The new network will improve access to rapid diagnosis and care for patients with OADs and UCDs. E-IMD will help make this happen by 1/ objectively evaluating management strategies and outcome of patients (patient registry, www.e-imd.ukhd.de), 2/ providing evidence-based diagnostic and management protocols, and 3/ empowering patients and patient organisations by providing up-to-date information in their own language (website, www.eimd.net). OTC deficiency, first described in 1962, was recognised initially because the child's urine stank of ammonia and characteristically vomited repeatedly failing to gain weight when her diet was changed from breast milk to an artificial formula with a higher protein intake in the first few weeks of life. This leads to dehydration and a low c.s.f. pressure. Over a period of time brain size is limited with premature closure of the fontanelles and the child is irritable and screams. The urine contains fine needles of orotic acid. Hypertonia leads to opisthotonus as illustrated.