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REACTIONS AND NEURITIS IN LEPROSY - ( H.D.)
NEURITIS AND REACTIONS IN LEPROSY - CONTINUED FROM :-
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Leprosy, primarily is a disease of the nervous system and, in particular, the peripheral (cooler) nerves. The central nervous system is not affected. Leprosy is often called a “skin disease” but, in fact, there is a type of leprosy in which ONLY nerves are involved, with no skin manifestations; it is called “Polyneuritic Leprosy”. For this reason, “Neuritis in Leprosy” is a most important subject.
The main nerves involved in affecting the hand in leprosy are - (1) the Ulnar at the elbow’s “funny bone”- at the medial epicondyle , - (2) the Median, just above the wrist joint and - (3) the Radial in what is called the “spiral groove” of the arm, (4) Cutaneous branch of the Radial nerve at the wrist. In relation to deformities of the foot, the main leg nerves involved are (1) Lateral Popliteal at the neck of the fibula, - (2) Posterior Tibial at the ankle - medial Malleolus. In both hand and foot deformities, other lesser nerves also are involved. Re. Facial deformities, the main nerves involved are - (1) Facial nerve at the zygoma and - (2) Great Auricular nerve over the sternomastoid muscle. It is thought that a lower temperature in these areas is the reason for these nerves being affected and at the particular sites. Once the nerves have become inflamed, extreme care must be exercised to prevent them being further damaged resulting in even more destruction of the nerve. Often, the traumatised nerve needs to be carefully protected with warm cotton-wool padding and the limb placed in a splint and / or sling.
NEURITIS IN LEPROMATOUS (“LL” of Multibacilliary ) LEPROSY:- In the “Lepromatous” form of the disease, Mycobacterium leprae can be found all over the body, in nerves, skin and other tissue, because, unlike the Tuberculoid, localised form, it is a generalised condition. Instead of the defence cells (macrophages) ingesting and digesting the bacilli, because of a lack of Cell-Mediated Immunity (CMI), they act as a convenient host in which the bacilli may even multiply, to be transported by the macrophages to all parts of the body. It has been known for up to 300 M.leprae to be found in one macrophage. In the very early stages of lepromatous leprosy, there may be little or no nerve damage and hence no sensory deficit, no sign of paralysis and no malfunctioning of the sweating mechanism. However, as the disease progresses and if treatment is irregular, serious damage can be suffered by the nerve which becomes swollen, inflamed and tender.
Erythema Nodosum Leprosum ( ENL) may also occur in the nerves, although Neuritis can occur without ENL. Oedema within the nerve results in a build-up of pressure, which, if not soon reduced , can seriously damage the nerve and even destroy it. Sometimes the nerve sheath has to be incised to relieve the pressure if corticosteroids are not sufficient in reducing the trauma.
NEURITIS IN TUBERCULOID (“TT” or Paucibacilliary) LEPROSY:- Unlike the generalised and infectious form of lepromatous leprosy, the Tuberculoid type is localised and may involve only one or two nerves. However, those nerves may be very seriously damaged, even in the early stages of the disease because of the strong immunological response. It must be remembered that nerve damage is due not so much to the presence of M.leprae germs but rather to the body’s intense immune response to the antigens liberated by the dead and dying leprosy bacilli. Whereas in Lepromatous leprosy, millions of leprosy bacilli may be seen under the microscope, in the Tuberculoid patient, it may be impossible to find even one bacillus (“Paucibacilliary” form of leprosy)
Remember that the nerve is damaged not by the leprosy bacillus (M.leprae) but by the the body’s immune / defence system reacting violently to the presence of antigens liberated by the dead and dying M.leprae. In fact, the nerve can be completely replaced by the inflammatory cells (“T” type lymphocytes) . Why cannot the leprosy germs by found in tuberculoid leprosy nerve damage? It is because the bacilli which gain entry to the nerves are ingested and digested (the action is called “phagacytosis”) by the large defence cells called Macrophages. It is during this phagocytosis that the nerves are destroyed. Often, this action is so acute that an abscess is formed inside the nerve, causing extreme suffering to the patient and sometimes, it may open to form a sinus.
NEURITIS IN BORDERLINE LEPROSY (BT - BB - BL ) :- “Borderline” is a term less used these days. It is the intermediate types of the disease in the Immunological Spectrum. Actually, nerve damage can be more intense in BT leprosy or that type, which in the immunological spectrum is nearer to the Tuberculoid end of the scale but not quite “TT”. In Borderline leprosy, nerves are traumatised in much the same way as in the Tuberculoid type, however, a higher build-up of bacilli is necessary to elicit a cellular response, conditional upon the position of the patient’s disease on the immunological spectrum. In BT, epithelioid cell granuloma is more diffuse than can be seen in TT. In BB, there is even more diffuse epithelioid cell granuloma, very few lymphocytes and no giant cells. In BL, macrophage granuloma can be seen with only a few epithelioid cells present.
REACTIONS IN LEPROSY :- There is much misunderstanding concerning “reactions” . While it is possible for a patient to suffer from reaction to a particular anti-leprosy drug, such as Dapsone, Rifampicin, Clofazamine etc., generally speaking, when we refer to “Reactions in Leprosy” we are thinking of those acute episodes experienced in the course of active leprosy infection which are related to Neuritis resulting from leprosy. Reactions can be experienced by sufferers of all the three main forms of leprosy - lepromatous, borderline and tuberculoid.
REACTIONS IN LEPROMATOUS LEPROSY :- Those suffering from the infectious, multibacilliary or lepromatous form of the disease may experience two types of reaction - (1) Erythema Nodosum Leprosum (ENL) and - (2) Progressive “Lepra Reaction”. “ENL” is the most common of all reactions and may be experienced by those with LL and BL types. We speak of ENL “episodes” because when such reactions occur, they generally are of short duration, maybe only for a few days, although, in some cases, the duration may be a few weeks. Recurrent attacks may occur over several months or years. The suffering can be most traumatic. ENL is caused by an antigen - antibody reaction occuring within the vessel wall. The antigen is the toxin released by the dead and dying M.leprae germs reacting with the “humoral” antibodies which circulate in the body’s fluids (humor) . This is “Humoral Immunity” which is not really effective in controlling leprosy. Signs and Symptoms of ENL are :- Neuritis-like nerve pain, fever, joint pains, erythematous, evanescent, tender skin nodules, orchitis, myositis, osteitis, periostitis, iridocyclitis. Sometimes the fever is very high. Acute complications of ENL are as follows (1) Ulceration of the skin lesions which may appear in crops - called “Ulcerative ENL”. (2) Blindness, when recurrent iritis or iridocyclitis is experienced. (3) Renal Damage and amyloidosis in particular, may be experienced in some patients. Treatment is only symptomatic because ENL is a self-limiting condition. Aspirin is still used. In the early days, antimony compounds were used and even the controversial drug Thalidomide , which later came to be used only for treating ENL in male patients or females unable to bear children. However, Thalidomide is only used in very rare circumstances and only in extreme cases of leprae reaction, when the regular drugs for controlling reaction - Clofazamine, Aspirin, Chloroquin and Prednisolone - fail to reduce inflammation and prevent nerve damage. It should also be remembered that in some countries, the use of Thalidomide is illegal and its use could have dangerous consequences in certain rare cases of exacerbated reaction where the patient’s life cannot be saved. Fortunately, Chloroquin, Aspirin, Clofazamineand hydrocortisone give relief in most cases. In (2) “Progressive Lepra Reaction”, experienced by those cases of advanced lepromatous leprosy, suffering can be very acute, with fever, ulcerated nodules and general malaise.
REACTIONS IN BORDERLINE LEPROSY :- In this middle-of-the-immunological-range form of leprosy, Reaction may serve to swing the patient more towards the LL end of the spectrum or to the opposite TT end, depending on the patient’s immunological response - whether it is “Humoral” immunity or “Cell-Mediated Immunity”. The two types of reaction are - (1) “Downgrading or Type 2” Reaction and (2) “Reversal or Up-grading Type 1” Reaction. If the patient’s resistance to the disease diminishes, the type of reaction is “Downgrading Type - 2”, resulting in a change in the patient’s immunological status with the M.leprae multiplying and spreading more throughout the body. In “Reversal Type-2” Reactions we see a reduction in the number of M.leprae bacilli and an improvement of the patient’s immunological status which moves more towards the Tuberculoid end of the spectrum. Signs and symptoms may be manifest in swelling and redness of the existing lesions with an increase in pain and tenderness of the nerves, also the formation of new skin lesions, which sometimes ulcerate. Some patients may also experience acute swelling of the hands and feet, also nerve paralysis and increase of anaesthesia of the skin.
REACTIONS IN TUBERCULOID (TT) LEPROSY :-
This occurs only rarely and is accompanied by an increase in Cell-Mediated Immunity (CMI) .
MANAGEMENT OF REACTIONS. - The 4 main principles of management are - (1) Prevent anaesthesia, paralysis and contracture by controlling the acute neuritis. (2) Take care of the eye to prevent blindness. (3) Use bactericidal drugs like Rifampicin to kill the bacilli and control the spread of the disease. (4) Control the pain and related trauma. In Mild reactions use Aspirin, Chloroquin and Antimonials. In severe reactions (most common in Type 1 reactions in BT and BB leprosy ) use corticosteroids such as Prednisolone for rapid control. Sometimes Reactions (especially in Type 2) may be precipitated or exacerbated by vaccination, hormonal changes as in pregnancy and even by psychological disturbances. These studies are only simple in nature and are designed to “whet the appetite” and stimulate a concern for leprosy sufferers. “In-Depth” details can be provided upon request. For a more detailed study, check out "TYPES 1 and 2 REACTIONS"
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