Clinical characteristics and course of spinal cord involvement in Behçet's disease.
Parenchymal neurological involvement in Behçet's disease (p-NBD) usually presents with a brainstem syndrome; occasionally spinal cord may also be involved. Files of patients with Behçet's disease and spinal cord involvement were reviewed retrospectively, in comparison with other types of p-NBD. Amongst 216 patients with p-NBD, 24 had spinal cord involvement (11%). Most commonly patients presented with sensory-motor symptoms, sphincter and/or sexual dysfunction evolving over days. Four of 10 patients showed single or multiple cervical and/or dorsal lesions on spinal MRI's and one showed dorsal atrophy. Although the clinical picture was variable, it tended to be severe; seven cases had primary progressive course, 11 cases had a secondary progressive course after initial attack(s), four had attacks with severe residual sequela and two had improvement after attacks. After a median follow-up period of 67 months, eight were independent and 14 were dead or dependent, whereas amongst the remaining patients with p-NBD, 113 patients were independent and 56 patients were dead or dependent (P < 0.05). Our study suggests that spinal cord involvement has even worse prognosis compared with other types of p-NBD. Therefore, recognition of spinal cord involvement in Behçet's patients should prompt early vigorous treatment.
Yesilot
N
N
Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Mutlu
M
M
Gungor
O
O
Baykal
B
B
Serdaroglu
P
P
Akman-Demir
G
G
D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D015331:Cohort Studies; D018450:Disease Progression; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D020335:Paraparesis / Q000209:etiology; D012678:Sensation Disorders / Q000209:etiology; D012735:Sexual Dysfunction, Physiological / Q000209:etiology; D013116:Spinal Cord / Q000473:pathology*; D014555:Urination Disorders / Q000209:etiology