Amyotrophic Lateral Sclerosis

An International Conference on "Therapeutic. Psychological and Research Aspects of Amyotrophic Lateral Sclerosis" was held in Varese. Italy from the 27th to the 31st March 1985. Health care professionals. scientists. patients and their families from twenty countries around the world participated in this meeting. The objectives of the Varese conference were the following: a. To provide a forum for the proponents of the various paths of research into ALS. b. To correlate the useful therapies employed regionally. for the purpose of developing a common guide for patients, families, and supporting professionals. c. To encourage self-examination by the health care professionals into the psychological barriers imposed by a diagnosis of terminal illness for which there is no known cause or cure. Not surprisingly, there were no announcements of "breakthroughs" or "miracle cures", which are nonetheless hoped for in the confrontation of a disease such as ALS. It is fair to say, however, that Varese provided the context for a thorough review of what is known about ALS and we hope that the papers will renew some of the enthusiasm which has characterized this conference. The book contains six sections: Basic Research Aspects, Diagnostic Tools, Clinical Management, Therapeutic Trials, Psychological Aspects and the Epidemiology of ALS. Certain aspects, such as pathological studies and animal models, have not been covered; these subjects were partially treated during the informal sessions. Nevertheless, the large number of papers bears evidence to the growing interest in ALS and to the success of the Varese meeting.

SECTION 1 BASIC RESEARCH IN ALS. - Deficient Repair of Alkylation Damage of DNA in Alzheimer s Disease and Amyotrophic Lateral Sclerosis Cells. - Immunoglobulin-Mediated Cytotoxic Effect of ALS-Plasma Towards Erythrocytes: Reflexion of a Pathogenetic Mechanism? . - Differentiation of Human Muscle Cells in the Presence of Neuronal Conditioned Medium (NCM), Nerve Growth Factor (NGF) and Spinal Cord Cells (SC). - GM2 Gangliosidosis with a Motor Neuron Disease Phenotype: Clinical Heterogeneity of Hexosaminidase Deficiency Disease. - Proteases, Their Inhibitors and the Extracellular Matrix: Factors in Nerve-Muscle Development and Maintenance. - The Role of Axoplasmic Transport in the Restoration of Synaptic Transmission and in the Process of Sprouting During Nerve Regeneration. - Purification of a Survival Factor for Spinal Neurons from Mouse Salivary Gland. - Neurite-Promoting Factors for Spinal Neurons: Their Possible Importance for the Understanding of Amyotrophic Lateral Sclerosis and the Spinal Muscular Atrophies. - Human Spinal Cord Neurons in Culture as a Tool to Study Amyotrophic Lateral Sclerosis. - Autoimmune Involvement in Motor Neurone Disease. - Cytotoxicity of Serum from Amyotrophic Lateral Sclerosis Patients on Spinal Cord Cells in Culture. - ALS Cerebrospinal Fluid Enhances Human Foetal Astroglial Cell Proliferation in Vitro. - The Electromyographic, Physiopathogenetic Evaluation of Amyotrophic Lateral Sclerosis. - The Pathogenetic Role of Metals in Motor Neuron Disease -The Participation of Aluminum. - SECTION 2 DIAGNOSTIC TOOLS. - The Syndromic Nature of Amyotrophic Lateral Sclerosis. - Familial ALS: Clinical, Genetic and Morphological Features. - Light and Ultrastructural Studies in Sural Biopsies of the Pseudopolyneuropathic Form of ALS. - Prosody Alterations inAmyotrophic Lateral Sclerosis. - Analysis of Extrinsic Factors Affecting Pallestesic Threshold (vt) of Amyotrophic Lateral Sclerosis Patients. - Spinal Cord Electrophysiological Activities After Small Doses of TRH in Control Subjects and ALS Patients. - Osmotic Haemolysis in Amyotrophic Lateral Sclerosis. - Some Remarks on Lung Function in Amyotrophic Lateral Sclerosis. - CT-Scanning of Skeletal Muscles in Amyotrophic Lateral Sclerosis. - Immunological Assessment in Sporadic Amyotrophic Lateral Sclerosis (ALS). - Changes in the Normal Pattern of H-Reflex Inhibition during Muscle Release in ALS. - Atypical Forms of Familial Amyotrophic Lateral Sclerosis. - SECTION 3 CLINICAL MANAGEMENT OF ALS. - The Management of Motor Neurone Disease. - The Treatment of Amyotrophic Lateral Sclerosis. - Nutritional Supplements in Amyotrophic Lateral Sclerosis. - Respiratory Monitoring and Treatment: Objective Treatments Using Non-Invasive Measurements. - Management of Bulbar Symptoms in Amyotrophic Lateral Sclerosis. - Clinico-Pathological Studies of Long Survival ALS Cases Maintained by Active Life-Support Measures. - First Results with Percutaneous Endoscopic Gastrostomy. - Home Nursing Care by Families for Severely Paralyzed ALS Patients. - Physical Therapy and Rehabilitation Techniques for Patients with Amyotrophic Lateral Sclerosis. - SECTION 4 THERAPEUTIC TRIALS. - A Study of Prognostic Factors in Motor Neuron Disease. - Therapeutic Trials in ALS - The Design of a Protocol. - Attempted Treatment of Motor Neuron Disease with N-Acetylcysteine and Dithiothreitol. - Therapeutic Trial with N-Acetylcysteine in Amyotrophic Lateral Sclerosis. - A Follow-up Electromyographic Investigation of ALS Patients Treated with High Dosage Gangliosides. - A Therapeutic Trial of a Thymic Factor in Amyotrophic Lateral Sclerosis (ALS). - Antiviral Therapy. - Therapeutic Trial of Intrathecal Thyrotropin-Releasing Hormone (TRH) and a TRH-Analogue in Amyotrophic Lateral Sclerosis (ALS). - SECTION 5 PSYCHOLOGICAL ASPECTS OF ALS. - Psychological Preparation of the Physician for ALS Patients. - Psychodynamic Aspects in Patients with Amyotrophic Lateral Sclerosis (ALS). - Severity Stages of ALS and Psychological Management. - On Behalf of the Patient. - SECTION 6 EPIDEMIOLOGY OF ALS. - Motor Neuron Disease: Epidemiologic Studies. - Epidemiology of Motor Neuron Disease in North-East Veneto Region: Venice, Padua, and Belluno Provinces (1972-1981). - Amyotrophic Lateral Sclerosis in Limousin (Limoges Area-France). - Amyotrophic Lateral Sclerosis in Finland. - Amyotrophic Lateral Sclerosis in Nova Scotia.