1 بسم الله الرحمن الرحيم

Childhood Epilepsy Dr. Mohammed AL- Jaradi Sana’a24-25/4/2013 2

3 Helpful tools for diagnosis Accurate history and description of events Provoking factors Electroencephalogram

4 Epilepsy syndrome A complex of signs and symptoms that define a unique epilepsy condition

Etiological categories of syndromes –idiopathic no underlying structural brain disease, presumed genetic –symptomatic seizures are the result of lesional or metabolic disease of the brain –probably symptomatic (cryptogenic) believed symptomatic but no etiology established 5

6 Seizures: focal & generalized Generalized seizure –a seizure whose initial semiology indicates, or is consistent with, more than minimal involvement of both hemispheres Focal seizure (partial, localization-related) –a seizure whose initial semiology indicates, or is consistent with, initial activation of only part of one cerebral hemisphere

7 Generalized seizure types Myoclonic Sudden, <100 ms involuntary contraction of muscle(s) or muscle groups Clonic Repetitive, rhythmic myoclonus at 2-3 Hz Tonic Sustained muscle contraction for seconds to minutes Generalized tonic-clonic Bilateral symmetrical tonic contraction, then bilateral clonic contractions Atonic Sudden, brief, 1–2 s decrease in tone without preceding myoclonic or tonic event Astatic Loss of posture (=drop) due to atonics, myoclonic, or tonic event Spasm Axial contraction, may be asymmetric

8 Generalized epilepsies Idiopathic generalized epilepsies Benign myoclonic epilepsy in infancy Epilepsy with myoclonic-astatic seizures Epilepsy with myoclonic absence seizures Childhood absence epilepsy Juvenile absence, JME, epilepsy with GTC only Generalized epilepsies with febrile seizures plus

9 Syndromes of infancy Early myoclonic encephalopathy; West syndromes Lennox-Gastaut syndrome Epilepsy with continuous spike waves in SW sleep Epileptic encephalopathy

10 Focal seizure types Focal sensory with elementary sensory symptoms with experiential sensory symptoms Focal motor with elementary colonic motor signs with symmetric tonic motor signs with automatisms Secondarily generalized seizures

11 Common Childhood Epilepsy Syndromes

12 Infantile spasms West syndrome Onset ages 3-12 months Brief axial contractions –usually bilateral, may be asymmetrical –typically flexor, may be extensor –usually in clusters, less likely random –typically on awakening, or when drowsy EEG shows hypsarrhythmia –multifocal spikes –high voltage, chaotic background

13 EEG Record

14 Infantile spasms “Symptomatic” cases “Symptomatic” cases –congenital infections –CNS malformations –metabolic disorders –genetic syndromes –tuberous sclerosis –perinatal asphyxia –postnatal trauma –acquired infections –immunizations Often “cryptogenic” no definite cause established but child is delayedno definite cause established but child is delayed Rarely “idiopathic” no cause established and child is normalno cause established and child is normal

15 Infantile spasms Short term treatment to stop spasms, improve EEG –ACTH {Adrenocorticotropic Hormone } effective, dose not established oral steroids not proven effective –Vigabatrin, especially in tuberous sclerosis potential retinal toxicity

16 West Syndrome Infantile Spasms Hypsarrhythmia on EEG Developmental regression that begins concurrently with the onset of spasms

17 Outcome Idiopathic cases: –Normal school 37% –Death 6% –Other seizure types 43% –Neurologic abnormality 31%

18 Outcome Symptomatic cases: –Normal school - none –Death 37% –Other seizure types 59% –Neurological abnormality 65% –Large number progress to develop Lennox Gastaut Syndrome

19 Rolandic epilepsy Onset ages 2-12 yrs, peak 5-10 yrs Characteristic seizures Infrequent simple partial seizures –tingling in mouth, on face, speech arrest – rare GTCS in sleep to many drug s

Resolve by puberty Characteristic EEG –high voltage centrotemporal spikes –usually bilateral Imaging normal Considered an idiopathic focal epilepsy –some evidence for genetic basis Treatment –may not be necessary –may respond 20

21 Centrotemporal spikes in Benign Rolandic Epilepsy

Presentation of “staring spells” Complex partial seizures Absence seizures Behavior staring

Complex Partial Seizures Commonly temporal lobe focus Begins in one area, then spreads enough to impair consciousness, but not to evoke a generalized tonic-clonic seizure Staring is often part of the initial spread Also can see automatisms…

Automatisms Coordinated involuntary movements Consciousness impaired Patient does not recall activity Simple –Lip smacking, chewing, uttering sounds, picking, tapping, walking straight or in circles Complex (behavior involved) –Undressing, chewing inedible objects, wandering, aggression

Treatment of complex partial seizures Use medications for focal onset seizures –Levetiracetam (Keppra) –Oxcarbamazepine (Trileptal) –Carbamazepine (Tegretol/ Carbatrol) –Lamotrigine (Lamictal) –Topiramate (Topamax) –Zonisimide (Zonegran) –Phenobarbital –Valproic Acid (Depakote) –Lacosamide (Vimpat)

Absence epilepsy

Childhood absence (petit mal) epilepsy Peak onset age 4-6 years Many seizures daily Seizures last seconds 70+% have associated automatisms –eyelid flutter –simple vocalizations –picking movements Typical EEG with 3 Hz spike wave Majority resolve by adolescence

slightly irregular 3 per second spike wave ▼

Treating absence seizures Ethosuximide (Zarontin) Valproic Acid (Depaken) Lamotrigine (Lamictal) Levetiracetam (Keppra)

Absence v. Complex Partial Sz Absence < 30 sec Non-convulsive status epilepticus Frequent (100’s a day) Sudden onset, sudden termination No post-ictal state Hyperventilation a trigger EEG: 3-4 Hz spike and wave Rare interictal abnormalities Complex Partial > 1 minute Rare non-convulsive epilepticus Occur ≤ daily Frequent simple, complex automatisms Evolve to other sz manifestations Post-ictal state EEG: Interictal focal abnormalities

Behavioral Staring Most commonly seen in children with ADD and MR Occurs when “bored” or over-stimulated Does not typically make the patient fall or stop an activity abruptly Can be stopped with close contact / stimulation Also can be seen in children with epilepsy!

32 Evaluation of a 1 st unprovoked seizure Good evidence recommends EEG –EEG can help diagnose the event –EEG can identify a specific syndrome –EEG can help with prognosis –timing of EEG not determine d Generalized burst Benign focal spike

immediate EEG may show abnormality or post ictal slowing –abnormal EEG best predictor of recurrence in neurologically normal children –abnormal neuro exam also strong predictor of recurrence 33

34 Evaluation of a 1 st unprovoked seizure Insufficient evidence for routine labs, LP, imaging Insufficient evidence for routine labs, LP, imaging –consider emergent imaging if postictal focal deficit, or not at baseline in several hours –consider nonurgent MRI with significant neuro abnormalities of unknown etiology a seizure of focal onset in children under 1 year of age –consider LP in the very young child (<6 months) in the patient who fails to return to baseline in any patient with meningeal signs if increased ICP suspected, image before LP

35 How likely is a 2 nd seizure? Evidence from multiple Class III studies Recurrence ranged from 14%-65% Most recurrences early (in 1 st year) Factors increasing recurrence risk –abnormal EEG –etiology remote symptomatic seizure recurrence >50% idiopathic seizure recurrence 30-50%

36 Summary of evidence: Treatment of 1 st unprovoked seizure Most children with a 1 st seizure have few or no recurrences 10% will have many seizures regardless of initial Rx Rx after 1 st vs. 2 nd seizure does not affect long term prognosis Rx in adults and children leads to decreased recurrences

37 Recommendations: Treatment of a 1 st Seizure Anticonvulsant treatment after a 1 st seizure must be individualized –treatment is not indicated for prevention of epilepsy –treatment may be considered if risks of recurrent seizure out weigh risks of Rx Treatment must take into account patient and family preferences

38 Febrile seizures The most common seizures in childhood In US affect 2-4% of children < age 5 yrs 1/3 have at least 1 recurrence Risk factors for recurrence first febrile seizure < 1 year of age low degree of fever at first febrile seizure family history of febrile seizures brief duration between fever onset and febrile seizure 4 risk factors = 70% recurrence No risk factors = 20% recurrence

39 Febrile seizures Simple –generalized –and <15 minutes duration Complex –focal –or >15 minutes –or recurrent within 24 hours

40 Evaluation of the child with simple febrile seizures Lumbar puncture –if meningeal signs –in infants < 12 months –with prior antibiotic Rx Blood studies not needed routinely Imaging not necessary EEG not necessary in simple febrile seizures

41 Treatment of the child with simple febrile seizures Effective in reducing recurrences but with potential toxicities –continuous Phenobarbital or valporic acid –intermittent diazepam

Thank you! شــــــــــــــــــــــــــــــــــــ ـكــــــــــــــــــــــــــــــــــــ ــــرا 42