Solitary sclerosis

Progressive myelopathy from solitary demyelinating lesion

William F. Schmalstieg, B Mark Keegan, Brian G Weinshenker

Research output: Contribution to journalArticle

30 Citations (Scopus)

Abstract

Objective: To present a case series of patients with progressive myelopathy in the setting of a solitary demyelinating lesion. Methods: We describe 7 patients evaluated over a 6-year period. All had progressive motor impairment attributable to an MRI lesion compatible with a demyelinating plaque in the brainstem or upper cervical spinal cord. At the time of evaluation, none met the International Panel imaging criteria for dissemination in space, and none described clinical symptoms consistent with relapses affecting other portions of the CNS. Results: Lesions identified were in the ventral cervicomedullary junction in 4 patients, the ventral spinal cord in 2 patients, and the pons in 1 patient. Median age at onset was 43 years (range 33-51 years). Median follow-up interval was 3 years (range 2-27 years). Six patients reached an Expanded Disability Status Scale (EDSS) score of 6.0 or worse. Median time to EDSS score of 6.0 was 7.5 years (range 1.5-26 years). Four had CSF findings characteristic of multiple sclerosis (MS). None had CSF, imaging, or serologic evidence of an alternative etiology of progressive myelopathy. In 3 patients, serial MRI scans of the brain and spinal cord demonstrated no accumulation of lesions. Postmortem examination of a fourth patient demonstrated an isolated pontine demyelinating lesion. Conclusions: Solitary demyelinating lesions may produce a progressive myelopathy similar to primary progressive MS. Demyelinating disease should be in the differential diagnosis of progressive myelopathy despite absence of dissemination in space.

Original languageEnglish (US)
Pages (from-to)540-544
Number of pages5
JournalNeurology
Volume78
Issue number8
DOIs
StatePublished - Feb 21 2012

Fingerprint

Spinal Cord Diseases
Sclerosis
Spinal Cord
Chronic Progressive Multiple Sclerosis
Pons
Lesion
Demyelinating Diseases
Age of Onset
Brain Stem
Multiple Sclerosis
Autopsy
Differential Diagnosis
Magnetic Resonance Imaging
Recurrence
Brain

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Solitary sclerosis : Progressive myelopathy from solitary demyelinating lesion. / Schmalstieg, William F.; Keegan, B Mark; Weinshenker, Brian G.

In: Neurology, Vol. 78, No. 8, 21.02.2012, p. 540-544.

Research output: Contribution to journalArticle

@article{726b141827b644029000dd8dbd917177,
title = "Solitary sclerosis: Progressive myelopathy from solitary demyelinating lesion",
abstract = "Objective: To present a case series of patients with progressive myelopathy in the setting of a solitary demyelinating lesion. Methods: We describe 7 patients evaluated over a 6-year period. All had progressive motor impairment attributable to an MRI lesion compatible with a demyelinating plaque in the brainstem or upper cervical spinal cord. At the time of evaluation, none met the International Panel imaging criteria for dissemination in space, and none described clinical symptoms consistent with relapses affecting other portions of the CNS. Results: Lesions identified were in the ventral cervicomedullary junction in 4 patients, the ventral spinal cord in 2 patients, and the pons in 1 patient. Median age at onset was 43 years (range 33-51 years). Median follow-up interval was 3 years (range 2-27 years). Six patients reached an Expanded Disability Status Scale (EDSS) score of 6.0 or worse. Median time to EDSS score of 6.0 was 7.5 years (range 1.5-26 years). Four had CSF findings characteristic of multiple sclerosis (MS). None had CSF, imaging, or serologic evidence of an alternative etiology of progressive myelopathy. In 3 patients, serial MRI scans of the brain and spinal cord demonstrated no accumulation of lesions. Postmortem examination of a fourth patient demonstrated an isolated pontine demyelinating lesion. Conclusions: Solitary demyelinating lesions may produce a progressive myelopathy similar to primary progressive MS. Demyelinating disease should be in the differential diagnosis of progressive myelopathy despite absence of dissemination in space.",
author = "Schmalstieg, {William F.} and Keegan, {B Mark} and Weinshenker, {Brian G}",
year = "2012",
month = "2",
day = "21",
doi = "10.1212/WNL.0b013e318247cc8c",
language = "English (US)",
volume = "78",
pages = "540--544",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "8",

}

TY - JOUR

T1 - Solitary sclerosis

T2 - Progressive myelopathy from solitary demyelinating lesion

AU - Schmalstieg, William F.

AU - Keegan, B Mark

AU - Weinshenker, Brian G

PY - 2012/2/21

Y1 - 2012/2/21

N2 - Objective: To present a case series of patients with progressive myelopathy in the setting of a solitary demyelinating lesion. Methods: We describe 7 patients evaluated over a 6-year period. All had progressive motor impairment attributable to an MRI lesion compatible with a demyelinating plaque in the brainstem or upper cervical spinal cord. At the time of evaluation, none met the International Panel imaging criteria for dissemination in space, and none described clinical symptoms consistent with relapses affecting other portions of the CNS. Results: Lesions identified were in the ventral cervicomedullary junction in 4 patients, the ventral spinal cord in 2 patients, and the pons in 1 patient. Median age at onset was 43 years (range 33-51 years). Median follow-up interval was 3 years (range 2-27 years). Six patients reached an Expanded Disability Status Scale (EDSS) score of 6.0 or worse. Median time to EDSS score of 6.0 was 7.5 years (range 1.5-26 years). Four had CSF findings characteristic of multiple sclerosis (MS). None had CSF, imaging, or serologic evidence of an alternative etiology of progressive myelopathy. In 3 patients, serial MRI scans of the brain and spinal cord demonstrated no accumulation of lesions. Postmortem examination of a fourth patient demonstrated an isolated pontine demyelinating lesion. Conclusions: Solitary demyelinating lesions may produce a progressive myelopathy similar to primary progressive MS. Demyelinating disease should be in the differential diagnosis of progressive myelopathy despite absence of dissemination in space.

AB - Objective: To present a case series of patients with progressive myelopathy in the setting of a solitary demyelinating lesion. Methods: We describe 7 patients evaluated over a 6-year period. All had progressive motor impairment attributable to an MRI lesion compatible with a demyelinating plaque in the brainstem or upper cervical spinal cord. At the time of evaluation, none met the International Panel imaging criteria for dissemination in space, and none described clinical symptoms consistent with relapses affecting other portions of the CNS. Results: Lesions identified were in the ventral cervicomedullary junction in 4 patients, the ventral spinal cord in 2 patients, and the pons in 1 patient. Median age at onset was 43 years (range 33-51 years). Median follow-up interval was 3 years (range 2-27 years). Six patients reached an Expanded Disability Status Scale (EDSS) score of 6.0 or worse. Median time to EDSS score of 6.0 was 7.5 years (range 1.5-26 years). Four had CSF findings characteristic of multiple sclerosis (MS). None had CSF, imaging, or serologic evidence of an alternative etiology of progressive myelopathy. In 3 patients, serial MRI scans of the brain and spinal cord demonstrated no accumulation of lesions. Postmortem examination of a fourth patient demonstrated an isolated pontine demyelinating lesion. Conclusions: Solitary demyelinating lesions may produce a progressive myelopathy similar to primary progressive MS. Demyelinating disease should be in the differential diagnosis of progressive myelopathy despite absence of dissemination in space.

UR - http://www.scopus.com/inward/record.url?scp=84858122827&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84858122827&partnerID=8YFLogxK

U2 - 10.1212/WNL.0b013e318247cc8c

DO - 10.1212/WNL.0b013e318247cc8c

M3 - Article

VL - 78

SP - 540

EP - 544

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 8

ER -