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.

Estudo do ciclo pupilar e de algumas manifestaçöes disautonômicas em portadores de hanseníase / Study of the pupil cycle time and some manifestations of autonomic involvement in leprosy patients

Em nosso estudo, realizou-se a avaliaçäo funcional da inervaçäo autonômica da íris de portadores das várias formas de manifestaçäo clínica da hanseníase através da medida do tempo do ciclo pupilar. Concomitantemente, procurou-se pesquisar a funçäo lacrimal, o comportamento da pressäo arterial diante das variaçöes posturais, as alteraçöes da temperatura corporal e a ocorrência de sintomas relacionados com o comprometimento do sistema nervoso autônomo. Foram incluídos 81 participantes, divididos em dois grupos: o Grupo I, composto por 63 portadores de hanseníase, e estratificado em cinco subgrupos (HT, HB, HBB, HBL e HL), dependendo da forma de manifestaçäo clínica da doença, e o Grupo II ou grupo controle, composto por 18 indivíduos hígidos. Limitamos a idade de todos os participantes entre 16 e 45 anos. Os portadores de hanseníase estavam em tratamento regular, tinham o tempo de diagnóstico inferior a 3 anos, grau de incapacidade 0 ou 1 e näo apresentavam surto reacional no momento da avaliaçäo. A medida do tempo do ciclo pupilar, realizada segundo a padronizaçäo de Miller & Thompson (1978), demonstrou ser um método ambulatorial näo invasivo, simples e objetivo para a avaliaçäo funcional da inervaçäo autonômica da íris. O tempo do ciclo pupilar observado nos integrantes dos Subgrupos HT e HBT foi estatisticamente semelhante ao dos indivíduos hígidos enquanto o tempo do ciclo pupilar dos integrantes dos Subgrupos HBB, HBL e HL apresentou um prolongamento significativo. Dentre os outros sinais disautonômicos pesquisados, apenas a frequência de disfunçäo lacrimal nos portadores das formas HBT e HL foi significativamente maior em relaçäo à dos indivíduos hígidos. Näo se verificou a ocorrência de hipotensäo arterial ortostática e nem de alteraçöes da temperatura corporal nos portadores de hanseníase. No entanto, a frequência de alguns sintomas disautonômicos foi significativamente aumentada no Grupo I em relaçäo ao Grupo II; esses sintomas foram mais frequentes no Subgrupo HL e menos no Subgrupo HT.

Respiratory dysautonomia in leprosy.

Autonomic neuropathy in leprosy has been reported recently. 25 cases of polar lepromatous leprosy with and without lepra-reaction and 25 normal control, have been studied for respiratory dysautonomia. In 12 cases out of 25 (48%) involvement of sympathetic respiratory reflexes has been observed. Possible causes of the same are discussed.

Cardiac dysautonomia in lepromatous leprosy.

Cardiovascular autonomic functions were studied in 32 patients of lepromatous leprosy, including 12 patients of lepra reaction. Fifty age and sex matched healthy subjects served as controls. Variable degree of autonomic dysfunction was observed in the study group. The derangement of autonomic functions in patients of leprosy can be explained on the basis of neurotrophic action of lepra bacilli that infiltrate the sympathetic and the parasympathetic fibres.

Autonomic neuropathy in leprosy.

The integrity of the autonomic control of the cardiovascular system was studied in 21 patients with lepromatous leprosy and in ten normal people using several simple tests based on cardiovascular reflexes. Impairment of both parasympathetic and sympathetic function was demonstrated in the lepromatous patients.