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)

Summer School on Disability & Development. Depok, West Java, Indonesia from 18 June to 13 July 2012

Leprosy Mailing List – April 2^nd, 2012 

Ref.:    Summer School on Disability & Development.  Depok, West Java, Indonesia from 18 June to 13 July 2012
From:  W. H. van Brakel, Amsterdam, The Netherlands

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Dear Salvatore,

Could you kindly circulate the following message on your list.

Summer School on Disability & Development

Join us this summer in Indonesia for the 3^rd Summer School on Disability & Development!  This international, English-medium course is offered by the University of Indonesia and the Athena Institute, VU University Amsterdam to be held in Depok, West Java, from 18 June – 13 July 2012.  Fees for disabled participants are negotiable.  The course is aimed at a Master-level of study, but the problem-based learning approach used and the large quantity of scientific papers and other documents provided allows participants to study in as much depth as they desire.  A good level of fluency in both spoken and written English is required.  Detailed information is available in the attached documents (Word, PDF).  The course is about disability in general, but leprosy is often used as a model of disablement.

With kind regards,

Wim van Brakel

Course Coordinator

Wim H. van Brakel, MD MSc PhD

Coordinator Disability & Development

Athena Institute

Faculty of Earth & Life Sciences

VU University Amsterdam

Tel. +3120 598 2529

Mobile +316 3035 9935

Email w.van.brakel(at)vu.nl

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

Global interruption of transmission by 2020

Leprosy Mailing List – March 13th, 2012 

Ref.:    << Global interruption of transmission by 2020 >> (?)
From: D. Soutar, London, UK

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Dear Salvatore,

In London on 30^th January, 2012 a major meeting on Neglected Tropical Diseases took place in London.   WHO took the opportunity of this meeting to launch a new document entitled:

Accelerating Work to Overcome the Global Impact of Neglected Tropical Diseases: A Roadmap for Implementation.  The link here is to the full version of the document but I would draw your readers’ attention to the paragraph on Leprosy/Hansen’s disease which is one of the diseases listed in the Roadmap as being targeted for“Global Elimination by 2020”.

http://www.unitingtocombatntds.org/downloads/partners/who_ntd_roadmap_2012.pdf

<< 5.3 Leprosy (Hansen disease)

Of the 122 countries considered endemic for leprosy, 119 have eliminated the disease as a public-health problem (defined as achieving a prevalence of less than 1 case/10 000 population).

The 213 000 cases known to remain are confined mostly to 17 countries reporting more than 1000 cases annually. This number reflects the more than 90% reduction in the number

of cases detected since 1985, mainly as a result of timely case-finding and multidrug therapy. Transmission continues in limited geographical areas of several countries that were

previously highly endemic. Vigorous case-finding and treatment would lead to global interruption of transmission by 2020, and reduce grade 2 disabilities in newly detected cases to

below 1/million population at global level. The development of methods to increase specificity of diagnosis, notably for paucibacillary leprosy, will enhance the elimination strategy. >>

In launching the Roadmap for Implementation WHO DG Margaret Chan herself expressed confidence that “almost all of these diseases can be eliminated or controlled by the end of this decade.”  Leprosy of course is one of the NTDs the Roadmap indicates is targeted for “Global Elimination by 2020”.  This is still based on the definition of elimination as less than one per 10,000 population and is the global target which was already achieved and announced as long ago as 2001.

It was interesting that yet again in such a high level forum people were returning to the thorny question of ‘control or elimination’.  Indeed, at least three of the key speakers on the panels posed the caveat of needing to be very clear about whether we are talking about ‘control’ or ‘elimination’.  Professor Christopher Whitty, for example, the UK Government’s Chief Scientific Adviser and Director of Research and Evidence, warned against falling in to the trap of ‘over-promising’ outcomes which might not in reality be attainable.  He stressed that as many diseases got closer to being eliminated, they required even more effort to cross the final hurdle.

With best regards

Doug

Douglas Soutar
General Secretary
International Federation of Anti-Leprosy Associations
Tel: 44 (0) 207 602 69 25 – Fax:  44 (0) 207 371 16 21 – Website: www.ilep.org.uk
E-mail: doug.soutar(at)ilep.org.uk

18th International Leprosy Congress, Brussels 16-20 September, 2013 – First announcement

Leprosy Mailing List – March 12th, 2012 

Ref.:   18^th International Leprosy Congress, Brussels 16-20 September, 2013 – First announcementFrom: M. Virmond, São Paulo, Brazil

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Dear All,

With great pleasure I forward to the leprosy mailing list the message from the President of the International Leprosy Association, Dr Marcos Virmond, with the first announcement of the 18^th International Leprosy Congress that will be held in Brussels, Belgium from the 16 to the 20 September 2013.

Kindly, see in attachment the files in Word and PDF formats.

Best regards,

S. Noto

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

"On service training in leprosy” in Mato Grosso and Mato Grosso do Sul, Brazil.

Leprosy Mailing List – March 8^th, 2012 

Ref.:    "On service training in leprosy” in Mato Grosso and Mato Grosso do Sul, Brazil.

From:  J Barreto, S. Paulo, Brazil

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Dear Salvatore,

Thank you in advance for sharing this experience of mine about << "on service training in leprosy" >> with the leprosy mailing list readers.

Since 2007, the German leprosy relief association (DAHW) and the Instituto Lauro the Souza Lima (ILSL) have adopted the methodology of "on service training in leprosy", in the states of Mato Grosso and Mato Grosso do Sul in Brazil.  The method consists of 3 steps:

First: evaluation of the previous knowledge about leprosy.

Second: a lecture, with explanation of what is, indeed, leprosy, i.e., its epidemiology, microbiology, classifications (Madrid, R&J, WHO), clinical diagnosis, laboratorial findings and differential diagnosis, management of the disease and its reactions, and prevention of incapacities.

Third: supervised practice, with recently diagnosed patients, with sensitivity tests (thermal, pain and tactile), palpation of nerve trunks, collection of skin smears and staining of slides "in loco", and evaluation of their household contacts.

Results of the last year (2011):

Number of "on service trainings": 13.

Number of municipalities which participated: 79. 

Number of health professionals trained: 1031, and among them 261 were physicians.

Number of patients evaluated: 588, and among them 123 (almost 20%) new cases were diagnosed.

The complete results of each work can be seen in the site www.dahwmt.org.br.  Finally, the amount of money which was spent to achievement of this results: less than U$ 70.000,00. 

And I think to myself: what could we do with U$ 1 million?

Regards,

Jaison

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

W.H.O. news release. Final [*] push needed to address leprosy in Western Pacific

Leprosy Mailing List – March 4^th, 2012 

Ref.:    W.H.O. news release.  Final [*] push needed to address leprosy in Western Pacific

From:  M. Vollset, Bergen, Norway

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Dear Dr. Noto,

Thank you very much in advance for circulating the enclosed message and link.  W.H.O. Western Pacific published a press release to mark the start of their three-day meeting on leprosy in the Western Pacific Region.
<< MANILA, 13 February 2012 – “Final push needed to address leprosy in Western Pacific - WHO's Western Pacific Regional Office today urged its Member States to make a final push to address leprosy in the Region.” >>

http://www.wpro.who.int/media_centre/press_releases/pr_20120213.htm 

Best regards,

Magnus Vollset

University of Bergen, Norway

[* after elimination in the year 2000 and the “final” push launched in 2005 this would be one more W.H.O. “final” push. (S. Noto)]

"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

Disability index for leprosy patients. The Bechelli’s index

Leprosy Mailing List – February 25^th, 2012

Ref.:    Disability index for leprosy patients. The Bechelli’s index

From:  E. Rangel, Rio de Janeiro, Brazil

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Dear Salvatore,

Thank you very much to Prof Smith for sharing on the leprosy mailing list the results of the Disability Grading Survey.  In our service, here at FIOCRUZ, I use the BECHELLI’s index.  Kindly find in attachment the references (Word document - PDF document)

Best regards,

Emanuel Rangel
Physiotherapist

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

Disability Grading Survey Results

Leprosy Mailing List – February 25^th, 2012 

Ref.:    Disability Grading Survey Results

From:  C Smith, Aberdeen, Scotland, UK

---

Dear Salvatore,

I would be grateful if you could post the results of the Disability Grading Survey on the LML.  I have attached versions in English and Portuguese kindly translated by Duane Hinders. 

Cairns

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@abdn.ac.uk 

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

---

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

---

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