[Leprosy and severe neuropathy in two native Spaniards]. / Lepra y neuropatía grave en dos nativos españoles.

INTRODUCTION: Leprosy is a widespread infectious disease in humans that is endemic to regions with poor sanitary conditions, especially in cases of overcrowding, malnutrition and bad hygiene. The disease is characterised by dermopathy, which is quite typical, but above all by neuropathy, which often becomes the most important element. In most cases, alterations to nerves are defined by sensory deficits that are predominantly distal and multiple neuritis in areas where nerve entrapment has taken place. CASE REPORTS: Two patients, both native Spaniards, presented largely overlapping clinical pictures, that is, a history of 'glove and stocking' type paresthesias and dysesthesias going back months or even years and functional impotence, which gave rise to a very pronounced gait disorder. In the two cases, the immunological situation was determined to be borderline lepromatous leprosy. The neurophysiological study revealed the presence of severe, diffuse sensory-motor axonal polyneuropathy that was predominantly distal, and several entrapped nerves. The dermatological illness was greatly improved by the treatment. The same was partially true, although to a satisfactory extent, of the neurological disease. CONCLUSION: We describe the cases of two Spaniards with borderline lepromatous leprosy with no past history of the disease, in whom neuropathy was the predominant symptom. We highlight the speed with which the neuropathies progressed, probably due to a change in 'polarity', and the severity of the neurological deficits in comparison with the dermopathy, in an unusual immunological situation. The growing number of native patients in the first world, even when there is no relevant history, suggests that we should not think of leprosy as something only occurring in immigrant patients from places where it is endemic, although the epidemiological relationship has still not been determined.

Lepra y neuropatía grave en dos nativos españoles / Leprosy and severe neuropathy in two native spaniards

La lepra es una infección humana extendida, endémica de regiones con malas condiciones sanitarias,en especial hacinamiento, malnutrición y mala higiene. La enfermedad se caracteriza por dermopatía, bastante típica; pero, sobre todo, neuropatía, que se convierte con mucha frecuencia en protagonista. La alteración del nervio viene definida en la mayor parte de casos por déficit sensitivos de predominio distal y multineuritis en lugares de atrapamiento nervioso. Casosclínicos. Dos pacientes, españoles indígenas, presentaron cuadros muy superponibles: parestesias y disestesias continuas en guante y calcetín de meses o incluso años de evolución, e impotencia funcional, en uno de los casos provocando un trastornode la marcha muy importante. En los dos se estableció la situación inmunológica como lepra borderline lepromatosa. El estudio neurofisiológico determinó la presencia de polineuropatía axonal sensitivomotora grave y difusa de predominio distal, y varios atrapamientos nerviosos. El tratamiento mejoró muy significativamente la enfermedad dermatológica y, de maneraparcial, aunque satisfactoria, la neurológica. Conclusión. Describimos dos españoles con lepra borderline lepromatosa sin antecedentes, en los que predominó sintomáticamente la neuropatía. Destacamos la rápida progresión de las neuropatías,probablemente por un cambio en la ‘polaridad’, la gravedad de los déficit neurológicos en contraste con la dermatopatía, en una situación inmunológica poco habitual. El número creciente de afectados indígenas del primer mundo, incluso sin antecedentesrelevantes, indica que no sólo debemos pensar en lepra en pacientes inmigrantes procedentes de lugares endémicos, aunque la relación epidemiológica está por determinar

Leprosy is a widespread infectious disease in humans that is endemic to regions with poor sanitaryconditions, especially in cases of overcrowding, malnutrition and bad hygiene. The disease is characterised by dermopathy, which is quite typical, but above all by neuropathy, which often becomes the most important element. In most cases, alterationsto nerves are defined by sensory deficits that are predominantly distal and multiple neuritis in areas where nerve entrapment has taken place. Case reports. Two patients, both native Spaniards, presented largely overlapping clinical pictures, that is, ahistory of ‘glove and stocking’ type paresthesias and dysesthesias going back months or even years and functional impotence, which gave rise to a very pronounced gait disorder. In the two cases, the immunological situation was determined to beborderline lepromatous leprosy. The neurophysiological study revealed the presence of severe, diffuse sensory-motor axonal polyneuropathy that was predominantly distal, and several entrapped nerves. The dermatological illness was greatly improved by the treatment. The same was partially true, although to a satisfactory extent, of the neurological disease. Conclusion. Wedescribe the cases of two Spaniards with borderline lepromatous leprosy with no past history of the disease, in whom neuropathy was the predominant symptom. We highlight the speed with which the neuropathies progressed, probably due to a change in ‘polarity’, and the severity of the neurological deficits in comparison with the dermopathy, in an unusualimmunological situation. The growing number of native patients in the first world, even when there is no relevant history, suggests that we should not think of leprosy as something only occurring in immigrant patients from places where it is endemic, although the epidemiological relationship has still not been determined