Chiropractic Care of a Pediatric Patient
with Myasthenia Gravis

This section is compiled by Frank M. Painter, D.C.
Send all comments or additions to:    Frankp@chiro.org

FROM:   J Manipulative Physiol Ther 2003 (Jul); 26 (6): 390–394 ~ FULL TEXT

Joel Alcantara, DC, Gregory Plaugher, DC, H. Jason Araghi, DC

This study was funded by Life Chiropractic College West,
Hayward, California, the Gonstead Clinical Studies Society,
Santa Cruz, California, and the International Chiropractic Pediatric Association,
Media, Pennsylvania.

Life Chiropractic College West,
2001 Industrial Blvd,
Hayward, CA 94545, USA;

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OBJECTIVE:   To describe the chiropractic care of a pediatric patient with complaints associated with myasthenia gravis.

CLINICAL FEATURES:   A 2–year-old girl was provided chiropractic care at the request and consent of her parents for complaints of ptosis and generalized muscle weakness (ie, lethargy), particularly in the lower extremities. Prior to entry into chiropractic management, magnetic resonance imaging of the brain and acetylcholine receptor antibody tests were performed with negative results. However, the Tensilon test was positive and the diagnosis of myasthenia gravis was made by a pediatrician and seconded by a medical neurologist.

INTERVENTION AND OUTCOME:   The patient was cared for with contact-specific, high-velocity, low-amplitude adjustments to sites of vertebral subluxation complexes in the upper cervical and sacral spine. The patient's response to care was positive and after 5 months of regular chiropractic treatment her symptoms abated completely.

CONCLUSION:   There are indications that patients suffering from disorders "beyond low back pain" as presented in this case report may derive benefits from chiropractic intervention/management.

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From the Full-Text Article:

Discussion

Myasthenia gravis is a disease of the neuromuscular junction. Specifically, an autoimmune response occurs, wherein antibodies for the acetylcholine receptors reduce the number available at the postsynaptic membrane either though increased antibody cross-linking and exocytosis, blockade of the receptor binding site, or damage of the postsynaptic membrane in conjunction with complement. [7] The result is a characteristic pattern of muscle weakness affecting initially the muscles innervated by the cranial nerves.

Childhood or adolescent onset MG accounts for approximately 11% to 24% of all patients with MG. [15, 16] The prevalence of autoimmune MG has been determined to be 4 to 10 of every 100,000 in the general population. [17, 18] In childhood or adolescent MG, 3 forms exist. They are juvenile MG, congenital MG, and transient neonatal MG. Juvenile MG is an acquired autoimmune disorder, wherein circulating antibodies attack the acetylcholine receptors on the motor endplates. [19] In congenital MG, various components of the neuromuscular junction are dysfunctional as a result of a heterogeneous group of inherited disorders. Receptor antibodies are not found, but this disorder does have other distinctive clinical or laboratory findings. [20] Spontaneous remissions are not expected in congenital MG. Juvenile MG is considered rare in children less than 1 year of age; however, its incidence increases with age. Gender bias for the disease is almost nonexistent during the prepubertal years (1.8:1 in favor of girls), but late onset favors females more than males (14:1). [21] There is recent limited evidence of familial tendency for MG. [22]

The diagnosis of MG is almost pathognomonic, with a history and physical examination findings of ptosis, diplopia, and weakness in the muscles of facial expression or mastication. [23] Generalized weakness increases with repeated muscle activity and is improved by rest. A recent report has attempted to quantify the effects on muscle strength with a reliable measurement instrument, the Quantified Myasthenia Gravis Strength Score, and this instrument has been recommended for both clinical and research settings. [24] Between 50% and 70% of patients with ocular myasthenia gravis will eventually develop generalized disease. [25] Laboratory testing with the Tensilon test is highly probable for MG if the test is unequivocally positive. Another test may involve radioimmunoassay for acetylcholine receptor antibodies. The test is approximately 90% positive for generalized MG, 50% positive for ocular MG, and a negative result does not rule out the possible diagnosis of MG. [7] In juvenile myasthenia, it is more common for children to not have a positive test (presence of antibodies) for the acetylcholine. [21]

Differential diagnoses for patients with symptoms as described above must account for other disorders with similar presenting complaints. These include drug-induced myasthenia, Lambert-Eaton myasthenic syndrome (LEMS), neurasthenia, hyperthyroidism, botulism, intracranial mass lesions, and progressive external ophthalmoplegia, to name a few. [1] Clinical findings, in addition to laboratory and other special studies, will assist in the differential diagnosis. For example, EMG or single-fiber EMG can assist in determining juvenile MG from myopathic disorders. With respect to the seronegative findings in the patient described, such findings are not unusual, particularly in patients with prepubertal onset. [26–28]

In addition, according to Anlar and others, [21, 29, 30] disorders such as juvenile-onset diabetes, asthma, thyroid disorders, and rheumatoid arthritis, are common comorbidities. Myasthenia gravis can also be associated with other autoimmune diseases (eg, Hashimoto disease). [31, 32]

Medical intervention

For juvenile MG patients, several medical approaches are available. One involves the use of acetylcholine esterase inhibitors. These inhibit the breakdown of acetylcholine to choline and acetate. Examples include pyridostigmine and neostigmine. Adverse reactions do occur due to possible overdose, and these include miosis, fasciculations, sweating, salivation, pallor, bradycardia, diarrhea, cramps, and increased weakness. Even in those who do not overdose their medications, significant side effects with considerable costs [33] can occur, including coronary spastic angina. [34] Another involves immunomodulatory approaches, where immunosuppressive agents are said to decrease fatalities and improve prognosis. An example is the use of glucocorticoids, such as prednisone. Adverse effects may include osteomalacia, hypertension, and body weight gain. Azathioprines are popular for use with adult MG patients, but their use in children is cautioned due to the adverse effect of secondary malignancies. Other immunosuppressive agents in use include cyclophosphamide and cyclosporin, but their adverse effects (ie, leucopenia and hemorrhagic cystitis, nephrotoxicity) limit their use. A third intervention involves plasmapheresis. This method removes the acetylcholine receptor antibodies from circulation. An alternative option from plasmapheresis is the use of intravenous immunoglobulin. Its action is thought to occur by blockade of Fc receptors on macrophages, binding of antibodies, reduction of receptor antibodies, prevention of its binding at the neuromuscular junction, or the stimulation of T suppressor cells. Thymectomy is also used as a treatment option. However, in consideration of the role of the thymus in immune function development, the recommended age is not until the patient is at least 10 years old, but as low as 5 years has been suggested as acceptable. [21] Studies in adults have also been inconclusive as to the efficacy of thymectomy due to multiple differences in baseline characteristics from various nonrandomized studies. Gronseth and Barohn [35] conclude that the benefit of thymectomy in nonthymomatous autoimmune MG has not been established conclusively. Guidelines have been developed for researchers attempting to study efficacy issues in the care of patients with myasthenia gravis. [36] One nonrandomized, retrospective study comparing surgical (ie, thymectomy) versus conservative treatments (ie, medications) has shown equivalent outcomes. [37]

The role of chiropractic care

Although anecdotes and patient testimonials have historically supported the role of chiropractic care in patients with complaints other than musculoskeletal-related conditions, the scientific literature is only now just beginning to document the role of chiropractic and other nonallopathic approaches in such patients. A MEDLINE search (1966–2001) using the subject headings chiropractic, alternative medicine, complementary medicine, and myasthenia gravis turned up an inconsiderable amount of available literature. Of those available, 1 paper was chiropractically related. Alcantara et al [38] described the successful chiropractic care of a 55–year-old patient with long-standing MG. Care was provided to sites of vertebral subluxation, utilizing the Gonstead Technique. Pease et al [39] described a case of persistent weakness in a 31–year-old woman with myasthenia gravis following therapeutic electrical stimulation. She was injured in an automobile accident and consulted a chiropractor 2 days later because her symptoms had not abated. Chiropractic treatment was described as consisting of short-wave diathermy, high-voltage electric stimulation, and spinal manipulation, continuing 3 times per week for 6 weeks. She then presented to her neurologist complaining of persistent fatigue, weakness, increased diplopia, cervical and occipital pain, and disrupted sleep. It was concluded that the patient’s symptoms were aggravated by the use of electrical stimulation. Other papers involved the role of acupuncture in patients with MG [40–42] and Chinese medicine. [43] Beekman and Oosterhuis [44] published their analysis on the use of alternative treatments by patients before and after MG was diagnosed and the influence on the diagnostic delay.

To discern the salutary effects of any intervention, several factors must be considered. Foremost it involves a consideration of the natural history of the disease, while another examines the specific and nonspecific effects of care. These issues have previously been discussed by Alcantara et al [38] from a chiropractic perspective. With respect to the natural history of juvenile MG, Andrews et al [45] found that the earlier onset of MG resulted in more favorable outcomes. Similar to findings in previous studies, [29, 46] they found an overall remission rate of 41%, particularly if onset was prepubertal. Less frequent spontaneous remissions are reported in older patients. In the study by Rodriguez et al, [29] they found from observations of 149 patients with juvenile MG studied from onset of disease for as long as 40 years, that spontaneous remissions occurred less in patients with onset after 11 years of age. Andrews et al [45] have speculated that the putative environmental trigger that precipitates juvenile MG in some patients may not be able to sustain an ongoing autoimmune response in the presence of immunosuppressive effects of androgens during adrenarche or due to the absence of stimulatory effects of circulating female sex steroids. If, indeed, putative environmental triggers play prominently in juvenile MG, the authors are interested in the possibilities of the vitalistic approach of chiropractic care in the care and prevention of individuals with this disease and other disorders. The patient in the present case report was involved in a motor vehicle collision prior to developing the MG symptoms. A case of MG in a 17–year-old football player appeared to develop following a minor head trauma sustained during a collegiate scrimmage. [46] The role of spinal or head trauma preceding the onset of symptoms in MG remains to be explored in both retrospective and prospective investigations. We encourage further research into this area.

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Conclusion

We report the successful chiropractic case management of a 2–year-old patient with antibody-negative, generalized MG. To the best of our knowledge, this is the first report in the scientific literature describing chiropractic in a pediatric patient with MG. Adjustive care was provided based on the objective and subjective parameters of vertebral and sacral findings, interpreted as vertebral subluxation complex. Prospective research into the efficacy of this approach to health care is encouraged. The possible role of an environmental trigger (eg, spinal trauma) antecedent to the development of MG symptoms and the salutary effects of chiropractic care need to be explored.

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