Clinical History
A 20-year-old female patient with a history of recent onset of oral and genital ulcers, blurred vision, and the development of moderate degree of quadriparesia with some degree of loss of touch and pain sensations in all extremities.
Imaging Findings
A 20-year-old female patient had a history of recent onset of oral and genital ulcers, blurred vision, and her ophthalmic examination revealed iridocyclitis with hypopyon bilaterally. An MRI study was performed upon the development of moderate degree of quadriparesia with some degree of loss of touch and pain sensations in all extremities. The MRI examination was performed with a 1.5 T MR scanner, SE T1, FSE T2, post-gadolinium SE T1 sagittal and axial images were obtained. MR images revealed two discrete spinal cord lesions with extensive peripheral edema, and with intense peripheral contrast enhancement following IV gadolinium injection. The neurological and other clinical findings as well as plaque lesions on MRI disappeared with intravenous steroid treatment within 8 weeks.
Discussion
Behcet's disease (BD) is a multisystem inflammatory vasculitic disorder of unknown origin with relapsing courses. The age of onset for BD is around third decade. Male to female ratio is 2 to 1. There are some major criteria listed as buccal and genital ulcerations (aphthous stomatitis, genital ulcers), ocular inflammations (relapsing iridocyclitis, hypopyon, choroiditis, papillitis, retinal vasculitis), skin lesions, and minor criteria listed as migratory thrombophlebitis, gastrointestinal (ulcers, perforation) and central nervous system findings, arthritis, family history for the diagnosis of BD. The neurologic involvement has been reported in the range of 5% to 10% in large series. BD may involve central nervous system (CNS) in two patterns, one is parenchymal CNS involvement which is the most common form, and the other is non-parenchymal CNS involvement. Brainstem is the most common place for BD in parenchymal CNS form, followed by basal ganglia, spinal cord, and cerebral hemispheres. Although CNS involvement is common in BD, isolated spinal cord involvement is reported to very rare. The patients show pyramidal signs, hemiparesis, behavioral changes and sphincted disturbance are the clinical findings in this group. Optic neuropathy, vestibulocochlear and peripheral nerve involvement are rare findings. The parenchymal distribution of lesions in BD appears to support the hypothesis of small-vessel vasculitis; mainly, venular involvement. The neuropathology is that of multifocal necrotizing lesions with marked inflammatory cell reactions, necrosis may affect both gray and white matter, probably secondary to vasculitis. In the non-parenchymal CNS involvement the syndrome of raised intracranial pressure due to dural sinus thrombosis is the main clinical manifestation. In 60% of the cases with parenchymal involvement, cerebrospinal fluid (CSF) is hypercellular with or without an elevated protein level, whereas in cases with non-parenchymal involvement CSF is usually normal except for the elevated pressure. Factors suggesting a poor prognosis are parenchymal (especially brainstem) involvement, repeated attacks, incomplete recovery, progressive disease course and a high level of protein and/or pleocytosis in the CSF during acute attack. MRI demonstrates BD lesions as well-defined areas of signal change associated with peripheral edema, and contrast enhancement following IV contrast injection. The lesions of multiple sclerosis, acute disseminated encephalomyelitis, sarcoidosis and other granulomatous inflammations, some pyogenic, viral and fungal infections may mimic the lesions of BD.
Differential Diagnosis List
Isolated spinal neuroBehcet's disease
Final Diagnosis
Isolated spinal neuroBehcet's disease
