ABSTRACT
Background: Spinal dural defects can result in superficial siderosis (SS) of the central nervous system. Closure of the defect can stop or slow the progression of the disease. Here, we evaluated, whether preoperative three-dimensional fast steady-state acquisition MR could adequately detect these defects and, thus, facilitate their closure and resolution. Case Description: A 65-year-old right-handed male presented with a 33-year history of the left C8 root avulsion and a 3-year history of slowly progressive gait difficulties and hearing loss. The T2*-weighted imaging revealed symmetrical hemosiderin deposition throughout his central nervous system. A left C6-C7 dural defect involving only inner layer was identified using a three-dimensional MR (3D-FIESTA). It was treated through a left C6-7 hemilaminectomy and successfully sealed with adipose tissue and fibrin glue. Subsequently, the progression of cerebellar ataxia was halted, nevertheless the sensorineural hearing loss worsened even over the next 2 years. Conclusion: 3D-FIESTA reconstruction was approved to be useful tool for identifying the tiny hole of the inner dural layer responsible for SS.
ABSTRACT
We report two patients with meningoencephalomyelitis without evidence of extra central nervous system (CNS) involvement. Brain MRI showed linear perivascular radial gadolinium enhancement patterns and spinal cord MRI showed longitudinal extensive T2-hyperintensity lesions. Pathological findings from brain biopsies were angiocentric T-cell predominant lymphoid infiltrates that lacked Epstein-Barr virus-positive atypical B cells. The patients were initially suspected to have isolated CNS-lymphomatoid granulomatosis (LYG). Thereafter, glial fibrillary acidic protein (GFAP)-immunoglobulin G were detected in their cerebrospinal fluid. This finding suggested autoimmune GFAP astrocytopathy. We speculate there is a link between isolated CNS-LYG and autoimmune GFAP astrocytopathy.
