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Biomedical subjects

R A Nardin

Publications and source records attributed to R A Nardin.

6 recordsLinked to original sources

A pilot randomized trial of oxandrolone in inclusion body myositis.

BACKGROUND: Inclusion body myositis (IBM) remains without effective therapy. As anabolic steroids have myotrophic properties, the authors studied whether a synthetic androgen, oxandrolone, would have efficacy in IBM. METHODS: A double-blind, placebo-controlled, crossover design was used. Patients received oxandrolone or placebo for 12 weeks followed by a minimum 2-month washout period, followed by 12 weeks of the alternative treatment. Maximal voluntary isometric contraction testing (MVICT), manual muscle testing (MMT), and functional performance testing were obtained before and after each treatment period, with the whole-body MVICT score as the primary outcome measure. RESULTS: Of 19 patients enrolled, 16 (14 men, 2 women; median age 68.5 years) had complete data for at least the first treatment period, with 13 completing the entire study. Whole-body MVICT improved by a median of 15.5 kg with drug and 4.1 kg with placebo (p = 0.06), whereas MMT demonstrated a median increase of 2.0 Medical Research Council points with drug and 0.9 point with placebo (p = 0.33). Upper-extremity MVICT demonstrated a significant treatment effect, with strength increasing a median 6.3 kg with drug vs 2.5 kg with placebo (p = 0.006). Stair climbing also increased a median of 1 step on average with drug versus no change with placebo (p < 0.001). Minimal adverse effects occurred. CONCLUSIONS: Oxandrolone had a borderline significant effect in improving whole-body strength and a significant effect in improving upper-extremity strength as measured by MVICT. Given these findings, further study of this drug, possibly in combination with an immunomodulating agent, is warranted.

Aged↗

Mitochondrial dysfunction and neuromuscular disease.

Mitochondrial diseases are a heterogeneous group of disorders with widely varying clinical features, due to defects in mitochondrial function. Involvement of both muscle and nerve is common in mitochondrial disease. In some cases, this involvement is subclinical or a minor part of a multisystem disorder, but myopathy and neuropathy are a major, often presenting, feature of a number of mitochondrial syndromes. In addition, mitochondrial dysfunction may play a role in a number of classic neuromuscular diseases. This article reviews the role of mitochondrial dysfunction in neuromuscular disease and discusses a rational approach to diagnosis and treatment of patients presenting with a neuromuscular syndrome due to mitochondrial disease.

Humans↗

Electromyography and magnetic resonance imaging in the evaluation of radiculopathy.

Electromyography (EMG) and magnetic resonance imaging (MRI) are commonly used in the diagnosis of cervical and lumbosacral radiculopathy, but the agreement between the two studies is unknown. We retrospectively studied 47 patients with a clinical history compatible with either cervical or lumbosacral radiculopathy who were evaluated with both an EMG and a spine MRI within 2 months of each other. Among these patients, 55% had an EMG abnormality and 57% had an MRI abnormality that correlated with the clinically estimated level of radiculopathy. The two studies agreed in a majority (60%) of patients, with both normal in 11 and both abnormal in 17; however, only one study was abnormal in a significant minority (40%), suggesting that the two studies remain complementary diagnostic modalities. The agreement was higher in patients with abnormal findings on neurologic examination, underscoring the difficulty of confirming the diagnosis in mild radiculopathy.

Adult↗

Fibrillations in lumbosacral paraspinal muscles of normal subjects.

Although paraspinal muscle fibrillations and positive sharp waves (PSWs) are used to help identify neuromuscular disorders, the frequency of these abnormalities in normal subjects is uncertain. We performed lumbosacral paraspinal electromyography in 65 normal subjects. Twenty-seven (42%) had fibrillations and/or PSWs, with the prevalence of these findings increasing with age (r = 0.830, P = 0.040). These data suggest isolated fibrillations and PSWs in lumbosacral paraspinal muscles, especially of older subjects, are nonspecific findings.

Adult↗

Sural/radial amplitude ratio in the diagnosis of mild axonal polyneuropathy.

As proximal nerves are relatively spared in length-dependent, axonal polyneuropathy, we theorized that a sural/radial amplitude ratio (SRAR) might be a sensitive indicator of mild polyneuropathy. In this study, sural amplitudes and SRARs in patients with signs of mild axonal polyneuropathy were compared to those of normal, age-matched control subjects. Sural and radial sensory responses were measured in a standard fashion in all subjects. Thirty polyneuropathy patients had an average SRAR of 0.29 as compared to 0.71 for the 30 normal subjects. An SRAR of less than 0.40 was a strong predictor of axonal polyneuropathy, with 90% sensitivity and 90% specificity, as compared to an absolute sural amplitude of less than 6.0 microV, which had sensitivity of only 66%. Additionally, unlike the sural amplitude, the ratio did not vary significantly with age. We conclude that the SRAR is a sensitive, specific, age-independent electrodiagnostic test for mild axonal polyneuropathy.

Adult↗

Myotonia in colchicine myoneuropathy.

Colchicine may induce a myoneuropathy in patients with renal insufficiency. To date, myotonia has not been described in this disorder. We recently studied 4 patients treated with routine doses of colchicine who, in the setting of renal insufficiency, developed a severe myoneuropathy characterized by prominent myotonic discharges on electromyography. In addition, 1 of the 4 patients had profound clinical myotonia. In the 3 patients in whom biopsies were performed, marked myopathic change with intracytoplasmic vacuolization was identified. All 4 patients improved rapidly with discontinuation of the medication. The patient in whom electrophysiologic studies were repeated had a complete resolution of the myotonic discharges. Colchicine myoneuropathy can present with prominent clinical and electrophysiologic myotonia that resolves completely with discontinuation of the medication.

Adult↗