Neurology Flashcards
Most common cause of seizures during the 1st 24 hours of life
HIE
Characteristics of benign neonatal myoclonus
Occurs during sleep, suppressible by touch
Jacksonian march occurs during which siezure type
Simple partial
Automatisms occur during which seizure type
Complex partial
Aura occurs prior to which seizure type
Simple partial
Recurrence rate for febrile seizure
33%
Age limit for febrile seizure
6 yo
Vaccinations associated with febrile seizure
MMR, DTaP
EEG findings of infantile spasms
hypsarrhythmia
West syndrome
infantile spasms + arrest of psychomotor development
Treatment for infantile spasms
ACTH, vigabratin
EEG findigns in Landau-Kleffner
during sleep
Characteristic of Landau-Kleffner
age 3-5 years, language regression
EEG findings in absence seizures
3/second spike and slow wave complexes
Treatment of absence seizures
ethosuximide, valproate
Juvenile myoclonic epilepsy age group
adolescents
Characteristics of juvenile myoclonic epilepsy
occur during waking, require lifelong treatment
EEG findings of juvenile myoclonic epilepsy
generalized poly spikes
Lennox-Gastaut prognosis
MR, intractable seizures
Characterisitics of temporal lobe seizures
complex partial seizure with automatisms
Characterisitics of benign focal epilepsy with centro-temporal spikes
age 8-10 yo, remit by 16 yo, nocturnal GTC
Phenytoin side effects
hirsutism, gingival hypertrophy
Topiramate side effects
weight loss, kideny stones, cognitive difficulties
Valproate side effects
weight gain, PCOS, pancreatitis, hepatotoxicity
Carbamazepine side effects
BM suppresssoin, hyponatremia
Breath holding spells association
iron deficiency anemia
Trigger for pallid breath holding spells
trivial head trauma, ocular pressure
PANDAS
tic disorder or OCD triggered by GAS infection
Stereotypies
non-purposeful repetitive movements in neurologically impaired children
Spasmus nutans
head tilt, head titubation, nystagmus (begins in 1st month and remits by 1-2 years)
Cataplexy
sudden loss of tone
Migraine epidemiology
More common in girls AFTER puberty
Acute confusional migraine trigger
mild head trauma
Basilar artery migraine symptoms
ataxia, nausea, dysarthria, hemiparesis, followed by occipital headache
Most prevalent neurocutaneous disorder
NF type 1
NF1 genetics
AD
Diagnostic criteria for NF1
cafe au lait macules, neurofibromas. axillary/inguinal freckling, optic glioma, lisch nodules, osseous lesions
NF1 association
learning disabilities (35%)
NF2 genetics
AD
NF2 presentation
hearing loss, late onset (avg. age 22 years)
Tuberous sclerosis genetics
AD
Disorder associated with infantile spasms
Tuberous sclerosis
Tuberous sclerosis findings
ash leaf spots, shagreen patch, adenoma sebaceum, subungal fibromas, MR, intracranial calcifications
High risk for Sturge-Weber
bilateral, V1, V2+V3, eyelids
Sturge-Weber complications
glaucoma, retinal detachment, MR, seizures
PHACE syndrome
posterior fossa brain malformation, hemagiomas, arterial anomalies, cardiac anomalies, eye abnormalities, sternal cleft
SMA genetics
AR
Muscular dystrophy genetics
XLR - Duchenne’s, Beckert
AD - myotonic dystrophy
Myotonic dystrophy genetics
maternal transmission, trinucleotide repeat disorder
Dandy-Walker
agenesis of cerebellar vermis, dilation of 4th ventricle, enlarged posterior fossa
Most common inherited ataxic condition
Friedreich’s ataxia
Friedreich’s ataxia genetics
trinucleotide repeat disorder (GAA)
Findings in Friedreich’s ataxia
dysarthria, high-arched foot, cardiomyopathy, afib, diabetes
Rett syndrome genetics
X-linked dominant
Wilson’s disease presentation
de novo psychiatric illness and a movement disorder
Alpers-Huttenlocher syndrome
DD, intractable seizures (may be focal), cortical atrophy, liver failure
Congenital myopathy with ptosis and ophthalmoplegia
myotubular myopathy
Congenital myopathy associated with malignant hyperthermia
central core, nemaline rod, minicore myopathies
Nemaline rod myopathy facies
elongated, narrow face with facial diplegia, lower jaw open
Difference between BMD and DMD
DMD = no dystrophin BMD = decreased dystrophin
Emery-Dreifuss MD natural history
slowly progessive weakness, survival depends on cardiac involvement
Myotonic dystrophy findings
distal weakness, ptosis, elongated facies, frontal balding, testicular atrophy, cataracts, heart disease
Congential muscular dystrophy that is more severely affected
merosin negative
Limb-Girdle muscular dystrophy findings
proximal muscle weakness (face sparing), muscle wasting
Ulrich muscular dystrophy
hypotonia, proximal joint contractures, hyperlaxity of hands, prominent calcenei (merosin negative)
Drug-drug interaction: Carbamazepine
macrolide antibiotics
Vital sign abnormality in hyponatremic seizures
hypothermia