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  • Neurodegeneration is the umbrella term for the progressive loss of structure or function of neurons, including death of neurons. Many neurodegenerative diseases including Parkinson’s, Alzheimer’s, and Huntington’s occur as a result of neurodegenerative processes. <br /> <br /> Neurocutaneous syndrome – NF,VonHippelLindau,Sturgeweber,tuberoussclerosis,ataxiatelengiectasia <br />

Bdak2 epilepsy Bdak2 epilepsy Presentation Transcript

  • WHAT IS EPILEPSY?  Epilepsy is a chronic neurological condition characterized by recurrent, unprovoked seizures, occurrence of at least 2 unprovoked seizures 24 hours apart.  Epileptic seizure → clinical manifestation of abnormal & excessive discharge of a set of neurons in the brain
  • CAUSES  Idiopathic (70 – 80%) – cause unknown but presumed genetic  Secondary  Cerebral dysgenesis /malformation  Cerebral vascular occlusion  Cerebral damage - Antenatal: Congenital infections, drugs, alcohol - Natal: HIE, birth trauma - Postnatal: IVH in prematurity, CNS infections, kernicterus, trauma, tumour  Cerebral tumours  Neurodegenerative disorders  Neurocutaneous syndrome
  • GENERALISED SEIZURES:- (Discharge arises from both hemisphere) Absence seizures Myoclonic seizure Tonic Tonic clonic Atonic seizures FOCAL – SEIZURES ARISE FROM ONE OR PART OF ONE HEMISPHERE Frontal seizures Temporal lobe seizures Occipital seizures Parietal lobe seizures ILAE 2010 CLASSIFICATION
  • TONIC CLONIC SEIZURES Tonic phase  The tonic phase begins with flexion of the trunk and elevation and abduction of the elbows. Subsequent extension of the back and neck is followed by extension of arms and legs.  Piercing cry may be present due to passage of air through closed vocal cords.  Autonomic signs are common during this phase and include increase in pulse rate and blood pressure, profuse sweating  This stage lasts for 10-20 seconds.
  • Clonic phase  tremor occurs at a rate of 8 tremors per second, which may slow down to about 4 tremors per second. This is because phases of atonia alternate with repeated violent flexor spasms. Each spasm is accompanied by pupillary contraction and dilation. Some patients may have tongue or cheek bites.  The atonic period lasts about 30 sec.  The clonic phase lasts for 30 sec. to 1minute. TONIC CLONIC SEIZURES
  • ABSENCE SEIZURES  Patient stares briefly and stop talking or ceases to respond.  Most of the patient are completely motionless while some feel some myoclonic movements in eye lids,facial muscles,fingers at a rate of 3 per sec..and this rate corresponds to the abnormality in EEG as generalized 3 per sec.spike & wave pattern.  Occurs at the age of 4-12 years  Prognosis is good.95% remission in adolescense
  • MYOCLONIC SEIZURES  These are brisque,brief muscular contractions  some of them involve only single muscle or a part of the muscle & some of them are so large that they include whole body or both the limbs. Myoclonic jerks are common in the morning involving entire body both the limbs and sometimes absence seizures are common. This is the most common form of idiopathic gen.epilepsy in begins at adolescence (15 yr).  4 to 6 Hz irregular spikes have been noted in EEG.
  • PARTIAL SEIZURE - FOCAL SEIZURE  Begin in a relatively small group of dysfunctional neurones in one of the cerebral hemispheres.  Mayb e heralded by an aura which reflects the site of the origin  May or may not be associated with change in consciousness or more generalised motor jerking.
  • Frontal seizures • Involve the motor cortex • May lead to clonic movements → travel proximally→ (Jacksonian March) • Asymmetrical tonic seizures → bizarre ,hyperkinetic & easily dismissed as non – epileptic events Temporal lobe seizure • Most common • Strange warning feeling/ aura with smell , taste abnormalities & distortion of sound & shape • Automatism → spread to the premotor cortex • Deja-vu & Jamais-vu • Consiousness may be impaired, length of event is longer than a typical absence
  • Occipital • Distortion of vision Parietal • Causes contralateral dysaesthesias (altered sensation ) • Distorted body image
  • SPECIAL EPILEPSY SYNDROME 1.INFANTILE SPASM:- -Most cases appears in 1st yr of life. - Single brief recurrent gross flexion movements of the limb …rarely extension movements -EEG shows multifocal,multiple small spikes. -On maturity it disappears(4 to 5yr) -CT & MRI mostly normal. -Later progress to LENOX GASTAUT SYND.
  • HISTORY TAKING HOPI:-  Two unprovoked seizures >24 hr apart suggest the presence of an epileptic disorder  Any aura?change in the behaviour?  Types of seizures -tonic clonic(tensing,then shaking,LOC) -atonic(drop attack) -absence(jus staring,not responding,blinking) -partial(maybe consciouss,only ½ limbs shaking/jerking  How long did it last?frequency?time of day?precipatating factor?  Any loss of consciousness?tongue bitting?  What did you do for the child?(appropriate first aid measures)  Post ictal:drowsy?sleeping?vomiting?
  •  PERINATAL HISTORY  Infection during pregnancy?TORCH  Birth history - birth asphyxia , birth trauma  Neonatal jaundice  POST NATAL HISTORY  Central nervous system (CNS) infection e.g. meningitis, encephalitis etc.  Head injury  Lead contact (lead fumes from burning batteries, pica)
  • PAST HISTORY  Age at 1st seizure?describe seizure?  h/o febrile seizures  When and how diagnosed?any event preceding seizure?  DRUG HISTORY 1. Anticonvulsant medications 2. How many? 3. Any increase in dosage?types? 4. Compliance,how often dose missed?what to do if missed? 5. Side effect? 6. Responding current medication or not
  •  Outpatient review 1. Frequency 2. Test done 3. Other investigations(eg.EEG to date)  Hospitalization 1. How many?reasons?  Any identified medical problems associated with seizures? 1. Any history of trauma,meningitis?encephalitis? 2. How are this problem managed?  FAMILY HISTORY 1. Of convulsion?inborn error metabolism?
  •  SOCIAL HISTORY:-  Impact on child: 1. Schooling 2. Athletic participation? 3. Self esteem 4. Does teacher know about the condition? 5. On family:financial burden  CONTINGENCY PLAN: 1. What to do in the event of a seizure?
  • PHYSICAL EXAMINATION •Consciouss level •Posture •Deformity •movement •Dysmorphism,head size and shape •Cranial nerves •Gait •Spine •Neurocutaneous signs 1. café-au-lait spots 2. Neurofibromata 3. adenoma sebaceum
  • DIAGNOSIS  The clinical diagnosis is more important then any tool… (H/O, Eye witnesses, substantiated by video if available) # EEG:-  EEG is most sensitive tool for diagnosis which shows electrical activity changes in the brain but it also require clinical correlation  Many children with epilepsy may have normal EEG and many children who will never have epilepsy have EEG abnormalities  Done for dx, classification, selection of anti-epileptic drugs and prognosis
  • MRI and CT -not required routinely for childhood generalized epilepsy.  To identify a tumour,vascular lesion or area of sclerosis. PET and SPECT.  To detect areas of hypometabolism in epileptogenic lesions  OTHER INVESTIGATIONS  Blood test and metabolic investigations(seizures related to feed and fasting).  Genetic studies  Lumbar puncture
  • PRINCIPLES OF ANTICONVULSANT THERAPY  Treatment recommended if ≥ 2 episodes→ recurrence risk 80%  Attempt to classify the seizure type & epileptic syndrome  Monotherapy as far as possible → most appropriate drug → increase dose gradually till epilepsy controlled, maximum dose reached / side effects occur  Alternative monotherapy (Add on the 2nd drug if 1st drug failed. Optimise 2nd drug, then try to withdraw 1st drug.  Rational combination therapy (usually 2 or maximum 3 drugs )  Combines drugs with different mechanism of action & consider their spectrum of efficacy, drug interactions & adverse affects.
  •  Monitor drug levels (carbamazepine, phenytoin, phenobarbitone) to check compliance → if seizures not well controlled/in situations of polypharmacy where drug interaction is suspected.  When withdrawal of medication is planned → seizure free for 2 years, consideration should be given to epilepsy syndrome, likely prognosis & individual circumstances before attempting slow withdrawal of medication over 3-6 months (longer if clonazepam/ phenobarbitone)  If seizures recur → last dose reduction is reversed & medical advice sought
  • INTRACTABLE EPILEPSY?  Re- evaluate the following possibilities  Is it a seizure /non epileptic event  Anticonvulsant dose not optimized  Poor compliance to anticonvulsant  Wrong classification of epilepsy syndrome → wrong anticonvulsant  Anticonvulsant aggravating seizures  Lesional epilepsy, hence a potential epilepsy surgery candidate  Progressive epilepsy or neurodegenerative disorder
  • REFERRAL TO PAEDIATRIC NEUROLOGIST  Poor seizure control despite monotherapy with 2 different anticonvulsants  Difficult to control seizures beginning in the 1st year of life  Seizures & developmental regression  Structural lesion on neuroimaging
  • ADVICE FOR PATIENTS  Educate and counsel on epilepsy.  Emphasize compliance if on anticonvulsant.  Don’t stop the medication by themselves.this may precipitate breakthrough seizures.  In photosensitive seizures-watch tv in brightly lit room.avoid sleep deprivation.  Use a shower with bathroom door unlocked  No cycling in traffic,climbing sports or swimming alone.  Know emergency treatment for seizure  Inform teachers and school abt the condition.
  • PARTIAL SEIZURES Simple partial Complex partial Sec.generalised FIRST LINE carbamazepine valproate SECOND LINE lamotrigine topiramate levetiracetam phenytoin Phenobarbitame clonazepam GENERALISED SEIZURES tonic clonic Clonic Absence Atpical absence Atonic,clonic Myoclonic Infantile spasms valproate valproate valproate Valproate,clonazepam ACTH,prednisolone lamotrigine topiramate levetiracetam phenytoin Phenobarbitame Clonazepam Lamotrigine Topiramate,clonazepam Topiramate,phenobarbitone, piracetam,levetiracetam,lam otrigine. Nitrazepam,valproate
  • SIDE EFFECTS AND SERIOUS TOXICITIES OF ANTICONVULSANT  CARBAMAZEPINE—drowsiness,dizziness,ataxia,diplopia,rash (serious toxicity—agranulocytosis Steven Johnson syndrome)  CLONAZEPAM---- hypotonia,salivary and bronchiol hypersecretion,paradoxical hyperactivity,aggresiveness  PHENYTOIN--- ataxia,diplopia,rash,gum hypertrophy,hirsutism (serious toxicity—megaloblastic anemia)
  •  PHENOBARBITONE---- cognitive dysfunction,ataxia,rash,behavioural disturbance serious toxicity—liver toxicity,steven johnson syndrome  VALPROATE---- epigastric pain,tremor,alopecia,weight gain,hair loss,thrombocytopenia serious toxicity—hepatic toxicity(<2 yrs age) hepatitis,pancreatitis, encephalopathy
  • STATUS EPILECTUS  Any seizures lasting > 30 minutes OR  Intermittent seizures longer than 30 minutes from which the patient does not regain consciousness
  • •Highest incidence in very young children • 70% of children with epilepsy experience at least one episode of SE Mortality rate 8 to 32%
  • CAUSES OF SE 1)Prolonged febrile seizures  Lasting for >30mins  Particularly in child younger than 3 years old  Associated with severe damage to the hippocampus in children(Hippocampus sclerosis)  Most common cause of SE  May be the initial manifestation of encephalitis, and epilepsy may be a long term complication of meningitis
  • CAUSES OF SE 2)Idiopathic status epilepticus  Includes epilepticus patients in whom SE followed sudden withdrawal of anticonvulsants(esp. benzodiazepines and barbiturates)  Given anticonvulsants on an irregular basis or who are noncompliant are more likely to develop SE
  • CAUSES OF SE(CONTINUED)  Acute head trauma  Brain tumor  Neurodegenerative disorders  Hepatic or renal encephalopathy  Storage diseases.
  • MECHANISM OF SE Inreased cerebral metabolic rate Increased in cerebral flow (half an hour) Inadequate oxygen tension and togetther with other factors lead to Neuronal injury STATUS EPILEPTICUS
  • MANAGEMENT OF STATUS EPILEPTICUS  Securing airway ,breathing and circulation (with continuous monitoring of vital signs ,ECG)  Determination and management of the underlying etiology(eg.hypoglycemia)  Laboratory studies(glucose,sodium,Ca)  Blood and spinal culture,toxic screens test for inborn error of metabolism  Antiepilectic drugs level  EEG(ruling out pseudostatus epilepticus)
  • SUMMARY  affects 1 in 200 children  Is classified as generalised or focal(partial) or an epilepsy syndrome of childhood  If suspected EEG is indicated  Most but not all requires antiepileptic drug therapy,which should be appropriate for the seizure,compromise as few drugs and with the least potential for unwanted effects as possible  Requires liaison with the school about the management of seizures and avoiding situations ehich could lead to injury.