TY - JOUR
T1 - Progressive solitary sclerosis
T2 - Gradual motor impairment from a singleCNSdemyelinating lesion
AU - Keegan, B. Mark
AU - Kaufmann, Timothy J.
AU - Weinshenker, Brian G.
AU - Kantarci, Orhun H.
AU - Schmalstieg, William F.
AU - Paz Soldan, M. Mateo
AU - Flanagan, Eoin P.
N1 - Publisher Copyright:
© 2016 American Academy of Neurology.
PY - 2016/10/18
Y1 - 2016/10/18
N2 - 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.
AB - 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.
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U2 - 10.1212/WNL.0000000000003235
DO - 10.1212/WNL.0000000000003235
M3 - Article
C2 - 27638926
AN - SCOPUS:84991741336
SN - 0028-3878
VL - 87
SP - 1713
EP - 1719
JO - Neurology
JF - Neurology
IS - 16
ER -