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Title of Journal: J Neurol

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Abbravation: Journal of Neurology

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Springer-Verlag

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DOI

10.1007/s11883-009-0029-3

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1432-1459

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Progressive ascending myelopathy atypical forms o

Authors: Viviana Nociti Anna Paola Batocchi Marco Luigetti Amelia Conte Vita Santa Lorusso Silvia Roiati Tommaso Tartaglione Alessandra Del Grande Mario Sabatelli
Publish Date: 2011/04/22
Volume: 258, Issue: 11, Pages: 1965-1970
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Abstract

The spinal cord can be affected by multiple heterogeneous disorders often difficult to diagnose We describe ten patients affected by a progressive ascending myelopathy with a poor prognosis The patients during the followup period underwent neurological examinations cerebrospinal fluid analysis hematological microbiological autoantibodies screening brain and spinal cord magnetic resonance imaging MRI and electroneurophysiological study At disease onset spinal cord MRI showed ≥1 myelopathic lesions extended for 2 segments and then evidenced a progressive spinal cord atrophy without any new lesion All patients showed an increase of the visual evoked potential P100 latency All of them showed two or more clinical recurrences of myelitis and then after a period ranging from 3 to 5 years from the disease onset a progressive course Five patients became unresponsive to intravenous highdose steroid treatments and/or intravenous immunoglobulins and to any other therapeutic attempts developed a progressive ascending myelopathy to tetraplegia and died from respiratory failure The other five patients are in progressive phase of the disease with an initial involvement of the upper limbs and show mild cervical spinal cord atrophy at MRI configuring the early stage of an ascending progressive myelopathy In our opinion the more suitable diagnosis is an atypical form of MS although is not possible to exclude a new nosological entity that could be included in the expanding range of spinal cord diseases

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