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


CONCLUSIONS
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.
Qiu
W
W
Australian Neuromuscular Research Institute, Sir Charles Gairdner Hospital, Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, 6009, Perth, WA, Australia. kermode@me.com
Wu
J-S
JS
Zhang
M-N
MN
Matsushita
T
T
Kira
J-i
JI
Carroll
W M
WM
Mastaglia
F L
FL
Kermode
A G
AG
D000328:Adult; D051401:Aquaporin 4; D001315:Australia / Q000453:epidemiology; D002391:Catchment Area, Health; D016208:Databases, Factual; D003711:Demyelinating Diseases / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D013116:Spinal Cord / Q000473:pathology; D013118:Spinal Cord Diseases / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D044465:White People / Q000706:statistics & numerical data*
D051401:Aquaporin 4; D007074:Immunoglobulin G