Bernard Hoffmann

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Through a survey of all departments of pediatrics, neurology and neuropathology in Germany, we calculated the incidence of all major forms of leukodystrophy. Only diagnoses based on specific biochemical tests in association with typical findings and/or neuroradiologically proven white matter involvement were accepted. In accordance with these strict(More)
Maxillonasal dysplasia or Binder's syndrome is an uncommon though easily recognizable congenital condition characterized by a retruded mid-face with an extremely flat nose. The facial deficiencies lead to functional as well as psychological problems. We report on 10 patients with maxillonasal dysplasia whose noses were corrected with onlay costal cartilage(More)
The long term courses of patients after traumatic brain injury (TBI) are particularly influenced by the quality of neuropsychological rehabilitation and social reintegration. Though we do have data from different European countries about the success of surgery and intensive care, we don't know much about the long term courses, mirrored by the patients,(More)
Hypertrophic cardiomyopathy (HCM) is a frequent, autosomal-dominant cardiac disease and manifests predominantly as left ventricular hypertrophy. Mutations in the cardiac beta-myosin heavy chain gene (MYH7) are responsible for the disease in about 30% of cases where mutations were identified. We clinically evaluated a large group of 147 consecutive HCM(More)
Guidelines for the treatment of traumatic brain injury (TBI) are subject of actual efforts in neurosurgery, anaesthesiology and traumatology as well as in the basic sciences. International collaborations such as European Brain Injury Consortium (EBIC), Pan-European-Advisory Board (PAB) and Euroacademy of Multidisciplinary Neurotraumatology (EMN) are working(More)
Early rehabilitation after traumatic brain injury has become a worldwide accepted interface between intensive care medicine and rehabilitation to aim for a better functional outcome of the surviving patients. So each chain can only be as strong as its weakest link, and there is still need for well defined quality standards depending on the medical demands(More)
Traumatic loss of bone substance or post - decompression defects require the reconstruction of the skull. In cases of simple geometry there are handy, secure and cost effective procedures such as using autologuous cryopreserved bone flaps or polymerized Methylmethacrylat. For large sized defects CAD - taylored implants developed to provide a comfortable(More)
Die klassische Galaktosämie aufgrund eines Galaktose-1-Phosphat-Uridyltransferase-Mangels ist eine angeborene Stoffwechselstörung mit einer Inzidenz von etwa 1:40.000 in der kaukasischen Bevölkerung. In der Neugeborenenperiode imponiert sie zunächst als Hepatopathie mit zerebraler Beteiligung. Unbehandelt führt sie zu Katarakt, Leberinsuffizienz,(More)
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