Skip to main content

Erkrankungen der neuromuskulären Übertragung

  • Chapter
Klinische Neurologie
  • 112 Accesses

Zusammenfassung

Die Myasthenia gravis ist eine erworbene Autoimmunerkrankung, bei der Antikörper den postsynaptischen Acetylcholinrezeptor zerstören. Die dadurch gestörte neuromuskuläre Übertragung führt zu einer vorzeitigen Ermüdbarkeit der Willkürmuskulatur, besonders unter Belastung. Überwiegend sind die okulären, faziopharyngealen und proximalen Muskelgruppen betroffen, jedoch kann die Muskelschwäche auch generalisiert auftreten.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Referenzen

Zitierte Literatur

  • Abicht A, Müller-Felber W, Fischer P et al. (1997) Congenital myasthenic syndromes: clinical and genetic analysis of 18 patients. Eur J Med Res 2: 515–522

    PubMed  CAS  Google Scholar 

  • Bady B, Chauplannaz G, Carrier H (1987) Congenital Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 50:476–478

    Article  PubMed  CAS  Google Scholar 

  • Bain PG, Motomura M, Newsom-Davis J, Misbah SA (1996) Effects of intravenous Immunglobulin on muscle weakness and calcium-channel autoantibodies in the Lambert-Eaton myasthenic syndrome. Neurology 47:678 – 683

    Google Scholar 

  • Besinger UA, Toyka KV, Homberg M et al. (1983) Myasthenia gravis: long term correlation of binding and bungarotoxin blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 33:1316–1321

    Article  PubMed  CAS  Google Scholar 

  • Bever LJ, Aquino AV, Penn AS et al. (1983) Prognosis of ocular myasthenia gravis. Ann Neurol 14: 516–519

    Article  PubMed  Google Scholar 

  • Compston DAS, Vincent A, Newsom-Davis J, Batchelor JR (1980) Clinical, pathological, HLA antigen and immunological evidence for disease heterogenity in myasthenia gravis. Brain 103: 579–601

    Article  PubMed  CAS  Google Scholar 

  • Dobkin BH, Verity A (1978) Familial neuromuscular disease with type 1 fiber hypoplasia, tubular aggregates, cardiomyopathy, and myasthenic features. Neurology 28: 1135–1140

    Article  PubMed  CAS  Google Scholar 

  • Engel AG (1986) Myasthenic syndromes. In: Engel AG, Banker BQ Myology, vol 2. McGraw-Hill, New York, pp 1955–1990

    Google Scholar 

  • Engel AG, Lambert EH (1987) Congenital myasthenic syndromes. Electroencephalogr Clin Neurophysiol 39 (suppl): 91–102

    CAS  Google Scholar 

  • Engel AG, Lambert EH, Gomez MR (1977) A new myasthenic syndrome with endplate acetylcholinesterase deficiency, small nerve terminals, and reduced acetylcholine release. Ann Neurol 1:315–330

    Article  PubMed  CAS  Google Scholar 

  • Engel AG, Lambert EH, Mulder DM et al. (1982) A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine induced ion channel. Ann Neurol 11: 553 – 569

    CAS  Google Scholar 

  • Engel AG, Walls TJ, Nagel A, Uchitel O (1990) Newly recognized congenital myasthenic syndromes. I. Congenital paucity of synaptic vesicles and reduced quantal release. II. High-conductance fast-channel syndrome. III. Abnormal acetylcholine receptor (AChR) interaction with acetylcholine. IV. AChR deficiency and short channel-open time. Prog Brain Res 84:125–137

    Article  PubMed  CAS  Google Scholar 

  • Grob D, Brunner N, Namba T (1981) The natural course of myasthenia gravis and effect of therapeutic measures. Ann NY Acad Sci 377: 652–669

    Article  PubMed  CAS  Google Scholar 

  • Jennekens FG, Hesselmann LF, Veldmann H et al. (1992) Deficiency of acetylcholine receptors in a case of endplate acetylcholinesterase deficiency: a histochemical investigation. Muscle Nerve 15:63 – 72

    Article  PubMed  CAS  Google Scholar 

  • Jerusalem F, Zierz S (1991) Muskelerkrankungen. Thieme, Stuttgart New York

    Google Scholar 

  • Lang B, Vincent A, Murray NMF, Newsom-Davis J (1989) Lambert-Eaton myasthenic syndrome: Immunglobulin G inhibition of Ca2+ flux in tumor cells correlates with disease severity. Ann Neurol 25:265 – 271

    Article  PubMed  CAS  Google Scholar 

  • Lecky BRF, Morghan-Hughes JA, Murray NMF et al. (1986) Congenital myasthenia: further evidence of disease heterogeneity. Muscle Nerve 9: 233 – 242

    Article  PubMed  CAS  Google Scholar 

  • Lindner A, Schalke B, Toya KV (1997) Outcome in juvenile-onset myasthenia gravis: a retrospective study with long-term follow-up of 79 patients. J Neurol 244: 515–520

    Article  PubMed  CAS  Google Scholar 

  • Mc Quillen MP (1966) Familial limb-girdle myasthenia. Brain 89:121–132

    Article  CAS  Google Scholar 

  • Melms A, Hohlfeld R (1998) Zur Ätiologie und Pathogenese der Myasthenia gravis. Akt Neurol 25 : S39 – S41

    Article  Google Scholar 

  • Molnar J, Szobor A (1990) Myasthenia gravis: effect of thymectomy in 425 patients: a 15-years experience. Eur J Cardio-thorac Surg 4:8

    Article  CAS  Google Scholar 

  • Morghan-Hughes JA, Lecky BRF, Landon DN, Murray NMF (1981) Alterations in the number and affinity of junctional acatylcholine receptors in a myopathy with tubular aggregates. A newly recognized receptor defect. Brain 104:279 – 295

    Article  Google Scholar 

  • Nagel A, Engel AG, Lang B, Newsom-Davis J, Fukuoka T (1988) Lambert-Eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigen modulation. Ann Neurol 24: 552 – 558

    Article  PubMed  CAS  Google Scholar 

  • Newsom-Davis J (1988) Lambert-Eaton myasthenic syndrome: a review. Monogr Allergy 25:480 – 485

    Google Scholar 

  • Nishida Y, Kobayashi M, Machi M et al. (1989) Congenital myopathy with myasthenic features and congenital cataract in two siblings. J Neurol 236:161 – 163

    Article  PubMed  CAS  Google Scholar 

  • Ossermann KE (1971) Studies in myasthenia gravis: review of a twenty year experience in over 1200 patients. Mt Sinai J Med 38:497–537

    Google Scholar 

  • Osterhuis HJGH, Newsom-Davis J,Wokke JHJ et al. (1987) The slow channel syndrome. Two new cases. Brain 110: 1061–1079

    Article  Google Scholar 

  • Patrick J, Lindstrom J (1973) Autoimmune response to acetylcholin receptor. Science 180:87 Iff

    Article  Google Scholar 

  • Schumpelick V, Janzen R (1984) Thymektomie bei Myasthenia gravis. Dtsch Med Wochenschr 109:1166–1172

    Article  PubMed  CAS  Google Scholar 

  • Smit LME, Jennekens H, Veldman H et al. (1984) Paucity of secondary synaptic clefts in a case of congenital myasthenia with multiple contractures: ultrastructural morphology of a developmental disorder. J Neurol Neurosurg Psychiatry 47:1091–1097

    Article  PubMed  CAS  Google Scholar 

  • Toyka KV, Drachman DB, Pestronk A, Kao I (1975) Myasthenia gravis: passive transfer from man to mouse. Science 190: 397 ff

    Article  PubMed  CAS  Google Scholar 

  • Toyka KV, Drachman DB, Pestronk A, Griffin DE, Kao I, Winkelstein JA (1977) Die pathogenetische Bedeutung von „myasthenogenen“ Immunglobulinen. In: Hertel G et al. Myasthenia gravis. Thieme, Stuttgart

    Google Scholar 

  • Toyka KV, Becker T, Fateh-Moghadam A et al. (1979) Die Bedeutung der Bestimmung von Antikörpern gegen Acetylcholin-Rezeptoren in der Diagnostik der Myasthenia gravis. Klin Wochenschr 57: 937

    Article  PubMed  CAS  Google Scholar 

  • Vincent A, Cull-Candy SG, Newsom-Davis J, Trautmannn A, Molenaar PC, Polak RL (1981) Congenital myasthenia: endplate acetylcholine receptors and electrophysiology in five cases. Muscle Nerve 4:306–318

    Article  PubMed  CAS  Google Scholar 

  • Walker MB (1934) Treatment of myasthenia gravis with physo-stigmine. Lancet 1:1200ff

    Article  Google Scholar 

  • Wekerle H, Hohlfeld R, Ketelsen UP, Kalden JR, Kalies I (1981) Thymic myogenesis, T-lymphocytes and the pathogenesis of myasthenia gravis. Ann N Y Acad Sci 377: 455 ff

    Article  PubMed  CAS  Google Scholar 

  • Wokke JHJ, Jennekens FGI, Molenaar PC, Vandenoord CJM, Oen BS, Busch HFM (1989) Congenital paucity of secondary synaptic clefts (CPSC) syndrome in 2 adult sibs. Neurology 39:648–654

    Article  PubMed  CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1999 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Hertel, K., Lindner, A., Zierz, S. (1999). Erkrankungen der neuromuskulären Übertragung. In: Berlit, P. (eds) Klinische Neurologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-08118-1_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-08118-1_12

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-08119-8

  • Online ISBN: 978-3-662-08118-1

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics