Neuro-Behçet syndrome presentıng as acute meningeal syndrome

Kara, Bülent; Somer, Ayper; Pişkin, Sanem; Aydınli̇, Nur; Salman, Nuran; Yalçın, Işık

doi:10.1016/j.jinf.2005.07.026

Cited by 14 publications

(22 citation statements)

References 6 publications

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“…Sixty-nine patients (53%) originated from the so-called “silk road” stretching from Morocco to Japan [14,17,18,28,30-33,35,37-42,44,48-50]. The large majority of these subjects (64/69 subjects) were from Western Asia, and only five were from other regions along this historical route [31,41].…”

Section: Resultsmentioning

confidence: 99%

“…We tabled the neuro-ophthalmological features for each of these subjects [14,26,27,33-38,40-42,44-48,50]. Data referring to the 14 cases having the parenchymal form are detailed in Table 2.…”

Section: Resultsmentioning

confidence: 99%

“…In 12/53 patients (23%), neurological and/or ophthalmological symptoms anticipated any other major or minor criteria for a diagnosis of BD [14,26,27,33,34,36,40,44,47,48], and in seven additional patients, only previous oral/genital aphtae were recalled by the patient [14,35,38,41,42,45,50]. NBD was diagnosed at presentation in 13 subjects [14,36,37], and before neurological involvement in 21 patients [14,46,48].…”

Section: Resultsmentioning

confidence: 99%

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Neuro-Behçet’s disease in childhood: A focus on the neuro-ophthalmological features

Mora

Menozzi

Orsoni

et al. 2013

Orphanet Journal of Rare Diseases

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Neuro-Behçet’s disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged ≤16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behçet’s disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal experience, a delayed diagnosis, and the consequently delayed immunosuppressive treatment, may favour permanent sequelae, in particular, optic atrophy.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Neuro-Behçet’s disease in childhood: A focus on the neuro-ophthalmological features

Mora

Menozzi

Orsoni

et al. 2013

Orphanet Journal of Rare Diseases

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“…In general ataxia, dysarthria, hemiparesis, bipolar pyramidal findings, cognitive disorder, and sphincter defect are detected during the examination. Headache and fever may occur in this type of involvement5). Siva et al reported that headaches in NB disease is at a high rate of 61%7).…”

Section: Discussionmentioning

confidence: 95%

A Neuro-Behçet's Case Operated with the Intracranial Mass Misdiagnosis

Tokgöz

Akpınar

Güney

et al. 2012

J Korean Neurosurg Soc

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Behçet's disease (BD) is an inflammatory systemic disorder with oral and genital ulcers, as well as ophthalmologic and cutaneous symptoms. Neurological manifestations in BD represent between 2.2% to 50% of the cases. The 25-year-old male patient, diagnosed with BD three years earlier, was admitted to our clinic with complaints of recurrent headaches. Tumor-like-parenchimal involvement was detected on a cranial magnetic resonance imaging. The lesion was removed surgically and then he suffered from right hemiparesis and epilepsy. Pathological examination of the lesion noted a demyelinating non-tumoural etiology. A neuro-Behğet's case with parenchymal involvement has been examined in light of the literature, in terms of a tumor and a demyelinating disease differential diagnosis.

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“…Behçet disease presenting as an acute meningeal syndrome has been reported. 18 Treatment of Behçet disease is aimed at decreasing active inflammation and becomes problematic given the chronic and recurrent nature of the disease. Corticosteroids are effective in treating acute inflammation but other immunosuppressive agents are commonly considered for long-term treatment.…”

Section: Behçet Diseasementioning

confidence: 99%