POSTNEURITIC OPTIC A T R O P H Y IN REPATRIATED PRISONERS
O F W A R
W A L T E R L . ROBERTS, M . D , A N D L I E U T . T . H . W I L L C O C K S O N , ( M C ) , U . S . N . Los Angeles,
During ,the war years, there were many reports of visual disturbances i n individ uals who were forced to live under inade quate dietary conditions.'"^ W e have had an opportunity to study such disturbances in men returned from Japanese prison camps. T h e incidence of permanent visual impairment was alarming a n d has been confirmed in many A r m y a n d Navy hos pitals. Reviewing publications of the past dec ade concerning nutritional diseases in which vision failure was an outstanding symptom, we find many convincing re ports. K r y l o v ' described 36 cases of pellagra in natives of the Caucasian mountains with accompanying opticnerve pathosis. H e observed paleness of the nerve head with scotomas for red and green. M o o r e * ' ' described a common disease in adolescent school boys in which loss of central acuity of vision associated with sore tongue and mouth were predominant features. Avitaminosis was suspected as the causative factor, because girls, who received supplementary feedings at school, appeared immune to the disease. A number of t h e patients w e r e treated with cod-liver oil, malt, marmith, and iron with successful results. Moore con cludes that the prognosis for the return of vision in cases of less than six months' duration was excellent, but if the dura tion were over six months, the prognosis must be guarded. M o o r e ' also described a syndrome of glossitis, stomatosis, pruritis of the ex ternal genitals, and dimness of vision with loss of central acuity. School chil dren were particularly affected. I n the
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author's opinion, based on the observa tion of some 300 cases, the disease was caused by the use of " g a r e , " a common and cheap food, with inadequate supple mentary food. O t h e r reports, notably those of Levine,^ of Fine a n d Lachman,'" R a m o n and A b b u , ' ' a n d Verma,'* point conclusively to the part avitaminosis can play in the pro duction of optic neuritis. Probably the most complete recent study made on this subject was that of Spillane a n d Scott.' T h e y described a peculiar neurologic syndrome consisting of retrobulbar neuritis, nerve deafness, and ataxic paralegia in 112 prisoners of war. T h e majority of these cases came from one camp. A nutritional neuropathy seemed the most probable explanation of the illness, since there was no evidence of poisoning, infection, or other cause. Peralta," Wilkinson and K i n g , ' and W h i t a c r e , " all describe similar syn dromes in people living under w a r condi tions. Although these described changes are thought to be nutritional in origin, the reponse to vitamin therapy has not been very satisfactory. A brief summary of six representative cases which we have been told are typical of those seen at other military hospitals will follow. I t should be emphasized that we saw all these patients from t w o to three years after the original visual symp toms developed a n d that we are observing the terminal effects of the nutritional neuropathy. W e had to rely entirely upon the history from the patients as to when the visual impairment started; what treat ment, if any, w a s given; whether or not other nutritional syndromes, such as def
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initc beriberi and pellagra, were present. W c arc presenting these cases as a con tribution to the number of similar, previ ously reported cases, hoping to emphasize the importance of an adequate diet espe cially for people living under war condi tions and to point out the marked visual incapacitation that can result from neglect to supply such a diet. CASE REPORTS
Case 1. J. M. B., P l / S g t . U.S.M.C., aged 45 years, was captured at W a k e Island on December 23, 1941. H e was confined in two camps in China and two camps in Japan. H e first noticed blurred vision in September, 1942. This blurring increased gradually until January, 1943. Since that time, he feels that there has been no change in his vision. H e stated that he had had no specific nutritional disease but suflered from severe malnutrition and lost considerable weight (the exact amount was not k n o w n ) . H e received some cod-liver oil and vitamin-C tablets iiitennittcntl)- with no improvement in his vision. Ocular examination at this hospital, three years after the onset of symptoms, showed vision in each eye to be 2/20, unimprovable. T h e media were clear, and the fundi showed the disc margins to be indistinct with slight temporal pallor. Periitietric examinations revealed the peripheral fields to be normal. T h e cen tral fields showed bilateral, absolute, cen tral scotomas of around five degrees. Case 2. J. D. S., Sgt., U.S.M.C., aged 25 years, was captured on Corregidor, May 6, 1942. H e was confined in two camps in the Philippines and one in Japan. H e first began to notice visual impair ment in January, 1943. At this time, his diet consisted of boiled rice, potatoes, and occasionally meat. H e received some vita mins and eye drops about six months after his vision began to fail. T h e r e was some
improvement in vision for a period of about three months. H e soon noticed a return of the blurred vision, however, and there was no change during the last 20 months of his internment. H e suffered from pellagra, beriberi, and diarrhea while he was a prisoner. Upon examination at this hospital three )e.irs after the onset of symptoms, vision in each eye was found to be 2/20, unim provable. Funduscopic examination showed a temporal pallor of the disc in each eye, with a granular appearance of both maculas. Perimetric examination re vealed bilateral, absolute, central scoto mas. T h e peripheral fields were normal. Case 3. I. C. S., S / S g t . U.S.M.C., aged 24 years, was captured on Bataan, on April 9, 1942. H e was confined in three Philippine prisons and one in Japan. H e first noticed impairment in his vision in April, 1943. At that time his diet con sisted of rice and thin green soup and, occasionally, meat. H e was given vitamin A in small quantities for his eye symp toms, with slight improvement. H i s vision gradually failed the last year of his con finement and has remained stationary since then. H e had "wet beriberi" at the onset of his visual loss. Upon examination in this hospital, three years after the onset of his symp toms, vision in each eye was found to be 2/20, unimprovable. Funduscopic ex amination of each eye revealed temporal pallor of both optic discs. T h e macula in each eye was granular in appearance, and the light reflex was absent. Perimet ric examination of both eyes revealed absolute central and paracentral scotomas, with normal peripheral fields. Case 4. K. K. K., P l / S g t . U.S.M.C., aged 35 years, was captured on December 23, 1941, on W a k e Island. H e was im prisoned in several camps in China and Japan. H e first noticed blurring of his vision in August, 1942. A t that time he
POSTNEURITIC OPTIC ATROPHY
was eating small quantities of rice, stew, watery fish stew, and, occasionally, vegetables. H e believed he received ascor bic-acid injections for 44 days in 1942. In 1943 and 1944, he received nine vita min pills daily for a total of 45 days. H e stated that there was improvement of his vision on this therapy. W h e n it was dis continued, however, the improvement was not sustained. H e had had beriberi, pel lagra, and malnutrition, with a weight loss of 37 pounds. Examination at this hospital 3J/2 years after the onset of the loss of vision, re vealed the vision in each eye to be 10/20, unimprovable. Funduscopic examination of each eye revealed slight temporal pal lor of the optic discs and granular macu las, with absent light reflexes. Perimetric examination showed absolute central scotomas bilaterally, and a contracted, left peripheral field. Case 5. H . M. E., Pfc. U.S.M.C., aged 31 years, was captured on Corregidor, May 6, 1942. H e was confined in three camps in the Philippines and one in Japan. His vision first became impaired in November, 1942, at which time he had had corneal ulcers. At the time of onset of symptoms, his diet consisted of rice and vegetable soup in small quantities. After the onset of symptoms, he was given one tablespoon of cod-liver oil a day for two months, plus one vitamin-A pill a day for two weeks. H e stated that his ulcers healed in three months but that his vision never did clear. H e had beri beri in 1943. Examination at this hospital, four years after the onset of the visual symp toms, showed this patient to have corneal opacities in both eyes. Vision in the right eye was 2/20, improvable with homatro pine dilatation to 6/20. Vision in the left eye was 6/20, improvable to 8/20, with homatropine dilatation and a —1.25 sphere. Slitlamp examination revealed
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central corneal opacities of both eyes, but the periphery of each cornea was clear. Funduscopic examination revealed slight temporal pallor of the optic discs. Peri metric examination showed some con striction of the right peripheral field with normal central fields. A n optical iridec tomy of the right eye was done, with visual improvement to 6/20. Case 6. J. B. G., B M L C U . S . N . , aged 26 years, was captured on Corregidor, May 8, 1942. H e was imprisoned in sev eral camps in Japan. I n September, 1942, he began to complain of a sore tongue and blurred vision. A t that time, he also no ticed some heaviness and weakness of his legs. In spite of these complaints, he was forced to do heavy physical work. T w o months later he complained of severe shooting pains in his legs, became sud denly paraplegic, and lost control of his sphincters. H e was diagnosed as having beriberi and was immediately transferred to Japan. At this time, he stated, he was almost blind. Six months after his arrival in Japan, he developed a corneal ulcer in his left eye. T h i s cleared u p after a long period of convalescence. Beginning in the spring of 1943, there was a gradual im provement in all of his symptoms for a period of six months. Since the fall of 1943, there has been little change in his conditiorf. H e was hospitalized continu ously until his liberation and, during that period, he lost 110 pounds in weight. Examination at this hospital, 3 j ^ years after the onset of his symptoms, revealed vision in the right eye to be 5 / 2 0 ; and in the left eye 1/20, unimprovable. Slit lamp examination revealed a large, cen tral scar of the left cornea, with an an terior synechia of the nasal pupillary por tion of the iris to the scar. T h e left lens showed some anterior-capsular opacities. Both fundi were seen and appeared nor mal. Perimetric examination revealed constriction of both peripheral fields, more
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marked in the right eye. T h e left eye showed an absolute central scotoma, and the right eye an absolute paracentral scotoma. DiscussiON
fields were constricted in only two cases; and all but one case showed bilateral absolute central or paracentral scotomas, These findings are similar to those described by other observers.
T h e existence of food-deficiency diseases in these cases was undeniable. All of these patients, except one, gave histories of having suflFered from beriberi or pellagra, and all had suffered severe malnutrition, with marked loss in weight, T h e r e has been some controversy as to which vitamin deficiency was responsible for this type of optic-nerve degeneration. M e l l o n b y " showed that vitamin-A deficiency in dogs was associated with
these patients were given intensive ^^^tary therapy upon liberation. Mco t h e r s " described the dietary management of the repatriates, and it is reasonable to assume that this regime was ^^'^^y standardized Ninety percent °^ ^he men were given six full meals a addition to ice cream and choco1^^^ bars. It was estimated that a 5,000«^^^«"^ diet was given, supplemented with the following vitamin intake:
severe degeneration of the afferent nerves, including the optic nerve. Rao^" observed myelin degeneration in the optic nerve of rabbits, rats, and fowls fed on diets lacking in vitamin A. Vitamin-B, deficiency has long been associated with
Vitamin A 15,000 units Vitamin B , (thiamin) 20 mg. Vitamin B¡ (riboflavin) 10 mg. (niacinamide) 60 mg. Vitamin C (ascorbic acid) . . . . 2 2 5 mg. Vitamin D 1,200 units
retrobulbar neuritis and optic-nerve degeneration. R e a , " Ramon and A b b u , " and Landor and Pallister" reported cases of optic-nerve degeneration in a vitaminB , deficiency syndrome. A large group of patients from the Japanese prison camps were carefully studied by McDamel and o t h e r s . " T h e clinical findings for these patients indicated definite deficiency in the vitamin-B complex, extrinsic factor, and protein. T h e funduscopic appearance of the cases herein reported was fairly uniform, All but one case showed slight temporal pallor of the discs; foveal light reflexes were absent; and the maculas had a granular appearance. Visual acuity varied from 1/20 to 10/20. T h e visual fields were recorded on a perimeter and Bjerrum screen at one meter. T h e peripheral
^ 3 determined by the visual examinations on repatriation, did the ^jj^^i examinations during this therapeu^ ^ ^ - ^ ^ 3^^^ ^.^^nge. This tends substantiate Moore's" observation that ^^e prognosis of the return of vision in ^^^^ longer than six m o n t h s ' duration ^^^^ guarded, SUMMARY Since the return of personnel from the Japanese prisoner-of-war camps, most ophthalmologists in A r m y and Navy hospitáis have seen permanent visual impairment which in all probability is nutritional in origin. W e have presented six typical cases of severe ocular disability the onset of which was concomitant with marked dietary deprivation.
REFERENCES ' ' ' '
Spillane, J. D., and Scott, G. I. Lancet, 1945, Sept. 1, p. 261. Peralta, Μ. Arch. Psychist. Nervenkrank, 1942, v. 114, p. 611. Wilkinson, P. P., and King, A. Lancet, 1944, p. 528. Ridley, Harold. Brit. Jour. Ophth., 1945, v. 29, p. 12.
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' Krylov, T. Soviet Vestnek Oftal., 1932, v. 1, p. 339. • Moore, D. G. F . W . African Med. Jour., 1932, v. 6. p. 28. ' Moore, D. F. Jour. Trop. Med., 1939. v. 42, p. 109. • Moore, D. G. F . W. African Med. Jour., 1934, v. 7, p. 119. ' Levine, J. Amer. Jour. Ophth., 1934, v. 12, p. 902. " Fine, M., and Lachman, G. S. Amer. Jour. Ophth., 1937, v. 20, p. 708. " Ramon, T. K., and Abbu. C. Jour. Indian Med. Assoc., 1941, v. 10, p. 417. " Verma, O. P . Indian Med. Gazette, 1942, v. 77, p. 646. " Whitacre, F . E. Jour. Amer. Med. Assoc., 1944, v. 124, p. 652. » Mellonby, E. Edinburgh Med, Jour., 1933, v. 40, p. 197. » Rao, M. N. Indian Jour. Med. Research, 1938, v. 25, p. 661. " Rea. R. L. Neuro-Ophthalmology. St. Louis, Mo., C. V. Mosley Co., 2nd Ed., 1941. " Landor, J. V. and Pallister, R. A. Trans, of the Royal Society of Trop. Med. and Hyg., 1935. v. 29, p. 121. " McDaniel, F . L., White, B. O., and Thompson, C. M. U. S. Naval Med. Bull., 1946, v. 46, p. 793.
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DAHLIA WHITBOURNE, M.B.* Kingston, Jamaica, BW.I.
Dimness of vision associated with im paired hearing was mentioned by H . H . Strachan' in accounts of peripheral neu ritis in Jamaica in 1888 and 1897. This condition occurred in adults as well as children. H . H . Scott,' in 1917, described a disease which he called the central neu ritis of Jamaica. H e described impaired vision and hearing, itching and burning of the eyes, photophobia, inflammatory le sions in the mouth, fissures, and saliva tion. In 1927, E . J e n n e r Wright* de scribed a new deficiency disease, the A - B avitaminosis of Sierra Leone. I n Jamaica, faulty vision due to optic-nerve lesions has been commonly complained of by adults. Inasmuch as many of these pa tients have had positive W a s s e r m a n n or Kahn tests, the condition in some cases has been ascribed by physicians to yaws or syphilis. I n 1934, during a post-hurri cane period of food shortage, there was an epidemic of cases of nutritional edema and "dark eyes" among children. These came tmder the care of D r s . L. M . Clark and W . N . Dickenson at the Kingston Public Hospital. T h e cases responded so * School Medical
Officer.
well to cod-liver oil plus a liberal diet that these doctors published a n article on this subject in 1936." A s far as is known, neither of these men was aware of simi lar diseases in Africa. A s school medical officer of Kingston, I treated several hundred similar cases more or less successfully with a mixture of cod-liver oil and iron plus a diet of pro tective foods for four years from 1934 to 1938. A t that time my attention was drawn to E . J e n n e r W r i g h t ' s " article and Fitzgerald Moore's description* of pel lagra in W e s t Africa. T h e substitution of dried brewers yeast for cod-liver oil was effective in nearly all cases, although some cases seemed to require both prod ucts for restoration of satisfactory vision ( 6 / 6 or 6 / 9 ) . T h e view was, there fore, taken that deficiency of no one vita min was responsible, but that both vita mins A and Β were implicated. A N A L Y S I S OF T H E CASES
T h e following are observations on 74 cases of retrobulbar neuritis treated at the School Q i n i c in Kingston during the year 1945.