Progressive solitary sclerosis: Gradual motor impairment from a singleCNSdemyelinating lesion

B. Mark Keegan, Timothy J. Kaufmann, Brian G. Weinshenker, Orhun H. Kantarci, William F. Schmalstieg, M. Mateo Paz Soldan, Eoin P. Flanagan

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Objective: To report patients with progressive motor impairment resulting from an isolated CNS demyelinating lesion in cerebral, brainstem, or spinal cord white matter that we call progressive solitary sclerosis. Methods: Thirty patients were identified with (1) progressive motor impairment for over 1 year with a single radiologically identified CNS demyelinating lesion along corticospinal tracts, (2) absence of other demyelinating CNS lesions, and (3) no history of relapses affecting other CNS pathways. Twenty-five were followed prospectively in our multiple sclerosis (MS) clinic and 5 were identified retrospectively from our progressive MS database. Patients were excluded if an alternative etiology for progressive motor impairment was found. Multiple brain and spinal cord MRI were reviewed by a neuroradiologist blinded to the clinical details. Results: The patients' median age was 48.5 years (range 23-71) and 15 (50%) were women. The median follow-up from symptom onset was 100 months (range 15-343 months). All had insidiously progressive upper motor neuron weakness attributable to the solitary demyelinating lesion found on MRI. Clinical presentations were hemiparesis/monoparesis (n 24), quadriparesis (n 5), and paraparesis (n 1). Solitary MRI lesions involved cervical spinal cord (n 18), cervico-medullary/brainstem region (n 6), thoracic spinal cord (n 4), and subcortical white matter (n 2). CSF abnormalities consistent with MS were found in 13 of 26 (50%). Demyelinating disease was confirmed pathologically in 2 (biopsy, 1; autopsy, 1). Conclusions: Progressive solitary sclerosis results from an isolated CNS demyelinating lesion. Future revisions to MS diagnostic criteria could incorporate this presentation of demyelinating disease.

Original languageEnglish (US)
Pages (from-to)1713-1719
Number of pages7
Issue number16
StatePublished - Oct 18 2016

Bibliographical note

Funding Information:
DISCLOSURE B. Keegan has served as a consultant to Novartis, Bionest, and Bristol Meyers Squibb and has research funded by Terumo BCT. T. Kaufmann is a consultant for SpineThera. B. Weinshenker: DSMB membership MS trials: Novartis (DSMB member for MS trials); Mitsubishi (DSMB member for MS trial); adjudication committee membership re NMO trials: MedImmune Royalties: RSR Ltd. and Oxford University re patent for NMO-IgG as a diagnostic test for NMO and related disorders. O. Kantarci, W. Schmalsteig, M. Paz Soldan, and E. Flanagan report no disclosures relevant to the manuscript. Go to for full disclosures.

Publisher Copyright:
© 2016 American Academy of Neurology.


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