Neuro Flashcards
Radial Nerve function
Extension of wrist arm and finger joints below shoulder! Forearm supination, thumb abduction.
Injury or Radial N
At passage through supinator canal (due to repetitive pronation/supination, direct trauma, or sublexation of radius)
Features or Fragile X
Neurobehavioral problem
Prominent forehead and chin
large ears
long, narrow face
Large testis
hyperlaxity of joints in hand
Cytogenic: small gap near tip of log arm of X chr
Fragile X Syndrome
X linked mutation of FMR1 gene
Huntington Disease
CAG trinucleotide repeats of huntington gene
Leads to gain of function mutation which is responsible for transcription repression via histone deacetylation
Cause of acute hypertensive intracerebral hemorrhage
Charcot-Bouchard aneurysm (chronic hypertension leads to progressive arteriolar hyalinization and fibrinoid necrosis)
Often involves deep structures (basal ganglia, thalamus, pons)
AVM
Most common cause of intracranial hemorrhage in children
Phenytoin
Seizure medication (blocks voltage gated sodium channels in neurons)
Side effects:
- gingival hyperplasia
- ataxia and nystagmus
- disruption of folic acid metabolism (megaloblastic anemia)
- p450 inducer
- coarse facial features, hirsutism
- fetal hydantoin syndrome
Locus Ceruleus
Located at posterior rostral pons near lateral floor of 4th ventricle
Responsible for producing almost all NE in brain
Ataxia-Telangiectasia
cerebellar ataxia
telangiectasia
sinopulmonary infections
mutation of ATM gene (AR) responsible for DNA Break Repair
cerebellar atrophy
Pink MacConkey agar
organisms that ferment lactose (e.coli, Klebsiella, Enterobacter)
Narcolepsy
Cataplexy (loss of muscle tone, abnormal facial movements)
Hypocretin-1 deficiency in CSF
REM sleep latency <15 min
Recurrent lapses into sleep multiple times within the same day at least 3*/week for 3 months
Cluster Headache
Behind one eye
15-90 minutes
Nasal congestion and lacrimation
Tension Headache
stress, band-like pattern (b/l), bull tight, persistent
Muscle tenderness
Femoral Nerve Block
At inguinal creases, anesthetizes skin and muscles of anterior thigh, femur, and knee
Gerstmann syndrome
- Agraphia (inability to write)
- Acalculia (inability to do math)
- finger agnosia
- left-right disorientation
Stroke of angular gyrus of dominant parietal lobe
s-100 positive tumors
Schwannoma and melanoma (derived from neural crest)
Wilson Disease
AR mut in ATP7B, hinders hepatocellular copper transport
Reduced secretion of ceruloplasmin and decreased secretion of copper
Copper, a pro-oxidant, causes hepatocellular injury
Neuro involvement includes movement abnl and psych symptoms
Rings around iris
HIV-associated dementia
- subcortical dementia (attention/memory problems, executive dysfunction)
- due to inflammatory activation of microglial cells
Intranuclear acidophilic inclusions
HSV
Intranuclear basophilic inclusion
CMV
Vitamin E Def neuro symptoms
Mimics Friedreich Ataxia
Loss of position and vibration sense
Loss of DTR
Ataxia
Friedreich Ataxia
AR degenerative dz
Loss of proprioception and vibration sense (damage to dorsal columns)
Ataxia (loss of spinocerebella tract)
Spastic weakness (loss of lateral corticospinal tract)
Hypertrophic cardiomyopathy
Kyphoscoliosis
high arched feet
Vitamin A Toxicity
Acute: nausea, vomiting, vertigo, double vision
Chronic: alopecia, dry skin, lyperlipidemia, hepatotoxicity, hepatosplenomegaly, visual difficulties pailledema
Teratogenic: microcephaly, cardiac anomalies, fetal death
Niacin deficiency
Pellagra (3 D’s)
- Dementia
- Dermatitis
- Diarrhea
Wernicke Encephalopathy
Chronic Thiamine deficiency (alcoholics)
- Opthalmoplegia
- Ataxia
- Confusion
Hemorrhage and necrosis of mammillary bodies
Baseline erythrocyte transketolase activity low
tx: thiamine supplementation with glucose infusion
Axonal Reaction
Change seen in neuronal body after axon is severed
- cellular edema
- nucleus displaced to periphery
- Nissl substance fine, granular, and dispersed throughout cytoplasm
Most common side effect of levodopa/carbidopa
Anxiety and agitation
N. meningitidis virulence factor that underlies disease severity
Lipooligosaccharide
endotoxin
Length and time constant
- Length constant: measure of how far along an axon electrical impulses can propogate without requiring active regeneration by ion channels
- Time constant:
measure of time it takes for membrane potential to respond to membrane permeability
demyelination decreases length constant and increases time constant
Nerve responsible for sensation at suprapubic and gluteal region
Iliohypogastric nerve (L1 root)
Often injured during appendectomy
Common Peroneal Nerve
responsible for ankle dorsiflexion, eversion and toe extension
Sensory to lateral leg and dorsal foot
Damage (compression or fracture of lateral neck of fibula) leads to foot drop
Diabetic mononeuropathy
CNIII central ischemia
Ptosis, down and out gaze, normal light and accomodation
Berry aneurysm of COW
Often seen in ADPKD
Rupture causes subarachnoid hemorrhage
Epidural hematoma from
fracture of temporal bone and tear of middle meningeal artery
Medulloblastom
sheets of primitive cells and many mitotic figures
Located in cerebellum (vermis)
MLF
In pons
Lesion: impaired adduction of ipsilateral eye during conjugate horizontal gaze
Subarachnoid Hemorrhage
Most commonly due to berry aneurysms
Baclofen
Agonist at GABA-B receptor for spasticity (Spinal cord dz, MS)
Tizanidine
alpha 2 agonist used for spasticity in spinal cord disease
NF1
AD mutation of NF1 TS gene
- cafe au lait
- CNS neoplasms
- cutanous neurofibromas (from schwann cells/neural crest origin)
Conversion disorder (functional neurologic symptom disorder)
Neurologic symptom incompatible with any known neurologic disease (often associated with stress)
Spinal Muscle Atrophy
mutation in SMN1 (assembly of snRNPs)
Impaired spliceosome function leads to degradation of anterior horn cell sin spinal cord with flaccid paralysis
Lobar hemorrhage
Cerebral amyloid angiopathy (beta amyloid deposition in vessel walls)
often occipital and parietal lobes
Marcus Gun Pupil
afferent pupillary defect
often due to optic tract lesion
Optic tract lesion
contralateral homonymous hemianopia and marcus gun pupil
C. Botulinum toxin
Inhibits ACH release from presynaptic terminals at NMJ and decreases CMAP (electric response of muscles)
3 Ds: diplopia, dysphagia, dysphonia
Pineal gland mass
- obstructive hydrocephalus (papilledema, h/a worse while lying, vomiting)
- Parinaud syndrome (limited upward gaze, b/ eyelid retraction, light-near dissociation)
Most commonly: germinoma
Orbital floor fracture
damage to infraorbital nerve
numbness to upper cheek, upper lip, upper gingiva
Battle sign
bruising over mastoid process
basilar skull fractures
Guillain-Barre
Ascending symmetric weakness following febrile illness
demyelination due to molecular mimicry
inflammatory infiltrate within endomesium and lipid laden macrophages
Thalamic stroke
contralateral pure hemisensory loss
VPL (spinothalamic and DCML input)
VPM (trigeminal)
PCA infarct
contralateral hemianopia
contralateral paresthesia and numbness (involvement of thalamus)
Middle cerebral artery stroke
mostly likely ischemic
contralateral motor and sensory deficits to upper > lower extremities and face
Uhthoff phenomenon
MS symptoms worsen with increasing body temperature (exercise, hot shower)
Risk factors of MS
HLA-DRB1
Location
Low vit D
Smoking
Most common cause of sporadic encephalitis in adults
HSV type 1
Temporal lobe edema
HSV type 1 encephaopathy
VHL disease
AD
capillary hemangioblastomas in the retina and/or cerebellum,
congenital cysts in kidney, liver and pancreas
increased risk for renal cell carcinoma (b/l)
Sturge-Weber Syndrome
cutaneous facial angiomas (overlyng V1 and V2 regions)
leptomeningeal angiomas
mental retardation
seizures
hemiplegia
skull radiopacities (tram-track calcifications)
Jugular foramen contents
Jugular vein CN IX (taste post 1/3 tongue) CN X (dysphagia/hoarseness, soft palate drop and deviation of uvula toward normal side) CN XI (SCM and trapz paralysis)
Gag reflex (IX, X)
Obturator nerve
Thigh adduction and sensation over distal, medial thigh (passes through obturator canal)
Restless leg syndrome
due to iron deficiency, uremia, diabetes or idiopathic
Tx: Dopamine agonist (pramipexole)
Tay Sachs
beta-hexosaminidase A deficiency
Accumulation of GM2 ganglioside in lysosomes
Lesch Nyhan
deficient HGPRT (hypoxanthine-guanin phosphoribosyltransferase) .of purine salvage pathway
Thiamine deficiency
Wernicke encephalopathy (ataxia, nystagmus, ophthalmoplegia, anterrograde amnesia)
Worsened with dextrose administration
Necrosis of mammillary body
Hemiballism
Movement disorder with wild, involuntary, large amplitude flinging movements of proximal limbs
due to basal ganglia lesion (subthalamic nucleus)
often due to lacunar stroke of htn or dm
Pancoast tumor
non-small cell lung cancers
Involvement of brachial plexus (ipsilat shoulder pain, upper limb parasthesia, areflexic arm weakness)
involvement of cervical sympathetic ganglia (ipsilateral ptosis, miosis, anhydrosis)
Chemotherapy n/v
area postrema at dorsal surface of medulla and cauda end of 4th ventricle
WNV neuro symptoms
Asymmetric flaccid paralysis
parkinsonian features (rigidity, bradykinesia, tremor, postural instability)
Wilson Disease
AR mut of APT7B
hepatic copper accumuation, leak from damaged hepatocytes, deposits in other tissues
Clinical findings (hepatitis, cirrhosis, parkinsonism, gait disturbances, depression, personality changes)
Diagnosis: Low ceruloplasmin, high copper urinary excretion
Kayser fleischer rings
Tx: Chelators (D-penicillamine, trientine)
zinc (interferes wit copper absorption)
Ventricles
Lateral ventricle foramen of monro third ventricle cerebral aqueduct fourth ventricle Magendie and luschka
3 mutations associated with early onset alzheimers
APP gene (chr 21)
Prenselin 1 gene (chr 14)
Presenilin 2 gene (chr 1)
Atrophy of alzheimers
Most pronounced in temporoparietal lobes and hippocampus
Treacher Collins Syndrome
Abnl development of 1st and 2nd pharyngeal arches
craniofacial abnl airway compromise, feeding difficulties, abnl ossicles
Peroxisome function
Oxidation of VLCFA
H2O2 degradation
Proteasome function
degradation of ubiquinated proteins
Zellweger sydrome
defected peroxisomal biogenesis
craniofacial abnl, hepatomegaly, neuro defects, elevated VLCFA and phytanic acid
X-linked adrenoeukodystrophy
Defective transport of VLCFA into peroxisomes
Present later with neuro deterioration, adrenal insufficiency
Normal pressure hydrocephalus
Wacky (dementia)
Wobbly (gait disturbances)
Wet (urinary incontinence due to stretching of descending cortical fibers))
Huntington disease
Acute onset personality changes (agression, flat affect, decreased memoriy and concentration)
Movement disorder (jerky movmeent of extremities)
B/l atrophy of caudate and putatmen and loss of GABA neurons in brain
Triptans
Serotonin agonists used for migraine tx
Foot Drop
common peroneal nerve damage
Parotid gland
CN VII courses through it
Motor innervation to muscles of facial expression (damage leads to facial droop)
Vertical diplopia
Double vision when looking down (reading, walking down stairs)
Seen with trochlear nerve palsy (SO innervation)
Neuroblastoma
px at 2 years with abdominal mass, ptosis, myoclonus, etc.
elevvated catecholamine metabolites (neural crest origin)
small round blue cells and homer wright rosettes
Biochemical change seen in alzheimers
Decreased ACh levels in hippocampus and nucleus basalis of meynert
Endogenous opioid peptides
Enkephalins, endorphins, dynorphins
Derivatives of POMC
- beta-endorphins (endogenous opioid)
- ACTH
- MSH
Chiari type 1
crebellar tonsils herniate through foramen magnum px in adulthood with paroxysmal occipital headaches and cerebellar dysfunction (dizziness, ataxia)
Chiari type 2
downward displacement of cerebellum (vermis and tonsils) thorugh formen mangum
px in infants with aqueductal stenosis, dysphagia, stridor, lumbar myeomeningocele
Dandy Walker malformation
hypoplasia/absense of cerebella vermis with cystic dilation of 4th ventricles and enlarged posterior fossa