Neuromuscular disorders

The symptoms of vitamin E deficiency in animals are numerous and vary from species to species (13). Although the deficiency of the vitamin can affect different tissue types such as reproductive, gastrointestinal, vascular, neural, hepatic, and optic in a variety of species such as pigs, rats, mice, dogs, cats, chickens, turkeys, monkeys, and sheep, it is generally found that necrotizing myopathy is relatively common to most species. In humans, vitamin E deficiency can result from poor fat absorption in adults and children. Infants, especially those with low birth weights, typically have a vitamin E deficiency which can easily be corrected by supplements. This deficiency can lead to symptoms such as hemolytic anemia, reduction in red blood cell lifetimes, retinopathy, and neuromuscular disorders. 

The aminoglycosides are used cautiously in patients with renal failure (dosage adjustments may be necessary), in the elderly, and in patients with neuromuscular disorders. 

Argov, Z., Mastaglia, F.L. (1988). Drug-induced neuromuscular disorders in man. In Disorders of Voluntary Muscle (Walton, J.N., ed.), pp. 981-1014, Churchill-Livingstone, Edinburgh. 

In some patients believed to suffer from the irritable bowel syndrome, an underlying enteric neuromuscular disorder has later been identified [133]. The bridge to infectious diseases is also of interest, with several entero-tropic viruses in focus, and reports of lymphocytic infiltration of enteric neural structures in patients with unexplained intestinal dysmotility require further studies. 

Krishnamurthy S, Schuffler MD Pathology of neuromuscular disorders of the small intestine and colon. Gastroenterology 1987 93 610-639. 

Hydrazine and all of its methylated derivatives appear to induce neuromuscular disorders at or near lethal doses, and all appear to be respiratory irritants. Jacobson et al. (1955) noted that the actions of hydrazine and its methylated derivatives were similar all are respiratory irritants and convulsants. In addition, monomethylhydrazine also induced severe intravascular hemolysis in dogs. 

Measuring muscle-evoked responses to repetitive motor nerve electrical stimulation permits detection of presyn-aptic neuromuscular junction dysfunction. In botulism and the Lambert-Eaton syndrome, repetitive stimulation elicits a smaller than normal skeletal muscle response at the beginning of the stimulus train, due to impaired initial release of acetylcholine-containing vesicles from presyn-aptic terminals of motor neurons followed by a normal or accentuated incremental muscle response during repeated stimulation. This incremental response to repetitive stimulation in presynaptic neuromuscular disorders can be distinguished from the decremental response that characterizes autoimmune myasthenia gravis, which affects the postsynaptic component of neuromuscular junctions. 

Jones, H. R. Jr, De Vivo, D. C. and Darras, B. T. (eds) Neuromuscular Disorders of Infancy, Childhood, and Adolescence - A Clinicians Approach. Butterworth-Heinemann. Philadelphia, 2003. 

Rustam H, Von Burg R, Amin-Zaki L, El Hassani S Evidence for a neuromuscular disorder in methylmercury poisoning—clinical and electrophysiological findings in moderate to severe cases. Arch Environ Health 30 190-195, 1975... 

Neuropathic disorders Administer with caution in individuals with peripheral motor neuropathic diseases (eg, amyotrophic lateral sclerosis, motor neuropathy) or neuromuscular junctional disorders (eg, myasthenia gravis, Lambert-Eaton syndrome). Patients with neuromuscular disorders may be at increased risk of clinically significant P.787... 

WARNING Systemic absorption of oral route may cause neuro/oto/nephrotox may result resp paralysis possible w/ any route of administration Uses Hepatic coma, bowel prq) Action Aminoglycoside, poorly absorbed PO -1- GI bacterial flora Dose Adults. 3-12 g/24- h PO in 3-4 doses Peds. 50-1 (X) mg/kg/24 h PO in 3-4 doses Caution [C, /-] Renal failure, neuromuscular disorders, hearing impair Contra Intestinal obst Disp Tabs, PO soln SE Hearing loss w/ long-term use rash, NA EMS Use neuromuscular blockers w/ caution, reduced dose may be necessary t bleeding risk w/ concurrent anticoagulant use OD May cause neuromuscular block and kidney failure calcium salts can be used to revise neuromuscular block... 

A couple undergoes genetic counseling for consultation on the possibility that they may have a child with Friedrich s ataxia, an autosomal recessive neuromuscular disorder. The disorder has been diagnosed in the wife s sister, and they are concerned that she is a carrier. The incidence of the disorder in the population is 1 in 25,000 live births. 

Drachman DB. Immunotherapy in neuromuscular disorders Current and future strategies. Muscle Nerve 1996 19 1239-1251. 

In patients with neuromuscular disorders, assess the respiratory response carefully... 

Treves, S., Anderson, A. A., Ducreux, S., Divet, A., Bleunven, C., Grasso, C., Paesante, S., and Zorzato, F. (2005). Ryanodine Receptor 1 Mutations, Dysregulation of Calcium Homeostasis and Neuromuscular Disorders. Neuromuscul Disord 15(9-10) 577-87. 

Considerable neuromuscular involvement also occurs in patients with AIDS.47 100 Peripheral neuropathies, myopathies, and various CNS manifestations (dementia, other psychological manifestations) can occur directly from HIV infection or secondarily, due to some other opportunistic infection.31 85 100 Likewise, peripheral neuropathies are a common side effect of certain anti-HIV drugs (didanosine, stavudine, zal-citabine), and myopathies are a side effect of zidovudine therapy.63 Patients with HIV disease often have painful symptoms such as joint pain, back pain, and pain related to neuropathies and myopathies.100 Hence, HIV disease can often be regarded as a degenerative neuromuscular disorder from the standpoint of a rehabilitation professional. Therapists can therefore help improve function and decrease pain in patients with HIV infection and AIDS.1 33... 

Wallgren-Petterson, C., and Laing, N. G. (2003). 109th ENMC International Workshop 5th Workshop on nemaline myopathy, 11-13 October 2002, Naarden, The Netherlands. Neuromuscular Disorders 13, 501-507. 

As a component of balanced anesthesia (intravenous administration) For control of ethanol or other sedative-hypnotic withdrawal states For muscle relaxation in specific neuromuscular disorders... 

Emery, A. E. H. (1998) Neuromuscular disorders clinical and molecular genetics, John Wiley, Chichester/New York. 

D Angelo MG, Bresolin N. 2006. Cognitive impairment in neuromuscular disorders. Muscle Nerve 34 16-33. 

Darras BT, Menache CC, Kunkel LM. 2003. Dystrophinopa-thies. Jones HR Jr, DeVivo DC, Darras BT, editors. Neuromuscular Disorders of Infancy, Childhood, and Adolescence, A Clinician s Approach. New York Butterworth Heine-mannn pp. 649-699. 

Ranum LP, Cooper TA (2006) RNA-mediated neuromuscular disorders. Annu Rev Neurosd... 

Dementia Syndromes Parkinsonism Plus Syndromes Neuromuscular Disorders Genetic/Metabolic Disorders... 

Jones JM, Datta P, Srinivasula SM, Ji W, Gupta S, Zhang Z, Davies E, Hajnoczky G, Saunders TL, Van Keuren ML, Fernandes-Alnemri T, Meisler MH, Alnemri ES (2003) Loss of Omi mitochondrial protease activity causes the neuromuscular disorder of mnd2 mutant mice. Nature 425 721-727... 

Livneh, A., Sarova, L, Michaeli, D., Pras, M., Wagner, K., Zakut, H., Soreq, H. (1988). Antibodies against acetylcholinesterase and low levels of cholinesterases in a patient with an atypical neuromuscular disorder. Clin. Immunol. Immunopathol. 48 119-31. 

A6. Aronson, S. M., and Volk, B. W., Serum aldolase activity in neuromuscular disorders. II. Experimental application. Proc. Soc. Exptl. Biol. Med. 94, 360 (1957). 

M18. Murphy, E. G., and Cherniak, M. M., Glutamic oxalacetic transaminase activity in the serum in muscular dystrophy and other neuromuscular disorders in childhood. Pediatrics 22, 1110 (1958). 

Okinaka, S., Sugita, H., Momoi, H., Toyokura, Y., Kumagai, H., Ebashi, S., and Fujie, Y., Serum creatine phosphokinase and aldolase activity in neuromuscular disorders. Trans. Am. Neurol. Assoc., 84th Meeting, Atlantic City 1959, p. 62. 

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