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001 CLINICAL FEATURES AT DISEASE ONSET IN 1000 PATIENTS WITH MULTIPLE SCLEROSIS
C. L. Hirst, T. P. Pickersgill, N. P. Robertson.Department of Neurology, University Hospital of Wales, Cardiff, Wales
Phenotypic variability contributes to difficulties developing patient specific interventions. Analysis of clinical features in early phase disease may allow prediction of disease severity and inform therapeutic decisions for clinicians and patients prior to onset of axonal degeneration.
A prospective clinic based cohort of 1000 patients seen over a 20 year period were identified. All patients met clinical criteria for multiple sclerosis and serial follow up data was available. Sex ratio 1M:2.4F, mean age at onset 31.6 years and mean age at diagnosis was 36.5 years. At presentation 78.8% patients recovered fully from initial relapse, 2.1% had progressive disease, 60.2% patients had symptoms of long tract involvement, 20.5% brainstem, 19.3% cerebellar and 10% optic nerve. Isolated optic neuritis occurred in only 3.2% patients. 174 patients presented with involvement of multiple anatomical sites. Mean time from onset to progression in 20% of patients was 10.65 years. The proportion of patients reaching EDSS 6.0 rose from 17% in those patients with disease duration of 0–5 years to 48% in the 15–20 year group.
These data suggest that primary progressive disease is an unusual presentation and that the therapeutic window for interventions targeted at the inflammatory component of disease closes for 20% of patients within 4 years of diagnosis, and 50% have developed a severe level of irreversible disability 15–20 years after disease onset.
002 THE CLINICAL CHARACTERISTICS AND TREATMENT OF IDIOPATHIC CENTRAL NERVOUS SYSTEM HYPERSOMNOLENCE
K. N. Anderson, I. E. Smith, J. M. Shneerson.Respiratory Support and Sleep Centre, Cambridge, UK
Idiopathic central nervous system (CNS) hypersomnia is defined as a cause of excessive sleepiness consisting of prolonged sleep episodes of non-rapid eye movement sleep. In comparison with narcolepsy, the clinical, polysomnographic, and immunogenetic features are poorly characterised and surprisingly little is known about the clinical course …
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