Izabel Feitosa da Mata Leite
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Adelina Mouta Moreira Neto
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Guilherme de Aguiar Moraes
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Lucas Cardoso Siqueira Albernaz
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Matheus de Campos Medeiros
Context: Behçet’s Disease (BD) is a multisystem chronic disorder characterized by perivasculitis within several tissues, including the Central Nervous System – Neuro-Behçet’s Disease, which accounts for 3 – 9 % of the BD patients. Neuro- Behçet’s Syndrome may present as brainstem or pyramidal syndromes, myelopathies, meningoencephalitis, intracranial hypertension and movement or psychiatric disorders. The objective of the present work is to report a case of Neuro-Behçet’s Disease, a rare and disabling disorder. Case report: 24-year-old male presenting focal neurological deficits – hemiparesis on the right side and motor aphasia, associated with unstoppable hiccups and visual blurring. His previous pathological history featured several self-limiting episodes of the same neurological presentation, as well as acute exacerbations of oral and genital ulcers. Erythema Nodosum and Folliculitis were his main skin lesions. The ophthalmological evaluation showed bilateral cicatricial chorioretinitis. Laboratorial diagnostic procedures revealed left shift leukocytosis, high ESR, high protein level in the CSF and negative serology for B and C hepatitis, HTLV, HIV and toxoplasmosis. P-ANCA, C-ANCA, ANA and Rheumatoid factor were all found negative. Magnetic Resonance Imaging of the brain showed multiple oval- shaped T2- hyperintensity foci, with adjacent vasogenic edema, in the brainstem and basal ganglia areas – suggesting vasculitis. Treatment involved pulse therapy with Methylprednisolone, followed by Azathioprine and Prednisone. The patient has had full recovery and no other relapses. Conclusions: This case illustrates the importance of investigating Neuro-Behçet’s Disease in patients with neurological symptoms and oral/genital ulcers. The goal is to establish adequate and early treatment to improve the quality of life.
IgA nephropathy in a patient with Behcet's syndrome--case report and review of literature