Autopsy case of neuro‐Behçet’s disease with multifocal neutrophilic perivascular inflammation

Abstract

We report here an autopsy case of neuro-Behçet's disease. The patient was a 28-year-old man, who developed a slight fever, right uveitis, and right sensory neural hearing loss at the age of 25. These symptoms relapsed repeatedly despite treatment. Eventually he was admitted to hospital because of progressing neurological deficits such as pyramidal symptoms, somatic sensorial and autonomic disorders, and bulbar palsy. The patient's condition deteriorated and he died of heart failure. Total clinical course was about three years. In postmortem examination, various-sized necrotic foci, often accompanied by gliosis and foamy macrophage infiltration, were scattered in the diencephalic region and brain stem. Meningitis was observed on the ventral side of the brain stem as well as inferior cerebral surface. Nonbacterial or non-fungal acute perivascular inflammatory foci were also present in the brain stem and cerebellar parenchyma. These histopathological findings suggest that a destructive multifocal neutrophilic inflammation might have caused the neurological deficits. Perivascular inflammation might be important to understanding the pathogenesis of neuro-Behçet's disease.

Characteristics of Neuro-Behçet's Disease in a Case-Series from a Single Centre in Northern Portugal

“…The preferential involvement of the white matter in some series can also be explained by geographic and ethnic differences [21]. This finding, consistent with small vessels disease/vasculitis (in keeping with the neuropathological findings in NB that showed an inflammatory perivasculitis) [22,23] has been helpful in supporting parenchymal involvement in BD. However, it lacks specificity, as it may also be described in other vasculitis and also in some inflammatory demyelinating diseases.…”

Pseudotumor cerebri como una manifestación excepcional de la enfermedad de Behçet: Caso clínico

“…Si bien la clásica histología del Behçet es una vasculitis de vaso pequeño, esto rara vez se demuestra a nivel de SNC, encontrándose zonas de células apoptóticas y un infiltrado inflamatorio perivascular 13 . En cuanto al estudio del compromiso neurológico en Behçet, el LCR puede ser normal y en caso de estar alterado los hallazgos más frecuentes son la pleocitosis y la hiperproteinorraquia.…”

Neurological Manifestations in Behcet Disease

“…In the progressive phase, authors found the inflammatory infiltration of lymphocytes and cytokines remains and cuts down. (Hirohata et al, 2008;Heo et al, 2008;Hadfi et al, 1996;Arai et al, 2006;Scardamaglia et al, 2001). …”