Slow progressive muscle wasting and weakness in childhood, developing usually before the fifth year. Peroneal muscular atrophy. Few survive after adolescence. “Waddling” gait, frequent falls, deformity. Another type: facio-scapular-humeral develops in early adult life. No cure possible.
Associated with a deficiency of taurine, (an amino acid) and Vitamin E.
Treatment. No specific therapy. Surgery sometimes able to correct. Herbs to support the constitution. Herbs contain vital complexes of minerals which may arrest progress of the disease.
Teas: Plantain, Alfalfa, Fenugreek seeds.
Tablets/capsules. Alfalfa, Kelp, Irish Moss, Saw Palmetto, Damiana.
For pain. See: ANTISPASMODIC DROPS. Wild Lettuce.
Evening Primrose. 4 × 500mg capsules, daily.
Diet. Bananas for potassium. Oats: oatmeal porridge.
Vitamins. A. B6. B12. C. E. Pantothenic acid.
Minerals. Dolomite, Potassium. Zinc.
Aromatherapy. Massage spine. Three drops each – Rosemary and Lavender in 2 teaspoons Almond oil. Treatment by or in liaison with a general medical practitioner.
A group of rare inherited muscle disorders which cause slow, progressive wasting away of muscle fibres. This degeneration may lead to disability and death.
The most common and severe form of muscular dystrophy is Duchenne muscular dystrophy. This is caused by a recessive gene carried on the X chromosome (see sex-linked inheritance). Boys only have one X chromosome, so if they inherit a copy of the defective gene from their mother they develop the disorder. Girls (with two X chromosomes) are not affected but become carriers of the defective gene. Affected boys walk with a waddle, find climbing difficult, and may have curvature of the spine. The disorder progresses rapidly: the ability to walk is lost by the age of 12, and few boys survive beyond the teenage years.
Becker’s muscular dystrophy starts later in childhood and progresses more slowly. Myotonic dystrophy affects the
muscles of the hands, face, neck, and feet, and causes learning difficulties. Limb-girdle muscular dystrophy mainly affects muscles in the hips and shoulders, and facioscapulohumeral muscular dystrophy affects muscles in the upper arms, shoulder girdle, and face. In this last form, severe disability is rare.
A diagnosis for Duchenne muscular dystrophy can be made with gene testing before symptoms develop. Once muscle weakness develops other tests become useful, including measurement of muscle enzymes and an EMG.
There is no cure, and physiotherapy is the main treatment. Remaining as active as possible keeps healthy muscles in good condition. Surgery to the
heel tendons may assist walking in some cases. The long-term outlook depends on the particular form.
Families in which a child or adult has developed any form of muscular dystrophy should receive genetic counselling.
See MUSCLES, DISORDERS OF – Myopathy.
a group of muscle diseases, marked by weakness and wasting of selected muscles, in which there is a recognizable pattern of inheritance. The affected muscle fibres degenerate and are replaced by fatty tissue. The muscular dystrophies are classified according to the patient’s age at onset, distribution of the weakness, the progression of the disease, and the mode of inheritance. Isolated cases may occur as a result of gene mutation. Confirmation of the diagnosis is based upon *electromyography and muscle biopsy.
One common form is Duchenne’s muscular dystrophy, which is inherited as a *sex-linked recessive character and is nearly always restricted to boys. It usually begins before the age of four, with selective weakness and wasting of the muscles of the pelvic girdle and back. The child has a waddling gait and *lordosis of the lumbar spine. The calf muscles – and later the shoulders and upper limbs – often become firm and bulky. Although the disease cannot be cured, physiotherapy and orthopaedic measures can relieve the disability. The identification of the gene abnormality raises the possibility of *gene therapy in the future. See also Becker muscular dystrophy; dystrophia myotonica.
a *sex-linked (X-linked) disorder in which affected males develop an increase in muscle size followed by weakness and wasting. It usually starts between the ages of 5 and 15, and 25 years after onset most patients are wheelchair-bound. Although most men become severely disabled, life expectancy is close to normal. The disorder is similar to Duchenne *muscular dystrophy but less severe. [P. E. Becker (20th century), German geneticist]... becker muscular dystrophy
a hereditary condition in which the corneal endothelium loses its functional ability, usually with age. It may result in thickening and swelling of the cornea (*bullous keratopathy) and thus affect vision. Cornea guttata, small whitish deposits of hyalin, are seen on the inner surface of the cornea and signify a reduced number of endothelial cells. A corneal transplantation (see keratoplasty) may become necessary in certain cases. [E. Fuchs (1851–1913), German ophthalmologist]... fuchs’ endothelial dystrophy
(SMA) a hereditary condition in which cells of the spinal cord die and the muscles in the arms and legs become progressively weaker. Eventually the respiratory muscles are affected and death usually results from respiratory infection. Most affected individuals are wheelchair-bound by the age of 20 and few survive beyond the age of 30. The gene responsible has been located: in affected children it is inherited as a double *recessive. There are three forms of the disease, based on severity of the symptoms and the age at which they appear. Type 1 (infantile spinal muscular atrophy) is the most acute and aggressive form of the condition (see Werdnig–Hoffmann disease). Type 2 develops between the ages of 6 months and 2 years and type 3 (Kugelberg–Welander disease), the least severe form, appears between 2 and 17 years of age.... spinal muscular atrophy
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