Neuro Flashcards by Hazel Baconga

test to measure neural tube defect while pregnant

AFP -> 16th to 18th week

acetylcholinesterase

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drugs that antagonize folic acid

trimethoprin
carbamazepine
phenobarbital
phenytoin
primidone

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downward herniation of medulla and cerebellar tonsils through foramen magnum
pooling of secretions
vocal cord paralysis
stridor

chiari crisis

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encephalocele
cleft lip and palate
microcephaly
microphtalmia
abnormal genitals
polycystic kidneys
polydactyly

meckel gruber

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absent cerebral convolutions

lissencephaly

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presence of unilateral or bilateral clefts within cerebral hemispheres

schizencephaly

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presence of cysts in brain

porencephaly

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most common location porencephaly

near sylvian fissure

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porencephaly assoc with

maternal cocaine 
maternal ab trauma
protein C
factor V leiden
von willebrand
perinatal alloimmune thrombocytopenia

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female
agenesis of corpus callosum
chorioretinal lacunae (punched out lesions in pigmented layer of retina)
infantile spasms

aicardia syndrome

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abnormal enlargement of occipital horns

colpocephaly

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colpochephaly assoc with

agensis of corpus callosum

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defective formation of prosencephalon

forebrain structures affected

holoprosencephaly

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sucking jaw movements with eye blinking

marcus gunn phenomenon

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paralysis of abducens nerve CN 6

mobius syndrome

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Limitation of abduction (outward movement) of the affected eye

duane retraction syndrome

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autosomal recessive
underdeveloped cerebellar vermis
molar tooth sign
hypotonia
ataxia
apnea

joubert syndrome

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scaphocepahly
cleft lip/palat
syndactyly of second and third toes
upturned nose
CHD
ambiguous genitals
agenesis of corpus callosum
decreased cholesterol levels
autosomal recessive

Smith–Lemli–Opitz syndrome

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CSF rate

20ml/hr

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amount of csf

50ml in infant

150ml in adult

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Incidence 1 in 50,000 live births

Round facies, prominent epicanthic folds, low-set ears, hypertelorism, characteristic cry No specific neuropathology

cri du chat

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Ptosis, scaphocephaly, inner epicanthic folds, anteverted nostrils Low birthweight, marked feeding problems

Smith-Lemli-Opitz

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Small for dates, petechial rash, hepatosplenomegaly, chorioretinitis, deafness, mental retardation, seizures Central nervous system calcification and microgyria

CMV

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Growth retardation, purpura, thrombocytopenia, hepatosplenomegaly, congenital heart disease, chorioretinitis, cataracts, deafness
Perivascular necrotic areas, polymicrogyria, heterotopias, subependymal cavitations

rubella

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Purpura, hepatosplenomegaly, jaundice, convulsions, hydrocephalus, chorioretinitis, cerebral calcifiation

toxoplasmosis

Growth retardation, ptosis, absent philtrum and hypoplastic upper lip, congenital heart disease, feeding problems, neuroglial heterotopia, disorganization of neurons

fetal alcohol

Growth delay, hypoplasia of distal phalanges, inner epicanthic folds, broad nasal ridge, anteverted nostrils

fetal hydantoin

premature closure saggital

scaphocephaly

most common craniosynostosis

scaphocephaly

skull that resembles cloverleaf

kleeblattschadel deformity

autosomal dominant syndrome with a highly variable phenotype Onset is usually in early childhood and remission is usually in mid-childhood It is characterized by multiple febrile seizures

generalized epilepsy with febrile seizures plus

characterized by febrile and afebrile unilateral clonic seizures recurring every 1 or 2 mo severe myoclonic seizure of infancy

Dravet syndrome

temporal discharges | loss of speech and verbal agnosia

landau kleffner epileptic aphasia syndrome

unilateral intractable partial seizures epileptia partia continua progressive hemiparesis of affected side

rasmussen's encephalitis

adolescent myoclonic drops things often

juvenile myoclonic epilepsy

seen in first three months of life mental retardation tonic seizure burst suppresion on EEG

ohtahara syndrome

infantile spasms that occur in clusters developmental regression hypsarrhythmia 2-12mo

west syndrome

2-10yrs old developmental delay multiple seizure types 1-2 hz spike and slow waves

lennox gastaut syndrome

treatment of landau kleffner syndrome

valproic acid | prednisone

four processes of seizures

1. underlying etiology 2. epileptogenesis (kindling) 3. epileptic state of increased excitability 4. seizure related neuronal injury

DOC focal seizure

oxcarbazepine | carbamazepine

DOC absence

ethosuximide

DOC juvenile myoclonic epilepsy

valproate | lamotrigine

DOC Lennox gastaut

valproate topiramate lamotrigine rufinamide

DOC infantile spasms

ACTH | adrenocorticotropic hormone

DOC west syndrome

ACTH | adrenocorticotropic hormone

side effect of ACTH adrenocorticotropic hormone

``` hypertension electrolyte imbalance infection hyperglycemia glycosuria ```

DOC Dravet

valproate | benzodiazepines (clonazepam)

DOC benign myoclonic epilepsy

valproate

side effect weight gain

valproate | carbamazepine

side effect gingival hyperplasia

phenytoin

side effect alopecia

valproate

side effect hyperactivity

benzodiazepines barbiturates valproate gabapentin

drug that can cause sjs like syndrome chinese HLA B 1502

carbamazepine

side effect rickets

phenytoin phenobarbital primidone carbamazepine

AED to avoid in IEM

valproate

how to give IVIG for west, dravet, lennox gastaut, landau kleffner

2g/kg over 4 consecutive days | then 1g/kg once a month for 6 months

when to d/c AED

2 years seizure free if benign epilepsy, 6 months

risk factors of seizure relapse after withdrawal

older age longe duration of epilepsy >1 AED used multiple seizure types

apneic focal motor seizures fifth day of life EEG sharp 4-7Hz theta pointu alterant

fifth day fits | benign idiopathic neonatal seizures

cerebellar hemangioblastomas retinal angioma autosomal dominant VHL gene

von hippel lindau disease

PHACE

``` posterior fossa hemangioma arterial anomalies cardiac anomalies/coarctation eye abnormalitites ```

dental ocular dermatologic (vesicular-> verrucous plaques-> hyperpigmented-> hypopigmented) cranial

incontinenta pigmenti

pathogen in acute cerebellar ataxia

varicella coxsakie echovirus

failure to thrive, photosensitivity, intermittent ataxia, nystagmus, and tremor autosomal recessive pellagra like

hartnup

``` steatorrhea fialure to thrive acanthocytosis ataxia retinitis pigmentosa decreased serum cholesterol and TG ```

abetalipoproteinemia or Bassen kornzweig

``` ataxia strabismus telangiectasia recurrent sinopulmonary infection low igA igG igE ```

ataxia telangiectasia

malignancy assoc with ataxia telangiectasia

leukemia lymphoma hodgkin brain tumor

``` autosomal recessive progressing ataxia explosive dysarthritic speech pes cavus scoliosis decreased DTR and vibratory involve spinocerebellar tracts , dorsal column of spine, cerebellum, medulla ``` vit E and co q10 slows progression

friedrich ataxia

dance like darting tongue milkmaid grip

chorea

chorea disappears with

sleep

chorea increases with

stress

clinical hallmark of sydenham chorea

emotional lability hypotonia chorea

tx for sydenham chorea

valproate carbamazepine dopamine receptor antagonist (haloperidol)

tremor | feature of cerebellar disease

intention tremor

tx of tremor

``` propranolol primidone (barbiturate) ```

excess deposition of copper in liver, brain progressive basal ganglia disease choreoathetosis parkinsonism

wilson disease

excess iron deposition in globus pallidus and substantia nigra "eye of the tiger" sign -> edema and necrosis of globus pallidus autosomal recessive

pantothenate kinase associated neurodegeneration or hallervorden spatz disease

seen at 1st mo of life, resolve by 3 yrs torticollis irritability ataxia

benign paroxysmal torticollis of infancy

tx for generalized dystonia

trihexyphenidyl | anticholinergic

arm more involved than leg hadn preference at early age cerebral palsy

spastic hemiplegia

commando crawl/rudder uses arms more in crawling periventricular leukomalacia cerebral palsy

spastic diplegia

CP from kernicterus

athetoid cp

1. recurrent strokelight episodes with hemiparesis 2. lactic acidosis 3. seizure

MELAS | mitochondrial myopathy encephalopathy lactic acidosis and strokelike episodes

``` progressive myoclonic epilepsy mitochondrial myopathy ataxia nystagmus dysarthria elevated lactate ragged red fibers in muscle biopsy ```

myoclonus epilepsy and ragged red fibers (MERRF)

``` feeding and swallowing problems delayed motor and language pyramidal signs weakness hypotonia ataxia nystagmus ```

leigh disease or subacute necrotizing encephalomyopathy

``` triad: 1. progressive external ophthalmoplegia 2. onset before 20yr old 3. pigmentary retinopathy PLUS ONE: heart block, cerebellar syndrome, CSF protein >100mg/dl ``` can be associ with endocrine problems

kearns sayre

``` congenitally infected arrest in brain growth dev delay weak pyramidal tract signs seizures ```

hiv encephalopathy

mood or personality change seizure dyskinesia sleep disturbance

anti NMDAR encephalitis

``` peroxisomal disorder cerebral pachygyria progressive loss of hearing and vision high forehead, hypoplastic supraorbital ridges, epicanthal folds, midface hypoplasia, and a large fontanel hepatomegaly renal cysts ```

zellweger syndrome or cerebrohepatorenal syndrome

``` defect galactocerebrosides B galactosidase excessive crying hyperpyrexia rigidity blindness deafness demyelinating disease ```

krabbe

``` lorenzo's oil (glyceryl trioleate and glyceryl trieucate) tanning without sun exposure ataxia swallowing disturbance adrenal insufficiency inflammatory demyelinating disease boy ```

adrenoleukodystrophy

``` x linked recessive deficient copper chubby cheeks, friable hairs severe mental retardation failure to thrive hypotonia generalized myotonic seizures ```

menkes disease | kinky hair disease

``` regression of language and motor tremor ataxic gait repetitive hand movements autistic Generalized tonic clonic seizure ```

rett syndrome

``` measles aggressive behaviour impaired cognitive Generalized tonic clonic seizure choreathetosis ```

subacute sclerosing panencephalitis

relapsing remitting course of episodes separated in time and space demyelinating

multiple sclerosis

``` hemiparesis optic neuritis focal sensory loss ataxia diplopia dysarthria bowel/bladder dysfxn discrete lesions on periventricular white matter on MRI ```

multiple sclerosis

treatment of multiple sclerosis

MPPT

pathogens associated with ADEM

``` influenza ebv cmv varicella enterovirus measles, mumps, rubella herpes mycoplasma pneumoniae ```

post vaccination ADEM

``` mmr jap b rabies smallpox dpt flu ```

``` lethargy fever headache vomiting encephalopathy visual loss ataxia motor/sensory deficit bladder/bowel dysfxn ``` MRI: swelling and variable enhancement within white and gray matter

ADEM | acute disseminated encephalomyelitis

tx ADEM

MPPT | then oral steroids

leading cause of acquired brain injury

artreial ischemic stroke

leading cause of term born cerebral palsy | congenital hemiplegia

perinatal stroke

virchow triad

1. Hypercoagulability 2. Hemodynamic changes (stasis, turbulence) 3. Endothelial injury/dysfunction

tx strep meningitis

10-14 days 3rd gen ceph or penicillin add Vanco if resistant

tx n. meningitidis meningitis

5-7 days | penicillin

tx hib meningitis

7-10 days

repeat LP in mengitis done for

neonates if indicated gram negative meningitis B lactam resistant strep meningitis

csf should be sterile after

24-48 hours after initiation of tx

tx gram (-) meningitis

3 weeks | or at least 2wks after csf sterilization

dexamethasone for __ meningitis

Hib

most common sequelae in bacterial meningitis

sensorineural hearing loss

chemoprophylaxis for neiserria meningitidis

rifampin 10mkdose q12 x 2 days

chemoprophylaxis for hib

rifampin 10mkdose q12 x 4 days

most common cause of helminthic/eosinophilic meningitis

rat lungworm | angiostrongylus cantonensis

emperic tx in brain abscess -unknown cause

vancomycin 3rd gen cephalosporin metronidazole

emperic tx in brain abscess - otitis media, sinusitis, mastoiditis

vancomycin 3rd gen cephalosporin metronidazole

emperic tx in brain abscess - penetrating head injury

vanco | 3rd gen ceph

emperic tx in brain abscess - cyanotic heart disease

ampicillin sulbactam OR 3rd gen ceph + metronidazole

tx citrobacter brain abscess neonate

3rd gen ceph + aminoglycoside

tx listeria brain abscess neonate

3rd gen ceph + aminoglycoside + ampicillin

emperic tx in brain abscess - immunocompromised

broad spectrum | amphotericin B

brain surgery for brain abscess not indicated if

<2cm <2weeks duration no signs of increased ICP neurologically intact

surgery of brain abscess indicated if

``` >2.5cm gas inside abscess fungus identified multiloculated located at posterior fossa ```

pseudotumor cerebri definition

ICP >200mm H2O in infants >250mm H2O in kids normal CSF picture

central cord syndrome | upper ext weakness (proximal first then distal)

syringomyelia

brown sequard syndrome

ipsilateral weakness, ataxia, spasticity | contralateral loss of pain and temperature

SCIWORA

spinal cord injury without radiographic bone abnormalities

injury T12 - L1 loss of sphincter control (urinary/rectal) flaccid sensory loss

conus medullaris syndrome

lower motor neuron signs in upper extremity upper motor neuron signs in lower extremity bladder dysfxn loss of sensation caudally upper extremities are weaker

central cord syndrome

motor and sensory deficit dysfxn in spine urinary retention first, then incontinence later weakness first, then spasticity after CSF: minimal PMN, protein mildly elevated, elevated myelin basic protein and immunoglobulin levels

transverse myelitis

weakness proximal > distal

myopathy

weakness distal > proximal

neuropathy

intact tendon stretch reflex

myopathy

decreased tendon stretch reflex

neuropathy

type 1 sma

wernig hoffman

most commonly sampled muscle

vastus lateralis | quadriceps femoris

most commonly sampled nerve

sural nerve

sensory or motor: sural nerve

sensory

myotubular myopathy arrest in

maturation of fetal muscle in the myotubular phase 8-15weks AOG

X-linked recessive disorder characterized by congenital cataracts, hypotonia, intellectual disability, proximal tubular acidosis, aminoaciduria, and low-molecular-weight proteinuria

oculocerebral syndrome | Lowe syndrome

floppy infant prone to malignant hyperthermia (dantrolene)

central core myopathy

muscle biopsy of duchenne

endomysial connective tissue proliferation scattered degenerating and regenerating myofibers mononuclear infiltrations

muscles do not hypertrophy contractures and wasting in scapulohumeralperoneal distribution absent myotonia cardiomyopathy

emery dreifuss muscular dystrophy

``` facial wasting inverted V shaped lip generalized muscle wasting myotonia slow gastric empyting/peristalsis heart block endocrine problems (hypothy, adrenal insuff, diabetes) low igG cataract ```

myotonic muscular dystrophy

diagnosis of myotonic musclular dystrophy

DNA analysis CTG repeat

kids that looks like body builder

myotonia congenita or thomsen disease

juvenile spinal muscular atrophy

kugelberg welander disease

prominent scapular winging in infants

facioscapulohumeral muscular dystrophy or Landouzy - Dejerine disease

factors that precipitate hypoK periodic paralysis

1. heavy carb meal 2. emotional stress 3. insulin 4. epinephrine 5. hyperaldosteronism 6. hyperthyroidism 7. amphotericin B 8. licorice

triad: 1. (micrognathia), low-set ears, and an abnormal curvature of the fingers called clinodactyly 2. periodic paralysis 3. potentially fatal cardiac ventricular ectopy (with long QT)

andersen tawil syndrome

``` floppy infant administration of general anesthetic fevere muscle rigidity metabolic and respiratory acidosis serum CK elevated 35, 000 acute tubular necrosis myoglobinuria ```

malignant hyperthermia

small cell lung cancer myasthenia gravis adult

eaton lambert

EMG in myasthenia gravis

decremental response in repeated stimulation

clinical test for myasthenia gravis >2yrs old

edrophonium (short acting cholinesterase inhibitor)

clinical test for myasthenia gravis used for <2yrs old

``` prostigmine methylsulfate (neostigmine) IM ```

tx myasthenia gravis caused by failure to release Acetylcholine

ephedrine or diaminopyridine

overdose of cholinesterase inhibitor

cholinergic crisis

myasthenia gravis cannot tolerate

NMJ blocking drugs | succinylcholine and pancuronium

cholinergic crisis

Salivation, Lacrimation, Urination, Defecation, Gastrointestinal Distress and Emesis

neurodegeneration of voluntary muscles weakness spasticity sensory intact

``` Amyotrophic lateral sclerosis (ALS) OR motor neurone disease (MND) OR Lou Gehrig's disease ```

stork leg

charcot marie tooth disease

nerves affected early in charcot marie tooth disease

peroneal and tibial nerves

findings in biopsy sural nerve charcot marie tooth disease

onion bulb formation proliferated schwann around axons interstitial hypertrophy neuropathy

poison burning paresthesias

arsenic

poison mononueritis complex ( motor neuropathy of peroneal, radial, median nerves)

lead

toxin puffer fish similar to

guillan barre syndrome

``` autosomal recessive reduced number of unmyelinated nerves (pain, temp, taste, autonomic) fungiform papilae in tongue absent swallowing problem excessive sweating tendon stretch reflex absent bradycardia urinary incontinence ```

familial dysautonomia | RIley Day

achalasia alacrima autonomic dysfunction

allgrove syndrome

landry ascending paralysis

guillain barre

acute external ophthalmoplegia ataxia areflexia

miller fischer

csf in guillain barre

protein 2x normal glucose normal normal cells

tx for guillain barre

ivig 0.4g/kg/day for 5 days

last function to recover in GBS

tendon reflexes

3 clinical features GBS that predict poor outcome

1. intubation 2. cranial nerve involvement 3. maximum disability at time of presentation

pathogen cause bells palsy

``` herpes varicella ebv lyme mumps mycoplasma ```

manifestation of bells

paretic upper and lower face taste anterior 2/3 affected unable to close eye NO paresthesia

tx bells palsy

prednisone 1mg/kg/dose x 1 wk then taper