Febrile convulsions and related epileptic syndromes

  Febrile convulsions Definition: Convulsions associated with febrile illness in children older than 1 month without febrile convulsions prior to onset, excluding central nervous system infections and electrolyte disturbances.  Febrile illness is defined as a patient with a body temperature above 38.4, and in some children, fever is evident only after the onset of convulsions.  Febrile convulsions and convulsions with fever are different concepts, and intracranial infections must be excluded in the diagnosis. For convulsive seizures with fever, lumbar puncture CSF is required whenever a serious bacterial infection is clinically suspected, especially septic meningitis (especially partially treated cerebral).  In addition, attention should be paid to the presence of pre-existing CNS abnormalities, triggering of convulsions due to fever, and the presence of temporary metabolic disturbances such as hypocalcemia and hypoglycemia.  2. generalized epilepsy with febrile seizures plus (GEFS+): The most common phenotype is FS+, and the rare phenotypes include FS with akathisia, FS with myoclonic seizures, FS with atonic seizures, etc.  Temporal lobe epilepsy 4. severe myoclonic epilepsy in infancy (SMEI) is also known as Drave’t syndrome.  It is a rare epilepsy syndrome, characterized by a family history of epilepsy or febrile convulsions, normal development before onset, seizures begin within 1 year of age, initially presenting as prolonged generalized or unilateral convulsive seizures induced by fever, mainly clonic seizures, EEG is normal, this stage is often diagnosed as FS. The EEG shows widespread spike-slow waves and multiple spike-slow waves, which are difficult to control seizures. After the onset of the seizure, the child may have delayed or regressed intellectual-motor development, ataxia, and cone bundle signs.  Myoclonic astatic epilepsy (MAE) is also known as Doose syndrome.  It usually starts at the age of 2-5 years with fever at the beginning of the seizure, and starts with FS, followed by myoclonic-atonic seizures, myoclonus, atypical aphasia, atonia, and myoclonic standing inability seizures. The EEG usually has irregular diffuse fast-spingy-slow complex waves or multi-spingy waves, and in myoclonic-atonic seizures, there may be spiky-slow complex waves with full conduction of 2-4H z. There is often developmental delay after the onset of the seizure, and the prognosis of intelligence is highly variable, from normal to severely impaired.