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

Abstract

We report the clinical and autopsy features of a 65-year-old Japanese man who clinically exhibited overlap of both neuro-Behçet's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA-B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1-weighted images, high intensity on T2-weighted and fluid-attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein-like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.

Myelopathy in Behçet's disease: The Bagel Sign

“…18 Although our findings at first may appear contradictory, it is possible that Motor Neuron pattern in BD could potentially evolve into ALS as has also been shown in other immune-mediated myelopathies such as multiple sclerosis when the right genetic setting is present. 18 Although our findings at first may appear contradictory, it is possible that Motor Neuron pattern in BD could potentially evolve into ALS as has also been shown in other immune-mediated myelopathies such as multiple sclerosis when the right genetic setting is present.…”

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

“…[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.…”

“…Di¤er yay›nda ise spinal tutulumu olan Nörobehçet sendromu ve beraberinde ALS olan bir olgu sunulmufltur. [11] Mrabet ve ark. 'n›n bildirdi¤i 5 hasta ve bizim olgumuz Uluslararas› Behçet Hastal›¤› Çal›flma Grubunun tan› kriterlerini karfl›lamaktayd›.…”

“…[12] Sato ve ark. 'n›n [11] olgusunda ise BS tan›s› üveit hikayesi ve HLA-B51 pozitifli¤i ile konmufl olup bu hastada BS tan›s›nda genelde olmas› gerekli olan oral ülser bulunmamaktayd›. Hastalar›n hepsinin erkek olmas› ALS'nin erkeklerde daha s›k gözlenmesinde dolay› beklenen bir durumdu.…”

“…Bu do¤rultuda, beraberinde NBS olan hastan›n immünsüpresif tedavi ile kraniyal lezyonlar› ve iliflkili klinik bulgular› gerilemiflken ALS klini¤i bu tedaviye cevap vermemifl ve hasta solunum yetmezli¤i ile eksitus olmufltur. [11] ‹stanbul'da yap›lan BS prevalans çal›flmas›nda BS s›kl›¤› 42/10.000 bulunmufltur. [14] ALS için ise Türkiye'de epidemiyolojik veri olmasa da tahmini hasta say›s› 6000-7000'dir.…”