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 Flanagan

Research output: Contribution to journalArticle

28 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1713-1719
Number of pages7
JournalNeurology
Volume87
Issue number16
DOIs
StatePublished - Oct 18 2016

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Sclerosis
Multiple Sclerosis
Spinal Cord
Demyelinating Diseases
Paresis
Brain Stem
Paraparesis
Pyramidal Tracts
Quadriplegia
Motor Neurons
Autopsy
Thorax
Databases
Biopsy
Recurrence
Brain
White Matter

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Progressive solitary sclerosis : Gradual motor impairment from a singleCNSdemyelinating lesion. / Keegan, B Mark; Kaufmann, Timothy J; Weinshenker, Brian G; Kantarci, Orhun H; Schmalstieg, William F.; Paz Soldan, M. Mateo; Flanagan, Eoin.

In: Neurology, Vol. 87, No. 16, 18.10.2016, p. 1713-1719.

Research output: Contribution to journalArticle

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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.",
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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.

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