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Acta Myol. 2009 July; 28(1): 34.
PMCID: PMC2859636

I-1. Diagnosis and long-term treatment of “untreatable” neuromuscular patients, an update

Personal examples of diagnosis and long-term treatment of seemingly “untreatable” neuromuscular diseases are as follows: a) Identification of Hyper-PTH-emic chronic fatigue and weakness can result in recover within hours-days following local-anesthetic out-patient surgical removal of an occult PTH adenoma; b) For severe muscle carnitine-deficient lipid myopathy, an “endodissolution”, cumulatively dramatic and sustained, 28 years, benefit to normality can be achieved with only a “Portagen“ diet devoid of long-chain fatty acids; c) Total-body irradiation of some otherwise-extremely-resistant dermatomyositis patients can have sustained, dramatic benefit; d) “Fever-responsive dysimmune neuropathy” treated long-term with interferon alpha-2A can show cumulatively excellent and sustained strength during 14 years of treatment, plus 6 years afterwards, the latter possibly suggesting cure; e) IVIG (i.v. IgG) can remarkably and cumulatively benefit various dysimmune diseases, e.g. CIDP (“Chronic Immune Dysschwannian Polyneuropathy”) and at least some patients with multiple sclerosis – if the proper schedule, dose, and infusion-rate are followed (contrary to some published recommendations that I consider incorrect). The peak-trough clinical response of each individual patient (not a pre-conceived IgG serum half-life) is critically relevant to allow the evanescent benefits of IVIG to summate. Maximal clinical benefit usually lasts only 1.5-2 weeks. To achieve incrementing summation of benefit, viz. avoiding a “Sisyphus Effect”, I use a schedule of 0.4 gms/kg/day x5-contigious days, every 3rd week, ± another “booster-dose” on Mon. or Tue. of the second skipped week. For worrisome patients with hypertension, cardiovascular concerns, migraine, or advanced age, my unique schedule of 0.4gms/kg/day on 2 non-consecutive days every week is safer. To avoid headache and vascular complications, infusion-rate is not over 100 ml/hr, and slower in fragile patients.

Only after presumed “maximal benefit” is achieved do I gradually increase spacing of infusions; f) “Diabetic”sensory-motor neuropathy in Type-2 diabetes often is much benefited by IVIG, as is “genetico-diabetoid-2 dysimmune neuropathy”; g) “CMT-oid CIDP” is sometimes gratifyingly-treatable with IVIG; h) Treatable “pseudo-ALS” patients must constantly be sought; i) For ordinary ALS, a new-old hypothesis of remote-pathogenesis, analogous in principle to the pathogenesis of B12-deficient myelopathy, will be presented.

Articles from Acta Myologica are provided here courtesy of Pacini Editore