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P78 Pediatrics
Neurology
Toronto Notes 2019
Heart problems, such as long QT syndrome and hypertrophic cardiomyopathy, are often misdiagnosed as epilepsy. Include cardiac causes of syncope in your differential diagnosis, particularly when the episodes occur during physical activity
Seizure Mimics
• Benign paroxysmal vertigo • Breath holding
• Hypoglycemia
• Narcolepsy
• Night terror
• Pseudoseizure • Syncope
• TIA
• Tic
Ketogenic Diet and other Dietary Treatments
for Epilepsy
Cochrane DB Syst Rev 2012;3:CD001903
Study: Systematic review of all studies of ketogenic and related diets. Included the review of 4 RCTs, 6 prospective studies, and 5 retrospective studies. Population: Adults and children with diagnosed epilepsy of any type.
Intervention: Ketogenic diet, control (placebo diet, any treatment with known antiepileptic properties). Main Outcome Measure: Seizure control at 3,
6, 12 mo.
Results: Studies showed a response rate of at least 38-50% seizure reduction at 3 mo. This response was maintained for up to a year. A range of side effects were reported. The most frequent were gastrointestinal effects (30%).
Conclusion: The ketogenic diet is a valid option for people with medically-intractable epilepsy.
Seizure Disorders
• seeNeurology,N18
Differential Diagnosis of Seizures in Children
• benignfebrileseizure
• CNS: infection, tumour, HIE, trauma, hemorrhage
• metabolic: hypoglycemia, hypocalcemia, hyponatremia
• idiopathicepilepsyandepilepticsyndromes
• others:neurocutaneoussyndromes,AVM,drugingestions/withdrawal • seizuremimics
Investigations
• labtests:CBC,electrolytes,calcium,magnesium,glucose
• toxicologyscreenifindicated
• EEG
• CT/MRI,ifindicated(focalneurologicaldeficitorhasnotreturnedtobaselineseveralhoursafterseizure) • considerLPiffirst-timenon-febrileseizure(notindicatedfordeterminingrecurrenceriskofbenign
febrile seizures or to determine seizure type or epileptic syndrome)
CHILDHOOD EPILEPSY SYNDROMES
Infantile Spasms
• brief,repeatedsymmetriccontractionsofneck,trunk,extremities(flexionandextension)lasting10-30s • occurinclusters;oftenassociatedwithdevelopmentaldelay;onset4-8mo
• 20%unknownetiology(usuallygoodresponsetotreatment);80%duetometabolicordevelopmental
abnormalities, encephalopathies, or are associated with neurocutaneous syndromes (usually poor
response to treatment)
• candevelopintoWestsyndrome(infantilespasms,psychomotordevelopmentalarrest,and
hypsarrhythmia) or Lennox-Gastaut (see below)
• typicalEEG:hypsarrhythmia(highvoltageslowwaves,spikesandpolyspikes,background
disorganization)
• management: ACTH, vigabatrin, benzodiazepines
Lennox-Gastaut
• characterizedbytriadof1)multipleseizuretypes,2)diffusecognitivedysfunction,and3)slow generalized spike and slow wave EEG
• onset commonly 3-5 yr of age
• seenwithunderlyingencephalopathyandbrainmalformations
• management:valproicacid,benzodiazepines,andketogenicdiet;however,responseoftenpoor
Juvenile Myoclonic Epilepsy (Janz Syndrome)
• myoclonusparticularlyinmorning;frequentlypresentsasgeneralizedtonic-clonicseizures • adolescentonset(12-16yrofage);autosomaldominantwithvariablepenetrance
• typicalEEG:3.5-6Hzirregularspikeandwave,increasedwithphoticstimulation
• management:lifelongtreatment(valproicacid);excellentprognosis
Childhood Absence Epilepsy
• multipledailyabsenceseizureslasting<30swithoutpost-ictalstatethatmayresolvespontaneouslyor become generalized in adolescence
• peakageofonset6-7yr,F>M,stronggeneticpredisposition
• typicalEEG:3Hzspikeandwave
• management:valproicacidorethosuximide
Benign Focal Epilepsy of Childhood with Rolandic/Centrotemporal Spikes
• focalmotorseizuresinvolvingtongue,mouth,face,upperextremityusuallyoccuringinsleep-wake transition states; remains conscious, but aphasic post-ictally
• onsetpeaksat5-10yrofage;16%ofallnon-febrileseizures;remitsspontaneouslyinadolescence without sequelae
• typicalEEG:repetitivespikesincentrotemporalareawithnormalbackground
• management:frequentseizurescontrolledbycarbamazepine,nomedicationifinfrequentseizures
General Approach to Treatment
• educationforpatientandparentsincludingeducationandprecautionsindailylife(e.g.buddysystem, showers instead of baths)