Request of information about a tool designed to measure and monitor ulcers

Ref.:   Request of information about a tool designed to measure and monitor ulcers.

From: D Andersen, Chennai, India

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Dear Dr Salvatore,

Thank you for adding me to the mailing list.  I am a student at Brigham Young University in the U.S. and I am conducting an internship with a leprosy rehabilitation organization called Rising Star Outreach; they operate outside of Chennai in India.

I am currently seeking a tool designed to measure and monitor ulcers.  I am evaluating an organizations health services which includes a mobile medical clinic that visits leprosy colonies bi-weekly to provide any treatments, as well as training and supplies for self-care.  So, the tool needs to be sensitive enough to measure the impact their programs have on the treatment of ulcers.  Any direction for finding such a resource would be greatly appreciated.

Thanks,

Dane Andersen
Project Evaluation and Assessment Team
Brigham Young University

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 DiaLep study - Combining care interventions for people affected by either leprosy or diabetes

Leprosy Mailing List – May 5^th, 2012

Ref.:    The DiaLep study - Combining care interventions for people affected by either leprosy or diabetes
From:  W de Bruin, Amsterdam, The Netherlands

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Dear Salvatore,

Some time ago a request to participate in the DiaLep study by filling in a questionnaire was posted in the LML.  We would kindly like to remind you to fill in this questionnaire.  With as many responses as possible we aim to achieve a large sample and a broad perspective of the opinions on this topic worldwide.

The DiaLep study aims to research  the possibility of combining care interventions for people affected by either leprosy or diabetes.  The questionnaire is available online at: https://www.surveymonkey.com/s/dialepstudy.

It will take approximately 15 minutes to complete the questionnaire.  We would appreciate if you could provide your input by 7 May 2012.  An offline version of the questionnaire is in attachment to this message; please only use it in case of a limited internet connection.

We encourage you to complete the questionnaire and to pass the link on to appropriate health-care workers, professionals and representatives of patient organisations active in the field of either leprosy or diabetes.

Many thanks!

Kind regards,

Willemijn de Bruin and Evelien Dijkkamp

Willemijn de Bruin

Leprastichting / Netherlands Leprosy Relief (NLR)

Postbus / P.O. Box 95005

1090 HA Amsterdam

The Netherlands

Tel:

Mob:

E-mail: W.d.Bruin(at)Leprastichting.NL

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

The DiaLep study

Leprosy Mailing List – April 12^th, 2012

Ref.:    The DiaLep study
From:  W de Bruin, Amsterdam, The Netherlands

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Dear Salvatore,

I would be very grateful if you could post the following request via the leprosy mailing list (LML). 

Currently, the Netherlands Leprosy Relief is researching the possibility of combining care interventions for people affected by either leprosy or diabetes.  A study and a questionnaire have been developed for people with expertise in leprosy or in diabetes.  The name of the study is the DiaLep study.  We kindly ask the interested LML readers to contribute to our study by filling in the questionnaire.

It is available online at: https://www.surveymonkey.com/s/dialepstudy .  It will take approximately 15 minutes to complete the questionnaire.  We would appreciate if you could provide your input by 7 May 2012.  An offline version of the questionnaire is in attachment to this message; please only use it in case of a limited internet connection.

We encourage you to complete the questionnaire and to pass the link on to appropriate health-care workers, professionals and representatives of patient organisations active in the field of either leprosy or diabetes.  With as many responses as possible we aim to achieve a large sample and a broad perspective of the opinions on this topic worldwide.

The contact information of my colleague and mine are as follow:-

Email addresses are  w.d.bruin(at)leprastichting.nl and e.dijkkamp(at)leprastichting.nl

Office phone number is  +31 20 595 05 00.

Many thanks in advance for your collaboration and support.

Kind regards,

Willemijn de Bruin and Evelien Dijkkamp

Willemijn de Bruin

Leprastichting / Netherlands Leprosy Relief (NLR)

Postbus / P.O. Box 95005

1090 HA Amsterdam

The Netherlands

E-mail: W.d.Bruin(at)Leprastichting.NL

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)

Clinical case. Borderline leprosy in reaction in a boy

  Leprosy Mailing List – March 28th, 2012 

Ref.:   Clinical case. Borderline leprosy in reaction in a boyFrom: P. Vijayakumaran, Chennai, India

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Dear Salvatore,

I refer to the clinical case circulated by via the Leprosy Mailing List – March 24th, 2012.  Thank you very much to Dr Barreto and Dr Cabral for sharing the interesting case history (not so interesting for the person affected).  I congratulate the team for providing all details for better understanding of the situation and also appropriate management of the condition. I am not a pathologist.  Here are my impressions:

·         The presentation was atypical so that it could not be related to leprosy.

·         Health staff are not aware of presentations of leprosy.

·         Leprosy referral hospital may have un-trained staff who cannot identify leprosy.

·         Multiple nerve involvement is characteristic of borderline leprosy.  That too with in a period of one year goes more in favour.

·         Bacteriological Index of 2+ at all sites (probably all selective sites – that means active lesions) may indicate that the disease has progressed beyond borderline tuberculoid leprosy (towards lepromatous leprosy).

·         Biopsy - 3+ positive and presence of globi are indicative of lepromatous side of leprosy spectrum.

·         Biopsy – Globi and macrophages are characteristics of lepromatous side of the spectrum.

Fig.1 & 2               : Any trained eye will suspect leprosy.

Fig.3 & 4               : Misleading presentation because of scaling and central healing.

Fig.5, 6 & 7          : Trained eyes should be able to suspect leprosy.

Fig.8                    : Explains importance of examination of peripheral nerves. Again trained eyes and hands comes to my mind.

Fig.15 & 16          : Clearly clinical presentation of BB leprosy. 

This child is fortunate to have intact nerve function. This also warrants close observation for possibility of fresh episodes of reactions and especially neuritis. 

I once again thank the authors and the LML for sharing their experience. 

With best wishes,

Dr.P.Vijayakumaran,

Regional Medical Coordinator South,

German Leprosy and TB Relief Association India,

#4, Gajapathi street, Shenoy Nagar,

Chennai – 600030, India

Clinical case. Borderline leprosy in reaction in a boy

 Leprosy Mailing List – March 24th, 2012 

Ref.:   Clinical case. Borderline leprosy in reaction in a boy.From: Barreto, J., S. Paulo, Brazil

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Dear Salvatore and Pieter,

Thank you very much for inviting me to circulate a clinical case on the leprosy mailing list.  I believe it is a good and useful initiative.  Herewith we (myself and José Cabral Lopez) present the case of a 5 years old boy from Brazil with borderline leprosy in reaction.  We will really appreciate any comment about the case.

Best regards,

Jaison

Silent neuritis (Quiet Nerve Paralysis)

Leprosy Mailing List – March 9th, 2012 

Ref.:    Silent neuritis (Quiet Nerve Paralysis)From: H Srinivasan, Chennai, India

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Dear Dr Salvatore Noto,

Ref.: Query by Dr Narayanakumar (Kumbakonam, India) about “Silent neuritis”

Thank you, Dr Narayanakumar. My response is as follows :-

The term “Silent neuritis” is used by many to refer to the occurrence of nerve function deficit (NFD), usually motor paralysis, without concurrent or immediately antecedent episode of acute neuritis.  I preferred the term “Quiet Nerve Paralysis” (QNP) to refer to this phenomenon.

While leprologists were aware of its occurrence, I drew attention to the fact that it was associated with the occurrence of deformity in a significant proportion of patients [1].  Here I will not go into the reasons why I preferred the term ‘Quiet Nerve Paralysis’ to ‘Silent Neuritis’.  Interested colleagues may refer to reference [2].  

During the course of my investigations, in the field and in the sanatorium, on the origin and progress of deformities in leprosy patients, I found that motor paralysis and associated deformity was five times more common in patients giving a history of remembered attack(s) of acute neuritis of the concerned nerve trunk than in those not giving such a history.  However, I also found that such patients accounted for only about 20% to 25% of those showing paralytic deformity.  Even allowing for faulty memory, it appeared that a sizable proportion of patients developed deformity without developing acute neuritis.  We designated such patients as having ‘Quiet Nerve Paralysis’.

This group of patients comprised:
1). untreated or inadequately treated patients;
2).Patients adequately treated in the past and discharged as ‘cured’; as well as
3).patients still under treatment.
We hypothesized that uncontrolled leprosy was the cause of nerve paralysis in the first group and instituted proper anti-leprosy therapy in them.  We considered QNP as the manifestation of relapse of leprosy in the second group and treated them again with anti-leprosy treatment of the day.  As for the third group, we felt that, in the absence of other explanations, they probably had “subclinically operating reactional pathology” in them and so treated them with a standard course of prednisolone for three to four months or more depending on their response.  Varying proportion of patients showed partial or complete restoration of nerve function in all the three groups, indicating that our conjectures were probably correct, at least in those patients.  Those in the first two groups who did not show any sign of recovery of nerve function after three months of anti-leprosy therapy were given a standard course of steroid therapy for what it was worth.  If I remember right, there was no clinical evidence suggestive nerve compression in these patients and so nerve decompression was not offered to them.

We subsequently tried to carry out a prospective trial of steroid therapy for QNP in the field, but the results were not reliable due to operational problems.

I should also point out that the patients were from South India, and the study was done during the ‘dapsone era’ when dapsone monotherapy was the standard anti-leprosy treatment.  I do not know what the situation is like in present conditions of years of intensive coverage of the patient population with MDT and fewer cases of active leprosy in the environment.

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

[1] Srinivasan H, Rao KS, Shanmugam N (1982).  Steroid therapy in recent “quiet nerve paralysis” in leprosy. Leprosy in India  54(3) :  412 – 419.
[2] Srinivasan H, Gupte MD.  Experiences from studies on Quiet Nerve Paralysis, Ch. 3  in The Peripheral Nerve in Leprosy and Other Neuropathies, (pp 30 – 35), Ed. by Noshir H Antia & Vanaja P Shetty, Delhi, Oxford University Press, 1997.   

The Grading or Index is NOT a Neurological examination/voluntary muscle test/sensory testing

Leprosy Mailing List – March 8^th, 2012 

Ref.:    The Grading or Index is NOT a Neurological examination/voluntary muscle test/sensory testing

From:  L F Lehman, Belo Horizonte, M G, Brazil.

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Dear Cairns,

Thank you for your message [LML March 6^th, 2012].  Yes, indeed the history of all is interesting. 

Dr. Palande can also give an important account of this early development as he was actually the originator, I believe of the idea, which than was adopted and adapted by W.H.O.  I do know talking with several of the persons involved in the beginning, the whole thing created quite a bit of discussion!

One other big areas of confusion is that many feel when they have done the Grading/Index they have done a "Neurological Examination – Voluntary muscle testing (VMT)".  It is important that readers clearly understand the Grading or Index is NOT a Neurological examination/VMT.  

Dr. Srinivasan [and Drs Schreuder and Naafs as well] points out that we need to combine Nerve palpation (pain responses) Sensory and Motor exams.  I am concerned that many programs have nerve palpation in one area of their clinical exam and then off in the physiotherapist part, the motor and sensory.  The Neurological exam is part of the Clinical exam and should combine all 3 parts.  Clinicians should know how to do and interpret results.  The neurological exam as well as education to help patients identify neurological changes is important.  Doing the Grading/Index each month may not be needed.  It is a concern that more time sometimes is spent on exams with little time spent working on self-care practice and education.  What is even more of a concern is that sometimes no action is taken when the exam results shows things have gotten worse.  The health team at times do not look at and/or do not know how to interpret the results.  Practice in interpretation and choosing what should be done is an important part of training/supervision.  Case studies can be helpful.

The other issue is that I see many programs record grading/indexes based on ONLY hand and foot exams and the eye is not even examined and/or perhaps the patient is asked "Do you see OK".  When I check the distance that fingers are counted, it varies greatly as people mark off the distance with their leg length of what they think is 6 meters.  It usually is less by at least a meter or 2.  

The quality of the Grading/Index has to be constantly monitored and clarified to try and get consistency.  Although it seems simple, I have seen it interpreted and additions slipped in without others knowing until close observation and inquiry is done.  I recall much effort was put into trying to get better definitions, clarity and consistency from 1997-2003 in Brazil.  National guidelines tried to include clearer definitions.   We worked hard at it and much improved however today this needs to be checked again.  I know this is something that all countries must monitor carefully.

Linda

Disability indices

Leprosy Mailing List – March 6^th, 2012 

Ref.:    Disability indices

From:  C Smith, Aberdeen, Scotland, UK

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Dear Emanuel,

Thank you for giving details of the Bechelli Index used in FIOCRUZ [LML Feb. 25^th, 2012].  I know it well but I had called it the Disability Index.  I used it extensively in the 1970s and 1980s in my doctoral thesis using the Disability Index 2 as the outcome in leprosy control – see the attached papers.

There were 3 indices proposed in the 1971 paper by Bechelli and Dominguez.  I wondered when the grading changed from a scale of 0,1,2, and 3 to a scale of 0,1 and 2.  The answer is in the WHO Expert Committee on Leprosy Report 768 published in 1988 and the change is described in the WHO Guide to Leprosy Control, 2^nd Edition in 1988.  The reason given for the change was that the grading system was considered to be ‘rather beyond the comprehension of primary health workers’.  1988 was the period when the uptake of MDT to replace dapsone mono-therapy was being advocated, and primary care was becoming involved in the delivery of leprosy services. 

The index you are using is the new 0,1 and 2 scale recommended by the Expert Committee in 1988 to calculate the Disability Index 2 (Bechelli Index 2) by adding all the scores for each eye, hand and foot and dividing by 6 to give an average.  The EHF score is actually the Bechelli Index 1 where the maximum score for each eye, hand and foot is added to produce the total score rather than the average score.         

The questions raised by Linda Lehman [LML March 1st, 2012] are very relevant about which index to use and when?  The Bechelli Index 2 which you use and the EHF score (Bechelli Index 1) are useful in monitoring progress in individual patients whereas the Grade 2 maximum score is more limited to monitoring early case detection in programmes with less expertise.

The important point is that we need to be clear which assessment we are using for which purpose, and that we all use them in the same way to be consistent and to provide comparable information.

Many thanks,

Cairns

"Disability Grading" is helpful to look at things form a GENERAL PUBLIC HEALTH perspective …

Leprosy Mailing List – March 1st, 2012 

Ref.:    "Disability Grading" is helpful to look at things form a GENERAL PUBLIC HEALTH perspective …

From:  L Lehman, Brazil

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Dear Salvatore,

Thank you for the posting of the results from the Disability Survey from Prof Cairns Smith - well done.

It highlights the areas of confusion and inconsistencies.  It shows us how to focus training and where we must monitor as new people come into health services.  I must personally say that the publication from Brandsma in 2003 of his "Proposed Grading" created confusion in several countries and health services I visited.  They thought it was an "Official W.H.O. Grading " that was supposed to be changed.  Brandsma's Proposal in Leprosy Review in 2003 is not acceptable by all but, could lead to further discussions as pointed out in this survey.

It might be worthwhile to discuss the PURPOSE of the Grading.  Many try to use it beyond its capabilities and Ebenso and Ebenso addressed this in their article in Leprosy Review a few years ago.  I do not believe it can substitute for doing a good clinical examination and documentation of the impairments found on the face, eyes, hands, feet and body.  It too does not look at the WHOLE person and the effects of the disease on their ability to do activities or participate socially - that is one of the reasons the SALSA and P-scale were developed and later an instrument to measure stigma.  

This "Disability Grading" is helpful to look at things from a GENERAL PUBLIC HEALTH perspective (providing it is done correctly and all are using the SAME criteria).  It can give us an idea of the following:

1. Early Diagnosis and if Health education is helping people identify and seek treatment early;

2. QUALITY of Treatment/Care.  Determine if New cases have been MANAGED adequately.  (In addition to multi-drug therapy (MDT), Completion of a cohort analysis comparing the Grades at Beginning and end of MDT Treatment gives one a better idea of quality of care).

I found when at the end of treatment the Grade was worse it usually was related to:

a. people not managing reactions well;

b. not doing adequate self-care education which included Grade 0 with reactions, Grade 1 & 2;

c. not having or using adequate protective footwear for those feet at RISK.  

The COHORT analysis of the grades at beginning and end of treatment may be one of the best QUALITY Indicators we could use on a more global level.  We do it for multibacillary and paucibacillary MDT completion rates, why can this not be considered?   I found when I did it with individual health services, it opened their eyes to areas in their management and care that needed attention.  

Another issue needing further discussion is the labeling of persons as "Disabled" based on the Grading.  This could be addressed at another time. 

Thank you again for this excellent work!

Linda Lehman

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