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Angeborene und endogene endokrine Myopathien

Congenital and endogenous endocrine myopathy

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Zusammenfassung

Eine klinisch manifeste Myopathie stellt eine seltene Manifestationsform einer erworbenen oder endokrinen hormonellen Dysbalance dar. Daher erfordert diese vom Kliniker einen Brückenschlag zwischen Neurologie und Endokrinologie. Asymptomatische oder Forme-fruste-Varianten sind sicher häufiger und ggf. auch unterdiagnostiziert. Oftmals ist das muskuläre Symptom das Leitsymptom, das zur Diagnose einer oft schon länger bestehenden Stoffwechselstörung führt. Die für den Muskelstoffwechsel unerlässlichen hormonabhängigen Stoffwechselvorgänge wie Kohlenhydrat-, Elektrolyt- und Proteinstoffwechsel sind bei Dysbalancen ursächlich für die muskuläre Beteiligung. Generell sollte zwischen iatrogenen und endogenen Ursachen unterschieden werden (z. B. Steroidmyopathie). Allen gemeinsam sind mehr oder minder ausgeprägte, proximal betonte Muskelschwächen, zum Teil verbunden mit Myalgien und Muskelatrophien. Die Therapie endokriner Myopathien besteht v. a. in der Behandlung der Grunderkrankung. Bei frühzeitiger Diagnostik und suffizienter Wiederherstellung der physiologischen Stoffwechsellage sind endokrine Myopathien langfristig kompensierbar und reversibel. Prognostisch maßgeblich bleiben jedoch die initiale Krankheitsschwere und die Krankheitsdauer der Endokrinopathie.

Abstract

Disorders in endocrinological pathways rarely lead to manifest acquired or endogenous myopathy so that an interdisciplinary evaluation between neurology and endocrinology is essential for these disorders. Asymptomatic or forme fruste variants may be more common and even underdiagnosed in these circumstances. Dysbalance disorders of protein synthesis, electrolytes and carbohydrates can lead to several rare forms of myopathy due to the dependence on hormonal metabolism. In general, the main neuromuscular symptom is proximal weakness, sometimes in addition to myalgia and muscle atrophy. Endocrine myopathies are usually reversible by treatment of the underlying disease. The severity of the endocrinopathy is of fundamental importance for the long-term clinical outcome.

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  1. Friedrich-Baur-Institut, Neurologische Klinik, Interdisziplinäres Zentrum für Neuromuskuläre Erkrankungen der Ludwig-Maximilians-Universität München, Ziemssenstr. 1a, 80336, München, Deutschland

    S. Wenninger &  B. Schoser

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  1. S. Wenninger
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  2. B. Schoser
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Wenninger, S., Schoser, B. Angeborene und endogene endokrine Myopathien. Z. Rheumatol. 70, 760–766 (2011). https://doi.org/10.1007/s00393-011-0787-5

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  • DOI: https://doi.org/10.1007/s00393-011-0787-5

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