Week 1 - Neuro Diseases / Disorders Flashcards
epidural hemorrhage / hematoma
traumatic vascular injury, between dura and bone, ruptured meningeal artery, appears lens shaped and clear on CT scan, person often experiences blow to head with momentary loss of consciousness and then acts normal only to go into a coma hours later, blow to the temple
subdural hemorrhage / hematoma
traumatic vascular injury, between dura and arachnoid layer, rupture of cerebral vein where it joins sinus, result of deacceleration injury in which the skull comes to a stop but the brain keeps moving, appears more blurred and follows curve of skull on CT scan, elderly falls
subarachnoid hemorrhage / hematoma
traumatic vascular injury, between arachnoid and pia, rupture of cerebral artery, caused by aneurysm, blood will be found in CSF, worst headache ever, possible berry aneurysm rupture
Babinski sign
plantar extensor response with fanning of toes when dorsal lateral foot is stroked, present in very young/old or with neurological defect because brain is not able to override reflex
Waaredenberg syndrome
neural crest cells fail to differentiate properly, white hair patch, pale skin, pale blue eyes, deafness, constipation
Hirschsprung disease or congenital megacolon
neural crest cells fail to migrate to rectum and sigmoid colon, normal peristalsis does not occur, fecal retention with ballooning, surgically repaired
craniorachischisis
neural tube defect, failure of the neural tube to close leaving exposed spinal cord
anencephaly or meroanencephaly
exposed brain or no brain, failure or rostral neuropore to close
spina bifida variations
failure of caudal neuropore to close
occipital encephalocele
anterior neural tube defect, brain and meninges protrude in occipital area
spina bifida
posterior neural tube defect, failure of vertebral arch formation, tuft of hair in lumbar region
meningocele
posterior neural tube defect, failure of vertebral arch formation, protruding meninges and CSF
myelomeningocele
posterior neural tube defect, failure of vertebral arch formation, meninges and CSF and spinal cord protuding
syringomyelia
non-NTD, cavitation of spinal cord in cervical or thoracic region, loss of pain and temp sensation in thoracic and cervical area
holoprosencephaly
non-NTD, forebrain does not divide into two hemispheres
hydrocephalus
non-NTD, CSF overproduction dilates ventricles, skull enlarges before sutures fuse
Dandy-Walker malformation
non-NTD, partial or complete absence of cerebellar vermis
Arnold-Chiari malformation
non-NTD, bone blocking normal passageway in skull, surgery required
Arnold-Chiari malformation type 1
asymptomatic until 30s
Arnold-Chiari malformation type 2
herniation in foramen magnum, myelomeningocele
Arnold-Chiari malformation type 3
occipital encephalocele, syringomelia
Arnold-Chiari malformation type 4
lack of cerebellar development
fetal alcohol syndrome
underdeveloped corpus collosum, very toxic to synpatogenesis 6months - 2 years, underdeveloped cortex
testing CN I olfactory (smell)
pass odors
testing CN II optic (acuity, fields, pupils)
chart, confrontation, eyes individually
testing CN III occulomotor (pupils, eye movements)
direct, conjugate, consensual, flash light, MR, IR, SR, IO
testing CN IV trochlear (superior oblique)
conjugate
testing CN VI abducens (lateral rectus)
conjugate
testing CN V trigeminal (facial sensation, corneal response, mastication)
bite down, cotton, pin, tuning fork, compare sides
testing CN VII facial (facial movement)
observe, squeeze eyes shut, smile, puff out cheeks, forehead muscle spared with a CNS lesion (stroke)
CNS lesion - facial appearance
forehead spared, wrinkles evenly
CNS lesion - facial appearance
forehead affected, wrinkles unevenly
testing CN VIII vestibulocochlear (hearing, balance)
dizziness, vertigo, finger rubbing, weber, rinne
testing CN IX glossopharyngeal
swallow, horseness, palate elevation
testing CN X vagus
swallow, horseness, palate elevation
neuronal storage diseases
autosomal recessive enzyme deficiency, substrate accumulates, loss of cognitive function, seizures
leukodystrophies
myelin abnormality, autosomal recessive, problem with lysosomal or peroxisomal enzymes, loss of motor, spasticity, hypotonia, ataxia, less cognitive problems
neuronal ceroid lipofuscinoses
deficient enzymes for protein modification, lipofuscin accumulates, blindness, mental and motor problems, seizures, childhood to adulthood
Tay-Sachs disease
hexosaminidase A deficiency, accumulation of gangliosides, Ash. Jews, early infancy, developmental delays, paralysis, blind, deaf, death, born normal, bulging lysosomes, cherry red spot on retina
Krabbe Disease
deficiency in galactosylceramidase, galactocerebrosides accumulate, converted to galactosylsphingosine, toxic to oligodendrocytes, loss of myelin and oligodendrocytes in CNS and PNS, fat globoid macrophages in brain, 3-6 months onset, muscle stiffness, weakness, stumbling, trouble swallowing, stiff hand change
B1 - thiamine deficiency
chronic, Wernicke encephalopathy, Korsakoff syndrome
Wernicke encephalopathy
alcoholism, acute, reversible, hemorrhage and necrosis in mammillary bodies, confusion, ophthalmoplegia, ataxia, nystagmus
Korsakoff syndrome
alcoholism, thalamic lesions, prolonged, irreversible, memory problems, confabulation, cystic spaces and hemosiderin macrophages in mammillary bodies
B12 deficiency
anemia, reversible, B12 administration
B12 deficiency
subacute spinal cord degeneration, numbness, axatia, weakness, reversible until paraplegia, myelin swelling, vacuolization, ascending and descending tracts
hypoglycemia
pyramidal neurons most vulnerable = pseudolaminar necrosis, hippocampus and cerebellum vulnerable
hyperglycemia
diabetes mellitus, ketoacidosis or hyperosmolar coma, dehydration, confusion, stupor, coma
carbon monoxide
hypoxia, forms carboxyhemoglobin preventing O2 from bonding, cortex / hippocampus / Purkinje cells vulnerable, demyellination of tracts, looks pink upon death
methanol (antifreeze)
retina, degeneration of ganglion cells, blindness, only 10ml to kill
ethanol (alcohol)
acute = reversible, chronic = irreversible, affect cerebellum on midline, truncal ataxia, unsteady gait, nystagmus, Bergmann gliosis - lot of astrocytes
Guillain-Barre syndrome
PNS, life threatening, inflammatory, flu-like, acute symmetric ascending paralysis over a week, loss of tendon reflexes and sensation, elevated CSF proteins, immune mediated demyelination (T cells and macrophages) especially in spinal / cranial nerve motor roots, loss of respiratory kills, remyelination follows
leprosy (Hansen Disease)
PNS, infectious, slow progressive infection of skin and nerves, mycobacterium leprae, forms granulomas, transmitted by respiratory droplet, tropical countries, deformities
Mycobacterium leprae
acid fast (does not gram stain), obligate intracellular bacteria, hard to kill, body forms granulomas around it
tuberculoid leprosy
PNS, localized - less severe, dry, scaly skin lesions, nerve degeneration, T-cell response with granulomas
lepromatous leprosy
PNS, serve, widespread, skin, nerves, eyes, mouth, testes, hands, feet, no immune response, leonine face, autoamputation of toes, foamy histiocytes
varicella-zoster virus
PNS, chickenpox dormant on sensory ganglia, reactivation = painful vesicular rash, neuronal destruction, multinucleate giant cell
hereditary neuropathy
progressive, disabling, motor, sensory, or autonomic, possible amyloid deposition or metabolic disorders
neuropathy type 1 (Charcot-Marie-Tooth disease)
demyelinating / remyelinating, muscle and sensation loss, pain intact, childhood / early adulthood, high arches, hammer toes, foot drop, legs with inverted bottle appearance, nerves with onion bulb appearance
diabetic peripheral neuropathy
common, distal nerves, stocking - glove, symmetric sensory and motor neuropathy, decreased pain sensation in distal extremities, ulcers on ball of foot
malignancy related neuropathy
mononeuropathy on one side of body common, ex: brachial plexus = lung neoplasm, obturator palsy = pelvic neoplasm, cranial nerve = brain tumor
paraneoplastic effect
polyneuropathy, collection of symptoms with cancer, small cell lung cancer, plasma cell malignancy
vasogenic cerebral edema
increased vascular permeability, intercellular spaces, localized or generalized
cytotoxic cerebral edema
cell membrane injury, intracellular fluid increased = big cells, hypoxia or metabolic damage, Tx mannitol
hydrocephalus
excessive CSF in ventricular system, infancy = head enlarged, after infancy = increased cranial pressure, five kinds: communicating / non-communicating / ex vacuo / increased CSF production / normal pressure
noncommunicating hydrocephalus
block in ventricular system (tumor, abcess, malformation), only part of ventricular system enlarged, CSF is not able to leave part of ventricular system and circulate in subarachniod space
communicating hydrocephalus
block in subarachnoid space, entire ventricular system enlarged, meningitis, hemorrhage, dural thrombosis
hydrocephalus ex vacuo
no ventricular block, ventricular system dilates due brain atrophy, Alzheimer’s Pick disease, massive hypoxia
hydrocephalus due to increased CSF
uncommon, choroid plexus papilloma
normal pressure hydrocephalus
elderly, gait problems, incontinence, dementia, large ventricles, no cortical atrophy, reversible, like communicating hydrocephalus, misdiagnosed as Alzheimer’s
increased intracranial pressure
brain volume increases, mass effect, compromised blood flow, herniation
brain herniation
part of brain gets pushed into another compartment, headache, vomiting, decreased consciousness, papilledema, fatal, tumor, abscess, hematoma, edema, encephalitis, hemorrhage, types - subfalcine, transtentorial, tonsilar
subfalcine herniation
cingulate gyrus under falx, compression of anterior cerebral artery, limb weakness, aphasia, speech problems
transtentorial herniation
uncinate, temporal lobe against tentorium, CN III - pupil dilation and ocular movement, brainstem - impaired consciousness, posterior cerebral artery - ischemia to visual cortex, contralateral cerebral peduncle
case - football player knocked out, normal CT scan, headaches all week, collapsed Friday night
left subdural hematoma, midline brain shift, decreased vision in right eye, limited left ocular motility, ptosis of left eye, caused a tentorial herniation with compression of CN III and posterior cerebral artery
tonsillar herniation
cerebellar tonsils through foramen magnum, compressed medulla, compromised vitals, life threatening
linear skull fracture
common, straight, not serious, heals
depressed skull fracture
inward, comminuted, damage brain
diastatis skull fracture
across a suture, suture widens, children
basal skull fracture
more force, distant hematoma, CSF from ear or nose, raccoon eyes, battle sign bruise behind ear, ex: temporal bone fracture in petrous part
concussion
parenchymal injury, altered consciousness, change in momentum, amnesia, confusion, headache, nausea, vomiting, dizziness, Grade 1 - 15 min, Grade 3 - LOC
direct parenchymal injury
tearing or bruising of parenchymal tissue, ex: laceration from bullet, contusion, coup injury, countercoup injury
diffuse axonal injury
twisting / shearing of axons in deep white matter of brain, angular acceleration / deceleration, persistent deficits or coma, shaken baby, boxers, can cause small hemorrhages and axonal spheroids
second-impact syndrome
second concussion before first one heals, brain swells rapidly, young athletes
chronic traumatic encephalopathy
killed Derek Boogard, progressive, degenerative, repetitive brain trauma, behavior and personality changes, dementia, tau proteins deposited
coup injury
contusion on brain at point of contact with fixed object, happens second
countercoup injury
contusion opposite point of impact with fixed object, often worse, happens first becaause brain remains suspended in CSF when skull deaccelerates into object
berry aneurysm
aneurysm at branch point in circle of Willis, most anterior cerebral artery, some middle cerebral artery, few posterior cerebral artery, Tx with coils
ischemia
local anemia due to lack of blood flow
infarction
severe ischemia leading to cell death and necrosis
thrombus
clot that blocks blood flow, can lead to ischemia or infarction, often extracranial
embolus
clot that forms in another location and travels to the brain and blocks blood flow, can lead to ischemia or infarction, often intracranial
hemorrhage
bleeding from blood vessel, can be associated with hypertension, can be intracerebral or aneuryism
middle cerebral artery syndrome
affects supratentorial structures supplied by middle cerebral artery
anterior cerebral artery syndrome
affects supratentorial structures supplied by anterior cerebral artery
lateral medullary syndrome (PICA)
effects infratentorial structures supplied by the inferior posterior cerebellar artery
medial medullary syndrome
effects infratentorial structures supplied by the anterior spinal artery and penetrating branches of the vertebral arteries
Weber’s syndrome
effects infratentorial structures
multiple sclerosis presentation
white female, 30s, light-headed, problems concentrating, forgetful, symptoms worse in summer (channels closer faster), problems typing, numb fingers, dizziness, depression
multiple sclerosis physical exam findings
normal EKG, orthostatic hypotension (BP lower and HR higher when standing), hyperreflexive 3+ = CNS problem (hyporeflexive = PNS), positive Babinski (CNS no longer inhibits reflex - extensor plantar reflex), struggle with rapid hand movement
multiple sclerosis lab results
normal - WBC, Hb, platelets, ESR, B12, folate, syphilis, TSH, electrolytes; lumbar puncture CSF - normal pressure, color, protein con, RBC, WBC - abnormal high IgG, high albumin, oligoclonal bands (shows IgG presence), abnormal visual evoked potential (slower and weaker), plaques on MRI
multiple sclerosis tx
prednisone, interferon B 1a (avonex); side effect immunosuppression
multiple sclerosis cause
autoimmune demyelinating disease, blood brain barrier become more leaky, immune cells - macrophages - attack myelin in CNS
multiple sclerosis areas affected
Cognitive function (esp. memory loss) - frontal and temporal lobes
Mood (especially depression) - limbic system
Sensory systems - vision occipital, hearing temporal, somatosensory postcentral
Motor control and coordination - precentral and cerebellum
Autonomic nervous system function (esp. cardiovascular system) - parasympathetic and sympathetic control of BP in heart and vessels
myasthenia gravis
autoimmune attack of nicotinic ACh receptors
trigeminal neuralgia
acute, excruciating, lightning like facial pain, near nose, lips, eyes, or ears, usually involves V2, pain caused by touch to lips or nose, nerve is compressed by superior cerebrellar artery, tx putting sponge between them
bell palsy
paralysis of facial muscles on affected side, facial nerve in distal facial canal or stylomastoid foramen, drooping lips and lower eyelid, loss of facial expression
craniosynostosis
premature closure of one or more sutures in the skull produces a misshapen skull
craniosynostosis
premature closure of one or more sutures in the skull produces a misshapen skull
brachycephaly
fusion of coronal suture, skull shortened front-to-back, flat head
brachycephaly
fusion of coronal suture, skull shortened front-to-back, flat head
oxycephaly, turricephaly, acrocephaly
fusion of coronal and lambdoidal sutures, tower skull
oxycephaly, turricephaly, acrocephaly
fusion of coronal and lambdoidal sutures, tower skull
plagiocephaly
fusion of coronal and lambdoidal sutures unilaterall, asymmetrical skull
plagiocephaly
fusion of coronal and lambdoidal sutures unilaterall, asymmetrical skull
scaphocephaly
fusion of sagittal suture, long skull front-to-back
scaphocephaly
fusion of sagittal suture, long skull front-to-back
trigonocephaly
premature fusion of metopic suture, forehead looks pointed
lambdoidal suture positional flattening
back of skull flattens from baby sleeping on their back, will self correct
microcephaly
small head due to brain that failed to grow, may be NTD, genetic, obstructed blood supply, prenatal drugs, maternal rubella / chicken pox
cranioschisis
meninges and / or brain herniate through skull defect, more common in occipital bone
meningocele
meninges only herniated
meningoencephalocele
meninges plus brain herniated
meningohydroencephalocele
meninges plus brain and ventricle herniated
Apert syndrome
autosomal dominant, craniosynostosis plus other defects like syndactyly of hands/feet and retardation, mutation of fibroblast growth factor receptor 1 and 2
acrania
no skull
anencephaly
no brain
meroanencephaly
poorly formed brainstem tissue present
hydrocephalus
enlarged ventricles due to too much CSF, if before sutures close = large head
congenital hydrocephalus
associated with spina bifida and Dandy-Walker malformation
acquired hydrocephalus
associated with hemorrhage, stroke, infection, tumor, and TBI
brachial cyst
form from remnants of brachial arch
syncope
fainting, problem in intermedolateral cell column, can be seen with MS
subarachnoid hemorrhage
image with axial CT, shows blood pooled in lateral ventricles and hemorrahge interhemispheric fissure
