Dados do Trabalho

Título

Neurological symptoms as initial manifestation in a patient with Behçet syndrome

RESUMO

Case Presentation: Male patient, 44 years old, no comorbidities. He started dysphagia for 3 months and, then, complete right paresis of III cranial nerve, dysarthria and bilateral dysmetria with gradual worsening. On admission, the presented foud were described, in addition with mouth ulcers refractory of oral treatment. An investigation was carried out with CSF suggestive of an inflammatory process, negative serology and neuroimaging with hypersignal on T2/FLAIR in the brainstem in the rostral region of the medulla, midbrain and left thalamus, besides positive pathergy test and biopsies of mucocutaneous and genital lesions with positive findings. Thus, given the central, chronic and progressive neurological condition, with involvement of the brainstem and important sequelae, treatment with methylprednisolone 1000 mg/day for 3 days was chosen, followed by maintenance with azathioprine at a dose of 2 mg/kg/day. The patient evolved with stagnation of sequelae and did not present new neurological manifestations, remaining with cerebellar symptoms and persistence of paresis of the III cranial nerve.
Discussion: Behçet's syndrome is a rare disease whose main characteristics are mouth ulcers and systemic manifestations that are mostly due to vasculitis.
Final Comments: Although rare, Behçet's syndrome should be included in the differential diagnosis of patients with progressive cerebellar syndrome, associated with the absence of demyelinating or neoplastic lesions, with negative infectious screening and CSF suggestive of an inflammatory process. Active research for dermatological changes in the patient's clinical history and physical examination should be carried out, as the delay in treatment can lead to the irreversibility of neurological sequelae.

Palavras Chave

Behçet syndrome, neurological manifestations, vasculitis, cerebelar ataxia, pathergy

Área

Neuroimunologia