Approaching a child that presented with involuntary movements or abnormal behavioural change suddenly
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Dr.C.S.N.Vittal Approach to Seizure Disorders in Children
Seizure ( Latin sacire , “to take possession of”) A transient occurrence of signs and/or symptoms resulting from abnormal excessive or synchronous neuronal activity in the brain.
Epilepsy Epilepsy is a disorder characterized by two or more unprovoked seizures occurring more than 24 hours apart beyond neonatal period.
A seizure is the event Seizure vs Epilepsy Epilepsy is the disease associated with spontaneously recurring seizures
Convulsion A convulsion is any seizure (not necessarily epileptic) characterized by excessive, abnormal muscle contractions, which are usually bilateral.
Some terminology A seizure occurring within a week of an acute brain insult (trauma, infection, toxic, metabolic or vascular insult) . Future risk of unprovoked seizures is only 3-10%. Acute Symptomatic Seizure ( Reactive seizures • Provoked seizures • Situation-related seizures) O ne that is not an acute symptomatic seizure. Unprovoked seizure Secondary to a distant brain injury, such as an old stroke. Remote symptomatic seizure Precipitated by a sensory stimulus such as flashing lights Reflex seizures Dr Neeta Naik, Guidelines for Diagnosis and Management of Childhood Epilepsy; Expert Committee On Pediatric Epilepsy, IAP ; Ind Ped , Vol 46 Aug 17, 2009: 681-698
Epilepsy – new definition Epilepsy is a disease of the brain defined by any of the following: A least two unprovoked (or reflex) seizures occurring >24 h apart 2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years 3. Diagnosis of an epilepsy syndrome Robert S fisher et al; A practical clinical definition of epilepsy: Epilepsia , 55($):475-482, 2014
Questions to Ask 2. What type of seizure? 3. Cause of seizure? 1.Was the spell in question a seizure? A detailed account of the event or spell from a witness in order to answer the three questions:
Pre-ictal Was there any warning before the spell? If so, what was the warning? Did the child complain of abdominal discomfort, fear or any other unpleasant sensations before the spell? What was the child doing before the spell? Was the child asleep or awake prior to the event? Was the child sleep deprived prior to the spell? Were there any triggers for the spell? Was the child well before the spell or was there a fever or illness?
Ictal Was the child responding during the spell or was consciousness impaired? Did the child remember anything that occurred during the spell? Were there any repetitive behaviors during the episode, such as lip smacking, pulling at clothing, and constant rubbing of objects. Did any body movements occur? Was there any perioral cyanosis? How long did the spell last? How many episodes has the child experienced?
Post-ictal How did the patient feel after the spell? Did the child seem confused and tired after the spell? How long did it take for the child to get back to baseline condition? Did the child suffer from a headache after the spell?
Other questions to ask: Has the child ever had any seizures before? Febrile seizures? Ask about past medical history, developmental history and current medications to rule out a symptomatic seizure. Is there any family history of seizures?
Motor tonic- clonic clonic tonic myoclonic myoclonic-tonic- clonic myoclonic-atonic atonic epileptic spasms 2 Non-Motor (absence) typical atypical myoclonic eyelid myoclonia Unknown Onset Motor Onset automatisms atonic 2 clonic epileptic spasms 2 hyperkinetic myoclonic tonic Non-Motor Onset autonomic behavior arrest cognitive emotional sensory focal to bilateral tonic-clonic Generalized Onset Focal Onset Aware Impaired Awareness Motor tonic- clonic epileptic spasms Non-Motor behavior arrest ILAE 2017 Classification of Seizure Types Expanded Version Unclassified 3 2 These could be focal or generalized, with or without alteration of awareness 3 Due to inadequate information or inability to place in other categories From Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia doi : 10.1111/epi.13671
Seizure Evaluation on First Visit 1 Seizure / seizure mimic ? 2 Is it unprovoked / provoked seizure ? 3 Generalized / focal seizure? 4 Etiology? 5 Any associated comorbidities? 6 Treatment received till now?
Symptomatic seizures are caused by: CNS Infection Meningitis Encephalitis Abscess CNS Trauma Acute trauma Previous trauma may lead to scar tissue formation Cerebrovascular Infarction Hemorrhage Arteriovenous malformation Venous thrombosis Hypoxic Hypoxic ischemic encephalopathy Metabolic Hypoglycemia Electrolyte disturbances Inborn errors of metabolism Neurologic effects of systemic disease Toxic Drugs Drug withdrawal Alcohol Alcohol withdrawal Lead poisoning Tumour Congenital CNS malformations Cortical dysplasia Lissencephaly Neurocutaneous syndromes (e.g. tuberous sclerosis) Fever – febrile seizures are discussed elsewhere
Differential Diagnosis Syncope Breath holding spell GERD Panic attack Conversion or pseudoseizures Benign sleep myoclonus Benign paroxysmal vertigo Motor tics Decorticate posturing Jitteriness in newborn
Physical Examination Vitals, including temperature Height, weight and head circumference Developmental stage of child in gross motor, fine motor, language and social domains. Signs of trauma Signs of increased intracranial pressure Skin lesions – may suggest a neurocutaneous diseases Fundoscopy – look for papilledema – suggests an increase in intracranial pressure (link to Signs of inc ICP) Neurologic exam
Blood tests Gen: CBC and differential Electrolytes Calcium, phosphorus, magnesium Blood glucose level Hemorrhagic basis – INR, PTT Toxic basis – blood levels of suspected drugs and metabolites Genetic disease – possible karyotype and other tests specific to illness Metabolic disease Ammonia, Lactate, Pyruvate, Amino acids, Urine organic acids
Differentiation of Seizures from Nonconvulsive Movements Jitteriness – Tremor No associated ocular movements or autonomic phenomena, Stimulus sensitivity, Tremor that is suppressed by flexing the limb. B enign neonatal sleep myoclonus Occurs in healthy newborns Only during sleep. Jerking ceases on wakening Apnea of prematurity In Preterms , apnea and bradycardia
Diagnosis History: Maternal drug abuse, Intrauterine infection, metabolic disease, natal history, etc. Complete Hemogram Blood: Sugar, Calcium, Magnesium, Na + , K + & HCO 3 , Elevated Ammonia, Lactate Levels, Culture & Sensitivity, plasma aminoacids , drugs
Lumbar puncture Infants <12 months with a first time febrile seizure to rule out meningitis Infants 12 to 18 months with a simple febrile seizure Any child with meningeal signs CSF: Analysis, Biochemical & C/s.
Imaging Cranial U/s.: hemorrhage, cysts, abnormal ventricles CT scan – indicated if head trauma is present/suspected MRI – indicated if the child has new or focal neurological deficits, recurrent seizures and/or papilledema
EEG EEG when abnormal can suggest the nature of the seizure tendency as focal or generalized, but does not determine whether or not a spell was a seizure or whether or not to treat,. 50% of patients with partial seizures show focal spikes (or slowing), up to 75% after repeat studies or sleep deprivation. 90% of patients with generalized seizures show generalized spikes, more with sleep deprivation, hyperventilation, or photic stimulation. A normal EEG would favor partial onset seizures in a patient with epilepsy. 1-2% of nonepileptics have spikes on their EEGs. 20% of patients with spikes on the EEG do not have epilepsy.
Step 6 Switch to another monotherapy or addon Management Strategies Step 1 Step 4 Select AED Step 3 Evaluate need for Tt Step 2 Establish seizure type/syndrome Confirm Diagnosis Step 5 Start monotherapy