title: Progressive solitary sclerosis: Gradual motor impairment from a single CNS demyelinating lesion.
abstract: 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.
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City. keegan.bmark@mayo.edu.
authors.forename: B Mark
authors.initials: BM
authors.lastname: Keegan
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Kaufmann
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: Brian G
authors.initials: BG
authors.lastname: Weinshenker
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: Orhun H
authors.initials: OH
authors.lastname: Kantarci
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: William F
authors.initials: WF
authors.lastname: Schmalstieg
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: M Mateo
authors.initials: MM
authors.lastname: Paz Soldan
authors.affiliation: From the Departments of Neurology (B.M.K., O.H.K., E.P.F.) and Neuroradiology (T.J.K., B.G.W.), Mayo Clinic, Rochester; Department of Neurology (W.F.S.), University of Minnesota, Minneapolis; and Department of Neurology (M.M.P.S.), University of Utah, Salt Lake City.
authors.forename: Eoin P
authors.initials: EP
authors.lastname: Flanagan
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging / Q000187:drug effects / Q000503:physiopathology; D003711:Demyelinating Diseases / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology*; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology*; D011446:Prospective Studies; D011712:Pyramidal Tracts / Q000000981:diagnostic imaging / Q000187:drug effects / Q000503:physiopathology; D012189:Retrospective Studies; D012598:Sclerosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology; D055815:Young Adult