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glycoscience pub vol1 no24

The scientific information in this journal is educational and is not to be used as a substitute for a doctor's care or for proven therapy.
PROVIDING SCIENTIFIC INFORMATION RELATED TO NUTRITIONAL SACCHARIDES AND OTHER DIETARY INGREDIENTS.
JULY 15, 2000
VOL 1, NO 24
EXTERNAL EDITORIAL BOARD
INTERNAL EDITORIAL BOARD
Tom Gardiner, PhD
Doris Lefkowitz, PhD
Stephen Boyd, PhD, MD Global Health Safety Environment and Associate Clinical Professor of Microbiology Kathryn Dykman, MD Regulatory Affairs Coordinator University of South Florida Shell Chemical Company (Retired) College of Medicine Bill McAnalley, PhD H. Reginald McDaniel, MD James C. Garriott, PhD, D-ABFT
Stanley S. Lefkowitz, PhD
Professor (Clinical Adjunct Faculty) Professor of Microbiology and Immunology University of Texas Health Science Center Texas Tech School of Medicine Eric Moore, DChem Consulting Toxicologist San Antonio, Texas Robert K. Murray, MD, PhD
Alice Johnson-Zeiger, PhD
Professor (Emeritus), Biochemistry Professor of Biochemistry (Retired) University of Toronto University of Texas Health Center Toronto, Ontario, Canada EDITOR IN CHIEF
Eileen Vennum, RAC Use of a Calcium Channel Blocker and
Glyconutrients to Treat FSH Muscular Dystrophy
Doris L. Lefkowitz, PhD and Stanley S. Lefkowitz, PhD

effects of three different channel blockers, diltiazem,nifedipine, and verapamil, were compared with respect to Muscular dystrophy is a chronic disabling disease with no the progression of disease in dystrophic hamsters.6 These known effective treatment. This report describes the effects investigators reported that of the three drugs employed in of a combination of the prescription drug, diltiazem this study, only diltiazem was effective. (labeled for use as a calcium channel blocker), and certain The following case report describes the beneficial effects glyconutrients on three patients with fascioscapulohumeral of a combination of diltiazem and glyconutritional supple- muscular dystrophy. All three patients experienced objec- ments (including Ambrotose® complex) on three subjects tive improvements in energy, facial muscle tone, strength, with FSHMD. The subjects and their families provided back- and other parameters on the combination therapy. ground and response information to the authors; a neurol-ogist also evaluated them periodically over a 6-month Fascioscapulohumeral muscular dystrophy (FSHMD) is interval following treatment. one of the main subtypes of muscular dystrophy, a group of The first subject was a 56-year-old female who exhibited genetically transmitted diseases. FSHMD is an autosomal primarily facial muscle involvement including eyelid weak- dominant disease characterized by progressive atrophy of ness, and an inability to smile, whistle, or enunciate certain symmetric groups of skeletal muscles, especially the muscles words. She was somewhat low in energy and also exhibited of the face, shoulders, and upper arms; there are no neural asymmetrical arm muscle strength against resistance with a or sensory disorders. While usually not fatal, the disease prominent decrease in the dominant extremity. Genetic typically progresses to all of the voluntary muscles. There is testing revealed a large deletion on chromosome 4, con- no cure and standard treatment consists only of physical firming the symptomatic diagnosis of FSHD. She started on therapy and orthopedic procedures to minimize deformity.1 low levels of glyconutrients (2-4 capsules/day). After There are numerous reports in the lit- approximately 1 month with no erature pertaining to abnormal regula- Muscular dystrophy is a chronic
appreciable symptomatic improve- tion of calcium in various muscular disabling disease with no known
ment, she stopped the glyconutrients dystrophies.2,3,4,5 In one study, the effective treatment.
and started 15 mg of diltiazem twice a The Official Publication of www.usa.GlycoScience.com: The Nutrition Science Site
Published by the Research and Development Department of Mannatech Incorporated, Coppell, Texas, USA. 2000 All rights reserved.
day for 4 days, followed by 15 mg Although some improvement was noted
FSHMD (including decreased facial three times per day for 4 days. The muscle tone and eyelid strength, with the individual approaches, it was
dosage was then increased to 30 mg and hand strength and mobility). He not as dramatic as the combination of
three times a day, a dosage that has could obtain flexion at the elbow diltiazem and glyconutrients.
been maintained to date. One only by using accessory muscles month after initiating the diltiazem, the subject noted (swinging his arm). By his late 40's, he was confined to a improvement in her energy levels. She then reinitiated the wheelchair. This subject was treated with the same schedule glyconutrient supplements (4-6 capsules/day) to see if they of glyconutritionals (Ambrotose® complex) and diltiazem as would provide further improvements. Following several the first subject, except that he consumed larger amounts of weeks of the combined treatment, all disease symptoms glyconutritionals (3 tablespoons/day). In addition, he took were alleviated. The subject exhibited a marked increase in at least five other nutritional products. These included sup- energy and facial muscle tone and arm muscle strength. In plements designed to support energy production and uti- addition, she could smile, whistle, and enunciate all words.
lization, the endocrine system, recovery from athletic exer- After 3 months of treatment, she was essentially asympto- tion, and a phytonutritional and vitamin supplements. He gradually exhibited noticeable improvements: facial muscle The second subject was a 25-year-old male. As a child, he tone was significantly improved, with increased strength of fatigued quickly and by late teens exhibited asymmetrical the eyelids as well as some ability to smile. The subject development of various muscle groups. Prior to treatment, exhibited prominent improvement in biceps strength and he exhibited low energy, ptosis, an inability to whistle, an could fully flex either arm. Hand strength and mobility were inability to smile, rounded shoulders, an inability to stand improved, with the subject being able to form a fist with his erect, abdominal protrusion, scapular winging, and difficul- right hand. A grip test showed an increase from 17 to 30 lbs ty raising his arms above his head. At age 23, genetic tests with the right and 14 to 21 lbs with the left hand. The indicated a large deletion on chromosome 4 indicative of quadriceps femoris seemed slightly stronger. It is interesting FSHMD. This subject started both the diltiazem according to to note that this subject forgot his supplements when he the same regimen as the first subject and the glyconutrients went on a 2-week vacation. Upon his return, he reported the at the same time. With regard to the glyconutritionals, he return of numerous symptoms, including: a loss of energy, took approximately 11/2 tablespoons each of two different generalized weakness (including an inability to hold his supplements each day: one was a glyconutritional head erect), difficulty driving using hand controls, and the (Ambrotose® complex) and the other was a supplement loss of a number of other functions. He immediately restart- designed to support energy production and utilization ed the supplements and is regaining lost functions. (EM.PACTTM sports drink). After several days the subjectreported an increase in energy. After several months, he DISCUSSION
could weakly whistle and his facial muscle tone overall was Two of the three subjects were initially treated with gly- markedly improved with no signs of ptosis. He could smile, conutritionals alone followed by diltiazem alone. albeit slightly asymmetrically. The left scapula was normal Although some improvement was noted with the indi- and the right one exhibited less than 10% abnormal mor- vidual approaches, it was not as dramatic as the combina- phology. His shoulders were no longer rounded and were tion of diltiazem and glyconutrients. The subjects reported essentially of equal size. The pectoralis muscles appeared feeling "more sustained" energy increases using the supple- increased in size and abdominal protrusion was less severe.
ment in conjunction with the diltiazem. Within 1 month of His overall posture was improved, with the subject now the initiation of the combination treatment, all three sub- standing erect and measuring 1 inch taller than he did jects showed improvement, with the most dramatic effects before treatment.
seen in the milder cases. Initially, all three subjects reported The third subject was a 59-year-old severely affected male.
increases in energy, which was followed by increased facial There was an extensive history of the disease in the family; muscle tone and other improvements. many of his relatives including his mother and grandmoth- FSHMD is a condition for which there is no effective er were affected, with the majority being wheelchair-bound.
treatment and for which deterioration is expected. Because His mother reported that the subject exhibited delayed of the unambiguous improvement seen with these three achievement of developmental milestones, such as not subjects, future investigation of calcium channel blockers being able to cry as an infant as well as not being able to sit concomitant with glyconutritional supplements needs to be up unassisted until past his 1st birthday. He was diagnosed stringently investigated using a double blind controlled with FSHMD at approximately 12 years of age. As he grew older, his left arm proximal muscles did not develop nor-mally and, by his late-twenties and early thirties, he had a wide waddling gait, could not walk steps, had trouble get- Ambrotose® complex and EM.PACTTM sports drink are ting up from a chair, as well as other classical symptoms of products of Mannatech, Inc.
1. Mosby's Medical, Nursing, & Allied Health Dictionary. 5th ed. St. Louis, Mo.: Mosby, 1998.
REFERENCE LIST (continued next page)
GlycoScience Vol. 1, No. 24 REFERENCE LIST (continued)
2. Duncan CJ. Role of intracellular calcium in promoting muscle damage: a strategy for controlling the dystrophic 3. Fong PY, Turner PR, Denetclaw WF, Steinhardt RA. Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin. Science. 1990;250(4981):673-676.
4. Imbert N, Cognard C, Duport G, et al. Abnormal calcium homeostasis in Duchenne muscular dystrophy myotubes contracting in vitro. Cell Calcium. 1995;18(3):177-186.
5. Hopf FW, Turner PR, et al. A critical evaluation of resting intracellular free calcium regulation in dystrophic mdx muscle. Am J Physiol. 1996;271(4 Pt 1):C1325-C1339.
6. Johnson PL, Bhattacharya SK. Regulation of membrane-mediated chronic muscle degeneration in dystrophic hamsters by calcium-channel blockers: diltiazem, nifedipine and verapamil. J Neurol Sci. 1993;115(1):76-90.
GlycoScience Vol. 1, No. 24

Source: http://www.fshd-selbsthilfe.de/Diltiazem.pdf

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