MRI findings of neuro-Behcet’s disease

Borhani‐Haghighi, Afshin; Sarhadi, Sirous; Farahangiz, Siamak

doi:10.1007/s10067-010-1650-9

Cited by 52 publications

(25 citation statements)

References 17 publications

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“…Facing upcoming patients with this constellation, neurologists and rheumatologists needed to conduct tests like magnetization transfer ratio; MR spectroscopy; MR tractography; CSF IL-6, TNF-alpha, and other inflammatory markers; anti-GM1 antibody; hexosaminidase level; and superoxide dismutase type 1 gene studies to shed light on the probable association of these two mysterious diseases. Knowing that both ALS [27] and progressive NBD [28] have no efficacious treatments re-necessitates the indication of these studies.…”

Section: Discussionmentioning

confidence: 98%

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

et al. 2012

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Neurological involvement may be seen in 5-30% of the patients with Behcet's disease (BD). Occasionally, parenchymal neurological involvement in BD can present as a spinal cord syndrome. However, motor neuron disease-like presentation is extremely uncommon. Here we are reporting five patients (all male; median age, 38) fulfilling both International Study Group criteria for BD and El Escorial criteria for amyotrophic lateral sclerosis (ALS). These patients were identified by a questionnaire sent to the members of the Neuro-Behcet Study Group of the International Study Group for BD. Three out of five patients had only motor presentations. In two patients, sensory and urinary manifestations were present as well. Spinal cord MRIs were normal in all, and brain MRIs were normal in four patients; one patient had nonspecific white matter changes. Two patients passed away 1-3 years after diagnosis of ALS, and two patients were lost to follow-up 3 and 11 years after admission; one patient is still alive 3 years after onset. The patients that are presented here might represent a rare form of neurological involvement in BD as well as sole coincidence. Larger prospective series are needed to further answer this issue.

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Section: Discussionmentioning

confidence: 98%

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

et al. 2012

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“…CNS involvement is one of the most severe manifestations of BD [14]. Sometimes it is difficult to differentiate from multiple sclerosis, stroke, intracranial hypertension, meningoencephalitis and myelitis.…”

Section: Discussionmentioning

confidence: 99%

“…Rarely, an inflammatory condition such as sarcoidosis, Wegener's disease, multiple sclerosis (Balo's type) or granulomatous pseudotumour may lead to similar lesions [8]. It has been suggested that histopathological examination may be done to rule out infectious or tumoural diseases and to avoid the inadequate use of immunosuppressive agents in previous reports [14,16,17].…”

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confidence: 99%

Tumefactive Neuro-Behcet’s Disease: A Case Report and Review of the Literature

Kuzu¹,

Ozer²,

Yücesan³

et al. 2017

J Neurol Neurosci

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Aim: Behçet's disease is a multi-systemic autoimmune disease, characterized with recurrent oral-genital ulcers and uveitis. Neuro-Behçet's disease (NBD) may rarely cause tumour like brain lesions. Methods:Here we present a case of forty-two-year-old Turkish male with tumour like brain lesion whose tissue biopsy result was compatible with necrotic inflammation, perivascular polymorphonuclear leukocytes (PMNL), lymphocytes and other inflammatory cells infiltration. Bacterial and fungal blood cultures were negative. The patient was diagnosed with NBD and was treated with intravenous methylprednisolone 1000 mg/day for 5 days. His neurologic examination improved significantly with the treatment. We evaluated histopathological properties of all biopsy proven cases reported so far in the literature including our case.Results: When tumour-like lesions were seen in patients who had Behçet's disease in past medical history, NBD should be considered for preventing unnecessary brain operation. Seventy-three percent of all biopsy proven tumefactive NBD cases had inflammatory cell infiltration in the tumour like lesion area. Conclusion:After excluding infectious aetiology, high dose IV methylprednisolone should be begun as soon as possible in spite of mass effect that is compatible with tumour in brain magnetic resonance imaging (MRI); the good response to steroid supports to the diagnosis of tumefactive NBD. Although most tumefactive NBD patients have inflammatory infiltration in biopsy material why some cases do not have any inflammatory cell infiltration in acute lesion area needs to be clarified in further research.

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“…1). Hemispheric lesions are exceedingly rare, but up to 71 % may fulfill MRI diagnostic criteria for multiple sclerosis [16]. Progressive infratentorial atrophy, tumor-like lesions in the brainstem, and preservation of corpus callosum help in the differential diagnosis [5,15,16].…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

et al. 2015

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A 22-year-old man presented with an acute altered mental status, left third cranial nerve palsy, ataxia, and right side weakness, all of which suggestive of brainstem encephalitis. Six months earlier, he had persistent fever, myalgia, and weight loss. Soon after fever resolution, oral and scrotal ulcers, usually painless, appeared accompanied by acneiform skin lesions in the chest and arms. At the time, antibiotic treatment led to partial regression of the lesions.Brain magnetic resonance imaging (MRI) showed hyperintense lesions on left midbrain, thalamus, and subcortical regions (Fig. 1). Cerebrospinal fluid analysis showed only mild lymphocytic pleocytosis. Pulse therapy with methylprednisolone 1 g/day for 7 days followed outstanding clinical improvement, although residual right hemiparesis and kinetic tremor remained. At discharge, he was on prednisone 20 mg/day and azathioprine 150 mg/day. Four months after therapy onset, a control brain MRI demonstrated resolution of most lesions along with signal alteration and enlargement of the olivary nuclei in the left medulla (Fig. 2a). At the time, he presented only minor oculopalatal myoclonus. Clinical stability and imaging pattern of the lesion suggested a benign finding, and no further investigation was pursued.Six years later, brain MRI showed an atrophic left midbrain peduncle with less evident olivary degeneration. In addition, there was significant atrophy of right cerebellar hemisphere (Fig. 2b) and shrinkage of midbrain (Fig. 2c, d). At that point, the patient had only mild right hemiparesis and oculopalatal myoclonus. Azathioprine 150 mg/day was his only medication, and he remains stable until this moment.

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MRI findings of neuro-Behcet’s disease

Cited by 52 publications

References 17 publications

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

Tumefactive Neuro-Behcet’s Disease: A Case Report and Review of the Literature

Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

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