Tumefactive demyelination of the spinal cord: a case report

Abstract

Study design: Case report. Objectives: We report on a 52-year-old male patient with tumefactive demyelination of the spinal cord. Setting: University Hospital and Jessenius Faculty of Medicine, Comenius University, Martin, Slovakia. Background: In contrast to relatively frequent tumefactive fulminant lesions in the brain, cases affecting the spinal cord in isolation have been reported less frequently. Methods: Description of the case report. Results: Clinical, neuroradiological and necropsy findings are described in a 52-year-old man with tumefactive fulminant demyelination of the spinal cord. Progression of the demyelination process produced paraplegia, mild paresis of the right upper limb, neurogenic bladder and sensitive loss over 2 weeks. MRI scans revealed several ovoid lesions in cervical segments and tumefactive T2-hyperintense signals with oedema and post-contrast enhancement located in thoracic segments Th3 to Th6. Cerebrospinal fluid (CSF) examination displayed lymphomonocytic pleocytosis with normal proteinorhachia, positive CSF oligoclonal IgG bands (OCB) and elevated IgG index (1.55). Serum anti-AQP4-Ab was not tested. Stored frozen CSF samples were later repeatedly examined with negative findings of anti-AQP4-Ab. Treatment with high-dose methylprednisolon and plasma exchange had limited effect. Immunosuppressive medication was interrupted because of an acute urinary infection. The patient died suddenly because of pulmonary embolism as a secondary complication. Histopathology of the spinal cord confirmed active demyelination. We considered that tumefactive demyelination could be a variant of neuromyelitis optica. Conclusion: Our case could be anti-AQP4-Ab-negative longitudinally extensive transverse myelitis, a variant of neuromyelitis optica.

Tumefactive Demyelinating Lesions in Children: A Rare Case of Conus Medullaris Involvement and Systematic Review of the Literature

“…Given the variegated nature of current nomenclature of intramedullary TDLs, we defined spinal cord tumefactive lesions as non-neoplastic, demyelinating, space-occupying lesions not due to neuromyelitis optica spectrum disorder, MOG antibody-associated disease, or acute disseminated encephalomyelitis, and with a characteristic radiographic appearance strongly suggestive of malignancy. Based on these criteria, 13 cases of solitary spinal cord TDL were found (Table 1 6,7,37,49,55 –61 ). As a group, the male-to-female ratio is 1:1 with a median age at onset of 42 years.…”

“…5 Owing to the scarcity of reported cases with spinal cord involvement, a comprehensive description of clinical and radiographic features with clear consensus nomenclature for tumorlike intramedullary TDL is currently lacking. 6– 8 Because cerebral TDLs have been described as a possible variant of multiple sclerosis and are often reported in this context, we defined spinal cord TDLs as larger than 2 cm, non-neoplastic, demyelinating, space-occupying lesions with a characteristic radiographic appearance strongly suggestive of malignancy not associated with neuromyelitis optica spectrum disorder, MOG antibody-associated disease, or acute disseminated encephalomyelitis.…”

A 25-Year-Old Clinically Isolated Syndrome Patient with Tumefactive Demyelinating Lesion in Cervical Cord: A Case Report and a Mini Review

“…There is not even a clear definition in this case. Reviewing the literature, the author found only five cases of cord involvement with TDL; unfortunately, one of these articles was not available (2)(3)(4)(5)(6). Another case was reported in a paper in Japanese, so only the information in the English abstract was used ( Table 1).…”

Longitudinally extensive transverse myelitis due to toxoplasma: An autopsy study

“…There are only few case reports of tumefactive demyelination affecting the spine, unlike the brain. [ 10 ]…”