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Epilepsy

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  • 1. EPILEPSY By Dr. M. saleem Laghari
  • 2. DEFINITION OF SEIZURE  A seizure (convulsion) is defined as a paroxysmal involuntary disturbance of brain function that may be manifested as an impairment of loss of consciousness, abnormal motor activity, behavioral abnormalities, sensory disturbances, or autonomic dysfunction.
  • 3. EPILEPSY  Epilepsy is defined as recurrent seizures unrelated to fever or to an acute cerebral insult.
  • 4. INCIDENCE/ETIOLOGY  Seizures are common neurologic problem in Paediatrics.  Seizures occur in 3-5 % of children.  Approximately 0.5-1.0% of children have epilepsy.  About 60% of all cases of epilepsy start in childhood.  In about half of all children with recurrent seizures, the workup will not disclose a specific cause.
  • 5.  There are many causes of epilepsy.  In general, any event having the potential to produce insult to the brain can result in epilepsy.  Mostly the cause is a static or non-progressive encephalopathy secondary to hypoxia, hemorrhage, CNS infection, head trauma, or developmental defects of the brain.
  • 6.  Labor and delivery complications are also less common cause of epilepsy.  Other important entities to be considered are tuberous sclerosis, neurofibromatosis, brain tumors, some inborn errors of metabolism, and certain degenerative brain diseases.  The chance of having a second seizure after an initial unprovoked episode is 30%.  The recurrence rate after the withdrawal of drugs is about 30%.
  • 7. CLASSIFICATION  Partial/Focal simple complex  Generalized Grand mal epilepsy Absence seizures myoclonic seizures Atonic seizures
  • 8. EVALUATION  Epilepsy is a clinical, not a laboratory diagnosis.
  • 9. HISTORY  Age of onset of first seizure.  Duration and frequency of seizures.  Factors that may have precipitated the seizures (e.g., fever.)  Seizures either focal or generalized in onset.  Presence of aura.  Postictal confusion, sleep, or (Todd) paralysis (on one side implies a focal onset).  Any developmental delay.  Previous drug treatment for epilepsy or for other illnesses.
  • 10. PHYSICAL EXAMINATION  Exclude some organic cause.  Take head circumference.  Observe the facial (dysmorphic) features.  Complete CNS examination for any focal neurological signs.  Development assessment.  Birthmarks such as café-au-lait spots or hypopigmented macules may point to a neurocutaneous syndrome.  Fundoscopy for papilledema, chorioretinitis, retinal hamartomas, and other congenital anomalies.
  • 11. MANAGEMENT OF EPILEPSY Principles of anticonvulsant therapy  Treat with the drug appropriate to the clinical type of epilepsy.  Do not use the anticonvulsant drug used previously without any success.  Start with the one drug of choice in appropriate dosage. Increase the dose until seizures are well controlled or signs of toxicity appear.
  • 12.  If seizures are not controlled with one drug of choice, second drug of choice is added. Do not stop first drug suddenly. Withdraw it gradually.  Advise the parents and the patient that the therapy will be prolonged but it will not produce any mental slowing. Changes in medications or their dosages should not be made without the advice of the physician. Sudden withdrawl of anticonvulsants may precipitate the seizures or even status epilepticus
  • 13.  Follow up of the patient and periodic neurologic re- evaluation is important.  If signs of toxicity appear, then reduce the drug by 25 % or add another drug.  Get frequent blood levels of anticonvulsant drugs as required.  After 2 -3 years of fits free interval, consider withdrawl of the anticonvulsant drug. Withdrawl should be gradual. In about 75 % of cases, seizures may not recur. If seizures recur during or after withdrawl, anticonvulsant therapy should be reinstituted and again maintained for at least 2 or more years. Generally, an EEG is indicated before withdrawl of an anticonvulsant. In case of an abnormal EEG, withdrawl may be deferred for 2 — 3 years.
  • 14. DETERMINATION OF BLOOD LEVELS OF ANTI-CONVULSANTS  Other drugs may affect blood levels of anticonvulsants. In such cases, adjustment of doses may be required.  Drug blood levels are indicated in a new patient, after a new drug is introduced, when expected control on a usual dosage has not been achieved, when seizures recur in a previously well-controlled patient, or when control is poor in a patient taking anticonvulsants being seen for the first time.  A low level may indicate inadequate dosage, drug interaction, or noncompliance with the prescribed regimen.  A high level may indicate slowed metabolism or excretion or drug interaction.
  • 15. EDUCATION ABOUT THE DISEASE  The patient and parents must be helped to understand the problem of seizures and their management.  Materials about epilepsy must be provided.  Advise that the child should be very careful in cycling on the road, unsupervised swimming, or locking the door of the bathroom when inside.  Encourage normal living but within reasonable bounds. Physical activities appropriate to their age should be allowed.  Loss of sleep should be avoided and emotional disturbances may need to be treated.  Proper attention should be given to infections and it is advised not to stop anticonvulsants in fever or in any other illness.
  • 16. ACTH AND CORTICOSTEROIDS  These are indicated in infantile spasms and in Lennox-Gastaut syndrome.  ACTH or oral corticosteroids are usually continued in full doses for 2 weeks to 2 months and after normalization of EEG and cessation of clinical seizures, tapered over 1 week.  Dose of ACTH gel is 2 - 4 units/kg/day IM in a single morning dose.  Prednisolone is given 2-4 mg/kg/day orally in two or three divided doses.
  • 17. KETOGENIC OR MEDIUM-CHAIN TRIGLYCERIDE DIET  A ketogenic diet should be recommended in myoclonic and absence seizures not responding to drug therapy. It is occasionally recommendd for inflantile spasms that do not respond to ACTH or corticosteroids therapy  Ketosis induced by ktogenic diet raises the seizure theshold.
  • 18. SURGERY  Seizures with primarily focal origin, neurosurgery should be considered for poorly controlled seizures.
  • 19. DRUGS USED IN EPILEPSY  Phenobarbitone  Phenytoin (dilantin)  Valproic acid (Epival / Epilim)  Carbamazepine (Tegral)  Lamotrigine (Lamictal)
  • 20. STATUS EPILEPTICUS Definition:  It is defined as a single generalized or series of tonic convulsions lasting 30 minutes or longer without intervening return of consciousness OR an epileptic seizure which is so prolonged or so frequently repeated as to create a fixed and lasting epileptic condition.  There are two types of status epilepticus: convulsive and non-convulsive. The latter consists of prolonged absence or complex partial seizures.
  • 21. INCIDENCE  It is estimated that 1.3-16% of all patients with epilepsy will develop status epilepticus at some point in their lives.  In some patients, it is the presenting initial seizure.
  • 22. ETIOLOGY  Status epilepticus occurs most frequently in patients younger than 1 year of age.  The etiology differs according to the age group. Two common causes are withdrawal of anti-epileptic drugs and hyperpyrexia secondary to a non-CNS infection in a patient with chronic static encephalopathy or seizure disorder.
  • 23. PATHOPYSIOLOGY  Brain damage occurs if the seizures remain for 60 minutes. Cell death results from excessively increased metabolic demands by continuously discharging neurons.  Areas of brain, which are most vulnerable to damage, are hippocampus, amygdala, cerebellum, middle cortical area, and thalamus. There is venous congestion, small petechial hemorrhages, and cerebral edema.  There is lactic acidosis, an alteration in the blood-brain barrier, raised intracranial pressure and fever due to prolonged seizures.  There are raised concentrations of calcium, arachidonic acid, and prostaglandins, which cause cell death.  There is dysfunction of the autonomic nervous system causing hypotension and shock
  • 24. TREATMENT  It is a medical emergency and requires prompt treatment.  The main goals of the treatment of status epilepticus are to: 1. Maintain adequate vital functions with prevention of systemic complications. 2. Terminate the seizure activity safely and quickly while minimizing treatment morbidity. 3. Evaluate and treat any underlying cause e.g., hypoglycemia, and meningitis
  • 25. INITIAL STABILIZATION  Airway: The highest priority is ensuring airway patency. Maintain oral airway. Intubation may be necessary.  Breathing: Give oxygen to prevent hypoxia.  Circulation: Assess pulse, blood pressure. Support with IV fluids. Monitor vital signs. Maintain perfusion.  Measure serum glucose by finger prick.  Check body temperature. Lowering body temperature is very important. Higher body temperature increases the neuronal discharges and the tissue damage associated with status epilepticus.  If vomiting occurs, turn the face to side and lower the head.
  • 26.  Venous access should be established as quickly as possible.  Blood is drawn for immediate complete blood count and measurement of electrolytes, glucose, calcium, and magnesium concentrations, toxic screen and levels of anticonvulsant medications if the child is already receiving therapy for seizures.  The initial stabilization phase of patient with status epilepticus is usually accomplished within the first 10 minutes after presentation to the emergency department. Specific anticonvulsant therapy is then started
  • 27. DRUG THERAPY  If finger prick sample indicates blood glucose < 50 mg/dl, treatment is initiated for hypoglycemia (2-4 ml/kg of 25 % dextrose; 0.5-l.Og/kg).  A benzodiazepine is the first drug used because of its rapid onset of action.  Because the half-life of diazepam is very slow, it must be followed immediately by. phenytoin. Dose is 20 mg/kg IV in normal saline
  • 28.  If seizure activity continues for another 5-6 minutes, give 1 ml/kg sodium bicarbonate to treat metabolic acidosis.  If status does not stop within 10 minutes the total dose of phenytoin being given phenobarbitone is the next drug.  If status is not responsive to a benzodiazepine followed first by phenytoin and then by phenobarbitone, the patient will require further therapy as given below.  Paraldehyde 0.3 ml/kg PR mixed with equal amount of mineral oil in a glass syringe.
  • 29.  Valproic acid 40-60 mg/kg/dose  Lidocaine 1-2 mg/kg IV up to 50 - 100 mg bolus over 2-4 minutes followed, if effective, by 3-5 mg/kg/hour as solution of 100-1000 mg in 250 ml 5% dextrose.  Halothane anesthesia is given by anesthesiologist, titrated to suppression of discharges on EEG. Note: When a drug is selected to be used, sufficient time must be allowed for the drug to act before more of the same medication or another medication is used. If a single agent does not control the seizures, a second drug may be needed. I/V and rectal administration are the routes of choice.
  • 30. POST-ICTAL MANAGEMENT  Give maintenance drug therapy by phenytoin (10 mg/kg/day) or phenobarbitone 5mg/kg/day).  LP should be deferred until the patient's vital signs are stable and the convulsion has stopped. If sepsis is felt to be likely, I/V antibiotics may be given immediately without waiting to do the lumbar puncture.  A history of trauma, evidence of raised intracranial pressure, focal neurologic signs, unexplained loss of consciousness or suspicion of cerebral herniation are indications for CT scan.