ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

PurposeIn tins retrospective study, positive results and side effects of long-term steroid treatment for Duchenne muscular dystrophy were analyzed. Results of an average follow-up period of 65 months (range, 49–79 mo) are described.MethodsNineteen male patients receiving steroids (0.9 mg/kg deflazacort) were compared with the natural history of Duchenne muscular dystrophy given in the literature. Additionally, 13 of these 19 patients were compared with an age-matched control group of 13 patients with Duchenne muscular dystrophy who were not receiving steroid treatment. They were not treated because of loss of independent walking before their first examination in our department or after informed consent with the parents. The same investigators followed all patients.ResultsMuscle strength, functional grade according to Vignos scale, and timed functional tests were significantly better in patients on steroids. The vital capacity was significantly improved in treated versus untreated boys; cardiologic examinations revealed no obvious difference. The main side effects of steroids in patients with Duchenne muscular dystrophy were obesity (2 of 13), cataracts (6 of 13), and short stature (II of 13). Rates of infectious diseases or of bone fractures resulting from osteoporosis were not increased.ConclusionLong-term steroid treatment in Duchenne muscular dystrophy (average. 65 mo) proved beneficial m terms of muscle strength, function, and tolerable side effects, as compared with untreated patients with Duchenne muscular dystrophy.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Graft versus host disease (GVHD) is a frequent complication of bone marrow transplantation (BMT). Peripheral neuropathies in association with chronic GVHD are uncommon. We report a patient with BHT for chronic myeloid leukemia, and chronic GVHD with severe sclerodermatitis resulting in bilateral medial antebrachial and saphenous sensory mononeuropathies.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Neuralgic amyotrophy (NA) is a syndrome of unknown etiology associated with pain in the necl“ and shoulder girdle followed by wasting and weakness in muscles supplied by the brachial plexus. Electromyographic studies typically show evidence of denervation in affected muscles. In this report, two cases of NA, both of which presented in the postpartum period, ace described. Both of the described cases demonstrated severe involvement of the anterior interosseous nerve. The course and electrophysiological assessment of postpartum NA is discussed.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Subcutaneous calcifications occur in a variety of diseases, including juvenile dermatomyositis. These calcifications cause disabling symptoms that do not always respond to immunosuppressant therapy. The calcium antagonist diltiazem reduces subcutaneous calcifications in CREST syndrome and in isolated cases of children with dermatomyositis. Our study was performed to determine the effects of diltiazem when used as adjunctive therapy in children with dermatomyositis.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Despite the more recent genetic classification of the novel myotonic dystrophies into at least two distinct myotome dystrophy (DM) loci (DM1, DM2/PROMM/PDM), the clinical characteristics of these dominant myotonic syndromes often blur. The multisystemic nature of these disorders leads to a spectrum of symptoms and signs th.it emphasize the similarities and differences between these diseases. This review focuses en the clinical, laboratory, and genetic findings in 3q-linked and unlinked Italian families with proximal myotonic myopathy (PROMM). From a neuromuscular and cardiac point of view, we present follow-up data fom 25 patients studied serially over a 6-year period. We also present data on central nervous system involvement following neuropsychologic arid neuroimaging studies in this same period of time.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Unexplained painful burning feet are common complaints among the elderly. Conventional elettrodiagnostic testing may be normal or only minimally abnormal and the lack of a universally accepted diagnostic test for small fiber dysfunction has posed a significant impediment to evaluation of these patients. The increasing application of newer diagnostic techniques, including quantitative sensory testing, tests of sudomotor function, and skin biopsy for intrnepidermal nerve fiber quantification, has permitted recognition and characterization of the idiopathic painful sensory neuropathies This article reviews the clinical features, evaluation, and management of this syndrome.

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2001

数据来源: OVID