McMaster ALS Conference课件.ppt

* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * Supportive TherapySleeping Rails Hospital bed Wedge cushion Baffle mattress (BiPAP) Be attentive to spousal stress Supportive TherapyBereavement 32 Families surveyed 6 months -7 years Often long lasting effects Family dynamics may be altered No clear relation of coping with elapsed time Supportive Treatments There are very important roles played by OT and PT, SLP, nursing, seating, social work, that we will touch on later. Specific Treatments Specific Therapy There is good evidence to support each of several pathological mechanisms in ALS. It is likely that several processes each contribute to the cell death in ALS. However, it is also likely that we are still missing a key or central piece in the puzzle. Until such time, we will not have a successful treatment. The ALS Mouse The SOD-1 mouse Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, et al. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 1994;264:1772-76 What causes ALS ? Excitotoxicity Oxidative Stress Neurofilament disorder Neurotrophic factor deficiency Immunologic disturbance Other Terminal death may be apoptotic or other Anti-excitotoxicityhuman trials +/- riluzole (Bensimon 1994; Lacomblez 1996) - BCAA (Plaitakis 1988; Italian Group 1993; Tandan 1996) - L-threonine (Testa 1992; Blin 1992) - dextromethorphan (Appelbaum; Ashmark; Blin; Gredal) - gabapentin (Miller 1996) - lamotrigine (Eisen 1993) - verapamil (Miller 1996) - nimodipine (Miller 1996; Ziv 1994) Anti-oxidantshuman studies - Acetylcysteine (Louwerse 1995) - vitamin E (Desnuelle 2001) - Creatine (preliminary) Neurotrophic factorshuman studies - CNTF (CNTF group 1996; Miller 1996) - Growth hormone (Smith 1993) - TSH (Brooke 1986; Caroscio 1986; Mitsumoto 1986) +/- IGF (Lai 1997) - BDNF (BDNF study group 1999) Re