Matthias Spranger

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Late-onset glycogen storage disease type 2 (GSD2)/Pompe disease is a progressive multi-system disease evoked by a deficiency of lysosomal acid α-glucosidase (GAA) activity. GSD2 is characterized by respiratory and skeletal muscle weakness and atrophy, resulting in functional disability and reduced life span. Since 2006 alglucosidase alfa has been licensed(More)
Nach Abschluss der Akutversorgung bei schweren neurologischen Erkrankungen werden Patienten in Deutschland in der sog. Phase B frühzeitig rehabilitiert. Über den Langzeitverlauf dieser Patienten existieren bisher keine Studien. Patienten der Phase B aus 9 Zentren wurden im Jahr 2002 prospektiv erfasst und nach 5 und 6 Jahren nachuntersucht. Als(More)
Glycogen storage disease type 2(GSD2)/Pompe disease is characterized by respiratory and skeletal muscle weakness and atrophy, resulting in functional disability and reduced life span. We present an open-label, investigator-initiated observational study of alglucosidase alfa enzyme replacement therapy (ERT) in 38 adult-onset GSD2 patients (20 female, 18(More)
Die myotone Muskeldystrophie ist eine autosomal dominante Multisystemerkrankung, die Muskulatur, Gehirn, Herz, Augen, Gastrointestinaltrakt und endokrine Organe betreffen kann. Die zugrundeliegende Genmutation ist eine Vermehrung von CTG-Triplets in der untranslatierten 3’Region eines Serin-Threonin-Kinase-Gens auf Chromosom 19q. Die Anzahl der CTG-Kopien(More)
1. Petito CK, Hart MN, Porro RS, Earle KM (11973) Ultrastructural studies of olivo-ponto-cerebellar atrophy. J Neuropathol Exp Neurol 32:503-522 2.PL Schiffman, Golbe LI (1992) Upper airway dysfunction in olivopontocerebellar atrophy. Chest 102:1291-1292 3. Longridge NS (1987) Bilateral cord paralysis in Shy-Drager syndrome. J Otolaryngol 16:146-148 4.(More)
Obwohl die kontinuierliche intrakranielle Hirndruckmessung (ICP-Monitoring) bei der intensivmedizinischen Behandlung zerebraler Ischämien immer häufiger angewandt wird, ist der Nutzen des Verfahrens bei diesem Krankheitsbild noch nicht etabliert. Der klinische Verlauf bei 48 Patienten mit den Zeichen erhöhten intrakraniellen Druckes aufgrund eines großen(More)
FOS and JUN proteins are transcription factors thought to be involved in coupling neuronal excitation to target gene expression. Cortical infarction of consistent size and location was produced by irradiating the rat brain with Xenon light through the intact skull for 20 min following systemic injection of the photo-sensitizing dye, rose bengal. To(More)
Inducible nitric oxide synthase (iNOS)-derived NO plays an important role in several neurological disorders. Understanding of mechanisms involved in the regulation of iNOS induction is of particular interest. Here, we investigated mechanisms of iNOS induction in rat astrocytes (AC) and in brain endothelial cells (BEC). We find that activation of AC or BEC(More)
Polyneuropathy (PNP) and aging both bring changes to the walking pattern of elderly people. However, the identification methods of PNP from gait patterns were not sufficiently investigated from a technical perspective. In this study an automated classification method was developed to discriminate the neuropathic gait from both young healthy and old healthy(More)
In this paper, a novel method for monitoring the changes in gait joint angle trajectories recorded using the low-cost and wearable Inertial Measurement Units (IMU) is presented. The introduced method is based on Dynamic Time Warping (DTW), an algorithm commonly used for evaluating the similarity of two time series which may vary in time and speed. DTW is(More)