A preliminary study of locognosia in people affected by leprosy.

Locognosia is the ability to localize a sensory stimulus on the body's surface and can be tested by graded filaments (Semmes-Weinstein monofilaments). This point localization of sensation (locognosia) wastested by SW filaments over four quadrants of the pulp of the fingers in ulnar/median and ulnar paralysis in 38 new patients affected by leprosy. The results were compared with standard testing of sensation at selected sites by Semmes Weinstein monofilament. Both pulp quadrant testing and standard site testing were done in leprosy patients and also in a group of controls. Sensation was tested in 73 hands in leprosy patients and 34 hands in controls. Results indicate a positive correlation between locognosia and standard SW filament testing. When locognosia and standard SW filament tests were compared, there was significant difference between the two tests to pick up abnormal sensation in leprosy patients both over the entire hand and over individual fingers. This preliminary study suggests that locognosia may be a useful tool to diagnose sensory impairment in leprosy. Further studies are required to corroborate this.

Digital neuropathy of the median and ulnar nerves caused by Dupuytren's contracture: Case report.

INTRODUCTION: Digital neuropathy is a pure sensory neuropathy of a digital nerve. It may be caused by acute or chronic local trauma or pressure, or accompany systemic illnesses such as rheumatoid disease, leprosy, Raynaud disease, dysproteinemia, or diabetes mellitus. We describe an extraordinary case of digital neuropathy of the median and ulnar nerves caused by Dupuytren contracture. CASE REPORT: A 56-year-old right-handed man was presented with numbness and tingling of the little finger of the right and ring finger of the left hand. The clinical and EMG findings in this patient were consistent with a lesion of the median and ulnar palmar digital nerves of the right and left ring and little fingers. CONCLUSION: Dupuytren tissue usually affects the palmar fascia, superficial to the digital nerves, and it may rarely affect the spiral cord in the digits. A spiral cord may cause sensory loss due to impingement of digital nerves or Dupuytren tissue may have been compressing the palmar digital nerves against the relatively inelastic deep transverse metacarpal ligament. As a result, digital neuropathy can develop in those with Dupuytren's contracture, and nerve conduction studies should also be performed to determine the condition. New studies are needed to provide better diagnostic criteria for the condition.

[Flexor digitorum superficialis tendon transfer for intrinsic paralysis in leprosy]. / Transfert tendineux palliatif utilisant le Flexor Superficialis Digitorum dans les paralysies intrinsèques d'origine lépreuse.

A follow up study was performed in the rehabilitation centre for patients with leprosy in Hôchiminhville - Vietnam. All patients had claw-hand deformity due to ulnar and median nerve intrinsic paralysis. Thirty-two affected hands (128 long fingers) were included in the study. A Bunnel-Littler tendon transfer procedure was performed using a four-tailed graft of the flexor digitorum superficialis of the third finger. Clinical evaluation included anatomical measurements of interphalangeal and metacarpal joints in complete extension and in the intrinsic position. In the open hand assessment, 48.5% reported good results, 14.8% medium results and 36.7% poor results. With the hand in the intrinsic position, 53.9% achieved good results, while 33.6% achieved medium results and poor in 12.5%. Poor functional outcome is related to a failure of this procedure and seems to be due to extensor tendon laxity, with or without stiffness of the interphalangeal joints. There were many anatomical deformities (27.3%) found at the time of follow up, notably boutonniere (51.4%) and mallet finger deformities (31.4%) The fourth and fifth fingers had the worst results. We have therefore decided to change our protocol for claw-hand correction and use the Bouvier test in deciding on our surgical indications. Preoperative physiotherapy is absolutely necessary to reduce stiffness of the interphalangeal joints.

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.

Assessment of results of opponensplasty.

One hundred and fifty-six opponensplasties carried out on 115 patients at Anandaban Hospital between 1987 and 1997 were reviewed. In most cases a flexor digitalis superficialis opponensplasty was performed. The outcome was assessed by measuring the finger to which the thumb could obtain a pinch grip, the gap between the thumb and little metacarpophalangeal joints, and the satisfaction of the patient. The objective assessments demonstrated excellent or good results in 89%. Good or fair patient satisfaction was obtained in 93%. Early complications were seen in seven cases (4%). Objective measurements of outcome and patient satisfaction were not always in agreement, indicating that objective measures do not adequately assess the success of surgery from the patient's perspective. We thus conclude that subjective measurements of results are an important measure of success and should be included in the evaluation of surgical results.

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.

Correction of claw fingers in leprosy--current status.

The operative procedures available for correction of claw fingers have been classified according to the principles involved in correction. The relative merits and demerits of the procedure have been briefly mentioned and an attempt has been made to define proper indications of these procedures.

[Peripheral nerve lesions of experimental leprosy in monkeys. VII. Intrafascicular-edema and small blood vessels].

Histopathological findings of intrafascicular-edema found in n. ulnaris running at forearm, palmside of hand and fingers of rhesus monkey 8664 and african green monkey 8175 were studied by semithin section method. These two monkeys were inoculated with leprosy bacilli from lepromata of mangabey monkey A022. The mangabey monkey A022 had been experimental leprosy after inoculum of the leprosy bacilli from lepromata of the mangabey monkey A015-natural infection. Period between date of inoculum and sacrifice of the rhesus monkey 8664 was about 2 years, and the african green monkey 8175 was about 5 years. Results found were: 1. Intrafascicular-edema found in n. ulnaris running at forearm, palmside of hand and fingers was remarkable at some of peculiar anatomical areas where were put on mechanical pressure. 2. The intrafascicular-edema inside many fascicles was remarkable around the small blood vessels. 3. Obsruction of the small blood vessels and congestion of blood stream at surrounded of the intrafascicular-edema with fibrous elements and cellular elements were observed inside some of fascicles. 4. Bundles of peripheral nerve fibers inside some of fascicles were pressed by the intrafascicular-edema, and the fibrous elements, especially collagen fibers, were increased around the pressed bundles of nerve fibers and each nerve fiber inside the fascicles. 5. Vacuolar degeneration and swelling of axon were observed at some of myelinated nerve fibers. 6. Not only the intrafascicular-edema but also a large amount of intracytoplasmic foamy structures with solid leprosy bacilli were observed inside many fascicles.(ABSTRACT TRUNCATED AT 250 WORDS)

[Failure to form wrinkles on the finger tips in the warm water test in lepromatous nerve damage]. / Fehlende Faltenbildung der Fingerkuppen im Warmwassertest bei lepromatöser Nervenschädigung.

In order to estimate the extent of nerval defects, we recommend the soaking test which may reveal affected areas indicated by the loss of wrinkling at the finger-tips after soaking the hands in warm water for half an hour. Thus we found impairment in the formation of wrinkles in 6 out of 7 tested patients suffering from different types of leprosy. In order to study the significance of the soaking test with regard to acute nerval signs and symptoms, one patient was reexaminated when he showed acute painful swelling of the right n. ulnaris. There was normal wrinkling observed at the affected hand, which did not differ from the outcome of the preceding test. This finding indicates that the soaking test is only appropriate to the examination of chronic nerval lesions in leprosy.

Lack of wrinkle formation in the fingertips of patients with Hansen's disease. Confirmation of previous observations.

Immersion of a hand in water at 38-40 degrees C for half an hour was performed in two phases with a one year interval in 38 patients with Hansen's disease and 20 controls. In all 20 controls wrinkles were produced. However, 32 of the 38 patients failed to show wrinkles in the fingertips. In 12, who participated in both phases, results of the second test confirmed those of the first. This may be an indication of irreversible damage. Due to the small number of individuals tested, no conclusions may be drawn in reference to groups and types of the disease. Provided that these results can be confirmed in larger groups of patients, this method could prove to be a tool in the diagnosis of Hansen's disease. Since the test is very easy to perform, it would be of great value in areas far from specialized medical centers.

Nerve conduction in leprosy: in vivo and in vitro study.

Sensory and mixed nerve conduction studies were performed on the distal segments of the radial, sural, median and ulnar nerves of leprosy patients from the entire spectrum of the disease. The values obtained were compared with those of age matched normal controls. All the clinically involved nerves, and about 30% of the clinically normal patient nerves showed a delayed conduction velocity. The conduction velocity difference was most significant (P less than 0.001) for the cutaneous branch of the radial, and sural nerves as compared to the ulnar and median. Five patients nerves that were clinically normal and showed a normal conduction velocity when tested in vivo, were biopsies and studied in vitro to determine the type of early nerve damage. C and A delta fibre involvement was found in all these nerves. It is concluded that leprosy is a diffuse neuropathy and there is early detectable nerve involvement even in clinically normal nerves of leprosy patients.

[Absence of the formation of wrinkles of the finger tips in leprosy]. / Fehlende Faltenbildung der Fingerkuppe bei Lepra.

Soaking of the hand of leprosy patients in water of 38 degrees--40 degrees C during half an hour does not lead to the formation of wrinkles, in contrast to healthy individuals. We tend to relate this lack of wrinkles to the damaged nerve ends which are often found in leprosy patients. The number of investigated individuals is small and repeated experiments are therefore required. Should the above results be established, the soaking test could serve as an additional tool for the diagnoses of leprosy.

Study of evolution of nerve damage in leprosy. Part II--Observations on the index branch of the radial cutaneous nerve in contacts of leprosy.

A total of 35 contacts from 20 families of lepromatous index cases were screened clinically. All of them were subjected for nerve conduction velocity (NCV) studies of both left and right index branch of the radial cutaneous (IRC) nerve. Fifteen of these nerves were biopsied. Out of 15 IRC nerves biopsied 10 had slightly reduced average NCV values and 5 were normal. Four nerves out of 10 where average NCV was reduced and 1 nerve out of 5 where NCV was normal showed significant ultrastructural changes and higher percentage of segmental demyelination (10%--13%). There was a striking similarity between the changes seen in the IRC nerves of early cases of leprosy and of contacts.

Study of the evolution of nerve damage in leprosy. Part I--Lesions of the index branch of the radial cutaneous nerve in early leprosy.

A total of six index finger branch of the radial cutaneous (IRC) nerves from three BL, two BB and one BT cases of leprosy, with less than 6 months history of disease were subjected for nerve conduction velocity (NCV) studies followed by biopsy. The biopsy was divided into three parts and subjected for light microscopy quantitative histology, electron-microscopy and fibre tease studies. The nerves revealed a slight reduction in NCV and a varying degree of segmental demyelination ranging from 10% to 35%. The earliest changes observed were the thickening and proliferation of Schwann cell processes of the unmyelinated fibres and degeneration of their axons regardless of the type of leprosy.

Skeletal deformities of the denervated hand in Hansen's disease.

Skeletal deformities of the denervated hand are subtle in their development and pose a more formidable problem than do primary deformities of Hansen's disease. Basic osseous changes consist of concentric, longitudinal, or the longitudinal varying methods of absorption. Knowledge of their pathogenesis provides the basis for their management. Prevention of deformity and conservative management of secondary complications are necessary to salvage as much of the hand as possible.

Quantitative, histologic and ultrastructural studies of the index branch of the radial cutaneous nerve in leprosy and its correlation with electrophysiologic study.

A correlative study of clinical, electrophysiological, quantitative, histopathologic and ultrastructural changes seen in 13 instances of the index branch of the radial cutaneous nerve was undertaken in leprosy patients. Testing by graded nylon proved to be more reliable than any other conventional clinical tests. The quantitative studies revealed that small-sized fiber loss was encountered in the early stages of nerve involvement in leprosy followed by loss of large-sized fibers with or without increase in small-sized fibers. Segmental dymyelination of small-sized fibers was seen in early stages of degeneration in leprosy nerves irrespective of type of leprosy. Wallerian degeneration was encountered in advanced stages with total destruction of the nerves. At the ultrastructural level clinically normal nerves of leprosy patients (LN series) showed minimal but significant changes, such as thickening of the basement membrane of perineurial cells, as well as an increase in the amount of collagen between the perineurial layers. Marked increases in the amount of endoneurial collagen were noticed. Axons devoid of myelin, probably demyelinated fibers, were occasionally observed. Slight proliferation of the basement membrane of the endothelial cells was also observed. These changes were of a more advanced nature in the clinically involved cases of leprosy (L series). Two BL types of cases from the L series, showed presence of M. leprae in Schwann cell cytoplasm of myelinated and unmyelinated fibers, perineurial cells, in endothelial cells and macrophages. In cases having impaired sensory modalities (L series), thickening of the perineurium was due to increase in the number of layers of perineurial cells in addition to the increase in collagen. Probably one of the ways in which unmyelinated fibers degenerate is by splitting the Schwann cell columns and extrusion of the axons. Collagen pockets were seen in the LN series of cases. These are probably the degenerated nonmyelinated fibers being replaced by collagen fibrils. Postoperative clinical charting was done on the patient whose IRC nerves were removed. It showed complete restoration of all modalities of sensation in the area of its distribution by the end of three months.

Clinical, electrophysiological, quantitative, histologic and ultrastructural studies of the index branch of the radial cutaneous nerve in leprosy. I. Preliminary report.

The index branch of the radial cutaneous nerve has been demonstrated as a constant nerve which can readily biopsied under local anesthesia and yields a nerve which is of suitable size for quantitative and qualitative studies both by light and electron microscopy. It supplies a limited but constant area where the sensory loss does not disturb the patient. Definite ultrastructural changes have been demonstrated in the clinically normal nerves of leprosy patients. These nerves have also revealed a loss in the small myelinated and unmyelinated fibers, which correlated with the common clinical findings of the absence of sweating and dissociated sensory loss in this disease. Gross damage has been encountered in nerves which showed only early signs of clinical damage even with refined methods of sensory testing. These nerves would have passed as normal on routine testing by No. 5 nylon. Regeneration of small fibers was noted following loss of large size fibers. Nerve conduction velocity may be a useful tool in early diagnosis.