Neuro Flashcards
Cracked pot sound on percussion of the skull indicating hydrocephalus
MacEwen sign
Malformation consisting of cystic expansion of the 4th ventricle int eh posterior fossa and midline cerebellar hypoplasia, resulting from a developmental failure of the roof of the 4th ventricle during embryogenesis
Dandy-walker malformation
Brain weight:volume ratio >98th percentile for age (≥ 2 SD above the mean)
Megalencephaly
Most common megalencephalic syndrome; cerebral gigantism
Sotos syndrome
Cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal, or occipital cortex dispersed over the membrane
Hydranencephaly
EEG findings in typical absence seizure
3 Hz spike-and-slow wave discharges
EEG findings in atypical absence seizure
1-2 Hz spike-and-slow wave discharges
__ seizures often occur at night and can be very numerous and brief
frontal lobe
EEG in patients with partial seizures usually show __ in the lobe where the seizure originates
focal spikes or sharp waves
Most common benign epilepsy syndrome with partial seizures
Child wakes up at night owing to simple partial seizures causing buccal and throat tingling and tonic or clinic contractions of one side of the face, with drooling and inability to speak but with preserved consciousness
Benign childhood epilepsy with centrotemporal spikes (BECTS)
EEG findings in BECTS
broad-based centrotemporal spikes that are markedly increased in frequency during drowsiness and sleep
Most common cause of surgically remediable partial epilepsy in adolescents and adults
Preceded by febrile seizures
Mesial temporal sclerosis
Form of chronic encephalitis that manifests with unilateral intractable partial seizures, epilepsia partialis continua, and progressive hemiparesis of the affected side, with progressive atrophy of the contralateral hemisphere
Rasmussen encephalitis
Typical absence seizures usually start at
5-8 yr
Consists of myoclonic and other seizures during the 1st yr of life, with generalized 3 Hz spike-and-slow wave discharges
Benign myoclonic epilepsy of infancy
Most common generalized epilepsy in young adults
Starts in adolescence with one or more of the ff: myoclonic jerks in the morning, often causing the patient to drop things; generalized tonic clonic or clonic-tonic-clonic seizures upon awakening; and juvenile absences
EEG shows generalized 4-5 Hz polyspike-and-slow wave discharges
Juvenile myoclonic epilepsy (Janz syndrome)
Starts during the first 2 mo of life with severe myoclonic seizures and burst suppression pattern on EEG
Early myoclonic infantile encephalopathy (EMIE)
Starts during the first 2 mo of life
Manifests as tonic seizures and is usually caused by brain malformations or syntaxin binding protein 1 mutations
Same EEG pattern as EMIE
Early epileptic infantile encephalopathy (EEIE) or Ohtahara syndrome
Starts as focal febrile status epilepticus and later manifests myoclonic and other seizure types
Severe myoclonic epilepsy of infancy or Dravet syndrome
Starts between the ages of 2 and 12 mo and consists of a triad of infantile spasms that usually occur in clusters, developmental regression and a typical EEG picture called hypsarrhythmia
West syndrome
Typically starts between the age of 2 and 10 yr and consists of a triad of:
- Developmental delay
- Multiple seizure types that as a rule include atypical absences and myoclonic, astatic and tonic seizures
- EEG findings 1-2 Hz spike-and-slow waves, polyspike bursts in sleep and a slow background in wakefulness
Lennox-Gestaut Syndrome
First line treatment of absence seizures
Ethosuximide
or valproic acid
First line treatment of complex partial seizures
oxcarbazepine
or carbamazepine
Drug of choice for juvenile myoclonic epilepsy
valproate
lamotrigine
Drug of choice for Lennox-Gestaut syndrome
valproate
topiramate
lamotrigine
rufinamide
Drug of choice for infantile spasms
ACTH
Drug of choice for West syndrome
ACTH
The most important factor for seizure relapse
abnormal EEG before medication is discontinued
Seizures that occur __ after AEDs are completely discontinued indicate relapse, and resumption is usually warranted
> 2-3 mo
EEG manifestations of status epilepticus Stage I Stage II Stage III Stage IV
Stage I: initial distinct electrographic seizures
Stage II: waxing and waning electrographic seizures
Stage III: continuous electrographic seizures
Stage IV: Continuous ictal discharges punctuated by flat periods
Periodic epileptiform discharges on flat background
Migraine that persists beyond 72 hr
status migrainous
Most commonly used preventive therapy for headache and migraine is
amitriptyline
Classification of tension type headaches
Infrequent <12 times per year
Frequent 1-15 times per mo
Chronic >15 times per mo
Criteria NF-1
CAFE SPOT
2/7:
- 6 or more cafe-au-lait macules over 5 mm in greatest diameter in prepubertal individuals and over 15 mm in greatest diameter in postpubertal individuals
- axillary or inguinal freckling consisting of multiple hyperpigmented areas 2-3 mm in diameter
- 2 or more Lisch nodules
- 2 or more neurofibromas or 1 plexiform neurofibroma
- osseous lesion such as sphenoid dysplasia or cortical thinning of long bones
- optic gliomas
- first degree relative with NF-1
mnemonic: CAFE SPOT
Criteria NF-2
1/4:
- bilateral vestibular schwannomas
- a parent, sibling or child with NF-2 and either unilateral vestibular schwannomas or any 2 of the ff: meningioma, schwannoma, glioma, neurofibroma, posterior lenticular opacities
- unilateral vestibular schwannoma and any 2: (same list as above)
- multiple meningiomas (2 or more) and unilateral vestibular schwannoma or any 2: (same list as above
Autosomal dominant disorder resulting from the loss of either tuberin or hamartin gene manifesting as numerous benign tumors (hamartomas)
tuberous sclerosis
Criteria tuberous sclerosis
2 major or 1 major plus 1 minor features
Major features of tuberous sclerosis
- cortical tuber
- subependymal nodule
- subependymal giant cell astroyctoma
- facial angriofibroma or forehead plaque
- ungula or periungual fibroma
- hypomelanotic macules (>3)
- Shagreen patch
- multiple retinal hamartomas
- cardiac rhabdmyoma
- renal angiomyolipoma
- pulmonary lymphangioleiomyomatosis
Minor features of tuberous sclerosis
- cerebral white matter migration lines
- multiple dental pits
- gingival fibromas
- bone cysts
- retinal achromatic patch
- confetti skin lesions
- nonrenal hamartomas
- multiple renal cysts
- hamartomatous rectal polyps
Lesons found along the wall of the lateral ventricles where they undergo calcification and project into the ventricular cavity, producing a candle-dripping appearance
subependymal nodules
most common neurologic manifestation of TSC
epilepsy, cognitive impairment, autism spectrum disorders
Hypomelanotic skin macules on the trunk and extremities seen in tuberous sclerosis
ash leaf lesions
Roughened, raised lesion with an orange-peel consistency located primarily in the lumbosacral region
Shagreen patch
Tuberous sclerosis
Treatment of subependymal giant cell astrocytomas in TBC patients not candidates for surgery
everlimus
Routine follow-up of TBC patients
brain MRI every 1-3 yr
renal imaging every 1-3 yr
neurodevelopmental testing at the time of beginning 1st grade
Sporadic vascular disorder and consists of a constellation of symptoms and signs including port-wine stain, leptomeningeal angioma and abnormal blood vessels of the eye leading to glaucoma
Sturge-Weber syndrome
Typical seizure in SWS
focal tonic-clonic and contralateral to the side of the facial capillary malformation
Pulsed dye laser therapy is used in?
clearing of port-wine stain in SWS
Autosomal dominant disorder with features of cerebellar hemangioblastomas and retinal angiomas
Also associated with cystic lesions of the kidneys, pancreas, liver, and epididymis as well as pheochromocytomas
Von Hippel-Lindau disease
Most common cause of death in Von Hippel-Lindau
renal carcinoma
Sporadic condition characterized by a facial nevus and neurodevelopmental abnormalities
Linear nevus syndrome
Components of PHACE syndrome
Posterior fossa malfromations Hemangiomas Arterial anomalies Coarctation of the aora and other cardiac defects Eye abnormalities
The most common brain abnormality in PHACE syndrome
Dandy-Walker malformation
Most common neuropathologic finding in spastic diplegia
PVL
Most common lesions seen on pathologic examination or on MRI in spastic quadriplegia
severe PVL and multicystic cortical encephalomalacia
Most common type of CP most likely associated with birth asphyxia
Athetoid CP/choreoathetoid, extrapyramidal, dyskinetic
Treatment for uncomplicated penicillin-sensitive S. pneumoniae meningitis
3rd generation cephalosporin or penicillin x 10-14 days
Treatment for uncomplicated N. meningitidis meningitis
penicillin x 5-7 days
Treatment for uncomplicated H. influenzae b meningitis
3rd generation cephalosporin x 7-10 days
Gram negative bacillary meningitis should be treated for __ or at least __ after CSF sterilization, which may occur after 2-10 days of treatment
3 weeks
or 2 weeks after CSF sterilization
Data support the use of IV dexamethasone in treatment of children older than 6 wk with acute bacterial meningitis caused by __
H. influenzae type b
Most common sequelae of bacterial meningitis
sensorineural hearing loss
Most common cause of viral meningoencephalitis
Enterovirus
Most common manifestation of CNS involvement in VZV infection
cerebellar ataxia
Part of the brain commonly affected by:
- HSV
- Arboviruses
- Rabies
- HSV - cortex, esp temporal lobe
- Arboviruses - entire brain
- Rabies - basal structures
EEG findings in viral encephalitis
diffuse slow-wave activity, usually without focal changes
Brain abscess most common in which age group?
4-8 yr and neonates
Most common pathogen in brain abscess in neonates
Citrobacter
A brain abscess can be treated with antibiotics without surgery if: 1. size of abscess 2. duration 3. 4.
- <2 cm
- <2 wk
- No signs of inc ICP
- Neurologically intact
Indications for surgery
- > 2.5 cm in diameter
- gas is present in the abscess
- lesion is multiloculated
- located in the posterior fossa
- fungus is identified
Main pathogen in purulent meningitis in newborn to 3 months
GBS E. coli L. monocytogenes Enterococci S. aureus and other Staph species
Main pathogen in purulent meningitis in 2 m to 6 yr
H. influenzae type b
S. pneumoniae
N. meningitidis
Main pathogen in purulent meningitis in >6 yr
N. meningitides
S. pneumoniae
Normal CSF pressure leukocyte count protein glucose
pressure 50-180 mmH2O
leukocyte <4, L: 60-70%, M 30-40%, N 1-3%
protein 20-45 mg/dL
glucose >50% of serum glucose
Diagnostic criteria for transverse myelitis
- bilateral sensorimotor and autonomic spinal cord dysfunction
- clearly defined sensory level
- progression to nadir of clinical deficits between 4 hr to 21 d after symptom onset
- demonstration of spinal cord inflammation: CSF pleocytosis, elevated IgG index, or MRI revealing GAD enhancing cord lesion
- exclusion of compressive, postradiation, neoplastic and vascular causes
Most common hereditary neuromuscular disease
Duchenne muscular dystrophy
