Safety and Tolerability of Anakinra in Combination With Riluzol in Amyotrophic Lateral Sclerosis

Inclusion Criteria: * Patients between 18 and 80 years of age * Clinical diagnosis of amyotrophic lateral sclerosis with predominant affection of the lower motor neuron or the clinical ALS variant of progressive muscular atrophy (PMA) * Clinical signs of lower motor neuron degeneration in at least one anatomic region beyond the brain stem * Sporadic and familial ALS * Onset of paresis six months to four years before study inclusion * Treatment with riluzol 100mg/d at least 1 month before study inclusion Exclusion Criteria: * Diagnosis of amyotrophic lateral sclerosis with predominant affection or the upper motor neuron without clinical signs of a concurrent affection of the lower motor neuron in at least one anatomic region beyond the brain stem (spastic ALS) - Diagnosis of primary lateral sclerosis (PLS) * Patients with known intolerance to anakinra, riluzol or one of the additives * Clinically severe hypoventilation syndrome with vital capacity \< 50% * Pregnancy or breastfeeding * Continuous non-invasive ventilation with ventilator-free time \< 2 hours - Tracheotomy and mechanical ventilation * Laboratory parameters outside the normal range that correspond to a clinically severe cardiovascular, pulmological, hematological, hepatological, metabolic or renal disease * Malignancies * Severe renal insufficiency (creatinine clearance \< 30 ml/min) * History of recurrent infections or a disease that may predispose to infections * Severe neutropenia (absolute neutrophil count \< 1.5 x 109/l) * Monoclonal gammopathy of unknown significance * Infections including infections with HIV and hepatitis B and C * Dementia and unable to give informed consent * History of epilepsy and epileptic seizures * Contraindication to E coli-derived proteins, anakinra or any components of the product * Concurrent therapy of anakinra and etanercept or other TNF blocking agents