The Use of Korean Medicine to Treat Patients with Spinobulbar Muscular Atrophy, Kennedy’s Disease - A Case Study

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Abstract

Objectives:

Studies involving patients with spinobulbar muscular atrophy (SBMA), which is often referred to as Kennedy’s disease, similar to those involving patients with progressive muscular disease (PMD), are rare. This paper reports a case study involving the use of Korean medicine to treat a patient with SBMA.

Methods:

We treated a patient with SBMA with unique symptoms by using various kinds of pharmacopuncture and herbal medicines for about two and a half years. After the treatment had ended, we evaluated the patient’s conditions and the side effects of the treatment.

Results:

After treatment, the patient’s symptoms were stabilized, and the patient suffered no abnormalities or side effects. No special changes in condition were noted during treatment period, and the patient was very satisfied with his response to treatment.

Conclusion:

Existing treatments have some considerable after effects and are difficult to apply in domestic clinics. In this regard, our findings should open possibilities for new clinical guidelines. Nevertheless, the limitations associated with this case study should be resolved, and more studies need to be conducted.

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Most cited references 7

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Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.

Malcolm Kennedy, H Sung, M Alter (1968)

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Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trial.

Sungyoung Auh, Ellen Levy, E. Roberta Ryder … (2011)

Spinal and bulbar muscular atrophy (SBMA) is caused by polyglutamine expansion in the androgen receptor, which results in ligand-dependent toxicity. Animal models have a neuromuscular deficit that is mitigated by androgen-reducing treatment. We aimed to assess the efficacy and safety of the 5α-reductase inhibitor dutasteride in patients with SBMA, and to identify outcome measures for use in future studies of the disease. We undertook a randomised, double-blind, placebo-controlled, single-site clinical trial in ambulatory, symptomatic men with genetically confirmed SBMA. Participants were assigned by random number table to receive dutasteride (0·5 mg per day) or placebo orally for 24 months. Patients and investigators were masked to treatment allocation. The primary outcome measure was quantitative muscle assessment (QMA). The final efficacy analysis included all patients who were compliant with study treatment at 24 months. This trial was registered with ClinicalTrials.gov, NCT00303446. 50 men were randomly assigned to treatment groups (25 dutasteride, 25 placebo), and 44 were included in the efficacy analysis (21 dutasteride, 23 placebo). At 24 months, the placebo group showed a decrease of 4·5% (-0·30 kg/kg) from baseline in weight-scaled muscle strength as indicated by QMA, and the dutasteride group had an increase in strength of 1·3% (0·14 kg/kg); the difference between groups (5·8%, 95% CI -5·9 to 17·6; p=0·28) was not significant. Prespecified secondary outcome measures of creatine kinase, muscle strength and function, motor nerve conduction, activities of daily living, and erectile function did not show a significant difference between the study groups in change from baseline. Quality of life, as measured by the physical component summary of the Medical Outcomes Study 36-item Short Form version 2, favoured dutasteride (change in score from baseline: placebo, -3·6%, vs dutasteride, 2·1%; p=0·01), whereas the mental component summary favoured placebo (3·3%vs -3·2%; p=0·03). The dutasteride group had fewer patients reporting falls than did the placebo group (9 vs 16; p=0·048); there were no other significant differences in reported adverse events. Our study did not show a significant effect of dutasteride on the progression of muscle weakness in SBMA, although there were secondary indications of both positive and negative effects compared with placebo. A longer trial duration or larger number of patients might be needed to show an effect on disease progression. Performance testing, QMA, and quality of life measures were identified as potentially useful endpoints for future therapeutic trials. Copyright Â© 2011 Elsevier Ltd. All rights reserved.

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Bulbar and spinal muscular atrophy (Kennedy's disease): a review.

J Finsterer (2009)

Bulbar and spinal muscular atrophy (BSMA) is an adult-onset, X-linked recessive trinucleotide, polyglutamine disorder, caused by expansion of a polymorphic CAG tandem-repeat in exon 1 of the androgen-receptor (AR) gene on chromosome Xq11-12. Pathogenetically, mutated AR accumulates in nuclei and cytoplasm of motor neurons, resulting in their degeneration and loss. Phenotypically, patients present with amyotrophic, proximal or distal weakness and wasting of the facial, bulbar and limb muscles, occasionally sensory disturbances, and endocrinologic disturbances, such as androgen resistance, gynecomastia, elevated testosterone or progesterone, and reduced fertility. There may be mild hyper-CK-emia, abnormal motor and sensory nerve conduction studies, and neuropathic and myopathic alterations on muscle biopsy. The golden standard for diagnosing BSMA is genetic analysis, demonstrating a CAG-repeat number >40. No causal therapy is available, but symptomatic therapy should be provided for tremor, endocrinologic abnormalities, sensory disturbances, or muscle cramps. The course is slowly progressive, the ability to walk lost only late in life, only few patients require ventilatory support, and life expectancy only slightly reduced.

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Author and article information

Contributors

Sungchul Kim:

ORCID: http://orcid.org/0000-0003-3580-5290

kscndl@hanmail.net

Journal

Journal ID (nlm-ta): J Pharmacopuncture

Journal ID (iso-abbrev): J Pharmacopuncture

Journal ID (pmc): J Pharmacopunct

Journal ID (doi): 10.3831

Title: Journal of Pharmacopuncture

Publisher: KOREAN PHARMACOPUNCTURE INSTITUTE

ISSN (Print): 2093-6966

ISSN (Electronic): 2234-6856

Publication date (Print): March 2017

Volume: 20

Issue: 1

Pages: 57-60

Affiliations

[1 ]Department of Acupuncture & Moxibustion Medicine, Wonkwang University Gwangju Korean Medical Hospital, Gwangju, Korea

[2 ]Clinical Research Center of Nervous & Muscular System Disease of Wonkwang University Gwangju Korean Medical Hospital, Gwangju, Korea

[3 ]Department of Radiology, Wonkwang University Gwangju Korean Medical Hospital, Gwangju, Korea

[4 ]Department of Nursing, Gwang-Ju Womens University, Gwangju, Korea

Author notes

* Corresponding Author Sungchul Kim. Wonkwang Gwangju Oriental Medical Hospital, 543-8 Juweol 1-dong, Nam-gu, Gwangju 503-310, Korea. Tel: +82-62-670-6441 Fax: +82-62-670-6767 E-mail: kscndl@hanmail.net

Author information

Sungchul Kim http://orcid.org/0000-0003-3580-5290

Article

DOI: 10.3831/KPI.2017.20.009

PMC ID: 5374340

SO-VID: ef972d87-5e5e-4f13-8c05-9e572b4aecbb

License:

This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

The Use of Korean Medicine to Treat Patients with Spinobulbar Muscular Atrophy, Kennedy’s Disease - A Case Study

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Abstract

Objectives:

Methods:

Results:

Conclusion:

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Most cited references 7

Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.

Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trial.

Bulbar and spinal muscular atrophy (Kennedy's disease): a review.

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