MYOPATHY IN SHEEP

2
841 Colonel Wright has made a valuable contribution to the literature on war wounds. I am putting it on the shelf alongside Jolly’s Field Surgery in Total War, the Field Surgery Pocket-Boolc, and the British Journal of Surgery’s war surgery supplements. Edinburgh. JAMES A. Ross. A FATAL CASE OF GLANDULAR FEVER SIR,-I was interested in the report by Dr. Shinton and Dr. Hawkins (Oct. 6). I note that their list of fatal cases does not extend beyond 1950. In 1952 Dr. Keith Millingen and I reported a fatal case of glandular fever with some unusual features.1 There are certain simi- larities between the two cases. In both the patient was a young man with typical sero- logical and hsematological findings (both having a slight leucopenia), and a raised blood-urea level. In our case, how- ever, the hepatic element was more in evidence, with definite jaundice. The liver showed perilobular fibrosis and cellular infiltration of the portal areas. The kidneys showed tubular degeneration with cloudy swelling, blood, and granular casts. All organs sectioned showed infiltration with mononuclear cells of glandular-fever type. It appears that in the case of Shinton and Hawkins the lymph-nodes were more severely and extensively involved than in our case, where the main lesions were in the coeliao and hepatic hilar glands. The heart and lungs manifested little change, apart from the presence of bile-stained fluid in the associated sacs. In neither case is the high terminal blood-urea level quite explained by the histological findings, although apparently more renal damage was seen in our sections. How much of the " hepatitis " in this case was directly due to the disease, or secondary to obstruction by hilar gland enlargement, was rather debatable. London, W.l. STANLEY F. MARSHALL. TETANUS SIR,-The management of a case of tetanus is such an exacting responsibility, the outcome of the individual case so doubtful, and personal experience of this rela- tively rare disease necessarily so limited, that one welcomes the publication of detailed case-reports. Reading recent accounts one wonders if the complexities of some modern treatment routines really constitute an advance. Our latest case of tetanus demanded two breakfasts yester- day morning (Oct. 11) ; he ate both without help and without masseter spasm. He is a 3-year-old boy ; his incubation period is indeterminate, for he had no culpable wound. Difficulty with feeding because of lock-jaw was first experienced on Sept. 19. Before transfer by ambulance to this hospital on Sept. 20 he was given rectal tribromethanol (’ Avertin ’) at the anaesthetic dose of 100 mg. per kg. estimated body-weight, and from that time till Oct. 8 he was under ,continuous tribromethanol ansthesia : this formed the basic treatment. A quiet room, meticulous attention to details of nursing (particularly directed to avoiding any unnecessary stimulation), aspiration of pharyngeal secretions, the bed raised at the foot, nightly gastric-drip feeds, and prophylactic penicillin were important adjuvants to the basic treatment. An elective tracheotomy would have been undertaken had difficulty been experienced with his air-way : the emergency tracheotomy set was kept at the bedside. An indwelling catheter in the rectum allowed tribrome- thanol to be given with minimum disturbance ; the level of anaesthesia varied from so deep that moderately painful stimuli were tolerated to so light that reflex swallowing and slight muscle spasm were observed : on three occasions he became so " light " that severe spasms and cyanosis occurred. At the height of his illness 1-0 g. of tribromethanol was needed two-hourly to maintain a satisfactory depth of anaesthesia (not all was retained), and a total of 109-3 g. was given in twenty days before he could be allowed to " come round," to be " tapered off " with chloral hydrate by mouth. Desperate diseases require desperate remedies ; but parenteral fluid therapy, muscle relaxants, intravenous 1. Marshall, S., Millingen, K. S. Brit. med. J. 1952, i, 1325. and inhalation anaesthesia, positive-pressure respiration, and even tracheotomy, have such theoretical and practical disadvantages in the management of tetanus that the simplicity and effectiveness of tribromethanol deserve to be remembered. Yet only a year or two ago we had the greatest difficulty in securing sufficient tribro- methanol to complete the treatment of another case, so little is this admirable substance now used in this country. As Auld and Forbes point out/ the mortality of 21% in 47 cases achieved by Cooke 2 using tribromethanol has yet to be bettered in a comparably large series. II)SWiell. R. M. MAYON-WHITE. MYOPATHY IN SHEEP SiR,-The paper last week by Dr. Bosanquet and her colleagues must surely have been of absorbing interest to all who are interested in human diseases of muscle. The pathological changes in the muscle-fibre which they describe and illustrate certainly bear a close resemblance to those seen in human cases of polymyositis. However, as they rightly point out, identical changes (patchy fibre necrosis and phagocytosis with signs of regeneration) are seen in a variety of other conditions, including rapidly progressive muscular dystrophy, virus infections, and severe myasthenia gravis as well as in a number of experimental conditions (vitamin-E deficiency, poisoning with plasmocid). It is apparent that the muscle-fibre can have only a limited repertoire of response to a variety of noxious influences. In passing, I should like to ask the authors whether their histological studies threw any light upon the cause of the muscular pseudohypertrophy which was sometimes visible macroscopically. Apparently it was not the result of infiltration with fat or fibrous tissue ; were swollen muscle-fibres present in sufficient number to be responsible for this change, and did they contain more myofibrils than the normal Differential diagnosis between muscular dystrophy and polymyositis in the human depends upon a combination of clinical, electromyographic, and pathological findings and cannot always be made on pathological findings alone. Much evidence is available to suggest that in many cases of polymyositis the disease is related to others in the " collagen " group, though some cases presenting a similar syndrome may be due to undefined toxic or meta- bolic factors.3 However, the fact that the condition in sheep appears to have a hereditary basis argues against " collagen" disease as a cause, while the comparative absence of perivascular cellular infiltrates is somewhat against an infective origin, though a virus aetiology is a possibility. On the evidence presented, a nutritional cause would seem at least as likely, particularly in view of the close resemblance of the clinical and pathological features to those seen in myopathic calves 4 and in other animals with vitamin-E deficiency.5 It would, however, be of the greatest interest to know whether treatment with cortisone, neostigmine, vitamin E, or modern antibiotics has been proved ineffective in these animals. A further possibility would be to suggest that this is a hereditary disease, akin to human muscular dystrophy. Dr. Bosan- quet and her colleagues draw an analogy between the myopathy they observed in sheep and the manifestations of facioscapulohumeral dystrophy described by Tyler and Stephens 6 in a large family. They cast doubts upon the validity of the term facioscapulohumeral as a descrip- tion for cases in the latter series and also suggest that available evidence concerning the mode of inheritance of muscular dystrophy is inadequate ; but in fact the 1. Auld, M., Forbes, G. B. Amer. J. Med. 1955, 18, 947. 2. Cooke, J. V. J. Pediat. 1953, 43, 220. 3. Walton, J. N. Proc. R. Soc. Med. 1956, 49, 107. 4. Blaxter, K. L., Brown, F., Macdonald, A. M. Brit. J. Nutr. 1953, 7, 287. 5. West, W. T., Mason, K. E. Amer. J. phys. Med. 1955, 35, 223. 6. Tyler, F. H., Stephens, F. E. Ann. intern. Med. 1950, 32, 640.

Transcript of MYOPATHY IN SHEEP

841

Colonel Wright has made a valuable contributionto the literature on war wounds. I am putting it on theshelf alongside Jolly’s Field Surgery in Total War,the Field Surgery Pocket-Boolc, and the British Journalof Surgery’s war surgery supplements.Edinburgh. JAMES A. Ross.

A FATAL CASE OF GLANDULAR FEVER

SIR,-I was interested in the report by Dr. Shinton andDr. Hawkins (Oct. 6). I note that their list of fatal casesdoes not extend beyond 1950. In 1952 Dr. Keith

Millingen and I reported a fatal case of glandular feverwith some unusual features.1 There are certain simi-larities between the two cases.

In both the patient was a young man with typical sero-logical and hsematological findings (both having a slightleucopenia), and a raised blood-urea level. In our case, how-ever, the hepatic element was more in evidence, with definitejaundice. The liver showed perilobular fibrosis and cellularinfiltration of the portal areas. The kidneys showed tubulardegeneration with cloudy swelling, blood, and granular casts.All organs sectioned showed infiltration with mononuclearcells of glandular-fever type.

It appears that in the case of Shinton and Hawkins thelymph-nodes were more severely and extensively involvedthan in our case, where the main lesions were in the coeliaoand hepatic hilar glands. The heart and lungs manifestedlittle change, apart from the presence of bile-stained fluidin the associated sacs. In neither case is the high terminalblood-urea level quite explained by the histological findings,although apparently more renal damage was seen in oursections. How much of the " hepatitis

" in this case was

directly due to the disease, or secondary to obstruction byhilar gland enlargement, was rather debatable.

London, W.l. STANLEY F. MARSHALL.

TETANUS

SIR,-The management of a case of tetanus is such anexacting responsibility, the outcome of the individualcase so doubtful, and personal experience of this rela-tively rare disease necessarily so limited, that one

welcomes the publication of detailed case-reports.Reading recent accounts one wonders if the complexitiesof some modern treatment routines really constitutean advance.

Our latest case of tetanus demanded two breakfasts yester-day morning (Oct. 11) ; he ate both without help and withoutmasseter spasm. He is a 3-year-old boy ; his incubation periodis indeterminate, for he had no culpable wound. Difficulty withfeeding because of lock-jaw was first experienced on Sept. 19.Before transfer by ambulance to this hospital on Sept. 20 hewas given rectal tribromethanol (’ Avertin ’) at the anaestheticdose of 100 mg. per kg. estimated body-weight, and from thattime till Oct. 8 he was under ,continuous tribromethanolansthesia : this formed the basic treatment. A quiet room,meticulous attention to details of nursing (particularlydirected to avoiding any unnecessary stimulation), aspirationof pharyngeal secretions, the bed raised at the foot, nightlygastric-drip feeds, and prophylactic penicillin were importantadjuvants to the basic treatment. An elective tracheotomywould have been undertaken had difficulty been experiencedwith his air-way : the emergency tracheotomy set was keptat the bedside.An indwelling catheter in the rectum allowed tribrome-

thanol to be given with minimum disturbance ; the level ofanaesthesia varied from so deep that moderately painfulstimuli were tolerated to so light that reflex swallowing andslight muscle spasm were observed : on three occasions hebecame so " light " that severe spasms and cyanosis occurred.At the height of his illness 1-0 g. of tribromethanol was neededtwo-hourly to maintain a satisfactory depth of anaesthesia(not all was retained), and a total of 109-3 g. was given intwenty days before he could be allowed to " come round,"to be " tapered off

" with chloral hydrate by mouth.

Desperate diseases require desperate remedies ; but

parenteral fluid therapy, muscle relaxants, intravenous

1. Marshall, S., Millingen, K. S. Brit. med. J. 1952, i, 1325.

and inhalation anaesthesia, positive-pressure respiration,and even tracheotomy, have such theoretical and practicaldisadvantages in the management of tetanus that thesimplicity and effectiveness of tribromethanol deserve tobe remembered. Yet only a year or two ago we hadthe greatest difficulty in securing sufficient tribro-methanol to complete the treatment of another case,so little is this admirable substance now used in this

country.As Auld and Forbes point out/ the mortality of 21%

in 47 cases achieved by Cooke 2 using tribromethanol hasyet to be bettered in a comparably large series.

II)SWiell. R. M. MAYON-WHITE.

MYOPATHY IN SHEEP

SiR,-The paper last week by Dr. Bosanquet and hercolleagues must surely have been of absorbing interest toall who are interested in human diseases of muscle. The

pathological changes in the muscle-fibre which theydescribe and illustrate certainly bear a close resemblanceto those seen in human cases of polymyositis. However,as they rightly point out, identical changes (patchy fibrenecrosis and phagocytosis with signs of regeneration)are seen in a variety of other conditions, includingrapidly progressive muscular dystrophy, virus infections,and severe myasthenia gravis as well as in a number ofexperimental conditions (vitamin-E deficiency, poisoningwith plasmocid). It is apparent that the muscle-fibrecan have only a limited repertoire of response to a varietyof noxious influences. In passing, I should like to askthe authors whether their histological studies threw anylight upon the cause of the muscular pseudohypertrophywhich was sometimes visible macroscopically. Apparentlyit was not the result of infiltration with fat or fibroustissue ; were swollen muscle-fibres present in sufficientnumber to be responsible for this change, and did theycontain more myofibrils than the normal

Differential diagnosis between muscular dystrophy andpolymyositis in the human depends upon a combinationof clinical, electromyographic, and pathological findingsand cannot always be made on pathological findings alone.Much evidence is available to suggest that in many casesof polymyositis the disease is related to others in the"

collagen " group, though some cases presenting a

similar syndrome may be due to undefined toxic or meta-bolic factors.3 However, the fact that the condition insheep appears to have a hereditary basis argues against"

collagen" disease as a cause, while the comparativeabsence of perivascular cellular infiltrates is somewhat

against an infective origin, though a virus aetiology is apossibility. On the evidence presented, a nutritionalcause would seem at least as likely, particularly in viewof the close resemblance of the clinical and pathologicalfeatures to those seen in myopathic calves 4 and in otheranimals with vitamin-E deficiency.5 It would, however,be of the greatest interest to know whether treatment withcortisone, neostigmine, vitamin E, or modern antibioticshas been proved ineffective in these animals. A furtherpossibility would be to suggest that this is a hereditarydisease, akin to human muscular dystrophy. Dr. Bosan-quet and her colleagues draw an analogy between themyopathy they observed in sheep and the manifestationsof facioscapulohumeral dystrophy described by Tylerand Stephens 6 in a large family. They cast doubts uponthe validity of the term facioscapulohumeral as a descrip-tion for cases in the latter series and also suggest thatavailable evidence concerning the mode of inheritanceof muscular dystrophy is inadequate ; but in fact the

1. Auld, M., Forbes, G. B. Amer. J. Med. 1955, 18, 947.2. Cooke, J. V. J. Pediat. 1953, 43, 220.3. Walton, J. N. Proc. R. Soc. Med. 1956, 49, 107.4. Blaxter, K. L., Brown, F., Macdonald, A. M. Brit. J. Nutr.

1953, 7, 287.5. West, W. T., Mason, K. E. Amer. J. phys. Med. 1955, 35, 223.6. Tyler, F. H., Stephens, F. E. Ann. intern. Med. 1950, 32, 640.

842

descriptions of Tyler and Stephens show that their caseswere suffering from facioscapulohumeral dystrophy in

typical form, while recent work has resulted in substantialagreement upon the mode of inheritance of the variousforms of human muscular dystrophy. 7 8 Furthermore,facioscapulohumeral dystrophy is generally the most

benign and chronic form of the disease, in which acutedegenerative changes in the muscle-fibre are very uncom-mon ; for practical purposes, changes approaching theseverity of those illustrated in the myopathic sheep areseen only in rapidly progressive cases of muscular

dystrophy, usually those of the Duchenne (pseudo-hypertrophic) type.

I do not wish to deny the fascinating possibility thatthe sheep myopathy may be akin to human musculardystrophy, but clearly much more information is neces-sary before this hypothesis can be tested. Controlledbreeding should be carried out to determine whether thedisease is inherited and, if so, the pattern of inheritance.A hereditary myopathy in mice, in which the histologicalchanges in the affected muscles are much more likethose in human muscular dystrophy, has been describedrecently. 9 The availability of at least two types of

experimental animal will undoubtedly open new avenuesof research which may throw light on many outstandingproblems in our understanding of diseases of muscle inman.

Royal Victoria Infirmary,Newcastle upon Tyne. JOHN N. WALTON.

TORSION OF THE TESTIS

SiB,—I was most interested to read about Mr. Scott’sthree atypical cases of torsion of the testes (Sept. 15).I am sure he is quite right in saying that many casesare missed because the clinician does not think of the

possibility. A sign that I have found most useful isthat the oedema of the scrotum is mainly anterior;but unfortunately by this time the testis is often

gangrenous.I was disappointed that Mr. Scott did not stress the

importance of fixing the opposite testis. I have alwaysmade a practice of this as I am convinced the conditionis due to a long congenital mesorchium, which is usuallybilateral, with the result that the opposite side frequentlyundergoes torsion at a later date. I have never found anydisadvantage in doing this at the same time as the

original operation.Hove, Sussex. S. HENRY C. CLARKE.S. HENRY C. CLARKE.

A RESIDENT’S COMPLAINT

SIR,-Before hospital management committees are

inundated with requests from medical staff to be paidrebates on the sums deducted for their board and

lodging whilst on holiday, I think that your readersshould be told that the reply received by Dr. Barber(Oct. 6) from the Ministry to his inquiry is completelymisleading.

First, in the case of house-officers, the terms and conditionsof service of hospital medical staff published in June, 1949,provided for a deduction of 9100 a year for emoluments. In

regard to these officers, it is therefore wrong for the Ministryto say that it is for the employing authority to fix a chargeequal to the value of the services provided.

Secondly, on the assumption that the Ministry’s letter waswritten after Aug. 1, 1956, it is wrong in relation to all medicalstaff because an agreement was reached earlier this year onthe deductions to be made for the board and lodging of allmedical staff. Hospital authorities have not yet, however,been enlightened on what the terms of this agreement areexcept for the amounts of the charges ; more than two monthsafter its effective date the terms of the agreement have stillnot been published.7. Stevenson, A. C. Ann. Eugen., Lond. 1953, 18, 50.8. Walton, J. N. Ann. hum. Genet. 1955, 20, 1 ; Ibid, 1956, 21, 40.9. Michelson, A. M., Russell, E. S., Harman, P. J. Proc. nat. Acad.

Sci., Wash. 1955, 41, 1079.

To my knowledge, the Ministry has always held the viewin regard to house-officers that the charge for board andlodging was fixed at an amount which took account of absenceson leave. The Nurses and Midwives Whitley Council has beensensible enough to regulate the position by agreement, butI think it is pertinent to point out that, whilst house-officersare charged :E125 for board and lodging and other servicesprovided, a sister pays E164 a year for these services and astaff nurse .S153. A student nurse pays only ;&bgr;6 a year less forher emoluments than does a house-officer.

It is always difficult to persuade someone that a chargefixed for board and lodging has made allowance forabsences on leave, and it is much more satisfactory tofix a higher yearly charge and make a rebate for absencesbecause justice will then be seen to be done.

Dartford, Kent. R. MORGAN.R. MORGAN.

INTERACTION OF DEPOLARISING ANDNON-DEPOLARISING RELAXANTS

Sir was interested to read the letter last weekby Dr. Hamer Hodges and Dr. Foldes. , , ,,

For some time I have suggested to my colleagues thatit is dangerous to administer both depolarising andnon-depolarising relaxants to a patient during anaes-

thesia, for on several occasions during the past twoyears I have seen prolonged apnoea due to this cause.

I have suggested that, because the apncea producedby the depolarising drug has worn off, it is wrong toassume that there is no residual effect on the end-plate,and that possibly some breakdown product is still

circulating which may act with the non-depolarisingrelaxant and give a dual block which is perpetuated byneostigmine.

I entirely agree with Dr. Hamer Hodges and Dr. Foldesthat curare-like drugs should never be administered aftera succinylcholine drip, but I would go further : in myexperience it is the small initial dose of succinylcholineused for intubation that may be the cause of prolongedapncea when non-depolarising drugs are used later in theanaesthetic.London, W.I. JOHN GORDON.JOHN GORDON.

CLINICOSIS

SiR,—A family doctor salutes the wisdom of " Richard

Cobbleigh " in your Widdicombe File last week. Out ofthe mouths of locum clinic officers....

Cousin George does not go far enough. The refereeingis not a personal insult-it is the cause of unnecessaryovertime, for the clinic gives advice cheerful in the

knowledge that by the time the parent has found thatthe advice does not work the clinic will be shut and thefamily doctor can take over the work he should havehad at first. In addition to his lack of knowledge of thefamily as a unit, the clinic doctor is handicapped byhis inability to learn by his mistakes, for these are

corrected by the family doctor who is thereby too busyto undertake the postgraduate education of office-hours doctors.

My remedy would be more drastic than Dr. Cobbleigh’s.Sell the clinics, stop all further building of clinics. Investthis money, and with the money so obtained providehealth visitors and part-time secretaries for the familydoctors. The money usually spent on running the clinicscan be used to provide general relief of rates and taxes.Allow the doctors so released from duplicated work to goto the underdoctored areas and join the ranks of whichI am proud to be a member as a family doctor.

Bitterne, E SELDON STOWELL.Southampton.

SIR,—Richard Cobbleigh should be his age. Thesecaustic things have been said by one branch of the

profession about another, ever since the cult of medicinebegan ; they are stale, they are half-truths, they are

terribly pharisaical. Why not go one further and acknow-

ELDON STOWELL.