[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.

High prevalence of vasomotor reflex impairment in newly diagnosed leprosy patients.

BACKGROUND: Initial nerve damage in leprosy occurs in small myelinated and unmyelinated nerve fibers. Early detection of leprosy in the peripheral nervous system is challenging as extensive nerve damage may take place before clinical signs of leprosy become apparent. PATIENTS AND METHODS: In order to determine the prevalence of, and factors associated with, peripheral autonomic nerve dysfunction in newly diagnosed leprosy patients, 76 Brazilian patients were evaluated prior to treatment. Skin vasomotor reflex was tested by means of laser Doppler velocimetry. Blood perfusion and reflex vasoconstriction following an inspiratory gasp were registered on the second and fifth fingers. RESULTS: Vasomotor reflex was impaired in at least one finger in 33/76 (43%) patients. The fifth fingers were more frequently impaired and suffered more frequent bilateral alterations than the second fingers. Multivariate regression analysis showed that leprosy reaction (adjusted odds ratio = 8.11, 95% confidence interval: 1.4-48.2) was associated with overall impaired vasomotor reflex (average of the four fingers). In addition, palmar erythrocyanosis and an abnormal upper limb sensory score were associated with vasomotor reflex impairment in the second fingers, whereas anti-phenolic glycolipid-I antibodies, ulnar somatic neuropathy and a low finger skin temperature were associated with impairment in the fifth fingers. CONCLUSIONS: A high prevalence of peripheral autonomic dysfunction as measured by laser Doppler velocimetry was observed in newly diagnosed leprosy patients, which is clinically evident late in the disease. Autonomic nerve lesion was more frequent than somatic lesions and was strongly related to the immune-inflammatory reaction against M. leprae.

The stapedial reflex as a topographical marker of proximal involvement of the facial nerve in leprosy. A pilot study.

This study aimed to determine the parameters necessary for a study of stapedial reflexes in leprosy patients to ascertain if the facial nerve is involved more proximally than the stylomastoid foramen. It involved leprosy patients with and without facial nerve involvement and non-leprosy controls. Clinical examination of the patients' ears, a tympanogram and audiogram to exclude conductive and sensorineural deafness, followed by the measurement of a stapedial reflex and the acoustic reflex threshold, were carried out. The number of absent reflexes and the acoustic reflex thresholds did not differ between the three groups of subjects. A definitive study would be logistically impossible. Suggestions are made as to more exact patient selection in order to demonstrate any stapedial reflex changes due to leprosy. The findings of this study do not suggest that facial nerve pathology extends proximally to the stylomastoid foramen, unless such proximal involvement is subclinical to the detection methods used.

Vasomotor reflex testing in leprosy patients, healthy contacts and controls: a cross-sectional study in western Nepal.

OBJECTIVE: To examine test characteristics of laser Doppler vasomotor reflex testing for leprosy and to determine the prevalence of abnormal responses in leprosy patients, healthy contacts and controls. DESIGN AND PARTICIPANTS: Cross-sectional study including 89 leprosy patients (mean age 35 years, 74% male), 36 healthy contacts (29 years, 64% male) and 47 controls (30 years, 68% male), for a total of 172 participants. SETTING: Leprosy hospital in an endemic region 200 km west of Kathmandu, Nepal. OUTCOME MEASURE: Finger-tip and toe-tip vasomotor reflexes elicited by inspiratory gasp were measured using a laser-doppler flow temperature technique. Results were expressed in per cent as the maximal reduction in bloodflow from baseline. RESULTS: For all 12 measurement sites there were highly significant (p > 0.0001 to < 0.004) differences between the three groups tested. Leprosy patients consistently had the lowest responses and controls the highest, with healthy contacts showing intermediate values. Thresholds defined as mean bloodflow reductions among controls minus 1.64 or minus 1.96 standard deviations provided optimal combinations of sensitivity and specificity. Using these cut-off values around 80% of leprosy patients, 50% of healthy contacts and 20% of controls had two or more abnormal reflexes (p < 0.0001 for differences between groups). CONCLUSIONS: In endemic regions, subclinical autonomic neuropathy may be an early but detectable marker for the risk of subsequent leprosy, making early treatment and prevention of transmission possible. Prospective studies are needed to establish the predictive value of abnormal vasomotor reflexes.

Impairment of fingertip vasomotor reflexes in leprosy patients and apparently healthy contacts.

Fingertip blood-flow velocity and its control by vasomotor reflexes were studied in leprosy patients and in healthy controls with a laser Doppler flowmeter. In newly registered patients, the flow was significantly lower than in the healthy controls, and even lower values were recorded in the long-standing patients with lower limb ulcers and/or deformity. The newly registered patients showed substantially impaired vasomotor reflex responses in the fingertips to cold challenge of the opposite hand or deep inspiratory gasp. Low blood flow and impairment of vasomotor reflexes were more prominent in those leprosy patients who showed clinical evidence of neuropathy and/or histological evidence of reaction in a punch biopsy of leprosy skin lesions. This aspect of dysautonomia to cold challenge was particularly prominent in apparently healthy, fully treated ex-patients. There was an unexpectedly high prevalence of impairment of vasomotor reflexes in newly registered and apparently healthy, adequately treated leprosy patients. The method is very sensitive, and it remains to be established whether the lesions it detects are nonprogressive residues, or previous nerve damage, or an indication of on-going nerve damage. A minority of leprosy contacts showed impairment of vasomotor reflexes. Those with two or more affected fingers were more likely to have had a higher level of exposure to Mycobacterium leprae than those with one or no affected fingers. The cause of this unexpected impairment of fingertip vasomotor reflexes in a minority of leprosy control workers has not yet been determined.

Impairment of vasomotor reflexes in the fingertips of leprosy patients.

A method is described for eliciting fingertip vasomotor reflexes by inspiratory gasp and contralateral hand cold challenge. The results of the two tests are reproducible on replicate testing and, when taken together, have proved reliable for detection of impairment of autonomic reflexes in 10 newly registered leprosy patients who did not have any obvious deformity. Similar, but less severe, impairment of vasomotor reflexes was noted in a group of 10 fully treated, apparently cured ex-leprosy patients, none of whom showed clinically obvious neuropathy. Both the new patients and the ex-patients were significantly different from healthy contacts and from healthy Europeans, who were indistinguishable by this test. Evidence is presented suggesting that impairment of these vasomotor reflexes is mainly due to damage to the efferent pathway in the peripheral nerves. The method might prove valuable for investigation of early nerve damage in new patients or during reversal reactions in leprosy at a stage before irreversible damage is done.