ABSTRACT
Severe relapses are common in neuromyelitis optica (NMO). Plasma exchanges (PE) were successfully used to treat acute demyelinating relapses resistant to corticosteroids. However, little is known about PE efficiency in NMO relapses, particularly in relation with the presence or not of specific antibodies. We here report two patients with NMO (one seropositive and one seronegative) with dramatic improvement after PE on both the optical and spinal involvement.
Subject(s)
Neuromyelitis Optica/therapy , Plasma Exchange/methods , Female , Humans , Male , Middle Aged , Neuromyelitis Optica/physiopathology , Recurrence , Spinal Cord/physiopathology , Treatment Outcome , Vision, OcularABSTRACT
The purpose of this report is to describe 29 cases of phakomatosis including 18 cases of tuberous sclerosis (Bourneville) and 11 cases of neurofibromatosis (von Recklinghausen) observed over a 10-year period at the Neurology Department of the University Hospital Centre in Conakry, Guinea. Findings during this period were consistent with those classically reported in the literature: high frequency of advanced skin lesions coalescing into massive tumours, occurrence of seizures of all types and development of a wide variety of complications as a result of late diagnosis. Our experience underscores the need for follow-up and surveillance of these patients by somatic studies based on neurological, ophthalmologic and tomographic data depending on clinical findings.
Subject(s)
Neurocutaneous Syndromes/diagnosis , Electroencephalography , Guinea , Humans , Neurofibromatosis 1/diagnosis , Seizures , Skin Diseases , Tomography, X-Ray Computed , Tuberous Sclerosis/diagnosisABSTRACT
Les auteurs rapportent 29 observations de phacomatoses dont 18 cas de sclerose tubereuse de Bourneville et 11 cas de neurofibromatose de Recklinghausen; observees dans le service de neuro l ogie du centre hospitalo-universitaire de Conakry; sur une periode de 10 ans. Dans cette peri o d e;les donnees classiques de la litterature sont retrouvees : frequence elevee des lesions dermatologiques evoluees atteignant de volumineuses tumeurs royales; des crises epileptiques sous toutes ses formes et les complica-tions diverses en raison du retard du diagnostic. Nos resultats soulignent la necessite de suivi et de surveillance de ces patients par un examen somatique (neurologique; ophtal- mologique) et un examen scannographique dicte par les donnees cliniques
