Multiple sclerosis, solitary sclerosis or something else?

Background: Inflammatory demyelinating diseases of the central nervous system represent a wide spectrum of entities and their classification cannot currently be regarded complete. Objective: Our aim is to describe a series of patients presenting with progressive myelopathy associated to a single dem...

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Bibliographic Details
Published in:Multiple sclerosis 2014-12, Vol.20 (14), p.1819-1824
Main Authors: Lattanzi, Simona, Logullo, Francesco, Di Bella, Paolo, Silvestrini, Mauro, Provinciali, Leandro
Format: Article
Language:English
Subjects:
Adult
Aged
Cohort Studies
Demyelinating Autoimmune Diseases, CNS - pathology
Demyelinating Autoimmune Diseases, CNS - physiopathology
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Multiple Sclerosis - pathology
Multiple Sclerosis - physiopathology
Myelitis, Transverse - pathology
Myelitis, Transverse - physiopathology
Spinal Cord - pathology
Spinal Cord - physiopathology
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Summary:Background: Inflammatory demyelinating diseases of the central nervous system represent a wide spectrum of entities and their classification cannot currently be regarded complete. Objective: Our aim is to describe a series of patients presenting with progressive myelopathy associated to a single demyelinating lesion of the spinal cord. Methods: We identified the patients affected by chronic progressive spinal cord dysfunction related to a single spinal cord lesion not satisfying the diagnostic criteria for any of the currently defined diseases. Results: Seven females and one male were included. The median age at onset of symptoms was 53 years (range 42–68) and the median follow-up was 8 years (range 5–12). Brain and spinal magnetic resonance imaging (MRI) scans detected only one single, circumscribed, T2 hyperintense, non-longitudinally extensive lesion at level of cervico-medullary junction or cervical cord, in the absence of Gadolinium enhancement or swelling. Cerebrospinal fluid (CSF) examination displayed neither oligoclonal bands nor raised IgG index. A response to immunosuppressive agents was observed in some of the patients. Serial control brain and spinal MRI did not reveal accumulation of new lesions. Conclusion: New entities or variants should be included among the inflammatory demyelinating diseases of the central nervous system, and their characterization may have relevant prognostic and treatment implications.
ISSN:1352-4585
1477-0970
DOI:10.1177/1352458514535129