Characterizing spinal muscular atrophy with electrical impedance myography.

Electrical impedance myography (EIM) is a non-invasive, painless technique for the evaluation of neuromuscular disease, and here we evaluate its potential application in spinal muscular atrophy (SMA). Twenty-one SMA patients and 18 healthy children underwent EIM of biceps brachii and tibialis anterior using a commercially available impedance device. Hand-held dynamometry and ultrasound assessment of subcutaneous fat thickness were also performed. All EIM parameters differed significantly between both SMA patients and normal subjects and between type 2 and type 3 SMA patients. In addition, EIM had an accuracy level as high as 93% for correctly categorizing patients as type 2 or type 3. Multiple regression analyses confirmed a strong association between EIM and dynamometry. These results confirm that EIM can accurately categorize patients with SMA. Because EIM requires no patient effort and is rapid to apply, it may serve a useful role in future SMA clinical trials.

Foot temperature in healthy individuals: effects of ambient temperature and age.

BACKGROUND: Patient complaints of excessively warm or cold feet are common in medical practice. Such symptoms can be caused by underlying vascular or neurologic disease, and measurement of foot temperature during daily activity and sleep could provide a deeper understanding of their actual thermal basis. METHODS: We used a Thermochron iButton to assess surface foot temperature variation and its relationship to ambient temperature during the day with activity and at night during sleep in 39 healthy individuals aged 18 to 65 years in a temperate region of the United States. We simultaneously used actigraphy to record leg movement. RESULTS: We identified a mean +/- SD awake temperature of 30.6 degrees +/- 2.6 degrees C and asleep temperature of 34.0 degrees +/- 1.8 degrees C, with values reaching as low as 15.9 degrees C in the winter and as high as 37.5 degrees C in the summer. Foot temperature was found to be independent of foot movement or sex; however, there was, as expected, a strong association between foot temperature and ambient temperature (r = .59, P < .001). Several measures of foot temperature variation demonstrated a significant or near-significant reduction with increasing age, including the Euclidean distance (r = -.38, P = .02) for awake periods and the variance (r = -.30, P = .06) during sleep. CONCLUSIONS: These results provide data on the normal variation of foot temperature in individuals living in a temperate climate and demonstrate the potential use of Thermochron iButton technology in clinical contexts, including the evaluation of patients with excessively warm or cold feet.

Electrical impedance myography in the assessment of disuse atrophy.

UNLABELLED: Tarulli AW, Duggal N, Esper GJ, Garmirian LP, Fogerson PM, Lin CH, Rutkove SB. Electrical impedance myography in the assessment of disuse atrophy. OBJECTIVE: To quantify disuse atrophy using electrical impedance myography (EIM), a noninvasive technique that we have used successfully to study neurogenic and myopathic atrophy. DESIGN: We performed EIM of the tibialis anterior of subjects with disuse atrophy secondary to cast immobilization and in their contralateral normal leg. Subjects were studied shortly after cast removal and again several weeks to months after the cast was removed and normal mobility was restored. SETTING: Outpatient neurology and orthopedic practices at a tertiary care medical center. PARTICIPANTS: Otherwise healthy subjects (N=10) with unilateral leg fracture. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Resistance, reactance, and phase measured at 50kHz. RESULTS: The main EIM outcome parameter, phase at 50kHz, was lower in the immobilized leg in 9 of 10 cases. Additionally, when normal mobility was restored, the phase of the casted leg increased relative to its initial measurement in all 10 cases, while it increased inconsistently in the contralateral leg. CONCLUSIONS: EIM may be a powerful tool for the assessment of disuse atrophy.

Localized muscle impedance abnormalities in amyotrophic lateral sclerosis.

OBJECTIVES: To assess changes in electrical impedance myography (EIM) parameters in amyotrophic lateral sclerosis (ALS). METHODS: Ten patients with ALS and a cohort of normal subjects underwent EIM testing of tibialis anterior. Montages using voltage and current electrodes placed at a distance (far) and in close proximity (near) were compared. The EIM parameters, resistance (R), reactance (X), and phase (theta) in the patients with ALS, were compared with normal values. RESULTS: theta measured at 50 kHz using the near montage was most sensitive to the presence of ALS, with 9 of 10 patients with ALS having smaller theta values than the calculated lower limit of normal. theta using the far montage and X using both the near and far montages were also sensitive to disease, whereas R did not seem to be useful. CONCLUSION: EIM is sensitive to muscle abnormalities in ALS, with theta measured with near montages providing the best results.

Impaired distal thermoregulation in diabetes and diabetic polyneuropathy.

OBJECTIVE: To determine how thermoregulation of the feet is affected by diabetes and diabetic polyneuropathy in both wakefulness and sleep. RESEARCH DESIGN AND METHODS: Normal subjects, diabetic subjects without neuropathy, diabetic subjects with small-fiber diabetic polyneuropathy, and those with advanced diabetic polyneuropathy were categorized based on neurological examination, nerve conduction studies, and quantitative sensory testing. Subjects underwent foot temperature monitoring using an iButton device attached to the foot and a second iButton for recording of ambient temperature. Socks and footwear were standardized, and subjects maintained an activity diary. Data were collected over a 32-h period and analyzed. RESULTS: A total of 39 normal subjects, 28 patients with diabetes but without diabetic polyneuropathy, 14 patients with isolated small-fiber diabetic polyneuropathy, and 27 patients with more advanced diabetic polyneuropathy participated. No consistent differences in foot temperature regulation between the four groups were identified during wakefulness. During sleep, however, multiple metrics revealed significant abnormalities in the diabetic patients. These included reduced mean foot temperature (P < 0.001), reduced maximal temperature (P < 0.001), increased rate of cooling (P < 0.001), as well as increased frequency of variation (P = 0.005), supporting that patients with diabetic polyneuropathy and even those with only diabetes but no diabetic polyneuropathy have impaired nocturnal thermoregulation. CONCLUSIONS: Nocturnal foot thermoregulation is impaired in patients with diabetes and diabetic polyneuropathy. Because neurons are highly temperature sensitive and because foot warming is part of the normal biology of sleep onset and maintenance, these findings suggest new potentially treatable mechanisms of diabetes-associated nocturnal pain and sleep disturbance.

Reference values for 50-kHZ electrical impedance myography.

Electrical impedance myography (EIM) is a method for non-invasively and quantitatively assessing muscle health, in which the major outcome parameter, phase (theta), decreases in diseased states. In order to create a set of normal reference values, we performed 50-kHZ EIM in 5 muscles of 87 healthy individuals, using theta as the major outcome variable. Because the distributions of data were mostly skewed, logarithmic transformations were performed, and the resulting data were fitted to quadratic functions. The lower limit of normal was set by plotting the lower 95% confidence interval of the curve for each muscle and then identifying age-specific reference values. We found that the distribution of data was similar to that for other neurophysiologic parameters. The lower limit of normal was easily defined, and relatively few values fell below the proposed lower limit. By using commercially available bioimpedance devices, these values will allow other investigators to explore the application of 50-kHZ EIM in clinical neuromuscular disease research.