“…We found a positive correlation between NB and vascular and gastrointestinal involvement in comparison with BD without neurological manifestations, as previously reported [15]. …”
Introduction: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology that may affect the CNS - Neuro-Behçet (NB). Our aim was to evaluate the frequency of neurological involvement and characterize a cohort of our NB patients. Methods: We retrospectively revised the clinical, laboratory and imaging data of a cohort of BD patients, followed in our hospital outpatient clinic. Results: We identified 138 BD patients. Twenty-five out of 138 had NB (15 female). Four patients presented with neurological symptoms. We identified a total of 37 attacks. Twenty-one attacks were classified as parenchymatous, four non-parenchymatous and 12 as other syndromes. Seventeen patients had CSF analysis performed (20 samples). Five samples were normal, 15 showed CSF pleocytosis. The most frequent finding on MRI performed in the acute phase was extensive lesions involving the brainstem. Two patients died due to the neurological involvement of BD. Conclusion: We found 18.1% prevalence of NB and a higher female-to-male ratio in our group than in other series. Gastrointestinal and vascular involvement was more frequent in the NB group. The fact that neurological involvement may be the first manifestation of BD with therapeutic implications and associated morbidity points out the relevance of an early diagnosis.
“…We found a positive correlation between NB and vascular and gastrointestinal involvement in comparison with BD without neurological manifestations, as previously reported [15]. …”
Introduction: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology that may affect the CNS - Neuro-Behçet (NB). Our aim was to evaluate the frequency of neurological involvement and characterize a cohort of our NB patients. Methods: We retrospectively revised the clinical, laboratory and imaging data of a cohort of BD patients, followed in our hospital outpatient clinic. Results: We identified 138 BD patients. Twenty-five out of 138 had NB (15 female). Four patients presented with neurological symptoms. We identified a total of 37 attacks. Twenty-one attacks were classified as parenchymatous, four non-parenchymatous and 12 as other syndromes. Seventeen patients had CSF analysis performed (20 samples). Five samples were normal, 15 showed CSF pleocytosis. The most frequent finding on MRI performed in the acute phase was extensive lesions involving the brainstem. Two patients died due to the neurological involvement of BD. Conclusion: We found 18.1% prevalence of NB and a higher female-to-male ratio in our group than in other series. Gastrointestinal and vascular involvement was more frequent in the NB group. The fact that neurological involvement may be the first manifestation of BD with therapeutic implications and associated morbidity points out the relevance of an early diagnosis.
“…The most common sites of lesions are the brainstem extending to the diencephalon and basal ganglia in NBD, while periventricular regions are more commonly involved in MS. If periventricular lesions are seen in NBD, they will be smoother and less frequent than those in MS. Brainstem lesions in NBD are larger than those in MS. 23 The most frequent pathologic feature of neuropsychiatric lupus is abnormal vessel walls without any inflammatory cell infiltration in the small vessels, particularly arterioles and capillaries. 24 It is obviously different with the abovementioned pathologic presentations of NBD.…”
Pathologic findings of central nervous system involvement in Behçet disease mainly include perivascular infiltration with lymphocytes, neutrophils and rarely, eosinophils, with or without signs of necrosis. The most common sites of brain involvement include the brainstem and basal ganglia. Nonetheless, other areas can also be involved.
“…In our prospective study with exclusion of all unsubstantiated cases, the prevalence of definite neurologic involvement among 96 Behçet patients was 6.3%. 28 In an autopsy series, 20% of patients with Behçet disease revealed pathologic signs of neurologic involvement. 29 Considering the abovementioned reasons, the prevalence of neurologic manifestation of Behçet disease must be between 5% and 30%.…”
Section: Epidemiology Prevalencementioning
confidence: 99%
“…[61][62][63] Eventually, pseudobulbar palsy and emotional incontinence can occur. 28 Recurrent facial palsies, 64 palatal myoclonus, 65 paroxysmal dysarthria, 66 intranuclear opthalmoplegia, 67 and isolated hypoglossal nerve palsy 68 have also been reported.…”
Neuro-Behçet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.
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