Sympathetic skin response in patients with Duchenne muscular dystrophy.
To verify an eventual disfunction of the palmar sympathetic skin activity in Duchenne Muscular Dystrophy, we studied the sympathetic skin response (SSR) with endogenous, Deep Breathing (DB-SSR), exogenous Startle (SE-SSR) and Electric type stimuli (EE-SSR) in a group of patients and a control group. At the same time as the SSR, the heart rate (HR) and the basic R-R interval variation (RRIV) as well as the EMG diaphragmatic activity were checked. In this way, significant differences in the latency (p = 0.02) and amplitude (p = 0.009) values of the EE-SSR and in the amplitude values of the SE-SSR (p = 0.001) and of the DB-SSR (p = 0.02) were noted in the DMD group compared to the control group. In the group of patients the rise in latency of the EE-SSR correlates with the age (p = 0.0001) and with the reduction of its amplitude (p = 0.03). This last parameter correlates with the stage of the illness (p = 0.02). In the control group the mean amplitude of the EE-SSR is approximately 30% greater than that of startle and 50% of deep breathing. Although with amplitudes homogeneously reduced, these ratios are also conserved in the DMD group. Moreover the HR is greater (p = 0.0001) whilst the RRIV is reduced (p = 0.02) in the group of the patients. However these parameters do not correlated either to the cardiac involvement or to the stage of the illness. The presence of the SSR for all the modalities of stimulation indicates the substantial integrity of the afferent and the efferent paths of the ANS. The alterations in latency and amplitude of the SSR in the patients could be the consequence of a lack of dystrophin at the level of the sudoriparous myopithelium in analogy to what has been demonstrated in the mdx mouse.
Department of Neurology, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
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Marcello
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Baratti
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Ortaggio
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Vescovini
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Zanoni
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Tugnoli
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De Grandis
D000293:Adolescent; D000328:Adult; D000344:Afferent Pathways / Q000503:physiopathology; D000367:Age Factors; D002648:Child; D003964:Diaphragm / Q000503:physiopathology; D016189:Dystrophin / Q000032:analysis; D004525:Efferent Pathways / Q000503:physiopathology; D004558:Electric Stimulation; D004562:Electrocardiography; D004576:Electromyography; D005071:Evoked Potentials / Q000502:physiology; D006225:Hand / Q000294:innervation; D006339:Heart Rate; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000503:physiopathology*; D011930:Reaction Time; D013216:Reflex, Startle; D012119:Respiration; D012867:Skin / Q000294:innervation*; D013564:Sympathetic Nervous System / Q000503:physiopathology*
D016189:Dystrophin