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

Abstract

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.

Myelopathy in Behçet's disease: The Bagel Sign

“…Therefore, our example with sustained clinical and radiological improvement did not represent a case of coexisting amyotrophic lateral sclerosis (ALS) with BD. Coexistence of ALS with BD was previously suggested 17 and also reported in a pathologically Figure 1A corresponds to a patchy peripheral smear-like enhancement pattern in axial images. (B) Axial T2W, axial T1W, and axial Gd1T1W images during myelopathy episode #3 from patient 2 are shown.…”

“…Coexistence of ALS with BD was previously suggested 17 and also reported in a pathologically FIGURE 3: Contrast-enhancement pattern of Bagel Sign. Patient 11 of our series had uniform, densely affected bilateral anterior horn cell columns, but underwent a dramatic improvement from paraplegia to a sustained mild monoparesis with almost complete resolution of imaging findings after immunomodulation.…”

Coexistence of amyotrophic lateral sclerosis with neuro‐Behçet's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient

“…Despite the fact that Behçet's disease and ALS are relatively rare and clinically distinct, at least five patients have been reported to show overlap of the clinical symptoms of the two disorders . Even considering their rarity, coincidental associations between them cannot be excluded.…”

Behçet's syndrome and amyotrophic lateral sclerosis: a case report and literature review

“…[9] Behçet sendromu ve ALS hastal›¤›n›n beraber oldu¤u olgular literatürde az say›da bulunmaktad›r. [10] ALS'nin, BS'nin nörolojik tutulumu mu oldu¤u yoksa rastlant›sal olarak m› beraber görüldü¤ü bilinmemektedir. Biz de BS ve ALS birlikteli¤ini bir olgu üzerinden literatür eflli¤inde tart›flmay› amaçlad›k.…”

“…[10,11] Mrabet ve ark. [10] bu birlikteli¤i araflt›rmak amac›yla Uluslararas› Nörobehçet Hastal›¤› Çal›flma Grubu üyelerine mektup yollayarak takip ettikleri BS-ALS hastas› olup olmad›¤›n› sormufllard›r. Kaç üyenin cevaplad›¤› belirsiz olsa da iki merkezden geri bildirim alm›fllar.…”