eMedicine Specialties > Neurology > Neuromuscular Diseases

Neuropathy of Leprosy: Follow-up

Author: Ramaratnam Sridharan, MD, FRCP, FAAN, Head of the Department of Neurology, Professor, Neurology, Chennai Neurospeciality & Research Institute
Coauthor(s): Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants; Lakshmi Narasimhan Ranganathan, MD, Assistant Professor, Department of Neurology, Stanley Medical College, India; Srinivasaraman Govindarajan, DMRD, DNB, Staff Physician, Barnard Institute of Radiology, Madras Medical College, India
Contributor Information and Disclosures

Updated: Feb 27, 2007

Follow-up

Deterrence/Prevention

• Periodically examine contacts of persons with MB leprosy. This is essential to detect and treat disease in its early stages.
• Several studies of 1 or more antileprosy drugs (eg, dapsone, long-acting injectable dapsone [acedapsone], rifampicin) as chemoprophylaxis failed to demonstrate any significant protection against leprosy. Therefore, at present, the only practical method of prevention is early detection and MDT in all patients with leprosy.
• BCG or other antileprosy vaccines may increase immunity to the disease and help in prevention.
• Isolation or leprosarium is not indicated. Viability of bacteria in skin biopsy samples decreases sharply within 3 weeks of the start of therapy with dapsone and rifampicin. Family members have already had prolonged exposure to the patient before diagnosis.

Complications

• Deformities and trophic changes may occur.
  
  
  • Deformities may be due to direct effects of proliferation of M leprae (eg, collapse of nasal septum and rarely, direct invasion of phalanges with pathologic finger fractures).
  • Deformities are usually due to neuropathy. These include crippling deformities of the hand, contractures due to paralysis, and recurrent injuries to the hands, feet, and eyes due to insensitivity. This leads to progressive absorption of extremities and/or blindness.
  • Common problems include ulcerations on plantar surfaces, sides of feet and toes, and hands. Lacerations, burns, abrasions, and hematomas are common on the hands. Shortening of digits on feet and hands may occur through destructive osteomyelitis, and fragments of bone may be discharged through ulcerated areas. Amputations may occur traumatically.
  • Impairment grading is as follows (WHO, 1982):
  • • Grade 0 - Normal, no impairments
    • Grade 1 - Peripheral anesthesia over hands and/or feet
    • Grade 2 - Trophic ulcers over hands and/or feet; mobile clawing of fingers or toes; minimal absorption of fingers and/or toes and wrist and/or foot drop
    • Grade 3 - Fixed deformities of fingers and/or toes; more than minimal absorption of fingers and/or toes and nasal collapse
• Lucio phenomenon is seen in certain Latin American patients with diffuse infiltrative LL leprosy. It is characterized by thrombosis of deeper subcutaneous arteries, resulting in necrosis of the skin and subcutaneous fat. Underlying tendons and muscles may be exposed. The outcome is often fatal.
• Secondary amyloidosis is a complication of severe LL disease, often related to the severity and frequency of ENL reactions.

Prognosis

• Progression of tissue and nerve damage can be limited, but recovery of lost sensory and motor function is variable and generally incomplete.
• Hyperpigmentation, hypopigmentation, and loss of skin organs persist.
• Intercurrent reactional states, poor compliance, and emergence of dapsone resistance can lead to clinical exacerbations or relapses that necessitate close follow-up.
• Much chronic debility results from repeated trauma to anesthetic digits and limbs. Careful counseling and consultations with physical and occupational therapy services are essential for optimal outcome.
• Rejection and isolation by community and family members leads to social and economic dislocation. Even when the disease is fully controlled, its stigma and social isolation persist.

Patient Education

• Educate the patient about the disease, its bacterial origin, low communicability, and possibilities for successful treatment. This knowledge improves their self-esteem and motivation to complete medical treatment.
• Educate the patients about the condition, the consequences of neuropathy and the proper self-care techniques. Periodic screening is recommended to detect signs and symptoms of neuropathic feet. Any change in status may require a change in the treatment protocol (possibly a different style of shoe or orthotic). The patient must realize the importance of taking responsibility for self-care to ensure healthy feet. All of the following activities should be explained and monitored on follow-up visits.
  
  
  • A person with insensitive feet must always wear shoes (no barefoot walking), but the shape and size of the shoe must be appropriate. The shoe must match the shape of the foot. Always have the feet measured when one buys shoes. Allow for a 0.5-in. space between the length of the longest toe and the shoe. One should be able to pinch a small area at the widest part of the shoe to determine sufficient width. The toe box (end of the shoe) should be roomy enough to accommodate the toes. Leather uppers are preferred because the leather conforms to the shape of the foot over time. Purchase shoes with a wedge and soft rubber sole. Wear shoes with a closure system; clogs, slip-ons, or loose-fitting shoes may easily come off the foot or rub red areas.
  • Gradually break in new shoes. Begin wearing new shoes no more than 2 hours the first time, and gradually prolong the time if no problems occur.
  • Always wear socks with shoes and inspect them daily. White cotton socks are preferred because they are more absorbent than socks made of other materials and because white easily shows evidence of skin breakdown and drainage.
  • Inspect shoes before and after wear to ensure that no objects have accidentally fallen into the shoe and that no sharp items have penetrated the sole. Do not wear high-heeled shoes, as they tend to put pressure on the forefoot.
  • Inspect the feet daily for redness, warmth, swelling, or new injury. Use a mirror to check the bottom of the feet. If any new injury or redness, swelling, or change in temperature is noted, it should be brought to the attention of a healthcare professional.
  • Cut toenails straight across. If nails are large and irregular in shape, professional care may be necessary. Do not cut calluses or corns or use corn removers. These are problems that a healthcare professional should address.
  • For dry skin, use a lotion that does not contain alcohol.
  • Never use heating pads or hot water bottles or stand too close to a heater or fireplace. Insensitive feet and lower legs may not detect when temperatures have reached dangerous, burning level.
• To enable acceptance, educate the patient's family, friends, and employers about leprosy.

Miscellaneous

Medicolegal Pitfalls

• Treatment progress and decreasing prevalence make differential diagnosis difficult, and neurologic expertise is required. Silent nerve lesions may be present long before symptomatic neuropathy appears. Detection of asymptomatic leprous neuropathy by means of careful clinical examination and electrophysiologic studies in persons at risk can aid early detection and treatment.
• Inexperience with the disease may delay diagnosis and treatment.
• Epidemiologic studies reveal that many cases occur within families. This pattern may lead to misdiagnosis of a hereditary neuropathy.
• Neuropathies, especially those involving small fibers of the peripheral nerve, may closely mimic leprosy.
• Differentiating leprous neuropathy from hereditary sensory neuropathy may be difficult. Both may involve family members, the clinical pictures may be similar, and the diagnosis may be missed unless skin smears or skin and/or nerve biopsy is done.
• Neuritic leprosy produces no obvious skin lesions, and skin smears are negative for AFB. Suspect this diagnosis when persons from areas of endemic disease present with nerve thickening and associated nerve deficit. In some studies, neuritic leprosy was reported in 5-15% of patients with leprosy. Some cases may have been treated partially; others may subsequently evolve to classic leprosy. Nerve biopsy is essential for diagnosis.
• In areas where the disease is not endemic, confirm the diagnosis by performing smears or biopsy and, if possible, by obtaining cultures in a mouse footpad. Consult local health authorities regarding treatment policies. Seek the help of referral centers performing and interpreting the results of nerve biopsy and in difficult cases, such as those in pregnant women, in patients who cannot tolerate standard drugs, and in patients with associated hepatic and/or renal disease or HIV infection.
• Pay special attention to prevent deformities, blindness, and damage to insensitive areas.
• Even in areas of endemic disease, not all persons with thickened nerves have leprosy; other neuropathies must be excluded.
• The involvement of the median nerve at the wrist, ulnar nerve at the elbow, and common peroneal nerve at the fibular head are common in leprosy and may lead to difficulty in distinguishing this condition from idiopathic entrapment neuropathy involving these nerves. A history of previous residence or travel to an area of endemic disease, a careful search for skin lesions, and a careful search for nerve enlargement, combined with an appropriate diagnostic workup for leprosy, may confirm the diagnosis. In leprosy, the nerve is often thickened and involves areas proximal to the entrapment site. Motor weakness and wasting are often more severe in leprosy than in a carpal tunnel syndrome. Idiopathic entrapment neuropathy must not be misdiagnosed as neuritic leprosy (ie, leprosy without skin lesions).

Special Concerns

• Nerve destruction in leprosy reactions
• • Significant nerve destruction occurs during the reactive phase of both the ENL and reversal reactions.
  • Acute granulomatous inflammation can destroy nerves to the extent of causing caseation necrosis of nerve tissue and irreversible paralysis. Swelling of nerves due to sudden increase in inflammatory cells and edema within an unyielding perineurium produces ischemia and paralysis.
  • High-risk patients may require corticosteroids for 6 months with MDT as a preventive measure. Nerve damage can occur even after clinical cure and release from treatment.
• Social rehabilitation
  • Social rehabilitation should focus on suitable vocational evaluation and placement of persons with deformities or insensitivity.
  • Many patients are in low socioeconomic groups. This disease may pose insurmountable problems for the patient.
  • Social service is helpful in achieving sources of financial support, home and community health services, and adequate housing.
• Intolerance, adverse effects, and contraindications related to MDT
  • After adverse events are conclusively established to be due to antileprosy drugs, other new antileprosy drugs can be used under direct supervision in a referral center. For patients who refuse clofazimine, educate the patient about the advantages of the drug, particularly the reversible nature of the discoloration produced by the drug; this information should be sufficient to encourage the patient to continue with clofazimine therapy. In exceptional cases, ofloxacin 400 mg or minocycline 100 mg daily may be used under supervision in place of clofazimine.
  • As an alternative, such patients may be treated with the monthly administration of 600 mg rifampin, 400 mg ofloxacin, and 100 mg minocycline (ie, ROM therapy) for 24 months. The following is recommended for adults with MB leprosy who do not tolerate rifampin: clofazimine 50 mg/d with ofloxacin 400 mg, and minocycline 100 mg for 6 months, followed by clofazimine 50 mg/d with minocycline 100 mg or ofloxacin 400 mg for at least an additional 18 months.
  • If the toxic effects of dapsone are severe in patients with PB disease, clofazimine may be substituted for dapsone at the same dosage as that used for patients with MB but for 6 months. In patients with MB, dapsone should be stopped, and treatment should be continued with rifampin and clofazimine at the standard dosages.
• Nonresponse to therapy
  • Nonresponse may be due to poor drug compliance or other concomitant, debilitating, intercurrent infections, including HIV infection.
  • When the patient's condition shows no improvement despite supervised drug administration, health education, and thorough investigation and management of intercurrent infections, an expert opinion may be necessary.
• Relapse
  • In MB leprosy, relapse is defined as the multiplication of M leprae, suggested by the marked increase (at least 2+ over the previous value) in the bacillary index at any single site, usually with evidence of clinical deterioration (new skin patches or nodules and/or new nerve damage). In most cases, this condition can be confirmed by the growth of M leprae in the mouse footpad system.
  • Recognition of relapse in PB leprosy is somewhat difficult because it is hard to distinguish it from reversal reaction. In theory, a therapeutic test with corticosteroids may be able to distinguish between the phenomena: Definite improvement within 4 weeks of corticosteroid therapy indicates a reversal reaction, and no response to corticosteroids during the same period suggests clinical relapse.
• Steroid therapy and leprosy
  • Indications for steroid therapy in persons with leprosy include neuritis, impending nerve palsies, iridocyclitis, epididymoorchitis, severe reversal reaction, ENL reaction, or systemic involvement.
  • No evidence suggests that immunosuppressive drugs, such as corticosteroids, accelerate multiplication of organisms located in dormant foci and cause reactivation of leprosy.
  • Whenever the duration of steroid therapy (for late reversal reaction or other medical conditions) is expected to exceed 4 months, clofazimine 50 mg/d can be started as a prophylactic measure. This treatment should be continued until the course of steroids is complete.
• HIV Infections and leprosy
• • Unlike the situation with tuberculosis and atypical mycobacteriosis, case-controlled studies have not revealed any significant association between HIV and leprosy.
  • HIV serodiagnostic tests based on ELISA and/or western blot may show significantly higher rates of false-positive results among patients with LL.
  • Care of patients with leprosy who also are infected with HIV is the same as that of any other patient, including treatment of reactions.
  • HIV-associated neuropathy might be confused with or exacerbate leprosy neuritis.
  • Neuropathy due to antiretroviral chemotherapy might be confused with leprosy.
  • Nonleprosy mycobacterioses in HIV-positive people might be confused diagnostically with leprosy.
  • National policies on BCG vaccination might be amended because of endemic HIV infection.
  • Slit-skin smear taking could spread HIV infection if proper precautions are not taken.
  • Leprosy workers in countries of endemic infection may become increasingly involved with problems of HIV counseling.
• Women, pregnancy, and leprosy
• • Hormonal changes in puberty and pregnancy cause nonspecific suppression of cell-mediated immunity with worsening of leprosy. Pregnancy also can be associated with reactions. Deterioration usually occurs in the second half of pregnancy or first 3 months after delivery. Late nerve damage associated with childbirth has been recorded, even in women in whom MDT is stopped. Therefore, the WHO recommends that MDT be continued during pregnancy.
  • Dapsone is not known to have any adverse effects on mother or fetus. Clofazimine, prednisolone, and thalidomide may affect both mother and fetus. Rifampicin is not recommended in the first trimester. Small quantities of antileprosy drugs are excreted through breast milk, but no adverse reactions have been reported as a result of this except for mild discoloration of infants due to clofazimine. Infants of mothers suffering from leprosy have lower birth weights and higher risk of contracting the disease.
• Tuberculosis and leprosy
  • MDT for leprosy is not adequate for treatment of tuberculosis. Therefore, an appropriate antitubercular regimen should be given, in addition to antileprosy MDT, to patients who have both leprosy and tuberculosis.
  • Except where daily rifampicin is part of antituberculosis treatment, monthly rifampicin is not needed as part of leprosy MDT.
  • If a patient with leprosy and tuberculosis is treated with a rifampicin-containing antitubercular regimen, the patient may run the risk of developing rifampicin-resistant leprosy. Hence, both diseases need to be treated simultaneously.
• Leprosy and human rights
• • Patients on MDT and those cured of disease should not have restrictions in terms of employment, education, and travel.
  • Any special legal measures that might increase prejudice against patients with leprosy or prevent patients with early cases from presenting themselves for diagnosis and treatment should be abolished.

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