Neurology
Terms
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- Seizure is a
- temporary disruptin of brian fuction resulting from abnormal, excessive, synchronous cerebral neuron discharge. Diagnosed with epilepsy when unprovked seizures recurrent
- Encephalopathy cause infection
- aids, varicella, mumps, measles, entrovirus, cyto, herpes simplex, lymes, TB
- Encephalopathy cause focal findings
- of (hemiparesis, ataxia, cranial nerve defects) on examination and seizures are more common with herpes symplex HSV encephalitis
- CP tx
- botulinum toxin motor point blocks - have helped with
- Jacksonian seizures
- are partial motor seizures - rhythmic twitching beings in one extermity and MARCHES proximally until entire limb involved
- white matter signs
- leukodystrophies, inherited progressive degenerative diseases resulting from abnomrally formed myelin, impaired conduction and rapid myelin breakdown. young patis with spasticity and developmental milestone loss.
- Tick paralysis
- resembles guillian barre - ticks
- generlized seizures
- bilateral hemisphereic involvment - impaired consciousness, symmetric bilateral activity and a POSTICTAL phase of confusion and lethargy
- Cerebral palsy is a
- nonprogressive disorder of movement and posture that results form a fixed lesion of the immature brain.
- Tonic-clonic
- what most people think of - tonic phase, sustained flexor or extensor contractions - episodes interspiresed with clonic activity, consiting of rhythmic symmetric generalized contractions of the trunk and extermity muscle
- Cheyne-Stokes respiration
- also known as periodic breathing) is an abnormal pattern of breathing characterized by oscillation of ventilation between apnea and hyperpnea
- Phakomatoses
- neurocutaneous diseases - lesions in the nervious system, skin, eyes. - three autosomal dom conditions - neurofibromatosis, tuberous sclerois, and von Hippel Lindau dz.
- hydrocephalus PE
- upward gaze paralysis (setting sun sign), diplopia (third or sixth cranial nerve palsies or both), Clonus, a postiive babinski test or excessively brisk deep tendon reflexis
- Partial Seizures types
- simople partial (intact consicousness) [motor, sensory, autonomic, psychic], Complex partial (impaired consciousness), Partial seizures with secondary generalization
- generalized seizures are always
- associated with impairment of consciousness
- cushing triad is
- hallmark of increased intracranial pressure.
- infantile spasms
- devasting generalized seizure syndrome, 2-7months age, recurrent mixed flexor-extensor spasms, last only a few seconds, but may repeate more than 100 time sin a row.
- ischemic strokes
- rare in children, think sickle cell, congenital
- spina bifida
- neural tube defects in the spinal region - incomplete fusion of vetebral arches -(myelomeningocele, meningocele, spina bifida occulta)
- gower sign
- DMD, first leaning on the hypertrophied calves and then pushing the truck up with the arms
- hydrocephalus DDx
- acute intraventricular bleed, diffuse brain edema - do a CT/MRI to tell difference
- glasgo coma score verbal 3
- inappropriate words, cries to pain
- headache that awake sleep
- suspicious for increased intracranial pressure. worse by bending, sneezing, or straining
- noncommunicating hydrocephalus example
- Chairi Type II malformation, and spina bifida occulta
- tabes dorsalis (neurosyphilis)
- postive rromberg test
- gray matter signs
- hypotonia, mental retardation, seizures, retinal degeneration, and ataxia are common
- microcephaly
- describes head circumference - greater than 2 standard deviations BELOW the mean head size for age. trisomy 21, prader-willi syndrome, or congenital infections/insults. seizures not uncommon
- Encephalopathy - metabolic disorders
- reccurrent episodes of mental status changes that clear when the acute process is corrected
- glasgo coma score motor 5
- localizes pain, withdraws to touch
- seizure other options
- remove focus surgically, ketogenic diet, vagal nerve stimulator.
- neural tube defects
- failur eof neural tube closure during the third and fourth weeks of gestation - alpha fetoprotein at 16-18wks - tx folic acid prior conception and early preg
- Hydrocephalus
- pathologic enlargement of the ventricles that occurs when CSF production outpaces absorption - secondary to outfllow obstruction
- adrenoleukodystrophy
- classic white matter disorder, characterized by areas of demyelination. associated with adreanl insufficency. psychomotor retardation progresses to spasticity, extensor posturing and death by early childhood
- complicated migraine
- when accompanied and/or followed by transient nuerologic deficits such as weakness/paralysis, sensory loss, difficulty speaking, or alterations in vision or mental status
- encephalopathy many causes
- disruption of any of these will lead to generalized cerebral dysfunction. Function normally : the brain needs adequate blood flow, oxygen, energy substrates, removal of metabolic waste and appropate electrolyte balance
- extrapyramidal CP
- rare. damage to basal ganglia, involved in regualtion of muslce tone. pts have involuntary choreoathetoid movements and postural ataxia
- Poliomyelitis
- viral illness affecting primarily the anterior horn cells of the spine. to avoid we use kill virus in vacine
- noncommunicating hydrocephalus is
- block exists somewhere within the ventricular system, and ventricles ABOVE the obstruction are selectively enlarged
- absence seizure tx
- ethosuximide
- Tuberous Sclerosis
- ash-leaf spots (flat, HYPO pigmented macules), shagreen patches (areas of abnormal skin thickening). diag with Wood Lamp.
- hydrocephalus history
- inappropriate increase in Head circumference, or bulging anterior funtanelle - perhaps poor feeding, irritablity, lethargy, apnea, and bradycardia.
- anecephaly
- born large skull defects virtually no cortex - neural tube defects
- migraine headaches caused
- by vasodilation of intracranial vessels in response to a vascular or neuronal stimulus
- glasgo coma score verbal 4
- confused
- headaches benign
- tension-type
- petit mal on EEG
- generalized, symmetric three-per-second spke and wave pattern
- complex partial seizures
- a partial seizure, result in alteration or impairement of consciousness. Semipurposeful movement continues without direction, or the child may begin lip pursing or picking at his or her clothes.
- rett's syndrome
- x-linked, girls, normal till 1 year, then microcephaly and developmental milestone regression. hand wringing, seizures, ataxia autisticbehavior
- Encephalopathy PE
- mental status changes, odd or inappropriate behavior, disorientation, a shortened attention span, cognitive deficits, lethargy, stupor and/or coma
- sturge weber's dz
- disorder of neurologic deterioration associated with portwine stain (nevus flammeus) - over the area innervated by the FIRST division of the trigeminal nerve (V). mental retardation, seizures, hemiparesis, and visual impairment.
- petit mal
- always being with children younger than 10y, brief staring episodes, associated with alterations in consciousness. child is unaware, may occur hundreds of times a day
- communicating hydrocephalus is
- all ventricles are proportionately enalrged, occurs when subarachnoid villi are dysfunctional or obliterated.
- ataxia (incorrdination) DDx
- includes labyrinthitis, acute ingestion, acute postinfectious cerebellar ataxia, ataxia-telangiectasia, and Friedreich ataxia
- acute epidural trauma
- unilateral, impaired-lucid-impaired, on CT with contrast biconcave, ipsilateral
- hydrocephalus Tx
- acetozolamide decreases CSF production effective short term, for mild cases. Indwelling shunts - complications - infection Stafphylococcus epidermidis.
- glasgo coma score motor 4
- withdraws to pain
- acute cerebellar ataxia
- viral illness may cause
- Complex febirle seizures
- last 10 to 15 minuts, recur within 24 hours, have focal features.
- goal treatment head trauma
- optimize cerebral perfusion pressure
- atonic seizure
- abrupt total loss of postural tone, lasting several minutes
- Guillain Barre's syndrome
- acute-onset, progressive, ascending weakness caused by autoimmune-mediated demyelination, more half develop after 7 to 21 days, initially numbness distal followed by progressive ascending weakness, deep tendon reflexes wane and disappear
- Friedreich ataxia
- later in childhood w/ progressive ataxia, weakness, and muscle wasting. Skeletal deformities invariably follow. Most die of cardiomyopathy-related heart dz. Autosomal recessive. chrom 9
- spina bifida occulta
- bony vetebral lesion occurs WITHOUT herniation of any spinal contents
- Cushing triad signs
- PE sign of hydrocephalus, a triad of bradycardia, hypertension and Cheyne-Stokes respirations
- encephalitis caused by herpes simplex virus
- present with focal neurologic findings and seizures. The characteristic EEG shows focal spking in the mediotemporal area, and temporal inflammatory lesions are demonstrated on MRI
- communicating hydrocephalus example
- subarachnoid hemorrhage and meningitis (TB, funci, ) - meningeal inflammation and scaring
- Encephalopathy - Reye's syndrome w/ asprin
- a rare mitochondrial disorder characterized by acute-onset encephalopathy and degenerative liver disease, may follow a viral illness, signs : vomiting, delirium, stupor, hypoglycemia, elevated transaminase, and ammonia levels
- von Recklinghausen's disease
- neurofibromatosis types 1
- diffuse axonal injury
- shearing forces on the white matter of the brain that occur with rapid deceleration of the head
- craniosynostosis
- premature fusion of one or more cranial sutures.
- glasgo coma score motor 3
- abnormal flexion
- raccon eyes
- basilar skull fractures are characterized by postauricular (battle sign) or periorbital (???)
- migraine tx
- sumatriptan or ergotamine both vasoconstrictors
- von Hippel Lindau's disease
- retinal angiomas (abnormal masses of the thin-walled capillaries), cerebellar hemangioblastomas, and associated neoplasms, including RENAL cell cafrcinoma and pheochromacytoma.
- hydrocephalus eval
- CT scan. LP should NOT be performed in the presence of markedly increased intracranial pressure becuase of the risk of herniation of the brainstem through the foreman magnum
- myoclonic seizures
- simple short jerks similar to those occasionally experienced by normal subjects while in light sleep
- encephaloceles
- are projections of cranial contents through a bony skull defect - - neural tube defects
- acute subdural trauma
- bilateral, direct trauma or shaking, seizures, retinal hemorrhages, appears on CT as crescent,
- spinal muscle atrophay
- inherited, degeneration of the anterior horn, SMA type 1 (Werdnig Hoffmann' dz) early infancy w/ gen hypotonia and weakness
- CP common form
- spastic CP (pyramidal, which is the consequence of injury to motor tracts in the brain. - increased muscle tone in the affected limbs.
- CP PE
- generally hypotonic first few months, later develping characteristic spasticity, fails motor developmental milestones.
- myelomeningoceles
- protruding sacs of neural and meningeal tissue, whiel meningoceles contain meninges only bowel and bladder sphincter dysfunction, and sensorimotor loss occurs below the lesion
- hypsarrhythmia
- EEG pattern diagnositc for infantile spasms is calld
- ataxia-telangiectasia
- autosomal recessive neurodegenerative disorder that presents in toddlers and progresses to wheelchair dependence.
- headache pseudotumor cerebri
- syndrome of recurrent headaches caused by increased intracranial pressures inthe face of normal neuro imaging idiopathic intracranial hypertension, uncommon, in overweight adolescent females, or tetracycline or corticosteroid use
- gray matter disorders
- tay-sachs, gaucher, and niemann-pick dz. lipid buildup in neuronal cell bodies.
- glasgo coma score eye 3
- to speach
- tourette's syndrome
- motor AND vocal tics, involuntary behaviors that are repetitive.
- neurofibromatosis type 2
- chrom 22, bilateral acoustic neurofibromatosis. hearing loss and vestibular disorientation. 8CN masses - neurofibromas, meningiomas, schwannomas, and astrocytomas
- brain hemorrhages history
- vomiting, severe headache, and mental status changes, suggest increased intracranial pressure. confusion, loss of consciousness, amnesia, seizures, and visual impairment
- headache tension type PE
- diffuse, constant, symmetric, and "bandlike".
- lennox gastaut's syndrome
- infantile spasms may evolve into this, characterized by frequent occurrence of mixed generalized deizures
- myasthenia gravis
- autoimmune disorder
- Duchenne type muscular dystrophy
- x-linked recessive dz of muscle tissue. presents early childhood with motor delay
- spina bifida and
- Chiari Type II malformation - an anatomic abnormality of the hindbrain that poses significant risk for hydrocephalus
- neurofibromatosis type 1
- two of the following must be present. 1) 6 or more cafe-au-lait spots > 5mm in children, and > 15mm adolescents/adults 2) axillary or inguinal freckling, 3) two or more lisch nodules (harmatomas) in the iris 4) two or more neurofibromas or one plexiform neurofibroma 5) a distinctive osseous lesion such as sphenoid dysplasia, 6) optic gliomas 7) affected first degree relative
- simple febrile seizure
- lasts less 5 to 10 minutes, not generlized, and does NOT recur during the precipitating illness epsiode. rapid rise temp the important determinant (over 102, child 6m to 5y)
- macrocephaly
- head circumference greater than 2 standard deviations. big brain, cranioskeletal dysplasias, storage disease, hydrocephalus possible.
- Partial seizures define
- small focus in one hemisphere is involved, child remains conscious and there is NO postictal phase
- signs of increased intracranial pressure
- include sixth nerve palsy and papilledema
- Generalized Seizures types
- absense (typcial, atypical), toinic, clonic, tonic-clonic, myoclonic. atonic, infantile spasms
- neural tube defects screen
- alpha fetoprotein level at 16 to 18weeks
- seizure disorder diagnosis
- based on historcal account
- Kernicterus causes
- extrapyramidal CP