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Vol. 5. Issue 4.
Pages 168-170 (July - August 2009)
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Vol. 5. Issue 4.
Pages 168-170 (July - August 2009)
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NeuroBehçet: a case
Neurobehçet: a propósito de un caso
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Germán Latorre González
Corresponding author
ger.latorre@gmail.com

Corresponding author.
, Ana Belén Escribano Gascón, Carlos López de Silanes de Miguel, Rocío García Cobos, Luis Ignacio Casanova Peño, Teresa Lapeña Montero
Hospital Clínico San Carlos, Madrid, Spain
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Article information
Abstract

Behçet's disease is an inflammatory systemic disorder, with oral and genital ulcers, as well as ophthalmologic and cutaneous disturbances. Five percent of the patients have neurological alterations. We present a case of neuroBehçet with a rare neurological involvement.

A 28-years-old male patient with recurrent oral and genital ulcer history and folliculitis presents 48 h progressive fever, headache, nausea, right sixth cranial nerve palsy, and right upper limb paresis. The CSF showed lymphocyte pleocytosis and high proteinorrachia, so antibiotic treatment was initiated. Cerebral MR found hyperintense enhancing lesions in the brainstem and left transverse sinus thrombosis. Patient improved with endovenous corticosteroids and was discharged asymptomatic.

Neurological involvement in Behçet's disease is rare and moreover the coexistence of parenchymatous and non-parenchymatous alterations in the same patient. It normally has a good response to immunosuppressant treatment.

Keywords:
Behçet's disease
NeuroBehçet
Neurological alterations
Resumen

La enfermedad de Behçet es un trastorno inflamatorio sistémico, caracterizado por aftosis orogenital así como por alteraciones oftalmológicas y cutáneas. Un 5% de los pacientes presenta manifestaciones neurológicas. Se presenta un caso de neurobehçet con participación neurológica inhabitual.

Varón de 28 años con antecedente de aftas orales y genitales recurrentes e historia de foliculitis, que acudió por cuadro de 48 h de fiebre, cefalea, náuseas, paresia del sexto par craneal y del miembro superior derecho. El análisis del líquido cefalorraquídeo reveló pleocitosis linfocitaria con hiperproteinorraquia, se inició tratamiento antibiótico de amplio espectro. Se realizó resonancia magnética cerebral, que mostró lesiones hiperintensas de predominio troncoencefálico con realce tras infusión de gadolinio y trombosis del seno transverso izquierdo. El paciente mejoró progresivamente con tratamiento intravenoso con corticoides hasta quedar asintomático.

La afectación neurológica en la enfermedad de Behçet es infrecuente y más aún la coexistencia de lesiones intraparenquimatosas y extraparenquimatosas. Habitualmente, presenta buena respuesta al tratamiento inmunosupresor.

Palabras clave:
Enfermedad de Behçet
Neurobehçet
Afectación neurológica
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Copyright © 2009. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
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