Zusammenfassung
Die neurogene Muskelatrophie (Denervationsatrophie) ist die am häufigsten an Muskelbiopsien gestellte Diagnose. Sie ist durch spezifische histologische Veränderungen charakterisiert, die eine Abgrenzung von den differenzialdiagnostisch wichtigen primären Myopathien, darunter den Muskeldystrophien, und den entzündlichen Muskelerkrankungen erlauben. Innerhalb der Gruppe der Denervationsatrophien zeigen die Motoneuronerkrankungen besonders charakteristische Atrophiemuster. Die Diagnose einer neurogenen Muskelatrophie am Biopsiematerial erfordert in der Regel den Einsatz spezieller Methoden der Enzym- und Immunhistochemie, aber auch die Semidünnschnitttechnik und ggf. die Elektronenmikroskopie. Mit einer kombinierten Muskel-Nerv-Biopsie können zudem Ausmaß und Progredienz der neuronalen Degeneration im motorischen und sensorischen System verglichen werden. Durch die enzym- und immunhistochemische Muskelfasertypenfärbung können außerdem selektive oder bevorzugte Atrophien der langsamen Typ-1- und der schnellen Typ-2-Muskelfasern erkannt werden, die differenzialdiagnostisch relevant sind.
Abstract
Neurogenic muscular atrophy (NMA) is the most frequent diagnosis obtained from reading a muscle biopsy. It is characterized by specific histological changes which distinguish NMA from other important muscle pathologies including the primary myopathies such as the muscular dystrophies as well as the inflammatory muscle disorders. Within the group of denervation atrophies, NMAs due to motor neuron diseases are associated with particular histological patterns. The diagnosis of NMA in muscle biopsies requires special methods, mainly enzyme and immunohistochemistry, but also resin histology and in some cases electron microscopy. Analysis of a combined muscle and sural nerve biopsy provides the opportunity to compare the extent of degeneration in the motor and sensory systems, respectively. Muscle fiber typing by enzyme and immunohistochemistry also leads to the detection of selective type 1 and type 2 muscle fiber atrophies which are relevant in the differential diagnosis of neuromuscular diseases.
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Weis, J., Nikolin, S. & Nolte, K. Neurogene Muskelatrophien und selektive Muskelfasertypenatrophien. Pathologe 30, 379–383 (2009). https://doi.org/10.1007/s00292-009-1171-y
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DOI: https://doi.org/10.1007/s00292-009-1171-y