Syringomyelia, limb hypertrophy and sympathetic overactivity: a rare association.

A 32-year-old man presented with uniform enlargement of right upper limb for 6 years. Examination revealed painful disorganised elbow joint along with sensory impairment in the affected limb with weakness of small muscles. The patient was given empirical antileprotic therapy from outside without any benefit. Ultrasonography showed pan-hypertrophic nature of local tissues. Although axonal type of sensorimotor neuropathy involving right ulnar and median nerve was detected in nerve conduction velocity study, biopsy of the same failed to confirm any axonal degeneration or evidence of leprosy. Considering the nature of sensory abnormality MRI of cervical spine was done which revealed a syrinx extending from C3 till D2. Tests for vasomotor tone showed positive results on the affected limb. Syringomyelia can rarely give rise to pan-hypertrophy of limb due to sympathetic overactivity, which is infrequently reported in literatures and deficient in logical grounds.

Electrophysiological evaluation of peripheral and autonomic involvement in leprosy.

OBJECTIVE: Motor and sensory nerve conductions, F responses, sympathetic skin responses and R-R interval variations (RRIV) were studied to determine the type of peripheral neuropathy among patients with leprosy. METHODS: Twenty-nine consecutive patients with leprosy (25 male, 4 female) hospitalized in the "Istanbul Leprosy Hospital" between January - December, 1999 were included in this study. Ten patients had borderline lepromatous leprosy, and 19 had lepromatous leprosy. None of the patients studied had the tuberculoid form. The mean age was 55 +/- 12 years. The control group consisted of 30 (26 male, 4 female) healthy volunteers (mean age: 58.1 +/- 7.8 years). All subjects included in the study underwent neurological examination and electrophysiological evaluation. Standard procedures were performed for evaluating sensory and motor conduction studies. Motor studies were carried out on both left and right median, ulnar, tibial and common peroneal nerves while median, ulnar, sural and superficial peroneal nerves were examined for sensory studies. Sympathetic skin response recordings on both hands and RRIV recordings on precordial region were done in order to evaluate the autonomic involvement. RESULTS: The lower extremity was found to be more severely affected than the upper, and sensory impairment predominated over motor. Of 58 upper limbs examined, no sympathetic skin responses was recorded in 46 (79.3%). Compared with the controls, the RRIVs of the leprosy patients were found to be reduced during both resting and deep forced hyperventilation. CONCLUSION: Our results indicate that leprosy causes a predominantly axonal polyneuropathy that is more severe in the lower extremities. Sensory nerve damage is accompanied by autonomic involvement.

R-R interval variation and the sympathetic skin response in the assessment of the autonomic nervous system in leprosy patients.

OBJECTIVES: The aim of this study was to evaluate possible autonomic nervous system (ANS) dysfunction in leprosy patients with the sympathetic skin response (SSR) and the heart rate (R-R) interval variation (RRIV) measurements which are easy and reliable methods for evaluation of autonomic functions. MATERIAL AND METHODS: We studied 37 lepromatous leprosy patients (mean age: 38 +/- 17 years, range 23-62 years, 20 females and 17 males) and 35 age-matched healthy subjects (mean age: 34.19 +/- 12.74 years, range 24-48 years, 20 females and 15 males). Non-invasive bedside tests (orthostatic test, Valsalva ratio), R-R interval variation (RRIV) during at rest and deep breathing, the SSR latency and amplitude from both palms, and nerve conduction parameters were studied in all the subjects. RESULTS: The mean values of RRIV in leprosy patients during at rest [mean RRIV in patients, 17.42 +/- 8.64% vs controls, 22.71 +/- 3.77% (P < 0.05)] and during deep breathing [mean RRIV in patients, 21.64 +/- 9.08% vs controls, 30.70 +/- 5.99% (P < 0.005)] was significantly lower compared with the controls. The mean latency of SSR in leprosy patients [mean SSR latency in patients, 1.72 +/- 1.13 ms vs controls, 1.30 +/- 0.41 ms (P < 0.05)] was significantly prolonged compared with the controls. The mean amplitude of SSR in leprosy patients [mean SSR amplitude in patients, 0.54 +/- 0.57 microV vs controls, 1.02 +/- 0.56 microV (P > 0.05)] was smaller compared with the controls, but this difference was not significant. The mean Valsalva ratio in leprosy patients [mean in patients, 1.11 +/- 0.13 vs controls, 1.16 +/- 0.07 (P > 0.05)] was smaller compared with the controls, but not statistically significant. The mean difference of systolic and diastolic blood pressure between supine rest and during standing in leprosy patients were higher compared with the controls [mean systolic pressure in patients, 7 +/- 6 mmHg vs controls, 6 +/- 8 mmHg (P > 0.05) and mean diastolic pressure in patients, 3 +/- 3 mmHg vs controls, 3 +/- 2 mmHg (P > 0.05)], but they did not reach statistical significance. Furthermore, lower RRIV and the prolonged SSR latencies in leprosy patients were closely correlated to some parameters of sensorimotor nerve conduction and each other [median nerve distal latency and RRIV, r = -0.67 (P < 0.05), ulnar nerve distal latency and RRIV, r = -0.59 (P < 0.05), RRIV and SSR latency, r = -0.33 (P < 0.02)]. These data indicate that leprosy patients have the functional abnormalities of ANS. CONCLUSION: We conclude that combined use of these two tests, both of which can be easily and rapidly performed in the electromyogram (EMG) laboratory using standard equipment, allows separate testing of parasympathetic and sympathetic function, and are very sensitive methods in assessing of ANS function in peripheral neuropathy in leprosy patients.

R-R interval variation and the sympathetic skin response in the assessment of the autonomic nervous system in leprosy patients

OBJECTIVES: The aim of this study was to evaluate possible autonomic nervous system (ANS) dysfunction in leprosy patients with the sympathetic skin response (SSR) and the heart rate (R-R) interval variation (RRIV) measurements which are easy and reliable methods for evaluation of autonomic functions. MATERIAL AND METHODS: We studied 37 lepromatous leprosy patients (mean age: 38 +/- 17 years, range 23-62 years, 20 females and 17 males) and 35 age-matched healthy subjects (mean age: 34.19 +/- 12.74 years, range 24-48 years, 20 females and 15 males). Non-invasive bedside tests (orthostatic test, Valsalva ratio), R-R interval variation (RRIV) during at rest and deep breathing, the SSR latency and amplitude from both palms, and nerve conduction parameters were studied in all the subjects. RESULTS: The mean values of RRIV in leprosy patients during at rest [mean RRIV in patients, 17.42 +/- 8.64 per cent vs controls, 22.71 +/- 3.77 per cent (P 0.05)] was smaller compared with the controls, but this difference was not significant. The mean Valsalva ratio in leprosy patients [mean in patients, 1.11 +/- 0.13 vs controls, 1.16 +/- 0.07 (P > 0.05)] was smaller compared with the controls, but not statistically significant. The mean difference of systolic and diastolic blood pressure between supine rest and during standing in leprosy patients were higher compared with the controls [mean systolic pressure in patients, 7 +/- 6 mmHg vs controls, 6 +/- 8 mmHg (P > 0.05) and mean diastolic pressure in patients, 3 +/- 3 mmHg vs controls, 3 +/- 2 mmHg (P > 0.05)], but they did not reach statistical significance. Furthermore, lower RRIV and the prolonged SSR latencies in leprosy patients were closely correlated to some parameters of sensorimotor nerve conduction and each other [median nerve distal latency and RRIV, r = -0.67 (P < 0.05), ulnar nerve distal latency and RRIV, r = -0.59 (P < 0.05), RRIV and SSR latency,...