Cortical and cerebellar atrophy, but no lesion in a patient with neuro-Behçet’s disease

Abstract

Neuro-Behçet's disease (NBD) is a rare clinical entity. There are no reports about cortical atrophy in NBD. We report a patient with NBD exhibiting only cortical and cerebellar atrophy without any lesions. A 38-year-old male was hospitalized due to gait disorder. He had experienced forgetfulness, irritability, sexual and behavioral dyscontrol, and incontinence for 5 years. Magnetic resonance imaging (MRI) showed cortical and cerebellar atrophy, but there were no lesions. In the differential diagnosis of NBD, physicians should take into account the presence of both brainstem and cerebellar and cortical atrophy even in the absence of typical lesions for NBD, particularly in the presence of cognitive dysfunctions.

Movement disorders in systemic autoimmune diseases: Clinical spectrum, ancillary investigations, pathophysiological considerations

“…Acute ataxic presentations generally show focal haemorrhagic or ischaemic cerebellar, brainstem or diencephalic lesions on MRI (with or without evidence of vascular occlusion). In contrast, chronic BD ataxia generally shows cerebellar or brainstem atrophy, with or without leukoencephalopathy [109,116,117,122]. Overall, CSF findings are generally non-specific.…”

“…Cerebellar ataxia is the most common manifestation of neuro-BD, occurring in 66.7% of BD patients, and generally post-dating the diagnosis [115]. Two phenotypes have been described: acute usually self-limiting ataxia (70%), and chronic progressive ataxia (30%), the latter usually accompanied by cognitive impairment [109,116,117]. The acute type mainly affects males (75%), with median age at onset of 39 years (range, 35 -55) [118][119][120][121], whereas the chronic type is more common in females (67%), with similar age at onset [116,117,122].…”

“…Two phenotypes have been described: acute usually self-limiting ataxia (70%), and chronic progressive ataxia (30%), the latter usually accompanied by cognitive impairment [109,116,117]. The acute type mainly affects males (75%), with median age at onset of 39 years (range, 35 -55) [118][119][120][121], whereas the chronic type is more common in females (67%), with similar age at onset [116,117,122]. Acute ataxic presentations generally show focal haemorrhagic or ischaemic cerebellar, brainstem or diencephalic lesions on MRI (with or without evidence of vascular occlusion).…”

Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

“…152 153 146 152 153 The syndrome of fatigue and sleep disturbance is common in Behçet's syndrome. Measurements of fatigue, anxiety and depression are higher in patients than in control populations and fatigue itself correlates with anxiety and depression; [153][154][155] scores of depression and sleep quality correlate with disease severity. 155 156 In another study, 64% of 117 patents studied had high fatigue scores and 63% poor quality sleep; serum concentrations of IL-6 and melanocyte stimulating hormone correlated with these clinical features.…”