P195: The value of EEG in the neurophysiological diagnosis of the Creutzfeldt-Jakob disease. Our iconographic contribution on variable genotypes

Abstract

Question: To investigate the features of sympathetic skin response in patients with amyotrophic lateral sclerosis (ALS) and determine a possible involvement of the autonomic nervous system in ALS. Methods: The data of outpatients and inpatients with definite ALS were collected in Peking Union Medical College Hospital from Jun, 2009 to Jun, 2012.Standard sensory and motor nerve conduction was performed in median nerve, ulnar nerve and tibial nerve in 95 patients with ALS. F-wave and sympathetic skin response were measured in median nerve. Parameters analyses mainly included sensory conduction velocity and amplitude, distal motor latency (DML), compound muscle action potential (CMAP) amplitude, SSR latency and amplitude. Findings were compared with those in 50 normal controls. Results: The SSR was present in 100% of the controls but was absent in 5.3% in the hands and12.6% in the feet in ALS patients. The mean amplitudes of the ALS patients (palmar SSR: 1.26±0.78 mv; plantar SSR: 0.76±0.66 mv) were lower than that of the controls (palmar SSR: 3.31±2.15 mv; plantar SSR: 1.73±0.98 mv) (P 0.05). The abnormality rate of SSR was 35.8% in 95 ALS patients, 13.5% in the hands, 35.8% in the legs. The abnormality of the plantar SSR is higher than that of the palmar SSR (P<0.01).The abnormality of SSR includes prolonged latencies, decreased amplitudes and wave absence. In limb-onset group the abnormality of SSR were increased. Logisitic regression revealed a strong direct association between onset site and SSR abnormality. SSR latency and amplitude are not correlated to either the duration of the disease or the age of the patients. Conclusions: An abnormal SSR can been observed in ALS. The autonomic nervous system in ALS patients may be early detected by the SSR.