[Pseudoparalytic myasthenia gravis. Diagnostic and therapeutic aspects in 60 separate cases].
Based on 60 of our own cases and on the medical literature the authors discuss the diagnostic, pathophysiological and therapeutic aspects of myasthenia gravis. Myasthenia is suspected in cases of motor weakness of changing intensity, diminishing by rest. The weak muscles are innervated by different peripheral nerves. At the beginning a weakness of upperlid-muscles, external eye muscles and bulbar muscles is particularly frequent. There is no sensory loss or other neurological symptoms. A transitory disappearance of motor weakness after an intravenous dose of Edrophonium (Tensilon) is a typical diagnostic sign. The effect is less evident with eye-muscle weakness. A typical appearance of potentials after repetitive stimulation of peripheral nerves as well as other characteristics in electrophysiological testing of muscles are of high diagnostic value. This allows differentiation from other types of muscle weakness. In the pathogenesis of myasthenia an autoimmune process related to a persistent thymus gland plays an important part. This leads to an ultrastructural change in the postsynaptic membrane of the muscle fibre. The postsynaptic membrane no longer reacts in a normal way to acetylcholine as a transmitter substance at the level of the motor endplate. Therefore the first step in the treatment of myasthenia consists of cholinesterase-inhibitors, specially Neostigmin (Prostigmin) and Pyridostigmin (Mestinon). Thymectomy is advised in all cases of myasthenia with the exception of the pure ocular form and of myasthenia in patients older than 60 years. The thymus gland is practically always persistent or hypertrophic in myasthenia. The suprasternal access is recommended. A thymoma should always be operated upon because of the danger of malignancy. In cases where thymectomy is not performed or not successful and if cholinesterase-inhibitors are not sufficiently efficient, treatment with corticosteroids or ACTH is recommended.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004491:Edrophonium; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009045:Motor Endplate / Q000503:physiopathology; D009119:Muscle Contraction; D009132:Muscles / Q000503:physiopathology; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D009220:Myositis / Q000175:diagnosis; D009388:Neostigmine / Q000627:therapeutic use; D009886:Ophthalmoplegia / Q000175:diagnosis; D011680:Pupil / Q000503:physiopathology; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D011950:Receptors, Cholinergic / Q000503:physiopathology; D013950:Thymus Gland / Q000503:physiopathology
D011950:Receptors, Cholinergic; D009388:Neostigmine; D004491:Edrophonium; D011729:Pyridostigmine Bromide
Die Myasthenia gravis pseudoparalytica. Diagnostische und therapeutische Aspekte anhand von 60 eigenen Beobachtungen