[From the beginning of a continuous partial epilepsy to the diagnosis and treatment of Rasmussen's syndrome].
Continuous partial epilepsy is a form of partial status epilepticus, which is characterized by the presence of repeated myoclonus affecting a muscle group. Its origin is cortical and it can last for hours, days, weeks and exceptionally, years. Within these forms of epilepsy we can distinguish two groups: the first group or Kojewnikow classic syndrome includes children with a known lesion in the rolandic region (the etiology is also known) and there is a stable neurological damage (unless the injury increases, e.g., tumors). This disease is characterized by the presence of motor partial seizures, sometimes they are followed by periods of well-localized myoclonus. The second group or Rasmussen syndrome is characterized by onset of seizures in previously healthy patients, starting with partial motor seizures, that later can be combined with myoclonus that affect different areas of the body. It is a progressive disease that leads to neurological damage. A case is presented of a 7-year-old patient investigated due to having partial seizures and progressive neurological degeneration. After performing imaging studies, neuropsychological studies, and laboratory tests, he was diagnosed with Rasmussen's syndrome. Finally, a palliative hemispherectomy was performed and the diagnosis was confirmed by a biopsy.
Servicio de Pediatría, CMI Teresa Herrera, Centro Hospitalario Universitario, A Coruña, España. natalia.fernandez.suarez@sergas.es
N
N
Fernández Suárez
B
B
Felgueroso Juliana
S
S
Castro Aguiar
D002648:Child; D004660:Encephalitis / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D004828:Epilepsies, Partial / Q000209:etiology; D006801:Humans; D008297:Male