Atypical manifestations in Brazilian patients with neuro-Behçet’s disease

Abstract

Type and frequency of systemic and neurologic manifestations of Behçet's disease (BD) vary with ethnicity. In Brazil, BD occurs as sporadic cases. We describe clinical and radiological features of 36 Brazilian patients of mixed ethnicity with neuro-Behçet's disease (NBD). Medical records of 178 BD patients were reviewed and 36 (20%) NBD patients were identified. Twenty-one NBD patients (58.3%) were female and 27 (75%) presented with parenchymal manifestations. Brainstem involvement was the most common neurologic syndrome (41.7%). Seizures (27.8%), isolated aseptic meningitis (16.7%), optic neuropathy (ON) (16.7%), cerebral venous thrombosis (CVT) (8.3%), peripheral neuropathy (2.8%), and spinal cord involvement (5.6%) were other neurologic manifestations observed among Brazilian NBD patients. Eighteen (50%) had at least one relapse, and isolated aseptic meningitis was the most common relapsing manifestation. No significant differences concerning the number of relapses between parenchymal and non-parenchymal groups were found. A multivariate model including disease duration, cell count in spinal fluid, cyclosporine use, immunosuppressive use at disease onset, age at NBD onset, and ON did not reveal any significant associations with NBD relapse. There was a low frequency of CVT and an unexpected higher number of isolated aseptic meningitis. Brazilian NBD patients present more parenchymal and atypical manifestations, and relapse more often than NBD patients from other populations.

Effectiveness and tuberculosis-related safety profile of interleukin-1 blocking agents in the management of Behçet's disease

“…On the other hand, nonparenchymal neurological BD is mostly secondary to cerebral venous sinus thrombosis [55,56]. Finally, the peripheral nervous system may rarely be involved in BD, causing peripheral neuropathies or multiplex mononeuritis [57]. Treatment of neurological BD, as for the management of the gastrointestinal involvement, depends mainly on experts' experience: corticosteroids, interferon-α, azathioprine, cyclophosphamide, methotrexate, and TNF-α antagonists can be used for parenchymal diseases, while corticosteroids are recommended for dural sinus thrombosis.…”

Cerebral Venous Thrombosis in Behçet's Disease Patients Compared to Other Causes of Cerebral Venous Thrombosis: a Retrospective Study

“…Other appearances are intracranial aneurisms, extracranial aneurisms/ dissections, tumor-like-NBD, and optic neuropathy. 3,5 Cerebral venous thrombosis (CVT) may be seen as a result of NBD. Actually, BD is one of the most common causes of CVT in the countries along the ancient Silk Road.…”

Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

“…Although NBD can present with tumor-like lesions in the central nervous system, involvement [1][2][3], pattern, and location of the medullary lesion in our patient strongly pointed to HOD, in particular because of progressive clini- …”

“…It mainly affects the skin and mucous membranes, leading to recurrent oral and genital ulcers, skin lesions, and uveitis [1][2][3]. Neurological involvement in BD is rare and occurs most often in the set of systemic disease [1][2][3][4][5]. Several neurological syndromes may ensue, but meningoencephalitis is the most common, representing 75 % of central nervous system lesions [1].…”