Síndrome de la persona rígida: manifestación de afectación inmunológica específica del sistema nervioso central / Stiff man syndrome: a specific immunological condition affecting the central nervous system

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[Anticonvulsant hypersensitivity syndrome with severe repercussions in the skin and kidneys due to carbamazepine]. / Síndrome de hipersensibilidad por antiepilépticos con repercusión cutánea y renal grave por carbamacepina.

INTRODUCTION: Anticonvulsant hypersensitivity syndrome (AHS) is characterised by fever, skin rashes and involvement of the internal organs. Owing to the low frequency with which it appears and its high clinical heterogeneity, it is not always suspected. Moreover, the symptoms often overlap with those of a vasculitis or of an infection. The most commonly associated antiepileptic drugs (AED) are the aromatic agents. We report the case of a female patient who developed AHS with several different AED and presented an especially severe kidney and skin disorder due to carbamazepine (CBZ). CASE REPORT: We describe the case of a 26-year-old woman who, after being diagnosed as suffering from secondarily generalised partial seizures, began treatment with 200 mg/12 hours CBZ. A few weeks later, she developed itchy skins lesions compatible with exanthematic pustulosis, together with acute kidney failure requiring haemodialysis. A biopsy study of the kidney revealed immunoallergic tubulointerstitial nephropathy, which is a lesion that has only very occasionally been reported in relation to CBZ therapy. The patient also presented a moderate rise in the level of transaminases and leukocytosis with eosinophilia. She was discharged from hospital without AED but suffered new seizures and was treated with phenytoin and, later, with valproic acid, both as monotherapy. With these drugs she developed AHS consisting in fever, rashes, eosinophilia and subclinical hepatitis. In epicutaneous tests with anticonvulsants, the three AED presented a positive reading, as well as others. The patient was treated with tiagabine, and there were no further hypersensitivity phenomena and a good control of seizures was achieved. CONCLUSIONS: AHS is an infrequent, but potentially serious, clinical entity and must therefore be suspected in patients taking AED who develop fever, rashes or disorders affecting the internal organs.

Síndrome de hipersensibilidad por antiepilépticos con repercusión cutánea y renal grave por carbamacepina / Anticonvulsant hypersensitivity syndrome with severe repercussions in the skin and kidneys due to carbamazepine

Introducción. El síndrome de hipersensibilidad por antiepilépticos (SHA) se caracteriza por fiebre, exantema y afectación visceral. Su baja frecuencia, junto con su gran heterogeneidad clínica, hace que no siempre se sospeche. Además, los síntomas se superponen frecuentemente a una vasculitis o a un cuadro infeccioso. Los fármacos antiepilépticos (FAE) asociados con mayor frecuencia son los aromáticos. Presentamos una paciente que desarrolló un SHA con varios FAE, en la cual fue especialmente grave la afectación renal y cutánea por carbamacepina (CBZ). Caso clínico. Se trata de una mujer de 26 años quien, tras el diagnóstico de crisis parcial secundariamente generalizada, comenzó un tratamiento con 200 mg de CBZ cada 12 horas. Pocas semanas más tarde, desarrolló lesiones cutáneas pruriginosas compatibles con pustulosis exantemática, junto con fallo renal agudo por el que precisó hemodiálisis. La biopsia renal mostró nefropatía tubulointersticial inmunoalérgica. Esta lesión se ha descrito en escasas ocasiones en relación con el tratamiento con CBZ. Presentaba una ligera elevación de las transaminasas y leucocitosis con eosinofilia. Se le dio de alta sin FAE, pero sufrió nuevas crisis, por lo que se trató con fenitoína y, posteriormente, ácido valproico, ambos en monoterapia; desarrolló con dichos fármacos un SHA consistente en fiebre, exantema, eosinofilia y hepatitis subclínica. En las pruebas epicutáneas con antiepilépticos, presentaron una lectura positiva los tres FAE, además de otros. Se trató con tiagabina, con la que no se presentaron nuevos fenómenos de hipersensibilidad y permitió un buen control de las crisis. Conclusión. El SHA es una entidad infrecuente, pero potencialmente grave, por lo que es imprescindible sospecharlo en pacientes con FAE que desarrollen fiebre, exantema y/o afectación visceral (AU)

Introduction. Anticonvulsant hypersensitivity syndrome (AHS) is characterised by fever, skin rashes and involvement of the internal organs. Owing to the low frequency with which it appears and its high clinical heterogeneity, it is not always suspected. Moreover, the symptoms often overlap with those of a vasculitis or of an infection. The most commonly associated antiepileptic drugs (AED) are the aromatic agents. We report the case of a female patient who developed AHS with several different AED and presented an especially severe kidney and skin disorder due to carbamazepine (CBZ). Case report. We describe the case of a 26-year-old woman who, after being diagnosed as suffering from secondarily generalised partial seizures, began treatment with 200 mg/12 hours CBZ. A few weeks later, she developed itchy skins lesions compatible with exanthematic pustulosis, together with acute kidney failure requiring haemodialysis. A biopsy study of the kidney revealed immunoallergic tubulointerstitial nephropathy, which is a lesion that has only very occasionally been reported in relation to CBZ therapy. The patient also presented a moderate rise in the level of transaminases and leukocytosis with eosinophilia. She was discharged from hospital without AED but suffered new seizures and was treated with phenytoin and, later, with valproic acid, both as monotherapy. With these drugs she developed AHS consisting in fever, rashes, eosinophilia and subclinical hepatitis. In epicutaneous tests with anticonvulsants, the three AED presented a positive reading, as well as others. The patient was treated with tiagabine, and there were no further hypersensitivity phenomena and a good control of seizures was achieved. Conclusions. AHS is an infrequent, but potentially serious, clinical entity and must therefore be suspected in patients taking AED who develop fever, rashes or disorders affecting the internal organs (AU)