|ECR 2019 / C-1223||
|Paediatric Epilepsy Imaging - OUT --> IN approach|
A seizure is a transient occurrence of signs or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Depending on the distribution of this discharges, this abnormal brain activity can have various manifestations.
Epilepsy describes a condition in which a person has a risk of recurrent seizures due to chronic, underlying process.
Pediatric group broadly includes neonates (<1 month), infants and children (>1 month and <12 years) and adolescents (12-18 years).
Classification of seizures (1):
1. Focal onset: Further described as having intact or impaired awareness, motor or nonmotor onset, or evolve from focal to bilateral tonic-clonic)
2. Generalized onset
a. Motor: Tonic-clonic, other motor (e.g. atonic, myoclonic)
b. Nonmotor: Absence
3. Unknown onset: can be further described as motor or nonmotor or unclassified.
Causes of pediatric seizures (refer to table 1) (1)
As above mentioned above, a wide range of causes can cause epilepsy in pediatric age group. In this exhibit, we propose an “Out to In approach” with various categories for effective evaluation of pediatric neuroimaging (Refer figure 1).
In this approach, first is evaluation with clinical history and examination (1).
· The first goal is to determine whether the event was truly a seizure – this is essential because in many cases diagnosis of a seizure is based solely on clinical grounds – examination and lab studies are often normal.
· Ask witnesses for when was an episode, its duration, number of episodes and details of aura if any to the patient.
· History to rule out trauma, infection, recent medications etc.
· History of febrile seizures, family history and developmental history to assess milestones
· Immunization history
· Also ask for a history of difficult labor, delayed baby cry, neonatal ICU admission.
1. Physical examination (1)
· Skin – Neurocutaneous syndromes / Phakomatoses – Developmental abnormalities that frequently involve skin along with increased risk of nervous system tumours – e.g. Neurofibromatosis (café-au-lait macules, plexiform neurofibroma, freckling in the axillary or inguinal region), Tuberous sclerosis (adenoma sebaceum, ash leaf spots, subungual and gingival fibromas, shagreen patches), Sturge Weber syndrome (port wine stain).
· Facial asymmetry (in hemiatrophy and hemimegalencephaly syndromes), sphenoid dyplasia.
· Eyes – Iris hamartomas / Lisch nodules (neurofibromatosis), retinal hamartomas (tuberous sclerosis), congenital cataract (metabolic, infections), white retinal reflex (retinoblastoma).
· Anthropometry: for growth assessment, Macro/microcephaly.
· Syndromic assessment (hypertelorism, epicanthal folds – Downs syndrome), if any pinna malformation, low set ears, high arched palate, webbed neck, macroglossia (hypothyroidism).
2. Systemic examination
· Organomegalies – Metabolic disorders, childhood leukemia.
· Neurological examination: Sensory system, motor system, and higher mental functions – for assessment for cerebral palsy, mental retardation etc.
1. Routine blood studies – Complete blood count, electrolytes, glucose, calcium, magnesium, renal and liver functions tests.
2. Toxins screen of blood and urine for suspected toxin cause in adolescents
3. CSF analysis if suspicion of meningitis or encephalitis
4. EEG study – To characterize seizures and its localization for focal seizures.
Refer figures (2 to 15) for various neurocutaneous syndromes presenting with seizures showing skin findings, MR findings, clinical diagnostic criteria’s and teaching points. Written consent was taken from all patients to demonstrate their skin findings in this exhibit. Anonymity is maintained to the best possible extent (2).