maternal grandmother and a maternal aunt are unaffected carri- ers. An uncle is blind without MS type symptoms but declined genetic testing. Conclusion: Altough rarely reported, the emergence of multiple- sclerosis-like symptoms in more than one generation has been pre- viously documented in families with LHON. This case demonstrates that multiple sclerosis-like symptoms may be part of the spectrum of LHON. doi:10.1016/j.jocn.2008.07.054 228. Characterisation of the spectrum of demyelinating disease in western australia Jingshan Wu a , Meini Zhang b , William M. Carroll a , Frank L. Mastaglia a , Allan G. Kermode a a Australian Neuromuscular Research Institute, Sir Charles Gairdner Hospital; Perth b First Hospital of Shanxi Medical University, Taiyuan, China The diversity of multiple sclerosis (MS) and the nosology of the conventional form of MS (CMS), optico-spinal MS (OSMS) 1 and neu- romyelitis optica (NMO) have been subject to controversy. This study retrospectively reviewed and analysed 842 cases with CNS demyelinating disease in Western Australia (WA). 2 The patient pop- ulation was predominantly Caucasian, representing approximately two-thirds of MS cases in WA. The study confirmed the relatively low frequency of OSMS as a proportion of total demyelinating dis- ease in WA, with 31 OSMS (3.7%) in contrast to 703 CMS (83.5%). It is likely however that our retrospective classification significantly underestimated the proportion of OSMS when compared to pro- spectively classified Asian cohorts. There were 11 OSMS cases that could also be classified as NMO. The clinical characteristics of OSMS in our study were compatible with so-called Asian MS: oligoclonal bands were less frequent in OSMS (29.4%) than in CMS (66.4%, p = 0.003), long spinal cord lesions (LESCL) were more prevalent in OSMS (22.2%) than in CMS (3.4%, P < 0.001), Brain abnormalities were seen in 48.4% of OSMS patients, and 96.2% of CMS patients (p < 0.001). This cross-sectional study presents the full spectrum of demyelinating disease in WA, and suggests that characteristics of the WA MS population were similar to those reported in Asian patients. References 1. Kira J. Multiple sclerosis in the Japanese population. Lancet Neurology 2003;2(2):117–27. 2. Characterisation of the Spectrum of Demyelinating disease in Western Australia. JS Wu, MN Zhang, WM Carroll, AG Kermode. Journal of Neurology, Neurosurgery and Psychiatry, in press. doi:10.1016/j.jocn.2008.07.055 229. Low Sensitivity of AQP4 IgG in MS, LESCL and NMO in Australians Jingshan Wu a , Takuya Matsushita b , William M. Carroll a , Jun-ichi Kira b , Frank L. Mastaglia a , Allan G. Kermode a a Australian Neuromuscular Research Institute, Sir Charles Gairdner Hospital; Perth b Department of Neurology, Neurological Institute, Graduate School of Medical Science, Kyushu University, Japan, Japan We investigated the prevalence of AQP4 IgG in classical multiple sclerosis (MS), Neuromyelitis optica (NMO) and MS with longitudi- nally extensive spinal cord lesions (LESCL) from a Western Australian MS cohort. There were only two cases (2%) found to be seropositive in a total of 96 samples. One positive case was found among 52 MS patients (1.9%), the other among the 18 LESCL or NMO patients (5.6%). Twenty three PPMS were all seronegative. There were 14 LESCL cases in this MS cohort with one AQP4 IgG positive (7.1%). In our WA MS cohort there were three NMO patients previously NMO-IgG negative (Mayo Clinic), one also AQP4 IgG negative. The other two cases were deceased. Our study showed a very low rate of anti-AQP4 antibody in Caucasian MS patients. Although one posi- tive case was associated with LESCL, the sensitivity of AQP4 IgG was still extremely low in comparison with other studies using the same test. These results emphasise the need for further study on the clini- cal utility of AQP4 serology 1 . We are preparing a comprehensive sero- logical and immunogenetic examination of 900 patients. Reference 1. Chong HT, Kermode AG, Tan CT. The role of anti-aquaporin-4 antibody in Asian patients with multiple sclerosis: Confusions and controversies. Neurology Asia 2003;12:135–9. doi:10.1016/j.jocn.2008.07.056 230. Use of respiratory function tests to predict survival in amyotrophic lateral sclerosis Fusun Baumann a , Nicole M. Hutchinson a , Robert D. Henderson a , Michael G. Brown b , James A. Douglas c , Pamela A. McCombe a a Department of Neurology, Royal Brisbane and Women’s Hospital b Department of Thoracic Medicine, Royal Brisbane and Women’s Hospital c Department of Thoracic Medicine, The Prince Charles Hospital Objective: The purpose of this study was to assess the ability of respiratory function tests (RFTs) to predict survival in ALS patients. Methods: Eighty patients with clinically-definite ALS underwent seated and supine forced vital capacity (FVC). The change in FVC from seated to supine (cFVC) along with maximal inspiratory pres- sure (MIP) and maximal expiratory pressure (MEP) were measured. The rate of decline in FVC was calculated in a subgroup of patients who had a minimum of two sequential RFTs. Probability of survival was calculated using the Kaplan-Meier method. Receiver operating characteristic curves were used in order to identify respiratory parameters that would best predict one-year survival. Results: Seated FVC, supine FVC, rate of decline in FVC, MIP and MEP were significantly associated with survival, whereas cFVC was not. An abnormal supine FVC (<80% predicted) was more sensitive (81%) for death at one year compared to seated FVC (70%). A rapid rate of decline in FVC (>3.6% predicted per month) and diminished MIP or MEP were highly sensitive (100%) for death at one year. Con- versely, a slow rate of decline and a normal MIP or MEP (>70 cmH20) were highly predictive for one-year survival. Rate of decline in FVC was the most highly correlated predictor of one-year survival. Conclusion: Our data support the importance of the rate of change in respiratory muscle function as predictive of survival rather than single measures. Comparison of the change in FVC from seated to supine has no advantage over supine measures alone. doi:10.1016/j.jocn.2008.07.057 478 Abstracts / Journal of Clinical Neuroscience 16 (2009) 462–481