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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
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
Bady B, Chauplannaz G, Carrier H (1987) Congenital Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 50:476–478
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
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
Bever LJ, Aquino AV, Penn AS et al. (1983) Prognosis of ocular myasthenia gravis. Ann Neurol 14: 516–519
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
Dobkin BH, Verity A (1978) Familial neuromuscular disease with type 1 fiber hypoplasia, tubular aggregates, cardiomyopathy, and myasthenic features. Neurology 28: 1135–1140
Engel AG (1986) Myasthenic syndromes. In: Engel AG, Banker BQ Myology, vol 2. McGraw-Hill, New York, pp 1955–1990
Engel AG, Lambert EH (1987) Congenital myasthenic syndromes. Electroencephalogr Clin Neurophysiol 39 (suppl): 91–102
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
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
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
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
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
Jerusalem F, Zierz S (1991) Muskelerkrankungen. Thieme, Stuttgart New York
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
Lecky BRF, Morghan-Hughes JA, Murray NMF et al. (1986) Congenital myasthenia: further evidence of disease heterogeneity. Muscle Nerve 9: 233 – 242
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
Mc Quillen MP (1966) Familial limb-girdle myasthenia. Brain 89:121–132
Melms A, Hohlfeld R (1998) Zur Ätiologie und Pathogenese der Myasthenia gravis. Akt Neurol 25 : S39 – S41
Molnar J, Szobor A (1990) Myasthenia gravis: effect of thymectomy in 425 patients: a 15-years experience. Eur J Cardio-thorac Surg 4:8
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
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
Newsom-Davis J (1988) Lambert-Eaton myasthenic syndrome: a review. Monogr Allergy 25:480 – 485
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
Ossermann KE (1971) Studies in myasthenia gravis: review of a twenty year experience in over 1200 patients. Mt Sinai J Med 38:497–537
Osterhuis HJGH, Newsom-Davis J,Wokke JHJ et al. (1987) The slow channel syndrome. Two new cases. Brain 110: 1061–1079
Patrick J, Lindstrom J (1973) Autoimmune response to acetylcholin receptor. Science 180:87 Iff
Schumpelick V, Janzen R (1984) Thymektomie bei Myasthenia gravis. Dtsch Med Wochenschr 109:1166–1172
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
Toyka KV, Drachman DB, Pestronk A, Kao I (1975) Myasthenia gravis: passive transfer from man to mouse. Science 190: 397 ff
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
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
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
Walker MB (1934) Treatment of myasthenia gravis with physo-stigmine. Lancet 1:1200ff
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
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
Editor information
Editors and Affiliations
Rights 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