Juvenile Behçet's disease: highlighting neuropsychiatric manifestations and putative genetic mechanisms.
Clin Neurol Neurosurg
; 109(5): 436-8, 2007 Jun.
Article
en En
| MEDLINE
| ID: mdl-17350161
ABSTRACT
Behçet's disease is a multisystem inflammatory disorder of unknown etiology. We report a 12-year-old boy who presented with features of raised intracranial tension and seizures and was found to have cerebral venous sinus thrombosis on evaluation. Behçet's disease was diagnosed based on occurrence of recurrent oral and genital ulcers in the past and characteristic skin lesions subsequently. He also showed significant personality changes including multiple attempts of deliberate self-harm. Pedigree analysis revealed that six family members spanning three generations had recurrent oral ulcers and three members satisfied the criteria for Behçet's disease. Clinical features varied amongst the family members and there was suggestion of genetic anticipation. The index case was carrying HLA-B37/B7 and the mother was carrying B37/B40. Our report sheds light on the genetics of Behçet's disease. Unusual features were early age of onset, cerebral venous sinus thrombosis, significant personality changes and strong family history with phenotypic heterogeneity.
Buscar en Google
Base de datos:
MEDLINE
Asunto principal:
Trastornos de la Personalidad
/
Síndrome de Behçet
/
Trombosis del Seno Sagital
/
Pruebas Neuropsicológicas
Tipo de estudio:
Diagnostic_studies
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
Clin Neurol Neurosurg
Año:
2007
Tipo del documento:
Article
País de afiliación:
India