Longitudinally extensive myelopathy in Caucasians: A West Australian study of 26 cases from the Perth Demyelinating Diseases Database

W. Qiu; J. S. Wu; M. N. Zhang; T. Matsushita; J. I. Kira; W. M. Carroll; F. L. Mastaglia; A. G. Kermode

doi:10.1136/jnnp.2009.172973

Longitudinally extensive myelopathy in Caucasians: A West Australian study of 26 cases from the Perth Demyelinating Diseases Database

W. Qiu, J. S. Wu, M. N. Zhang, T. Matsushita, J. I. Kira, W. M. Carroll, F. L. Mastaglia, A. G. Kermode

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Abstract

Objectives: To characterise West Australian cases of longitudinally extensive myelopathy (LEM). Methods: Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed. Results: LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4-C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG. Conclusion LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.

Original language	English
Pages (from-to)	209-212
Number of pages	4
Journal	Journal of Neurology, Neurosurgery and Psychiatry
Volume	81
Issue number	2
DOIs	https://doi.org/10.1136/jnnp.2009.172973
Publication status	Published - Feb 2010

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Surgery
Clinical Neurology
Psychiatry and Mental health

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10.1136/jnnp.2009.172973

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title = "Longitudinally extensive myelopathy in Caucasians: A West Australian study of 26 cases from the Perth Demyelinating Diseases Database",

abstract = "Objectives: To characterise West Australian cases of longitudinally extensive myelopathy (LEM). Methods: Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed. Results: LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4-C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG. Conclusion LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.",

author = "W. Qiu and Wu, {J. S.} and Zhang, {M. N.} and T. Matsushita and Kira, {J. I.} and Carroll, {W. M.} and Mastaglia, {F. L.} and Kermode, {A. G.}",

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doi = "10.1136/jnnp.2009.172973",

language = "English",

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T1 - Longitudinally extensive myelopathy in Caucasians

T2 - A West Australian study of 26 cases from the Perth Demyelinating Diseases Database

AU - Qiu, W.

AU - Wu, J. S.

AU - Zhang, M. N.

AU - Matsushita, T.

AU - Kira, J. I.

AU - Carroll, W. M.

AU - Mastaglia, F. L.

AU - Kermode, A. G.

PY - 2010/2

Y1 - 2010/2

N2 - Objectives: To characterise West Australian cases of longitudinally extensive myelopathy (LEM). Methods: Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed. Results: LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4-C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG. Conclusion LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.

AB - Objectives: To characterise West Australian cases of longitudinally extensive myelopathy (LEM). Methods: Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed. Results: LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4-C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG. Conclusion LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.

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Longitudinally extensive myelopathy in Caucasians: A West Australian study of 26 cases from the Perth Demyelinating Diseases Database

Abstract

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