[Early onset Neurobehçet's disease].
BACKGROUND
Behçet's disease is difficult to diagnose in infancy due to the absence of early clinicopathological pathognomonic symptoms. There have been reports of patients in whom the onset of the disease occurred during the first months of their lives.


METHODS
We describe here the case of a 12-year-old female from Pakistan whose first symptoms appeared at the age of 6 months in the form of fever and skin lesions. The first neurological symptoms appeared at the age of 5 years in the form of ophthalmoplegia, followed by ataxia and dysmetry. Magnetic resonance imaging of the head was a valuable aid in the study of the vascular alterations. She also presented abdominal pains, arthromyalgia, ulcers in the mouth and severely delayed weight gain. Yet, we were finally guided towards a diagnosis by the appearance of ulcers on the genitals, which do not usually occur until puberty, as well as the findings from the skin biopsy study. The serological study revealed the presence of antigen B08. Establishing therapy with methylprednisolone and later with azathioprine brought about the practically complete remission of the clinical picture.


CONCLUSIONS
Behçet's disease, which has been infrequent in our country up till now, is a condition to be taken into account, especially in young female patients from countries with a high incidence rate of oral and genital ulcers, accompanied by neurological symptoms. Knowledge of this syndrome and performing appropriate complementary studies are essential to be able to reach an early diagnosis and for establishing suitable treatment.
Servicio de Neuropediatría, Consorci Hospitalari Clínic-Sant Joan de Déu, Esplugues de Ll., Barcelona, Spain. mmoraledac@hotmail.com
M
M
Moraleda-Cibrián
M A
MA
Aracil-Martínez
G
G
Ercilla-González
J
J
Ros-Viladoms
V
V
Cusí-Sánchez
E
E
González-Pascual
M
M
Pineda
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001528:Behcet Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male