Objectives: Liquorpheresis (CSF ®ltration) has been shown to be of bene®t in various neurologic disorders, including sporadic ALS. Liquorpheresis in patients with familial ALS, has not been investigated so far. Methods: A 52-year-old woman with familial ALS is reported who underwent liquorpheresis during 4 consecutive days. During this period, 875 ml CSF were ®ltered o via an intrathecal catheter and a combined mechanical and ionic ®lter by means of a bi-directional syringe pump. Results: Immediately after treatment and 2 weeks later there was subjective, but no objective, improvement of her symptoms, assessed by the Norris score and measurements of the elbow extension, vital capacity, F-waves of both ulnar nerves and automatic EMG of the right brachial biceps and anterior tibial muscles. Conclusion: Liquorpheresis does not seem to be helpful in the treatment of familial ALS.