Neurology Flashcards
HA red flags that prompt further eval
dramatic increase in HA severity HA that awakens CH from sleep change in est. HA pattern gradually increasing frequency & severity suggests increasing ICP
PE finding for intracranial HTN
papilledema
PE for HA
growth, HC, BP
intracranial HTN (papilledema)
focal neurologic signs
general- rhinitis, dental abscess, bruit, head trauma, hematoma, skull step-off
red flags in hx/PE for HA may entail further investigation with what
head imaging (CT for hemorrhage, MRI for tumor & cerebellar imaging)
electroencephalography (EEG)
+/- sleep deprivation
tension HA
diffuse
symmetric
often related to fatigue
cluster HA
extreme deep pain in & around one eye
migraine
triggered by stess
vomiting
FH
migraine classification
migraine w/ aura (visual or otherwise)
“ “ w/o aura
complicated migraine (transient focal abnormality)
migraine equivelent
paroxysmal torticollis
attacks of head tilt, +/- vertigo, vomiting
requires r/o posterior fossa pathology
benign paroxysmal vertigo
attacks of unsteadiness w/ nystagmus & vomiting followed by sleep
may precede development of typical migraine
requires r/o epilepsy & CNS tumor
cyclic vomiting
protracted attacks of vomiting, 1-4x/hr, up to 5 days
requires r/o epilepsy, GI d/o, urea cycle d/o
abdominal migraine
attacks of migraine lasting 1-72 hrs, untreatable by other means
midline, dull, mod-severe pain
assoc. w/ anorexia, n/v, pallor
dx often by response to anti-migraine therapy
requires r/o other GI d/o
confusional migraine
episodic disorientation/ combativeness, sometimes followed by HA
requires r/o drug abuse, epilepsy, CNS ischemia
Therapy for migraine acute episode
sleep
acute tx- acetaminophen, ibuprofen, sumatriptan, other triptans
rescue tx- NSAIDs, promethazine, metoclopramide
migraine prophylaxis
create mgnt plant to prevent stressors
biofeedback (stress reduction)
physical modalities (massage, PT, exercise)
meds (usu. involves a neurologist)
cyproheptadine
antihypertensives (propranolol, verapamil)
TCAs- amitriptyline, nortriptyline
anticonvulsants- valproate, topiramate, gabapentin
HA summary
r/o underlying pathology address exacerbating factors mgnt plan meds (start w/ acetaminophen, ibuprofen) abortive agents daily prophylaxis (neurologist)
pseudotumor cerebri
increased ICP in absence of identifiable intracranial mass/ hydrocephalus
postulated to be d/t impaired CSF reabsorption
Risk factors of pseudotumor cerebri
obesity female sinus thrombosis head injury chronic CO2 retention SLE
Acute S&S of intracranial HTN in pseudotumor cerebri
HA pulse synchronous tinnitus pain behind the eye pain w/ eye movements transient visual obscurations blurred vision/double vision CN VI paresis vomiting macrocphaly altered behavior
Chronic S&S of intracranial HTN in pseudotumor cerebri
growth impairment
optic atrophy
visual field loss
total blindness
Diagnosing pseudotumor cerebri (one of exclusion)
CT- r/o hydrocephalus
MRI- r/o intracranial mass, hydrocephalus
Ophthalmologic- papilledema, optic n. changes
LP- measurement of opening pressure, normal CSF pnl & cx
Tx of pseudotumor cerebri
tx underlying causes- wt loss is mainstay of therapy, tx anemia
medical tx-
diuretics
acetazolamide (Diamox) causes peripheral paresthesias &
metabolic taste to carbonated soft drinks
furosemide (Lasix)
glucocorticoids
lumbar puncture
surgical tx- optic n. sheath decompression, lumboperitoneal shunt
Seizures
a sudden, transient disturbance of brain function, manifested by involuntary, motor, sensory, autonomic, or psychic phenomena, alone or in a any combo often accompanied by alteration of LOC
epilepsy
repeated seizures w/o evident cause
recurrent, unprovoked seizures
classification of seizures
symptomatic- cause is identified/ presumed
idiopathic- cause is unknown/ presumed to be genetic
Examples of symptomatic seizure
infxn (meningitis/encephalitis) trauma metabolic (hypoglycemia, hyponatremia) hypoxic tumor malformation (hydrocephalus)
nonepileptic paroxysmal events
benign nocturnal myoclonus shudder attacks tics & Tourette's syndrome sleep orders- night terrors, sleepwalking/talking, cataplexy, narcolepsy nightmares migraine- BPV, paroxysmal torticollis conversion rxn & pseudoseizure GERD masturbation hypoglycemia temper tantrums & breath-holding syncope & vasovagal events paroxysmal dystonia/ choreoathetosis
Seizure
hx- events prior, during, after exam lab- CBC, metabolic abnormalities CT scan/ MRI EEG- not sensitive or specific, may help classify seizure type to determine therapy, may reveal subclinical seizures
Seizure Tx
ABCs- protect against self-injury, airway clearance, monitor ABCs
anticonvulsants- if seizures are prolonged/hindering ABCs
education- seizure precautions, meds, daily life swimming, adequate sleep, driving, pregnancy
Seizure meds
abortive meds:
benzodiazepines
lorazepam parenterally
diazepam orally/rectally
midazolam nasally, PO, rectally
phenobarbital & fosphenytoin
LT anticonvulsants- different drug classes used for different types of epilepsyfebrile seizure is a convulsion assoc. w/ a temp > than
38.0 C
MC type of CH seizure
febrile seizure
incidence 2-5% of CH under 5 yo
peak occurrence 18-24 mo
When are febrile seizures most likely to occur (age)
6 mo-5yo
seizure in younger infant should concern you
some CH under 5 yo who develop febrile seizures may have persistant febrile seizures further on into CH
Febrile seizures are not d/t
CNS infxn/ inflammation
no acute systemic metabolic abnormality that may produce convulsions
no hx of previous febrile seizures
Criteria for simple febrile seizure (SFS) (65-90%)
duration under 15 min, total duration less than 30 min if in series
no recurrence in 24 hrs
no focal features
Complex febrile seizure (CFS)- meets 1+ criteria
duration > 15 mins
recur in series for total duration > 30 min or more than 1 seizure in 24 hr period
focal features
etiology of febrile seizure
infxn: HSV 6 (roseola), influenza, rotavirus
immunization-related fever
predisposing factors
heredity, inhibitory NT production, maternal alcohol intake &
smoking during pregnancy raises risk 2-fold
fever variability in febrile seizures
degree of fever is widely variable
ht of fever does not seem to correlate w/
onset of seizure
often the first sign of illness
simple febrile seizures MC are generalized & tonic-clonic
DDx for febrile seizure
chills/rigors b/c of fever meningitis encephalitis intracranial tumor metabolic d/o meurologic d/o (developmental delay)
fevers in infants under 3 mo old
10% who appear well have a T > 38 C have a serious bacterial infxn or meningitis
fevers in CH 3-36 mo of age
2% who have a T above 39 C are found to have bacteremia
AAP recommends strong consideration of LP in febrile seizure if
infant <12 mo who have had 1st seizure w/ fever
febrile seizures occurring on/after 2nd day of illness
Diagnostic eval for febrile seizure
EEG no usually indicated for SFS if hx & PE benign
neuroimaging
electrolytes, glucose, Ca2+, BUN should be measured if hx & PE indicate (vomiting, diarrhea, edema, dehydration) or w/ CFS
search for underlying cause of fever: CBC, blood cx, UA, urine cx
Febrile seizure Tx
ABCs
anticonvulsants if seizure >10 min, watch for respiratory compromise, drugs in office/ED- midazolam, lorazepam, fospheytoin, rectal diazepam
Antipyresis in preventing febrile sizures
around the clock use not shown to be of benefit wo all CH w/ febrile seizures
unknown if some subset benefits
intermittent use warranted
Anticonvulsant therapy for febrile seizures
not recommended in CH w/ 1+ SFS
Continuous phenobarbital/ valproate reduce the risk of
recureent febrile seizures but have significant side effects
SE of phenobarbital
transient sleep disturbances
decreased memory
reduced concentration
SE of valproate
suppressed hematopoiesis
renal toxicity
pancreatitis
hepatotoxicity
recurrence risk of febrile seizures
young age at onset hx of FS in 1st degree relative low degree of fever while in ED brief duration bet onset of fever & initial seizure all 4 factors- 70% risk 0-4 factors- <1yr old
febrile seizures & risk of epilepsy
2% chance (twice as high as gen. pop)
FH of FS, ethnicity, gender not risk factors
infantile spasms
seizures that have characteristic clinical findings in age group 4-8 months
sudden adduction & flexion of limbs,
head & trunk
usually occurs in clusters when pt is
irritable/ fatigued
Cryptogenic infantile spasm (40%)
no etiology evident, normal development before seizures
Symptomatic infantile spasms (60%)
association w/ perinatal & prenatal event or other identifiable cause
have poor responses to anticonvulsants & poor intellectual prognosis
EEG with infantile spasms
waking state EEG reveals chaotic high-voltage slow waves, random spikes & background disorganization
Tx of infantile spasms
anticonvulsant therapy
ACTH may be helpful
ketogenic diet
neurocutaneous d/o’s
d/o’s of tissue arising from neuroectoderm
skin findings
brain, spinal cord, eye manifestations
hamartomas
hamartoma
nml tissue growing at abnormal sites/ abnormally rapidly
neurofibromatosis type 1
NF-1>NF-2
cafe´ au lait spots
neurofibromatosis (small, rubbery, usu. on skin)
plexiform neurofibroma (diffuse thickening of n. trunk)
Lisch nodule (hamartoma located in iris)
Complications of neurofibromas
scoliosis learning disabilities HTN (renal artery stenosis) d/o's of the hypothalamus tumors- brain, eye
neurofibromatosis type 2
bilateral acoustic neuroma unilateral 8th nerve mass neurofibroma meningioma glioma Schwannoma juvenile posterior subcapsular lenticular opacities
Tuberous sclerosis
AD (autosomal dominant) d/o characterized by proliferation of tubers from abnormal production of protein hamartin & tuberin
Clinical manifestations of tuberous sclerosis
may present w/ seizures (infantile spasms)
calcified tubers in periventricular areas
mental retardation
hypopigmented skin lesions
ash leaf macules
shagreen patch
adenoma sebaceum
subungual/ periungual fibroma: commonly appear during adolescence
ash leaf spot
hypopigmented macule/patch
Wood’s lamp highlights appearance
shagreen patch
roughened, raised lesion w/ an orange peel, leathery texture
often located in lumbosacral region
sebaceous adenomas
tiny, red, hyperpigmented nodules over the nose & cheeks
Sturge-Weber dz
anomalous development of the vascular bed during early cerebral vascularization.
overlying leptomeninges richly vascularized & the brain beneath becomes atrophic & calcified
Clinical manifestations of sturge-weber dz
facial nevus (port-wine stain) seizures mental retardation intracranial calcifications hemiparesis
CT scan findings of Sturge-Weber dz
cerebral atrophy
intracranial calcifications
Guillain-Barre Syndrome
m>f
all ages, but rarely < 2 yo
acute idiopathic acquired inflammatory demyelinating polyradiculoneuropathy
d/o is thought to result from a post-infectious immune mediated process that predominantly affects motor nerves
Pathophys of Guillain-Barre syndrome
most likely grouping of d/o’s w/ peripheral n. demyelination as a common mechanism mediated by humoral factors & cell mediated phenomenon
In 2/3s of cases Guillain-Barre syndrome occurs after infxn with…
Campylobacter sp CMV, EBV Haemophilus influenzae Mycoplasma pneumoniae viruses
Clinical manifestations of Guillain-Barre syndrome
fine paresthesias & tingling of toes & fingertips pain may be prominent feature ascending weakness/paralysis LE symmetric weakness ascends to arms & cranial nerves
Guillain-Barre syndrome exam
diminshed/absent DTR paralytic ileus poor respiratory effort bladder dysfunction autonomic dysregulation HTN, HoTN, tachycardia/bradycardia, abnormal sweating
Dx of Guillain-Barre Syndrome
electrophysiologic studies
mildly elevated ESR
CSF
elevated protein
Tx of Guillain-Barre Syndrome
IVIG
plasmapheresis
physical therapy
watch for complications: respiratory failure, nutrition, autonomic disturbance
transverse myelitis
inflammation of the spinal cord w/ perivascular cuffing by lymphocytes thought to have immunologic basis pathogenesis (proposed) cell mediated immune response direct invasion of the spinal cord autoimmune vasculitis
Clinical manifestations of transverse myelitis
BACK PAIN AT LEVEL OF LESION
ABRUPT ONSET of progressive weakness; legs weak & flaccid
areflexia
sensory disturbances in LE’s below level of spinal lesion
hx of preceding viral infxn
sphincter disturbances
Lab findings in transverse myelitis
lumbar puncture- non specific increased protein pleocytosis w/ lymphocytes MRI (often diagnostic) dwelling of area of spinal cord EMG normal early in dz may show denervation at level of lesion
Tx of transverse myelitis
meds (controversial benefit) corticosteroids IVIG plasmapheresis supportive care physical therapy
infant botulism
acute, flaccid paralytic illness caused by the neurotoxin produced by soil orgnaism Clostridium botulinum
95% of cases between 3 wks & 6 mo of age
almost 1/2 of cases are from CA
Clostridium botulinum
gram + spore forming anaerobe
found in fresh & cooked agricultural products
botulinum toxin is heat labile
forms of botulism
wound
food-borne
infantile
botulism pathogenesis
toxin carried by blood to peripheral cholinergic synapses
binds irreversibly, blocking Ach release & causing impaired neuromuscular & autonomic transmission
loss of motor endplate function
recovery requires re-growth of terminal unmyelinated motor neurons
Infantile botulism clinical signs
symmetric, descending paralysis cranial nerve palsies poor feeding weak suck feeble cry ptosis obstructive apnea
lab confirmation of infant botulism
detection in serum
detection of C. botulinum in feces
CSF normal
EMG defect in neuromuscular transmission
tx of infant botulism
supportive care
tube enteral feeding
respiratory support
positioning to avoid aspiration
mechanical ventilation
botulism immune globulin (BIG)
reduces duration of illness if given earlytics
quick, repetitive, irregular, briefly suppressible movements
-facial: blinking, grimaces, twitches
-trunk & extremities: twisting, flinging
usu. last 1 mo-1yr
stress/anxiety tend to increase the amt of tics
Tx of tics
teach parents natural hx
disregard
monitor for co-morbidities: school problems, ADHD, OCD, sleep difficulties
Tourette’s syndrome is not d/t
direct physiological effects or a substance or a general medical condition
Diagnostic Criteria for Tourette’s syndrome
- tics occur many times a day (bouts) nearly q day/intermittently throughout a period of >1 yr (never a tic free period of >1 yr; never a tic free period of >3 consecutive mo)
- multiple motor & 1+ vocal tics present at some time
- onset before 18yo
Tx of Tourette’s syndrome
non-pharm: counseling, adjustment of school & family members
-meds: haloperidol, fluoxetine, clonidine
spinal muscular atrophy (SMA) types I, II, III
group of d/o’s characterized by degeneration of motor neurons w/ compensation from re-innervation of an adjacent motor unit
upper motor neurons NOT involved
SMA type I
Werdnig-Hoffman dz clinical manifestations
severe hypotonia generalized weakness thin muscle mass involvement of the tongue (fasciculations) perservation of extraocular muscles
Labratory Dx of Werdnig-Hoffman dz (SMA type I)
serum CK may be mildly elevated (different from muscular dystorphy)
muscle bx- areas of atrophy, adjacent areas of hypertrophy
molecular genetic dx
Tx of Werdnig-Hoffman dz (SMA type I)
no tx to delay progression
funtional aids in type II or type III
supportive care
feeding NG feeds
respiratory support
O2
pulmonary toiletmicrocephaly
head circumference more than 2 std deviations below the mean for age
macrocephaly
HC > 2 std deviations above the mean for age
DDx for microcephaly
chromosomal d/o (trisomy 13, 18, 21) non-chromosomal genetic d/o congenital malformation placental insufficiency perinatal hypoxia/trauma metabolic degenerative dz (Krabbe, Tay-Sachs) toxins (fetal alcohol syndrome, meds, maternal PKU) infxns (TORCH, toxoplasmosis, rubella, CMV, herpes, HIV) radiation familial
DDx for macrocephaly
pseudomacrocephaly
increased ICP (w/ dilated ventricles-hydrocephalus, with mass effect-tumor, arachnoid cyst, porencephalic cyst)
benign familial macrocephaly
megalencephaly (large brain)- neurocutaneous d/o, gigantism, metabolic d/o, lysosomal storage dz, leukodystrophy- progressive white matter degeneration
thickened skull
