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