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Neuromuscular Disease(2) Epilepsy

Neuromuscular Disease(2) Epilepsy. Department of Pediatrics Soochow University Affiliated Children’s Hospital. Seizures (p130). Main contents: 1) Emphasize the clinical features of epileptic seizure and epilepsy. 2) Introduce the management of epilepsy and status epilepticus.

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Neuromuscular Disease(2) Epilepsy

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  1. Neuromuscular Disease(2) Epilepsy Department of Pediatrics Soochow University Affiliated Children’s Hospital

  2. Seizures (p130) Main contents: 1) Emphasize the clinical features of epileptic seizure and epilepsy. 2) Introduce the management of epilepsy and status epilepticus. 3) Emphasize the features of febrile seizure. 4) Introduce the features of seizure-like disorders.

  3. Topic 1. Epileptic seizures Seizure prevalence: □Seizures occur in 0.5-1% of the population. □if febrile seizures are included, affect up to 3-5% of children. □prevalence of epilepsy is 0.3%-0.6%.

  4. Topic 1. Epileptic seizures Clinical features: □investigation will not reveal an underlying cause in the majority of cases. □involving episodic involuntary movement and behavioural or sensory activity. □often associating with loss of consciousness. □with an manifestation of abnormal electrical activity in the brain.

  5. Topic 1. Epileptic seizures In making the diagnosis of epilepsy: □a detailed history is fundamental. □the clinical examination is usually unremarkable, but must exclude: 1) raised intracranial pressure. 2) hypertension 3) neurocutaneous stigmata 4) metabolic or storage disorder. □EEG is important in the diagnosis of EP.

  6. Topic 1. Epileptic seizures EEG (electroencephalogram) □is a recording of the electrical activity of the brain. □may provide useful information on the diagnosis of epilepsy, the seizure type, its treatment and prognosis.

  7. partial seizures (interictal : T3、T4)

  8. partial seizures ( fit : FP1、F3)

  9. Topic 2. Classification of seizures Table Types of epileptic seizure Epileptic seizures can be divided into partial or generalized types

  10. Topic 2. Classification of seizures Partial seizures □Simple partial seizures (SPS): 1) brief tonic or clonic movements of the face and extremities. 2) without impaired consciousness. □Complex partial seizures (CPS): 1) strange sensations or complex semi-purposeful movements. 2) with altered or impaired consciousness.

  11. Topic 2. Classification of seizures Absence seizures (typical absences, petit mal) • usually affect girls. • sudden loss of consciousness for less than 30 seconds. • with staring and eyelid flickering but no aura or postictal state. • produce 3 per second spike and generalized discharges on the EEG.

  12. Topic 2. Classification of seizures Atypical absence seizures • associated with myoclonic movements. • less than 3 per second discharges on EEG . • adverse outcome is expected in those with 1) multiple seizures. 2) a positive family history. 3) low IQ(<90).

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  15. Topic 2. Classification of seizures Tonic-clonic seizures □the most common type of convulsion. □commencing with an aura and followed by 1) loss of consciousness. 2) tonic contractures. 3) rhythmic clonic movements. 4) above movements lasting several minutes. □cyanosis, tongue biting and incontinence of urine during the fit. □a postictal phase (e.g. headache, a period of sleep).

  16. Topic 2. Classification of seizures Myoclonic epilepsy □sudden loss of muscle tone, which may result in injury. □some forms: a positive family history and a benign course. □others: mental retardation and are refractory to treatment.

  17. Topic 2. Classification of seizures Infantile spasms (West syndrome) □Up to 20% are idiopathic; the remainder are secondary to : 1) hypoxic damage. 2) CNS infection. 3) trauma. 4) neurocutaneous syndromes. 5) storage diseases. □usually commence around 3-6 months of age.

  18. Topic 2. Classification of seizures Infantile spasms (West syndrome) □repetitive, symmetrical contractions of the neck, trunk and limbs 1) flexor spasms (mostly). 2) extensor contractions (minority). 3) combination. □The EEG is usually hypsarrhythmia. □around 40% have evidence of cerebral palsy and 80% have significant cognitive disability. □the treatment difficult, especially in non-idiopathic types. □Steroids and vigabatrin have been shown to be effective.

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  21. Topic 2. Classification of seizures Lennox-Gastaut syndrome (LGS) □Multiple seizures often include myoclonic, tonic, atypical absences . □Most patients with significant motor and cognitive impairment. □LGS is often refractory to therapy and only 10% have a reasonable outcome. □Occasionally, recourse to surgical resection is necessary.

  22. Topic 3. Management of epilepsy 1.general therapeutics 2.causative therapeutics any underlying cause and complications must be controlled.

  23. Topic 3. Management of epilepsy 3.therapeutic principle of AED (antiepileptic drug) 1)An expectant approach can be applied for: □a single, non-febrile seizure. □no abnormal physical signs. □a normal EEG. 2)Having established a diagnosis of epilepsy, AED is started early. 3)The drug of choice is dependent on the seizure type.

  24. Topic 3. Management of epilepsy 4)Monotherapy principle: □Having established a diagnosis of epilepsy, a single anticonvulsant is started. □If the child is still experiencing more than one seizure per month, a second drug is added in combination. After a long period of control (a few months), the initial drug can be discontinued. a second single drug also belongs to monotherapy.

  25. Topic 3. Management of epilepsy 5)Note side effects: After AED is started, the dose built up over a number of weeks until fit control is adequate without drug-related side effects.

  26. Topic 3. Management of epilepsy 6)Serum anticonvulsant levels: □It should be used only as a guide to management, but may be helpful especially with VPA,PHT,PB. □for most anticonvulsants, there is very little correlation with serum levels, efficacy and side effects.

  27. Topic 3. Management of epilepsy 7)Cessation of medication can be considered after a 1-2-year fit-free period in those patients without risk factors. □Weaning should take place over a minimum of 3 months. □up to 70% of these patients will have no further fits.

  28. Indicative of a good outcome: □ a single seizure type. □ short duration. □ infrequent occurrence. □ no neurological impairment. Indicative of a poor outcome: □ multiple seizure types. □ prolonged duration. □ frequent episodes. □ additional neurological impairment. □ refractoriness to AED. □ early onset. Topic 3. Management of epilepsy

  29. Topic 3. Management of epilepsy 4.Surgery □is developing as a viable treatment option for intractable epilepsy. □particularly in those with a demonstrable clinical, radiological or EEG focus.

  30. Topic 3. Management of epilepsy 5.Vagal nerve stimulation (VNS): □has been shown to reduce seizures by up to 50% on average. □with intractable seizure disorders. □multiple anticonvulsants have previously failed to achieve satisfactory control.

  31. Topic 4. Non-epileptic seizures Non-epileptic seizures include: □those related to a fever or to a CNS insult such as trauma, infections and metabolic derangement. □They do not necessarily lead to lifetime seizure activity.

  32. Topic 4. Non-epileptic seizures Febrile convulsions (FC) characteristic : □FC are the most common seizures in childhood, usually affecting 4% of children. □children aged 6 months to 6 years. □The convulsion is generally triggered with spikes of fever and usually responds to cooling and antipyretic measures. □They comprise symmetrical, generalized tonic-clonic seizures, associated with loss of consciousness and no focal neurological signs. □seizures lasting less than 15 minutes, □often with a positive family history.

  33. Topic 4. Non-epileptic seizures Following a febrile convulsion, all children should be examined to exclude: • a serious underlying infection. If in doubt, a lumbar puncture should be performed. • an underlying metabolic or CNS abnormality. All those with atypical febrile convulsions require nvestigation with neuroimaging and an EEG.

  34. Topic 4. Non-epileptic seizures Febrile convulsion treatment: • the administration of rectal diazepam during subsequent convulsions. • If indicated, prophylactic VPA or PB. • There is a slightly increased risk of non-febrile seizure activity later in life in 10% of children. • There is no place for routine anticonvulsant therapy in typical febrile seizures.

  35. Topic 4. Non-epileptic seizures Febrile convulsion adverse factors : • continuing seizure activity include complex or atypical initial seizures. • a positive family history of epilepsy. • preceding neurological abnormality.

  36. Topic 5. Status epilepticus status epilepticus (SE) definition: • constant seizure activity persisting beyond 30 minutes. • recurrent fits with no resumption of consciousness persisting beyond 30min.

  37. Topic 5. Status epilepticus SE causes: • usually after prolonged FS. • patients with preceding neurological or metabolic disease. • also occurs in those patients without a CNS insult.

  38. Topic 5. Status epilepticus SE complication: □cerebral hypoglycaemia. □lactic acidosis. □Hypoxia. □5% of patients results in death.

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