LEONTIASIS OSSIUM

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457 known to affect the central nervous system. The serum of a patient who had recovered in 1933 from louping-ill gave positive complement-fixation and neutralisation tests with’the viruses of both louping-ill and Russian spring-summer encephalitis, as did the serum of a patient who had an encephalitic illness while working with the two viruses in the laboratory. There is therefore, according to Casels and Webster, a striking similarity between the two viruses in their clinical effects, modes of transmission, and immunological responses. SUMMARY Two cases of louping-ill in man are reported, with evidence suggesting that the infection was acquired naturally and not in the laboratory as in previous cases. The clinical picture of louping-ill in man is that of meningo-encephalitis, usually following a prodromal influenza-like illness and followed by complete recovery in most cases. i It is suggested that the illness would be more often diagnosed if it were suspected whenever a meningo- encephalitis appears in a person exposed to louping-ill virus. The evidence linking louping-ill with Russian spring- summer encephalitis is given. The credit for diagnosis in case I lies essentially with the patient, Mr. William Lyle Stewart, of the Department of Agriculture, King’s College, Newcastle-on-Tyne. To him we are indebted also for a clear history of his symptoms, for advice on how the diagnosis could be established, and for sources of further information. lhanks are also due to Dr. W. S. Walton, medical officer of health for Newcastle-upon-Tyne, and to Dr. George Hurrell and Dr. E. G. Brewis, medical superintendents of the Newcastle General Hospital and the City Hospital for Infec- tious Diseases respectively, for facilities provided ; Dr. R. Norton and Dr. Emslie Smith for bacteriology ; Mr. G. F. Rowbotham for neurosurgical advice ; Dr. S. Whately Davidson for radiological reports ; Dr. W. H. H. Merivale for biochemistry ; Dr. Paul Szekely for helping one of us (G. D.) with translation of the literature ; and Dr. T. H. Bates, Dr. L. Richmond, and Dr. G. W. L. Kirk for reference of the patients and information given. REFERENCES Brownlee, A., Wilson, D. R. (1932) J. comp. Path. 45, 67. Casals, J., Webster, L. T. (1943) Science, 97, 246. &mdash; &mdash; (1944) J. exp. Med. 79, 45. Findlay, G. M., Elton, C. (1933) J. comp. Path. 46, 126. Gordon, W. S., Brownlee, A., Wilson, D. R., MacLeod, J. (1932) Ibid, 45, 106. Hurst, E. W. (1931) Ibid, 44, 231. MacLeod, J., Gordon, W. S. (1932) Ibid, 45, 240. Pool, W. A., Brownlee, A., Wilson, D. R. (1930) Ibid, 43, 253. Rivers, T. M., Schwentker, F. F. (1934) J. exp. Med. 59, 669. Somorodintsev, A. A. (1944) Amer. Rev. Soviet Med. 1, 400. Wesemeier, K. (1938) Dtsch. Arch. klin. Med. 182, 451. Wiebel, H. (1937) Kiln Wschr. 16, 632. LEONTIASIS OSSIUM C. BERKELEY WAY M.C., M.B. Lond. MEDICAL OFFICER, HENDON COTTAGE HOSPITAL, LONDON WITH the improvements that have taken place in dentistry and in the surgery of the nasal accessory sinuses, cases of leontiasis ossium should become rare. The skull from the case reported here has been accepted by the museum of the Royal College of Surgeons. The patient was a spinster, who died in 1944 at the age of 73. Her appearance and health were normal until the age of 20, when she had a bicuspid tooth stopped. She had no pain, but a few months afterwards a hard swelling developed in the gum above the tooth, followed later by similar changes in the cheek. Much dental treatment followed, but the swelling steadily increased, spreading to the other cheek and into the frontal region. Beyond occasional headaches the condition caused her no physical or social inconvenience. She was a charming, witty, and well-beloved lady who continued her work as-a book-keeper until four months before her death, with only one short period of ill health. In 1939 she had a transient hemiplegia : blood- pressure 220/95 mm. Hg; left knee-jerk exaggerated and left plantar reflex extensor; otherwise no physical signs. She was working again in six weeks, the left knee -j erk remain- ing slightly greater than the right and the left plantar reflex indefinite until her death. The B.P. con- tinued high, and there was some cardiac enlarge- ment. Four months before her death she left her office well, but later was found crawling up the stairs to her flat. From then onwards muscular control slowly left her; deaf, ness, which had not been present before, became intense ; and her mental faculties were much slowed. However, if one Fig. 1-" Lion-like" head of patient with leontiasis ossium. COU1Q Wal1i,SIleWOUlU tJVt::::11tJUi:tt.UY y Nrv v.euc zc w lUU.y y aama OIpp.LVp.L.la<l.iC&iacute; answer to a question. Swallowing became difficult, hypostatic congestion supervened, and she died in coma. Her nares and intranasal spaces were wide, but there was no crusting. She never had a cold. Her ear drums were normal. Bone conduction was lost. Her mandible was not unduly small in comparison with her sister’s. Radiogram of Fig. 2-Anteroposterior and lateral radiograms of skull. the pelvis was normal. For the condition of the skull see figs. 1 and 2. COMMENTS In view of the hyperpiesia, it can be fairly said that the condition caused little inconvenience to her health and was only slightly contributory to her death. The history appears to follow the usual lines in that a chronic septic osteoperiostitis follows a sinus infection and one by one the accessory air chambers are filled with masses of inflammatory cancellous bone protruding out- wards and causing great disfigurement. Bony changes spread to the skull (cancellous hyperostosis of Virchow) ; the mandible sometimes is involved, but the base of the skull usually escapes. In the present case, however, the base is involved and the mandible is normal. The lion- like head is well depicted in the photograph. The absence of colds and the dryness of the nose suggest that the mucosa lining the sinuses is the major source of nasal secretion. " ... Can there be values greater than the mere attainment of health ? One reason why we have not been more successful in directing people toward healthful living is that sub- consciously many have decided, and quite correctly, that there are more important things than merely being healthy." - W. W. BAUER, M.D., director, bureau of health education, American Medical Association, in Motivation in Health Education. London, 1948 ; p. 20.

Transcript of LEONTIASIS OSSIUM

457

known to affect the central nervous system. The serumof a patient who had recovered in 1933 from louping-illgave positive complement-fixation and neutralisationtests with’the viruses of both louping-ill and Russianspring-summer encephalitis, as did the serum of a

patient who had an encephalitic illness while workingwith the two viruses in the laboratory. There is therefore,according to Casels and Webster, a striking similaritybetween the two viruses in their clinical effects, modesof transmission, and immunological responses.

SUMMARY

Two cases of louping-ill in man are reported, withevidence suggesting that the infection was acquirednaturally and not in the laboratory as in previous cases.The clinical picture of louping-ill in man is that of

meningo-encephalitis, usually following a prodromalinfluenza-like illness and followed by complete recoveryin most cases.

i It is suggested that the illness would be more oftendiagnosed if it were suspected whenever a meningo-encephalitis appears in a person exposed to louping-illvirus.The evidence linking louping-ill with Russian spring-

summer encephalitis is given.The credit for diagnosis in case I lies essentially with the

patient, Mr. William Lyle Stewart, of the Department of

Agriculture, King’s College, Newcastle-on-Tyne. To him weare indebted also for a clear history of his symptoms, foradvice on how the diagnosis could be established, and forsources of further information.lhanks are also due to Dr. W. S. Walton, medical officer of

health for Newcastle-upon-Tyne, and to Dr. George Hurrelland Dr. E. G. Brewis, medical superintendents of theNewcastle General Hospital and the City Hospital for Infec-tious Diseases respectively, for facilities provided ; Dr. R.Norton and Dr. Emslie Smith for bacteriology ; Mr. G. F.Rowbotham for neurosurgical advice ; Dr. S. WhatelyDavidson for radiological reports ; Dr. W. H. H. Merivalefor biochemistry ; Dr. Paul Szekely for helping one of us(G. D.) with translation of the literature ; and Dr. T. H.Bates, Dr. L. Richmond, and Dr. G. W. L. Kirk for referenceof the patients and information given.

REFERENCES

Brownlee, A., Wilson, D. R. (1932) J. comp. Path. 45, 67.Casals, J., Webster, L. T. (1943) Science, 97, 246.

&mdash; &mdash; (1944) J. exp. Med. 79, 45.Findlay, G. M., Elton, C. (1933) J. comp. Path. 46, 126.Gordon, W. S., Brownlee, A., Wilson, D. R., MacLeod, J. (1932)

Ibid, 45, 106.Hurst, E. W. (1931) Ibid, 44, 231.MacLeod, J., Gordon, W. S. (1932) Ibid, 45, 240.Pool, W. A., Brownlee, A., Wilson, D. R. (1930) Ibid, 43, 253.Rivers, T. M., Schwentker, F. F. (1934) J. exp. Med. 59, 669.Somorodintsev, A. A. (1944) Amer. Rev. Soviet Med. 1, 400.Wesemeier, K. (1938) Dtsch. Arch. klin. Med. 182, 451.Wiebel, H. (1937) Kiln Wschr. 16, 632.

LEONTIASIS OSSIUM

C. BERKELEY WAYM.C., M.B. Lond.

MEDICAL OFFICER, HENDON COTTAGE HOSPITAL, LONDON

WITH the improvements that have taken place in

dentistry and in the surgery of the nasal accessory sinuses,cases of leontiasis ossium should become rare. The skullfrom the case reported here has been accepted by themuseum of the Royal College of Surgeons.The patient was a spinster, who died in 1944 at the age of

73. Her appearance and health were normal until the ageof 20, when she had a bicuspid tooth stopped. She had nopain, but a few months afterwards a hard swelling developedin the gum above the tooth, followed later by similar changesin the cheek. Much dental treatment followed, but theswelling steadily increased, spreading to the other cheek andinto the frontal region. Beyond occasional headaches thecondition caused her no physical or social inconvenience.She was a charming, witty, and well-beloved lady whocontinued her work as-a book-keeper until four months beforeher death, with only one short period of ill health.

In 1939 she had a transient hemiplegia : blood-

pressure 220/95 mm. Hg; left knee-jerk exaggeratedand left plantar reflex extensor; otherwise no physicalsigns. She was working again in six weeks, the left

knee -j erk remain- --

ing slightly greaterthan the right andthe left plantar reflexindefinite until herdeath. The B.P. con-tinued high, andthere was somecardiac enlarge-ment. Four monthsbefore her deathshe left her officewell, but later wasfound crawling upthe stairs to her flat.From then onwardsmuscular controlslowly left her; deaf,ness, which had notbeen present before,became intense ; andher mental facultieswere much slowed.However, if one

Fig. 1-" Lion-like" head of patient withleontiasis ossium.

COU1Q Wal1i,SIleWOUlU tJVt::::11tJUi:tt.UY y Nrv v.euc zc w lUU.y y aama OIpp.LVp.L.la<l.iC&iacute;answer to a question. Swallowing became difficult, hypostaticcongestion supervened, and she died in coma.Her nares and intranasal spaces were wide, but there was

no crusting. She never had a cold. Her ear drums werenormal. Bone conduction was lost. Her mandible was not

unduly small in comparison with her sister’s. Radiogram of

Fig. 2-Anteroposterior and lateral radiograms of skull.

the pelvis was normal. For the condition of the skull seefigs. 1 and 2.

COMMENTS

In view of the hyperpiesia, it can be fairly said that thecondition caused little inconvenience to her health andwas only slightly contributory to her death.The history appears to follow the usual lines in that a

chronic septic osteoperiostitis follows a sinus infectionand one by one the accessory air chambers are filled withmasses of inflammatory cancellous bone protruding out-wards and causing great disfigurement. Bony changesspread to the skull (cancellous hyperostosis of Virchow) ;the mandible sometimes is involved, but the base of theskull usually escapes. In the present case, however, thebase is involved and the mandible is normal. The lion-like head is well depicted in the photograph. The absence ofcolds and the dryness of the nose suggest that the mucosalining the sinuses is the major source of nasal secretion.

" ... Can there be values greater than the mere attainmentof health ? One reason why we have not been more successfulin directing people toward healthful living is that sub-

consciously many have decided, and quite correctly, thatthere are more important things than merely being healthy."- W. W. BAUER, M.D., director, bureau of health education,American Medical Association, in Motivation in HealthEducation. London, 1948 ; p. 20.