RESUMEN
Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia disorder in which children, usually 3-8 years of age who have developed age-appropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. There are no controlled clinical trials investigating the therapeutic options for LKS. Only open-label data are available. Early diagnosis and initiation of prompt medical treatment appear to be important to achieving better long-term prognosis.Several antiepileptic drugs have been reported to be beneficial in treating this syndrome. These include valproic acid (valproate sodium), diazepam, ethosuximide, clobazam, and clonazepam. Reports on the efficacy of lamotrigine, sultiame, felbamate, nicardipine, vigabatrin, levetiracetam, vagal nerve stimulation, and a ketogenic diet are few and more experience is needed. Carbamazepine and possibly phenobarbital and phenytoin have been reported to occasionally exacerbate the syndrome. As initial therapy, valproic acid or diazepam is often empirically chosen. Subsequently, other antiepileptic drugs, corticosteroids, or intravenous immunoglobulin (IVIG) therapy are often used. Corticosteroid therapy should probably not be delayed more than 1-2 months after the initial diagnosis. Various corticosteroid regimens including oral prednisone and, recently, high doses of intravenous pulse corticosteroids, as well as corticotropin (adrenocorticotropic hormone) have been reported to be effective in LKS. Oral corticosteroids are used more often and usually need to be maintained for a long period of time to prevent relapses. The use of IVIG has been associated with an initial dramatic response in only a few patients. In our experience, a long-term worthwhile improvement has been noted in only 2 of 11 patients. These two patients had an immediate response to IVIG initially and after relapses before eventually achieving a long-term sustained remission. Surgical treatment by multiple subpial transection, which is reserved for patients who have not responded to multiple medical therapies, has been followed in selected cases by a marked improvement in language skills and behavior. However, a widely accepted consensus about suitable candidates for this surgery and about its efficacy is still lacking. Speech therapy, including sign language, and a number of classroom and behavioral interventions are helpful in managing LKS, and should be used in all patients.
Asunto(s)
Hormona Adrenocorticotrópica/uso terapéutico , Anticonvulsivantes/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunoglobulinas Intravenosas/uso terapéutico , Síndrome de Landau-Kleffner/tratamiento farmacológico , Terapia Conductista , Niño , Preescolar , Humanos , Síndrome de Landau-Kleffner/terapia , Psicocirugía , LogopediaRESUMEN
We report a case with calculation-induced idiopathic generalized epilepsy (IGE) that, unlike most patients with IGE, was refractory to medications. This patient had a family history of (1) a similar condition in a relative of hers who, however, did not have identical manifestations, and (2) a mother who had migraine. Our observations illustrate that the occurrence of IGE in families usually follows rather complex patterns of inheritance and that some of them can be refractory to therapy.
Asunto(s)
Epilepsia Generalizada/diagnóstico , Epilepsia Generalizada/genética , Epilepsia Refleja/diagnóstico , Epilepsia Refleja/genética , Matemática , Adolescente , Adulto , Edad de Inicio , Anticonvulsivantes/uso terapéutico , Niño , Hijo de Padres Discapacitados , Diagnóstico Diferencial , Electroencefalografía/estadística & datos numéricos , Epilepsia Generalizada/tratamiento farmacológico , Epilepsia Refleja/tratamiento farmacológico , Salud de la Familia , Femenino , Humanos , Masculino , Trastornos Migrañosos/genética , Epilepsia Mioclónica Juvenil/diagnóstico , Linaje , Insuficiencia del TratamientoRESUMEN
We report a case of recurrent partial seizures that were often precipitated by looking up a flight of stairs and included spitting as well as repetitive affectionate kissing automatisms. These seizures were shown by long-term video/EEG monitoring to be of right temporal origin and completely subsided after right temporal lobectomy. This case is unique because: (1) The patient had partial rather than primarily generalized pattern-induced seizures. (2) Affectionate kissing automatisms were a part of his partial seizures and, to our knowledge, have not been reported in the literature before.