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Biomedical subjects

Robert C Griggs

Publications and source records attributed to Robert C Griggs.

15 recordsLinked to original sources
PubMed2006

The current status of treatment for inclusion-body myositis.

There is no established treatment that improves, arrests, or slows the progression of inclusion-body myositis (IBM). Many anti-inflammatory, immunosuppressant, or immunomodulating agents have been administered to patients with IBM but the design of clinical trials was such that it can only be concluded that none produced rapid improvement. The natural history of the disease is for stabilization or improvement in a third of patients for 6 months or more. Thus some agents that did not produce dramatic benefit may have been prematurely abandoned. However, because high-dose prednisone worsens strength while decreasing inflammation but increases amyloid accumulation, alternative targets for intervention and novel treatment strategies are needed.

Anti-Inflammatory Agents↗
PubMed2006

Limb-girdle muscular dystrophy in the United States.

Limb-girdle muscular dystrophy (LGMD) has been linked to 15 chromosomal loci, 7 autosomal-dominant (LGMD1A to E) and 10 autosomal-recessive (LGMD2A to J). To determine the distribution of subtypes among patients in the United States, 6 medical centers evaluated patients with a referral diagnosis of LGMD. Muscle biopsies provided histopathology and immunodiagnostic testing, and their protein abnormalities along with clinical parameters directed mutation screening. The diagnosis in 23 patients was a disorder other than LGMD. Of the remaining 289 unrelated patients, 266 had muscle biopsies sufficient for complete microscopic evaluation; 121 also underwent Western blotting. From this combined evaluation, the distribution of immunophenotypes is 12% calpainopathy, 18% dysferlinopathy, 15% sarcoglycanopathy, 15% dystroglycanopathy, and 1.5% caveolinopathy. Genotypes distributed among 2 dominant and 7 recessive subtypes have been determined for 83 patients. This study of a large racially and ethnically diverse population of patients with LGMD indicates that establishing a putative subtype is possible more than half the time using available diagnostic testing. An efficient approach to genotypic diagnosis is muscle biopsy immunophenotyping followed by directed mutational analysis. The most common LGMDs in the United States are calpainopathies, dysferlinopathies, sarcoglycanopathies, and dystroglycanopathies.

Adolescent↗
PubMed2004

Neurology 2004.

Explore the source record for details and available documents.

Diagnostic Techniques, Neurological↗
PubMed2003

Treatment of idiopathic inflammatory myopathies.

PURPOSE OF REVIEW: This article reviews the results of recent therapeutic trials in dermatomyositis, polymyositis, and inclusion body myositis and suggests an approach to treating patients with inflammatory myopathy. RECENT FINDINGS: We reviewed 10 double-blind, placebo-controlled therapeutic trials in patients with inflammatory myopathy. Only one, using intravenous immunoglobulin in refractory dermatomyositis, indicated benefit. A brief trial of azathioprine in polymyositis and eight studies using various treatments in inclusion body myositis did not show benefit. SUMMARY: There have been no adequate double-blind, placebo-controlled therapeutic trials of dermatomyositis and polymyositis. It is generally accepted, however, that these disorders respond to immunosuppressive agents. Prednisone is usually the initial treatment. There is no agreement on how prednisone should be administered and even less agreement about other agents. Inclusion body myositis, which now appears to be the most common (in adults), is unresponsive to immunosuppressive and immunomodulating therapies. There are candidate treatments for inclusion body myositis and a need for additional double-blind, placebo-controlled therapeutic trials in all patients with inflammatory myopathy.

Controlled Clinical Trials as Topic↗
PubMed2002

Inclusion body myositis.

Inclusion body myositis (IBM) is an inflammatory myopathy with distinctive clinicopathologic features. The etiology of IBM remains elusive. The immune-mediated basis for this disease has been challenged by evidence implicating a number of divergent etiologic factors. These factors include mitochondrial deletions, nitric oxide induced oxidative stress, myonuclear breakdown, and abnormal accumulation within muscle fibers of brain-specific Alzheimer type proteins. The treatment of IBM with conventional immunosuppressive agents has been disappointing. Therapeutic approaches currently under study or consideration are beta-interferon and synthetic anabolic hormones.

Female↗