P7-15 The effect of chronic intermittent hypoxia in ALS mice model

Abstract

Patients were diagnosed with IBP if there was no limb progression over six months. Patient demographics, clinical features and neurophysiological biomarkers, including cortical excitability using paired pulse, threshold tracking, transcranial magnetic stimulation, were recorded. IBP patients were compared to more typical bulbar-ALS patients. Results: From a cohort of 250 consecutive referrals, 32 patients with bulbar onset disease (11 males, 21 females, age 64.1±9.0 years) were identified. Twelve patients had IBP (3 male, 9 female, age 61.1±8.2 years) and twenty had bulbar-ALS (8 males, 12 females, age 65.9±9.3 years). IBP was characterized by female predominance and predominantly upper motor neuron bulbar features. In contrast, patients with bulbar-ALS had more prominent lower motor neuron bulbar features. The compound motor action potential amplitude was preserved in patients with IBP compared to patients with bulbar-ALS (7.1mV compared to 4.2mV, P< 0.05) as was the neurophysiological index (1.22 compared to 0.45, P< 0.05). Furthermore, cortical excitability was normal in patients with IBP and abnormal in patients with bulbar-ALS. Conclusions: Patients with IBP were predominantly female with prominent upper motor neuron bulbar features. Neurophysiologically, IBP patients had no evidence of peripheral lower motor neuron dysfunction and had normal cortical excitability. Neurophysiological biomarkers such as SICI may prove useful for patient diagnosis, selection and monitoring in clinical trials.