A pediatric patient with neuro-Behçet’s disease

Hatachi, Saori; Nakazawa, Takashi; Morinobu, Akio; Kasagi, Shinpei; Kogata, Yoshinori; Kageyama, Goichi; Koshiba, Masanori; Kumagai, Shunichi

doi:10.1007/s10165-006-0507-1

Cited by 9 publications

(9 citation statements)

References 15 publications

(16 reference statements)

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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]. In addition to the large number of Asian subjects, 24 patients (26%) were from Europe: 14 were French [12,32,36,48], eight were Italian [29,34,46,47], and two were from the UK [22,43].…”

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%

“…NBD was diagnosed at presentation in 13 subjects [14,36,37], and before neurological involvement in 21 patients [14,46,48]. Nine patients had a positive family history of BD (17%) [14,35,44,48], 30 patients had a negative family history [14,26,42,48], and information was not specified in 14 patients [27,34,36-38,40,41,45-47]. …”

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%

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

View full text Add to dashboard Cite

show abstract

“…Hearing loss in high frequencies in Behc¸et's disease is an indicator of cochlear involvement. 30,85,86 The Churg-Strauss syndrome is a systemic and pulmonary vasculitis defined by its association with severe asthma and hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with alveolar hemorrhage, renal and nervous system damage, causing mononeuropathy or polyneuropathy, encephalopathy, and stroke.…”

Section: Vestibular Paroxysmiamentioning

confidence: 99%

“…Vestibular paroxysmia also accounts for 4% of all patients with dizziness in the dizziness unit of Hüfner et al 26 Although vasculitic disorders are uncommon in the general population, vertigo may be seen in almost up to 50% of patients with different vasculitic syndromes such as systemic lupus erythematosus, Cogan's syndrome, ChurgStrauss syndrome, Behc¸et's disease, Wegener granulomatosis, giant cell arteritis, and Susac syndrome. [27][28][29][30][31][32][33] CLINICAL SYNDROMES…”

Section: Epidemiologymentioning

confidence: 99%