craniopharyngioma
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Primary brain tumor
- calcification is common (tooth like enamel) - derived from remnants of RAthke's pouch |
Craniopharyngioma
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Pain due to irritation of structures sech as dura, cranial nerves, or extracranial structures, but not the brain parenchyma it's self
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Headache
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- unilateral 4-72 hours of pulsating pain w/nausea, photophobia, or phonophobia.
- aura |
Migraine
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Migraine is caused by?
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- irritation of CN V
- release of substance P, CGRP, vasoactive peptides |
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Treat meant for migrane
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- propranolol
- NSAIDS - sumatriptan |
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- bilateral headache
- more than 30 mins long - not aggravated by light or noise; no aura |
Tension Headache
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- unilateral; repetitive brief headaches
-periorbital pain - asso: w/ipsilateral lacrimation, rhinorrhea, Horner's |
Cluster headache
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Treatment for Cluster headaches
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sumatriptan
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- inner ear etiology
- delayed horizontal nystagmus |
Peripheral vertigo
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Cause of Peripheral vertigo
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- Meniere's disease
- semicircular canal debris - vestibular nerve infection |
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- brainstem or cerebellar lesion
- immediate nystagmus in any direction; may change directions |
Central vertigo
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Neurocutaneous disorder
- V(1) ophthalmic distribution - leptomeningeal angiomas - pheochromocytomas - port-wine stain (nevus flammeus) - glaucoma, seizure, retardation |
Sturge-Weber syndrome
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Neurocutaneous disorder
- Hamartomas in CNS, skin, organs - rhabdomyoma - subependymal giant cell astrocytoma - ash leaf spots |
Tuberous sclerosis
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Neurocutaneous disorder
- cafe-au-lait spots - lisch nodules (pigmented iris) - optic gliomas |
Neurofibromatosis type I
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Neurofibromatosis type I cause?
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mutation in NF-1 gene on chromosome 17
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Neurocutaneous disorder
- cavernous hemangiomas in skin, mucosa, organs - bilateral renal cell carcinoma - hemangioblastoma in retina |
von-Hippel-Lindau disease
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von-Hippel-Lindau disease is caused by?
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mutated tumor suppressor VHL on chromosome 3
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Simple partial seizure
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motor, sensory, autonomic, and psychic
(consciousness left intact) |
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Complex partial seizure
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motor, sensory, autonomic, and psychic
(consciousness imparied) |
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Absence seizure (petit mal, 3Hz)
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blank stare
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Myoclonic seizure
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quick, repetitive jerk
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Tonic-clonic (grand mal)
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alternating stiffening and movement
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Tonic seizure
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stiffening
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Atonic seizure
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"drop" seizure; often mistaken for fainting
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- Demyelination of CNS due to destruction of oligodendrocytes
- Rapidly progressive and fatal - seen in 2-4% of AIDS Pts |
Progressive multifocal leukoencephalopathy (PML)
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Virus associated w/Progressive multifocal leukoencephalopathy (PML)
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JC virus
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-Multifocal perivenular inflammation and demyelination
- seen after infection (VZV; measles) - seen after vaccination (rabies, smallpox) |
Acute disseminated (postinfectious) encephalomyelitis
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- autosomal-recessive lysosomal storage disease
- due to arylsulfatase A deficiency - buildup of sulfatides leads to impaired production of myelin sheath |
Metachromatic leukodystrophy
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Dysmyelinating disease
- hereditary motor and sensory neuropathy (HMSN) |
Charcot-Marie-Tooth disease
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- ascending muscle weakness
- facial paralysis in 50% of case - Autonomic function affected (cardiac, hypo/hypertension) |
Guillain-Barre syndrome
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associated w/infection; autoimmune attack of peripheral myelin due to molecular mimicry
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campylobacter and herpesvirus
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Multiple sclerosis (MS) findings
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- increased protein (IgG)
- oligoclonal bands are diagnostic - MRI is gold standard - periventricular plaques (reactive gliosis) |
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Treatment for Multiple sclerosis (MS)
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- Beta- interferon
- immunosuppressant therapy |
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Familial form associated w/Early onset Alzheimer's
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APP (21); presenilin-1 (14); presenilin-2 (1)
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Familial form associated w/Late onset Alzheimer's
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ApoE4 (19)
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Protective association of Alzheimer's
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ApoE2 (19)
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Alzheimer's histologic/gross findings
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- decreased ACh
- senile plaques (extracellular; beta-amyloid) - neurofibrillary tangles (intracellular; phosphorylated tau protein) |
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- Dimension, aphasia, parkinsonian aspects, change in personality
- spares parietal lobe and posterior 2/3 pf superior temporal gyrus |
Pick's disease
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Pick's disease histologic/gross findings
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- intracellular, aggregated tau protein
- frontotemporal atrophy |
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intracellular, aggregated tau protein
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Pick bodies
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Parkinsonism w/dementia and hallucinations
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Lewy body dementia
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"Parkinsonism w/dementia and hallucinations" histologic/gross findings
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alpha-synuclein defect (Lewy body dementia)
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rapidly progressive (weeks to months) dementia w/myoclonus
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Creutzfeldt-Jakob disease (CJD)
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Creutzfeldt-Jakob disease (CJD) histologic/gross findings
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- spongiform cortex
- prions (alpha helix -> beta sheet [resistant to proteases]) |
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Second most common cause of dementia
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multi-infarct
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Papilledema
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- increased intracranial pressure
- elevated optic disk w/blurred margins - bigger blind spot |
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Extraocular nerves
- eye looks down and out - pupillary dilation - ptosis |
CN III damage
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Extraocular nerves
- eye drifts upward causing vertical diplopia - problems reading newspaper and going down stairs |
CN V damage
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Extraocular nerves
- medially directed eye |
CN VI damage
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Extraocular muscle
- eye drifts upward causing vertical diplopia - problems reading newspaper and going down stairs |
Superior Oblique
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Extraocular muscle
- medially directed eye |
Lateral Rectus
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- afferent pupillary defect
- due to optic nerve damage or retinal detachment - decreased bilateral pupillary constricition |
Marcus Gunn pupil
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