ENL or relapse in a BL/LL patient?

Leprosy Mailing List – May 3^rd, 2012

Ref.:   ENL or relapse in a BL/LL patient?
From: G Warren, Sydney, Australia

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Dear Salvatore,

I refer to the letter from Dr Kawuma in Uganda (LML May 3^rd, 2012).

Yes, definitely down grading reaction can occur and it frequently did in the “old days” when a patient was receiving one drug only, when the patient developed resistance to that drug.  In the 1960s it was usually dapsone but, also downgrading reaction occurred to some of the other drugs in which initial improvement had appeared to occur and then the patients would down grade till we changed the antileprosy drug therapy.  It usually required several years for such resistance to show up.

It also occurred in patients who had been given multidrug therapy because they were not responding to dapsone and so were considered dapsone resistant; though there was no ability to test for resistance to dapsone.  After a period often a year or two the disease seemed under control and the patient stopped drug therapy (often just by dropping out himself but sometimes by completing the 12 months recommended for MDT).  But after several years the patient would reappear with what was said to be ENL; but on careful testing one would find the bacteriological index (BI) was higher than it had been at the last test.

It is fascinating how one can separate between new lesions of downgrading BL/LL and the lesions of ENL that look similar.  Restarting adequate anti-leprosy medication especially including the use of clofazamine, seemed to rapidly deal with the problem that was apparently resistance to dapsone and the multidrug therapy had not been given long enough.

I hope that will help.  When a patient returns with what is queried to be ENL or relapse in a BL/LL patient who has completed the recommended 12 months MDT it is wise to check the lesions for infiltration, by pressing a glass slide onto the lesions.  The pressure of a slide will define the edge of the lesion.  If it is ENL there will be a well localised small patch of infiltration but, if it is a new lesion ie true relapse, it will not be so definite and not so erythematous.

We need to be on the watch for such relapses!

Grace Warren

Previously Superintendent Hong Kong Leprosarium 1960-1975.

The downgrading reaction would only be an issue at first presentation

Leprosy Mailing List – May 3^rd, 2012

Ref.:   The downgrading reaction would only be an issue at first presentation.
From: H J Kawuma, Buluba, Uganda

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Dear Salvatore,

I am glad that this longstanding puzzle has come up for debate again.

Dr. Bryceson, as usual, has put the arguments clearly for many of us to appreciate [LML April 26^th, 2012].  It appears the downgrading reaction would only be an issue at first presentation as we would not expect it in a person on treatment.  Might it also be a manifestation of treatment failure?

Dr. Bryceson must be aware of the reasons behind the shift to referring to type 1 reactions as "Reversal Reactions" at some stage.  Would he kindly remind the readers of those reasons as well?

Best wishes,

H Joseph Kawuma
GLRA, Uganda 

Ungrading and downgrading reactions; do these concepts help us?

Leprosy Mailing List – May 3^rd, 2012

Ref.:   Ungrading and downgrading reactions; do these concepts help us?
From: J A da Costa Nery, Rio de Janeiro, Brazil

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Dear Salvatore,

It´s always a pleasure to follow these reports, so we can think upon them.  In my whole life studying leprosy, these nomenclatures [upgrading and downgrading reactions] always bothered me.  I find them, as a clinician, very hard to distinguish on a daily basis.

I don’t really know if these concepts help us in the programme and, not all doctors know how to use that kind of nomenclature while treating reactional episodes.  The discussion on appearance of either upgrading or downgrading type 1 reaction [type 1 reaction is also commonly referred to as reversal reaction or RR] is very relevant to the knowledge on leprosy in the state of art.  Nevertheless, such a goal is more likely to be achieved by basic researchers, by means of immunologic tests; not by clinicians.  Moreover, it is not always possible, given that cellular proliferation and activation tests are not deemed as a golden standard assessment.  On the other hand, histopathology is also helpful for the definition of RR, although such examination is not very useful to evaluate the characteristic of such a reaction (either upgrading or downgrading). 

It’s always good to hear new opinions so we can discuss.  Lately I´ve been studying cases of relapse in leprosy, and I´ll watch out for these type 1 reactions, which are treated with long term corticosteroids,  can they be included in the downgrade?

We have a large experience with a number of RR cases per year, and our most relevant concern relates to the long-term use of corticosteroids and risk of disability. 

Best wishes,

Dr. José Augusto da Costa Nery
Fiocruz 

What would be the meaning of the downgrading type 1 reaction in terms of the management of the patient?

Leprosy Mailing List – May 1^st, 2012

Ref.:   What would be the meaning of the downgrading type 1 reaction in terms of the management of the patient?
From: H K Kar, New Delhi, India

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Dear Dr C Shumin,

Thanks for your message [LML April 23rd, 2012].  The term downgrading type 1 reaction is basically downgrading of the spectrum of the disease with some amount of inflammation over some of the lesions.  In those cases simply multi-drug therapy (MDT) has to be started immediately.  These patients need close watch, since they are likely to develop upgrading type 1 reaction.

Regards

Dr (Prof.) H K Kar
Dean, PGIMER, Dr R M L Hospital
Consultant & HOD
Department of Dermatology, STD & Leprosy
P.G.I.M.E.R. and Dr Ram Manohar Lohia Hospital
Baba Kharag Singh Marg
New Delhi-110001

In my opinion those hypothetic concepts of upgrading and downgrading reaction only confuse

Leprosy Mailing List – April 30^th, 2012

Ref.:   In my opinion those hypothetic concepts of upgrading and downgrading reaction only confuse

From: P AM Schreuder, Maastricht, The Netherlands

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Dear Salvatore,

I have problems with the explanation of Prof. Bryceson [LML April 26^th, 2012]:

<< "Add more lymphocytes (as in upgrading) and the reaction, as measured by titrated thymidine incorporation, increases.  Add more antigen (as in downgrading) and the reaction increases." >>

What we, however, see is that most reactions happens the first six months after starting with MDT.  You would expect an enormous increase in antigen, while at the same time the patient has a so-called "upgrading" reaction.  In my opinion those hypothetic concepts of upgrading and downgrading reaction only confuse.

  

Kind regards,

Pieter AM Schreuder

Difference between Jopling’s downgrading and upgrading reactions.

Leprosy Mailing List – April 29^th, 2012

Ref.:   Difference between Jopling’s downgrading and upgrading reactions.

From: J A. Barreto, S Paulo, Brazil

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Dear Dr Noto,

Many thanks for circulating our clinical case of about “Borderline leprosy in reaction in a boy from Brazil” (LML March 24th, 2012).  Herewith I would like to comment on the difference between Jopling’s downgrading reaction and upgrading reaction.

Initially, the most important feature is the presence of viable (“solid” or globi) bacilli.  In downgrading reaction, there are viable bacilli, despite the presence of a granulomatous epithelioid reaction; which is seen on the tuberculoid side of the leprosy spectrum.  Actually a “granulomatous epithelioid reaction” can be found on the following three distinct conditions: 

First condition

True TT leprosy (rare).  In this case, bacilloscopy in biopsy specimens ranges from 0 to 1+, and bacilli are usually found, when present, inside dermal nerve branches.

Second condition

BT leprosy (most common).  In this case, bacilloscopy in biopsy is positive, usually 2+ or 3+, inside dermal nerve branches, macrophages (less common), sub-epidermal area and smooth muscle of hair follicles.

Third condition

Type 1 reaction.  Borderline tuberculoid (BT) and mid borderline (BB) leprosy can show epithelioid cells, which in turn means that macrophage differentiation and antigen processing was done, due to IL2 plus IFN-gamma and TNF alfa functions.  What does it mean the presence of epithelioid cells together with viable (solid or globi) bacilli?  This is easy to understand: it means that the macrophage differentiation was not proper and bacilli are still multiplying.  This pattern is typical of the non-treated borderline group, where cellular immunity is partial, and is the reason why most BT patients downgrade to borderline lepromatous (BL), progressively or during reactions.  According to Ridley, indeed, most of BL patients results from downgraded BT.

Coming back to the clinical case we presented; the boy had globi under the epidermis, and it means that this is a downgrading reaction.  Upgrading reaction will never show globi, that is to say aggregations of viable (solid or well stained) bacilli.  This boy also did not receive antibiotics.  Clinicians in the past had already noticed that downgraded reaction occurred in untreated borderline patients [B Naafs personal communication]. 

Unfortunately, leprosy knowledge has been lost since the Ridley and Jopling (R&J) Classification was forgotten, and a new classification (W.H.O.) based only on the number of lesions is nowadays the rule. 

Best regards,

Jaison A. Barreto

Dermatologist and Dermatopathologist

More details can be found on the suggested bibliography.

(Ridley DS. Skin biopsy on leprosy. 2ed. 1987 and Hastings RC. Leprosy 2ed. 1994)

Downgrading and upgrading type 1 reactions. Do they exist?

Leprosy Mailing List – April 26^th, 2012

Ref.:   Downgrading and upgrading type 1 reactions.  Do they exist?
From: A Bryceson, London, UK

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Dear Salvatore,

I refer to Prof. Kar’s message << Downgrading type 1 reaction? >> [LML April 10th, 2012]

The problem in understanding type 1 reactions lies with the nomenclature, not the immunology.  Cell mediated immunity is the process that, if all goes well, controls the leprosy infection.  More cell mediated immunity (CMI) results in upgrading and increased control; less CMI results in downgrading and decreased control.

I think of a type 1 reaction as a hypersensitivity reaction between antigen and specifically sensitized lymphocytes.  Imagine the patient, or a nerve, to be a test tube containing antigen and lymphocytes.  Add more lymphocytes (as in upgrading) and the reaction, as measured by titrated thymidine incorporation, increases.  Add more antigen (as in downgrading) and the reaction increases.  Thus it is possible to have a type 1 reaction associated with upgrading and a type 1 reaction associated with downgrading.

Clinically, the reactions are indistinguishable.  The history, clinical examination and bacillary index indicate whether the underlying disease is upgrading or downgrading, and thus the prognosis.  If downgrading continues (the patient does not receive anti-leprosy treatment) the infection is uncontrolled and the concentration of antigen in the test tube continues to rise and will eventually suppress the reaction.

We might have a clearer understanding of reactions associated with shift along the borderline tuberculoid (BT) – borderline lepromatous (BL) spectrum if we were to replace the terms upgrading reactions and downgrading reactions with the terms type 1 reaction associated with upgrading, and type 1 reaction associated with downgrading.

With best wishes,

Anthony

There are no accepted criteria for the diagnosis of downgrading type 1 reaction

Leprosy Mailing List – April 23^rd, 2012

Ref.:   There are no accepted criteria for the diagnosis of downgrading type 1 reaction.
From: C Shumin, Shandong, China

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Dear Dr. Kar,

Thank you very much for your comments on the downgrading type 1 reaction [LML April 10^th, 2012].  I agree with you.  So far there are no accepted criteria for the diagnosis of downgrading type 1 reaction.  If yes, what would be the meaning of the downgrading type 1 reaction in terms of the management of the patient?

Warm regards,

Dr. Chen Shumin

Head of Leprosy and STD Control Unit 

Shandong Provincial Institute of Dermatology

Downgrading type 1 reaction?

10 April 2012

Ref. Downgrading type 1 reaction?

From: H K Kar, New Delhi, India

Dear Dr Noto,

Thank you very much to Drs Barreto and Cabral for presenting their case (LML March 24th, 2012). A very good case of a child with untreated mid borderline (BB) leprosy presenting with downgrading (DG) type 1 reaction.

Many of us do not accept the existence of DG type of type 1 reaction. Many of the so called untreated borderline patients presenting with DG type 1 reaction are in true sense upgrading (UG) type 1 reaction. On taking detailed history we come to know that in the immediate past they took some antibiotics responsive to M. leprae for other infections which precipitated UG type 1 reaction. In true sense these reactions should be considered as Up-grading type 1 reaction, rather than DG type 1 reaction.

With Regards.

Dr (Prof.) H K Kar

Consultant & HOD

Department of Dermatology, STD & Leprosy

P.G.I.M.E.R. and Dr Ram Manohar Lohia Hospital

Clinical case. Borderline leprosy in reaction in a boy

 Leprosy Mailing List – April 6th, 2012 

Ref.:   Clinical case. Borderline leprosy in reaction in a boy.From: Warren G., Melbourne, Australia

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Dear Dr Noto,

I would like to congratulate Dr Barreto and Dr Cabral on the excellent case presentation (LML 24 March 2012) that opens the way to discussion of several important points.  There were some interesting picture of biopsies, but one wonders what type of lesions were biopsied.  One would expect those type of pictures from the BT end of spectrum but I wonder if any biopsy was taken from a vague lesion?

1. The lesions appeared at age 4 with no known contacts of leprosy, but we do not know the incidence of leprosy in the area or if the patient had lived elsewhere.  The state of Mato Grosso is well known for his leprosy endemicity.  Was any (extended) contact examination done?

2. The condition was diagnosed as eczema at several occasions, by different health staff and even by the local leprosy reference centre.  It does accent in endemic areas that any skin lesion not reacting to normal treatment needs to be followed up and the possibility of leprosy should be taken into account.  Even the local leprosy clinic did not think about this possibility.  This is an aftermath we are seeing in many place now that the statement that leprosy is eliminated has resulted in many early cases being missed.  WHAT WE DO NOT LOOK FOR WE WILL NEVER SEE!  One should be highly suspicious of eczema that continued for twelve months not reacting to treatment.

3. I am interested in the degree of affection of the nerves.  There is no mention of sensory changes (in the lesions, hands and feet).  Perhaps a little hard  to test, in a 5 year old. The history implies no motor nerve deficit, but the biopsy showed bacilli in nerves and some nerves were easily palpable and visible.  The use of steroids  is certainly indicated.  We see that the inflammation has settled clinically in 2 months so hopefully he should not develop further nerve involvement.  The steroids cannot reverse any real damage to the actual fibres that has occurred, but there is no point in continuing the steroids as their job is to reduce the inflammation.  However, it will not encourage regrowth of damaged fibres. 

4. Long experience with dozens of patient with this type of reaction has shown me that 10-12 weeks of steroids is all that most need and then the continued use of MDT, possibly with extra clofazimine to prevent and control further reaction.  I certainly did find that in lepra reaction in the BL/BBish type one often had both types of reaction at the same time (acute redness and even ulceration of the lesions that looked real BB/BT and ENL on the BL/LL ones).  In the pictures of the patient in question there is definitely a suggestion of BLish type lesions on wrists and also the ear lobe) and even around the knees.  Yes, he has downgraded, but lesions now are right across the spectrum and I would not be surprised if there was some early ENL in those arm and face (ear lobe) lesions or, that ENL comes once the steroid is discontinued.  Hence the suggestion that extra clofazimine may be of help. 

5. Steroids are often continued for a prolonged period and does a lot of harm to the patient’s own metabolism.  I have seen too many patients die because they had had long periods of steroids and for various reasons they were not restarted when medical complications arose (e.g. one teenage boy got a severe flue 6 months after 5 years of steroids stopped and he did not get adequate medical care and he died within a week). 

6. The other problem that is often forgotten is that steroids are effective  in preventing inflammation.  At the same time, the body’s own abilities to control the infection are slowed down by the steroids and this affects the ability of the body to reduce the degree of infection caused by M. leprae.  In fact in a well controlled drug trial severe LL patients were given Rifampycin daily under supervision for 5 years.  After that time the disease appeared controlled, but nerve biopsy and culture revealed M. leprae that were fully sensitive to the Rifampycin.  It is now accepted that the antibiotics while being able to kill the bacteria in the blood and other tissues, but cannot eliminate them from the nerves.  Once MDT is completed the bacteria come out, start multiplying again and the patient will relapse in years time. Hence, once definite durations for MDT were suggested by W.H.O., we  started counting the months recommended  as those not on steroids.  If a patient had 3 months steroids he had 24 plus 3 months of MDT for Multibacillary leprosy.  This certainly seemed to prevent many relapses.  In patients with less natural resistance ie the LL/BL type of disease that is well developed at diagnosis, I feel they need longer MDT than that recommended by W.H.O. to ensure no relapse. 

7. Hence I hope that the boy affected will have at least twelve months full MDT after the steroids are stopped.  He is obviously borderline in type and so should have some ability to  eliminate and control the infection. 

8. I am interested to see if he was given tricyclics.  Yes, I use them a lot on adults and think they are excellent in helping to minimise tension and fear of the disease.  However, I must confess  I rarely give tricyclics to small children though have frequently use Phenobarb with good results.  They  can help reduce the duration that one needs to give the steroids.

I do hope your presentation will encourage others to look more carefully for diagnosisand use steroids wisely.

Yours sincerely,

Grace Warren

Previously  Superintendent Hong Kong Leprosarium ( 1960-75)
Advisor in leprosy and   Reconstructive surgery in Asia  ( 1975-95)

Neuritis, acute and chronic, in leprosy

Leprosy Mailing List – February 28^th, 2012 

Ref.:    Neuritis, acute and chronic, in leprosy

From:  H Srinivasan, Chennai, India

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Dear Dr Noto,

The following are my views regarding the queries raised about "Acute neuritis" in leprosy.  I think it will help starting from the definition of neuritis and then considering acute and chronic neuritis.  I also report some relevant aspects of histopathology.

Definition of leprosy neuritis

Leprosy neuritis is an inflammatory mononeuropathy occurring in leprosy.  In can be acute or chronic.

Acute leprosy neuritis 

Acute leprosy neuritis describes the clinical state characterized by pain occurring in an obviously thickened peripheral nerve trunk such as ulnar, median, lateral popliteal nerve etc.  It may occur in cutaneous nerve trunks also, but here there is no risk of disability and deformity, although the condition may be quite distressing to the patient.  Acute neuritis is of rapid (i.e., acute) onset, over the course of a few hours to a few days.  It may have been present for a few days to a few weeks by the time the patient is seen by the physician or paramedical worker.

It may be moderately severe or severe.  In moderately severe acute leprosy neuritis patient complains of severe pain, but the movement of adjacent joint is not restricted and sleep is not disturbed because of pain.  In severe acute leprosy neuritis patient complains of severe pain and the movement of adjacent joint is restricted due to the pain and patient admits that pain disturbs sleep. 

Often, acute neuritis occurs in a background of chronic neuritis.  Acute neuritis may occur along with cutaneous manifestations of type I or type II reaction, or as an isolated clinical manifestation of the reactional process.

The term “acute leprosy neuritis" when used in the histopathological context indicates presence of foci of polymorpho nuclear leucocyte infiltration in the nerve (micro or macro “hot abscess”).

Chronic leprosy neuritis

 “Chronic leprosy neuritis” is the clinical condition where there has been long standing ‘mild’ (patient admits to having pain in the nerve only on asking about it) to ‘moderate’ nerve pain (complains of pain even without asking about it, but says it is not severe) in one or more peripheral nerve trunks of the limb(s).   

Histologically, every case of leprosy shows some evidence of chronic neuritis at some site in the peripheral nervous system.  Leprosy is not diagnosed without such evidence.   

Clinical examination

On examination, the concerned nerve trunk is obviously thickened (swollen), and very tender (very painful on palpation), such that the patient is afraid of palpation of the nerve.  Range of active movement of the adjacent joint is restricted because of pain; and/or passively increasing the range aggravates pain in the nerve.  There may be clinical nerve function deficit relating to the affected nerve trunk, which may be pre-existing or of recent origin along with the attack of acute neuritis or, pre-existing nerve function deficit may have worsened coincident with the attack of acute neuritis or, there may not be any clinically identifiable nerve function deficit.  

Indications for Steroid therapy

Onset or worsening of clinical nerve function deficit relating to the affected nerve trunk (eg., sensory loss, muscle weakness or paralysis) along with acute neuritis or even while the condition is under treatment with other drugs is an absolute indication for immediate institution of steroid therapy in adequate dosage.  Continued severe nerve pain even in the absence of increasing nerve function deficit or in a destroyed nerve trunk (with no possibility of the nerve recovering) despite adequate analgesic therapy is often relieved by steroid therapy.

Nerve conduction studies

One does not wait for or depend on nerve conduction studies for diagnosing and treating acute neuritis.  They may be used, when available, for monitoring efficacy of therapy.  Nerve conduction velocity (NCVs) may be within normal limits when only slow conducting fibres are damaged.  Marginal improvement in NCVs without clinical improvement is of no material benefit to the patient.

Early detection of leprosy neuritis

Patient is the best person to suspect early the possibility of acute neuritis and report for treatment without delay.  So the patient should be trained to look for and suspect acute neuritis as well as onset/worsening of nerve function deficit of his or her thickened nerve trunks.  The paramedical and medical personnel must be sensitized to show concern and examine the patient very carefully and sympathetically when a patient reports for suspected acute neuritis and not play down or neglect the patient.  It goes without saying that they must know how to examine such patients.

H Srinivasan, FRCS
Surgeon (Retd)
25 First Seaward Road
Chennai - 600 041
INDIA

Periodical assessment of the patient by a combination of: - always look, palpate, compare and test

Leprosy Mailing List – February 15^th, 2012 

Ref.:    Periodical assessment of the patient by a combination of: - always look, palpate, compare and test.

From:  F Ross, UK

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Dear Salvatore,

Thanks for your publication of this excellent response from Drs Naafs and Schreuder to the question of acute neuropathy (LML Feb. 14^th 2012 [enclosed]).  If applied it will save a lot of pain and disability.

Best regards.

Felton Ross.

----- Original Message -----

From: Salvatore Noto

To: Salvatore Noto

Sent: Tuesday, February 14, 2012 10:26 AM

Subject: (LML) Proposal for "Guidelines for the management of acute neuritis in leprosy" - Part I. Definition, clinical signs and electrophysiology

Leprosy Mailing List – February 14^th, 2012

Ref.:    Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition, clinical signs and electrophysiology

From:  B Naafs, Munnekeburen;  P A M Schreuder, Maastricht, The Netherlands

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Dear Salvatore,

Very much, “thank you” to Dr Antoine Mahé (LML January 18^th, 2012) and to colleagues contributing to the topic about “acute leprosy neuritis”.  It is difficult to answer all Dr Mahé’s questions because one is always subjective!  Herewith are our answers:-

1) What is your definition of 'acute leprosy neuritis’?

<< Definition of acute neuritis:-  Acute neuritis in leprosy is the occurrence within a few days of increase in pain, or tenderness, decrease in voluntary muscle test (VMT) and sensory testing (ST) scores, severely diminished motor nerve conduction or nerve block in peripheral nerves serving the eye lids, face, hands and feet.  Since patients are not seen every day, “acute” goes up to 3 months.  >>

2) Which are the clinical symptoms and signs to be taken into account for justifying the implementation of a specific therapy of acute neuritis (i.e., systemic steroids):

<<. >>

2a Presence of pain: spontaneous? Provoked by palpation?  Or by movement?

<< All of them are important and justify therapy but, especially tenderness at palpation. >>

2b Recent occurrence of neurological dysfunction (sensory, motor, autonomic)?

<< All of them are important but, most decrease in VMT and ST scores, because these are easy to measure. >>

2c Definition of "recent"?  With or without pain?

<< We would consider “recent” when within 3 months. >>

 3) Relevance of electromyography and nerve conduction studies?

<< Nerve conduction studies are relevant but, they are not widely available. >>

4) Strategies for early detection of incipient neuritis during follow-up of known patients?

<< Periodical assessment of the patient by a combination of: - always look, palpate, compare and test.  Look at the face and eyes: do the eyes blink?  Any degree of lagophthalmos?  Any deviation of the mouth?  Look at hands and feet:- any dryness, atrophy or wounds?  Palpate* the peripheral nerves of predilection of leprosy.  Comparealways the two sides.  Test by performing the voluntary muscle test and sensory test on eyes, hands and feet.

We hope these answers help. 

Best regards,

Ben Naafs and Pieter Schreuder

* Description of the palpation of the peripheral nerves of predilection of leprosy is available on line at: - The Diagnosis of Leprosy – text and slides <<http://atlasofleprosy.hsanmartino.it/ >>

Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition, clinical signs and electrophysiology

Leprosy Mailing List – February 14^th, 2012 

Ref.:    Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition, clinical signs and electrophysiology

From:  B Naafs, Munnekeburen;  P A M Schreuder, Maastricht, The Netherlands

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Dear Salvatore,

Very much, “thank you” to Dr Antoine Mahé (LML January 18^th, 2012) and to colleagues contributing to the topic about “acute leprosy neuritis”.  It is difficult to answer all Dr Mahé’s questions because one is always subjective!  Herewith are our answers:-

1) What is your definition of 'acute leprosy neuritis’?

<< Definition of acute neuritis:-  Acute neuritis in leprosy is the occurrence within a few days of increase in pain, or tenderness, decrease in voluntary muscle test (VMT) and sensory testing (ST) scores, severely diminished motor nerve conduction or nerve block in peripheral nerves serving the eye lids, face, hands and feet.  Since patients are not seen every day, “acute” goes up to 3 months.  >>

2) Which are the clinical symptoms and signs to be taken into account for justifying the implementation of a specific therapy of acute neuritis (i.e., systemic steroids):

<<. >>

2a Presence of pain: spontaneous? Provoked by palpation?  Or by movement?

<< All of them are important and justify therapy but, especially tenderness at palpation. >>

2b Recent occurrence of neurological dysfunction (sensory, motor, autonomic)?

<< All of them are important but, most decrease in VMT and ST scores, because these are easy to measure. >>

2c Definition of "recent"?  With or without pain?

<< We would consider “recent” when within 3 months. >>

 3) Relevance of electromyography and nerve conduction studies?

<< Nerve conduction studies are relevant but, they are not widely available. >>

4) Strategies for early detection of incipient neuritis during follow-up of known patients?

<< Periodical assessment of the patient by a combination of: - always look, palpate, compare and test.  Look at the face and eyes: do the eyes blink?  Any degree of lagophthalmos?  Any deviation of the mouth?  Look at hands and feet:- any dryness, atrophy or wounds?  Palpate* the peripheral nerves of predilection of leprosy.  Comparealways the two sides.  Test by performing the voluntary muscle test and sensory test on eyes, hands and feet.

We hope these answers help. 

Best regards,

Ben Naafs and Pieter Schreuder

* Description of the palpation of the peripheral nerves of predilection of leprosy is available on line at: - The Diagnosis of Leprosy – text and slides <<http://atlasofleprosy.hsanmartino.it/ >>

Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition, clinical signs and electrophysiology

Leprosy Mailing List – February 11^th, 2012 

Ref.:    Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition, clinical signs and electrophysiology

From:  L Reni, Genoa, Italy

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Dear Salvatore,

Thank you very much to Dr Antoine Mahé for introducing such an important topic (LML January 18^th, 2012).  Herewith are my answers:-

1) What is your definition of 'acute leprosy neuritis' ?

<< "Acute leprosy neuritis", my definition:-

It is an acute inflammatory mononeuropathy presenting with pain spreading, from a point of entrapment (for example at the ulnar groove or in the cubital tunnel), along the nerve.

There is tenderness and/or swelling of the nerve; its palpation evokes a typical “electric shock” along the nerve with paraesthesia in the area of its cutaneous distribution (Tinel's sign).

The pain may be isolated or accompanied by neurological dysfunctions (sensitive and/or motor and/or autonomic).  The neuritis may be considered acute if symptomatology and/or signs have arisen within a few weeks.  >>

2) Which are the clinical symptoms and signs to be taken into account for justifying the implementation of a specific therapy of acute neuritis (i.e., systemic steroids):

<< The implementation of a specific therapy is necessary whenever a peripheral nerve lesion is suspected. >>

 2a Presence of pain: spontaneous? Provoked by palpation?  Or by movement?

<< The pain may be absent.  If present, it may be spontaneous, provoked by palpation or by movement. >>

2b Recent occurrence of neurological dysfunction (sensory, motor, autonomic)?

<< Neurologic dysfunction may be sensory (sensory deficit or paraesthesia) and/or motor and/or autonomic. >>

 2c Definition of "recent"?  With or without pain?

<< "Recent" means within a few weeks.  Pain may be absent. >>

3) Relevance of electromyography and nerve conduction studies?

<< Electromyography is useless.  Nerve conduction studies are useful; it allows the diagnosis in dubious cases, detecting the nervous lesion, while waiting echography and surgical approach.  Nerve conduction has proved to be useful in our experience demonstrating focal modifications in quite modest or dubious cases and previous to the appearance of clinical symptoms. >>

4) Strategies for early detection of incipient neuritis during follow-up of known patients?

<< The patient needs instructions about the possible symptoms leading to a dramatic evolution.  Sensory testing and voluntary muscle test are performed periodically.  Nerve conduction study is useful. >>

Best regards,

Lizia

Dr Lizia Reni

Department of Neurology

University of Genoa,

Genoa, Italy

Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition

Leprosy Mailing List – February 2^nd, 2012 

Ref.:    Proposal for "Guidelines for the management of acute neuritis in leprosy” – Part I. Definition

From:  H K Kar, New Delhi, India

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Dear Dr Noto,

Thank you very much to Dr Mahé and to the Association of the French speaking leprologists for this initiative (LML 18^th Jan. 2012).  Herewith I am trying to give my opinion on the first question:

1) What is your definition of 'acute leprosy neuritis' ?

My definition:-

<< Sudden onset of acute inflammation of the nerve presenting with acute pain in peripheral nerve or nerve tenderness and/or swelling due to nerve abscess with or without recent onset of neurological deficit of usually 6 months duration in a leprosy patient is called as “acute leprosy neuritis”.

Best regards,

Dr (Prof.) H K Kar
Consultant & HOD
Department of Dermatology, STD & Leprosy
P.G.I.M.E.R. and Dr Ram Manohar Lohia Hospital
Baba Kharag Singh Marg
New Delhi-110001

Disability Grading Survey – available in English, French and Portuguese - deadline extended to 17th February

Leprosy Mailing List – February 9^th, 2012 

Ref.:    Disability Grading Survey – available in English, French and Portuguese - deadline extended to 17^th February

From:  C Smith, Aberdeen, Scotland, UK

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Salvatore,

We have had an excellent response to the Survey on disability grading – so far 237 people have completed it.  I would like to extend the deadline to 17^th February for anyone who has still to complete the survey – it is available in English, French and Portuguese.

http://www.abdn.ac.uk/~sme577/Leprosy  (English)

http://www.abdn.ac.uk/~sme577/Leprosy%20French/

http://www.abdn.ac.uk/~sme577/Leprosy%20Portuguese/

I have asked a panel of experts to give their answers and I will circulate this with the survey finding on 24^th February.

Many thanks,

Cairns Smith.

School of Medicine and Dentistry,

University of Aberdeen,

Polwarth Building,

Foresterhill,

Aberdeen AB25 2ZD,

Scotland, UK

Telephone - (44) 1224 437266

Email: w.c.s.smith(at)abdn.ac.uk

Further information

Accurate assessment of disability due to leprosy using the WHO Disability Grading is important.  It is used to monitor diagnosis (it indicates whether diagnosis is early or late) and it is used as an indicator of the effectiveness of treatment (the progress of patients during MDT is monitored using the WHO disability grading).   

The WHO disability grade is a simple method to assess impairments in the Hands, Eyes and Feet of patients taking MDT.  It is described in the Operational Guidelines for the Enhanced Global Strategy (2011-2015) – see page 22 – 25.  The overall WHO Disability Grade (the WHO maximum Disability Grade) should be recorded for each patient, but it is also recommended that a patient’s progress through treatment should be assessed using the EHF score. The EHF score is when the grading for both eyes, both hands and both feet are added together.  

It is important that the WHO Disability Grades are accurate and reliable.  There is wide variation in the disability grading between and within countries.  This may be due to real differences or due to differences in the way the Grading is implemented.   We would like to check to see if disability assessments are conducted and recorded in the same way everywhere. To help assess this situation, we would like as many people as possible to complete the following 20 questions (your answers will be completely anonymous).  We will circulate the answers on the LML

Original English:  http://www.abdn.ac.uk/~sme577/Leprosy