Ephedrine for myasthenia gravis, neonatal myasthenia and the congenital myasthenic syndromes. 2014

Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
Community Genetics Section, Clinical Genetics, VU University Medical Center, Amsterdam,Netherlands.

BACKGROUND Myasthenia is a condition in which neuromuscular transmission is affected by antibodies against neuromuscular junction components (autoimmune myasthenia gravis, MG; and neonatal myasthenia gravis, NMG) or by defects in genes for neuromuscular junction proteins (congenital myasthenic syndromes, CMSs). Clinically, some individuals seem to benefit from treatment with ephedrine, but its effects and adverse effects have not been systematically evaluated. OBJECTIVE To assess the effects and adverse effects of ephedrine in people with autoimmune MG, transient neonatal MG, and the congenital myasthenic syndromes. METHODS On 17 November 2014, we searched the Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE. We also searched reference lists of articles, conference proceedings of relevant conferences, and prospective trial registers. In addition, we contacted manufacturers and researchers in the field. METHODS We considered randomised controlled trials (RCTs) and quasi-RCTs comparing ephedrine as a single or add-on treatment with any other active treatment, placebo, or no treatment in adults or children with autoimmune MG, NMG, or CMSs. METHODS Two review authors independently assessed study design and quality, and extracted data. We contacted study authors for additional information. We collected information on adverse effects from included articles, and contacted authors. RESULTS We found no RCTs or quasi-RCTs, and therefore could not establish the effect of ephedrine on MG, NMG and CMSs. We describe the results of 53 non-randomised studies narratively in the Discussion section, including observations of endurance, muscle strength and quality of life. Effects may differ depending on the type of myasthenia. Thirty-seven studies were in participants with CMS, five in participants with MG, and in 11 the precise form of myasthenia was unknown. We found no studies for NMG. Reported adverse effects included tachycardia, sleep disturbances, nervousness, and withdrawal symptoms. CONCLUSIONS There was no evidence available from RCTs or quasi-RCTs, but some observations from non-randomised studies are available. There is a need for more evidence from suitable forms of prospective RCTs, such as series of n-of-one RCTs, that use appropriate and validated outcome measures.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D009157 Myasthenia Gravis A disorder of neuromuscular transmission characterized by fatigable weakness of cranial and skeletal muscles with elevated titers of ACETYLCHOLINE RECEPTORS or muscle-specific receptor tyrosine kinase (MuSK) autoantibodies. Clinical manifestations may include ocular muscle weakness (fluctuating, asymmetric, external ophthalmoplegia; diplopia; ptosis; and weakness of eye closure) and extraocular fatigable weakness of facial, bulbar, respiratory, and proximal limb muscles. The disease may remain limited to the ocular muscles (ocular myasthenia). THYMOMA is commonly associated with this condition. Anti-MuSK Myasthenia Gravis,MuSK MG,MuSK Myasthenia Gravis,Muscle-Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle-Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Generalized,Myasthenia Gravis, Ocular,Anti MuSK Myasthenia Gravis,Generalized Myasthenia Gravis,Muscle Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Anti-MuSK,Myasthenia Gravis, MuSK,Ocular Myasthenia Gravis
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002800 Cholinesterase Inhibitors Drugs that inhibit cholinesterases. The neurotransmitter ACETYLCHOLINE is rapidly hydrolyzed, and thereby inactivated, by cholinesterases. When cholinesterases are inhibited, the action of endogenously released acetylcholine at cholinergic synapses is potentiated. Cholinesterase inhibitors are widely used clinically for their potentiation of cholinergic inputs to the gastrointestinal tract and urinary bladder, the eye, and skeletal muscles; they are also used for their effects on the heart and the central nervous system. Acetylcholinesterase Inhibitor,Acetylcholinesterase Inhibitors,Anti-Cholinesterase,Anticholinesterase,Anticholinesterase Agent,Anticholinesterase Agents,Anticholinesterase Drug,Cholinesterase Inhibitor,Anti-Cholinesterases,Anticholinesterase Drugs,Anticholinesterases,Cholinesterase Inhibitors, Irreversible,Cholinesterase Inhibitors, Reversible,Agent, Anticholinesterase,Agents, Anticholinesterase,Anti Cholinesterase,Anti Cholinesterases,Drug, Anticholinesterase,Drugs, Anticholinesterase,Inhibitor, Acetylcholinesterase,Inhibitor, Cholinesterase,Inhibitors, Acetylcholinesterase,Inhibitors, Cholinesterase,Inhibitors, Irreversible Cholinesterase,Inhibitors, Reversible Cholinesterase,Irreversible Cholinesterase Inhibitors,Reversible Cholinesterase Inhibitors
D004809 Ephedrine A phenethylamine found in EPHEDRA SINICA. PSEUDOEPHEDRINE is an isomer. It is an alpha- and beta-adrenergic agonist that may also enhance release of norepinephrine. It has been used for asthma, heart failure, rhinitis, and urinary incontinence, and for its central nervous system stimulatory effects in the treatment of narcolepsy and depression. It has become less extensively used with the advent of more selective agonists. Ephedrine Hydrochloride,Ephedrine Renaudin,Ephedrine Sulfate,Erythro Isomer of Ephedrine,Sal-Phedrine,Ephedrine Erythro Isomer,Hydrochloride, Ephedrine,Renaudin, Ephedrine,Sal Phedrine,SalPhedrine,Sulfate, Ephedrine
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D018663 Adrenergic Agents Drugs that act on adrenergic receptors or affect the life cycle of adrenergic transmitters. Included here are adrenergic agonists and antagonists and agents that affect the synthesis, storage, uptake, metabolism, or release of adrenergic transmitters. Adrenergic,Adrenergic Agent,Adrenergic Drug,Adrenergic Neuron Agents,Adrenergic Release Inhibitors,Adrenergic Synthesis Inhibitors,Sympathetic Transmitter Releasers,Adrenergic Drugs,Adrenergic Effect,Adrenergic Effects,Adrenergic Neurohumor Depleters,Adrenergic Neuron Drugs,Adrenergics,Agent, Adrenergic,Agents, Adrenergic,Agents, Adrenergic Neuron,Depleters, Adrenergic Neurohumor,Drug, Adrenergic,Drugs, Adrenergic,Drugs, Adrenergic Neuron,Effect, Adrenergic,Effects, Adrenergic,Inhibitors, Adrenergic Release,Inhibitors, Adrenergic Synthesis,Neurohumor Depleters, Adrenergic,Neuron Agents, Adrenergic,Neuron Drugs, Adrenergic,Release Inhibitors, Adrenergic,Releasers, Sympathetic Transmitter,Synthesis Inhibitors, Adrenergic,Transmitter Releasers, Sympathetic
D020294 Myasthenic Syndromes, Congenital A heterogeneous group of disorders characterized by a congenital defect in neuromuscular transmission at the NEUROMUSCULAR JUNCTION. This includes presynaptic, synaptic, and postsynaptic disorders (that are not of autoimmune origin). The majority of these diseases are caused by mutations of various subunits of the nicotinic acetylcholine receptor (RECEPTORS, NICOTINIC) on the postsynaptic surface of the junction. (From Arch Neurol 1999 Feb;56(2):163-7) Congenital Slow-Channel Myasthenic Syndrome,Myasthenic Syndromes, Congenital, Slow Channel,Postsynaptic Congenital Myasthenic Syndrome,Presynaptic Congenital Myasthenic Syndrome,Slow-Channel Congenital Myasthenic Syndrome,Congenital Myasthenia,Congenital Myasthenia Gravis,Congenital Myasthenic Syndrome,Congenital Myasthenic Syndromes,Congenital Myasthenic Syndromes, Postsynaptic,Congenital Myasthenic Syndromes, Presynaptic,Congenital Slow-Channel Myasthenic Syndromes,Myasthenia Gravis, Congenital,Myasthenic Syndrome, Congenital, Slow-Channel,Postsynaptic Congenital Myasthenic Syndromes,Presynaptic Congenital Myasthenic Syndromes,Slow-Channel Congenital Myasthenic Syndromes,Congenital Myasthenias,Congenital Slow Channel Myasthenic Syndrome,Congenital Slow Channel Myasthenic Syndromes,Gravi, Congenital Myasthenia,Myasthenia, Congenital,Myasthenias, Congenital,Myasthenic Syndrome, Congenital,Slow Channel Congenital Myasthenic Syndrome,Slow Channel Congenital Myasthenic Syndromes,Syndrome, Congenital Myasthenic,Syndromes, Congenital Myasthenic
D020941 Myasthenia Gravis, Neonatal A disorder of neuromuscular transmission that occurs in a minority of newborns born to women with myasthenia gravis. Clinical features are usually present at birth or develop in the first 3 days of life and consist of hypotonia and impaired respiratory, suck, and swallowing abilities. This condition is associated with the passive transfer of acetylcholine receptor antibodies through the placenta. In the majority of infants the myasthenic weakness resolves (i.e., transient neonatal myasthenia gravis) although this disorder may rarely continue beyond the neonatal period (i.e., persistent neonatal myasthenia gravis). (From Menkes, Textbook of Child Neurology, 5th ed, p823; Neurology 1997 Jan;48(1):50-4) Antenatal Myasthenia Gravis,Neonatal Myasthenia Gravis,Myasthenia Gravis, Neonatal, Persistent,Myasthenia Gravis, Neonatal, Transient,Myasthenia Gravis, Persistent, Neonatal,Myasthenia Gravis, Transient, Neonatal,Neonatal Myasthenia Gravis, Persistent,Neonatal Myasthenia Gravis, Transient,Persistent Neonatal Myasthenia Gravis,Transient Neonatal Myasthenia Gravis,Myasthenia Gravis, Antenatal

Related Publications

Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
January 2023, Handbook of clinical neurology,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
December 2019, Continuum (Minneapolis, Minn.),
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
May 2013, Ideggyogyaszati szemle,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
August 2009, Zeitschrift fur Rheumatologie,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
October 1992, Current opinion in neurology and neurosurgery,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
November 1984, Annals of neurology,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
January 1990, Revista chilena de pediatria,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
September 2020, Journal of veterinary internal medicine,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
July 1980, Journal of neurology, neurosurgery, and psychiatry,
Charlotte Vrinten, and Angeli M van der Zwaag, and Stephanie S Weinreich, and Rob J P M Scholten, and Jan J G M Verschuuren
September 2016, Arquivos de neuro-psiquiatria,
Copied contents to your clipboard!