333TRANSACTIONS OF THE ROYAL SOCIETY OF

TROPICAL MEDICINE AND HYGIENE.Vol. 44. No. 3. December, 1950.

THE LATHYRISM SYNDROME.*BY

C. GOPALAN, M.D., PH.D.,Nutrition Research Laboratories, Coonoor, South India.

Lathyrism has been reported as occurring in " epidemic " form in certainparts of India, where lathyrus forms part of the dietary of the population.Thus outbreaks of the disease have been recorded in Central Provinces(BUCHANAN, 1902), Rewa (ACTON, 1922 ; YOUNG, 1927), Gilgit (MCCARRISON,1926; MACKENZIE, 1927), United Provinces (SCOTT, 1930), Punjab (SHAH,1939), and Bhopal (SHOURIE, 1945). There has been no report of cases oflathyrism from South India where lathyrus is not consumed. MINCHIN (1940),however, reported a series of cases of " primary lateral sclerosis " clinicallyindistinguishable from lathyrism, from the Government General Hospital,Madras.

The present investigation shows that cases presenting the clinical featuresof lathyrism occur in a sporadic form even in South India. Since, however,South Indian diets do not contain lathyrus, it would seem that lathyrismsyndrome is not solely confined to the lathyrus-eating population.

The following report is based on 11 cases investigated by the writer and 50other cases collected from the records of the Government General Hospital.

INCIDENCE.

Economic status.—The disease was met with among the poorest classes.The cases investigated by the writer had family incomes of less than Rs. 50/-per mensem. The others were cases from the general wards of the hospitalwhere only poor patients are admitted.

Dietary habits.—All the patients were subsisting on grossly inadequatediets. The dietary consisted mainly of rice gruel with occasional helping ofcheap vegetables ; milk, meat and eggs were rarely, if ever, consumed. Therewas no evidence that lathyrus seeds had ever formed part of the diet of thesepatients.

Age.—The majority of subjects were young adults. The incidence wasparticularly heavy between the ages of 20 and 40, 41 of the 61 cases belongingto this age period. There were six cases in this series which were below 20years and only four above 50. The youngest patient was a boy of 6 years andthe oldest a man of 59.

* I am grateful to Dr. V. N. PATWARDHAN, Director, Nutrition Research Laboratories,Coonoor, and Sir GEORGE MCROBERT for their helpful interest in this work.

334 THE LATHYRISM SYNDROME

Sex.—There was a striking preponderance of males over females in theseries, the proportion of males to females being three to one.

Locality.—The disease was not apparently confined to or predominant inaiy particular portion of the province. The present series included Tamilsand Telugus from different districts.

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15

THE ONSET.

The onset of the disease was variable. In nine cases, the history obtainedwas that the patient had retired to bed apparently normal, and on waking upfound inability to use his lower limbs. In 21 other cases the onset was sub-acute, the disability progressing to its full-fledged state within 10 to 20 days.In the remaining 19 cases, the disease set in insidiously ; there was graduallyincreasing- weakness and stiffness of the lower limbs, the process being spreadout ever 1 or 2 months. In four cases there was a history of fever of shortduration (4 to 7 days) immediately preceding the attack. Several patients inthis series had had the disease for many years when they were first seen. Therewas no history of remissions and exacerbations of the disorder in these subjects.Eleven patients in the series had suffered from paraesthesias of the lowerextremities immediately preceding, during and for some time after the onsetof the disease. These took the form of tingling, pins and needles, numbness,cramp-like pains and burning sensation in the soles of the feet.

CLINICAL FEATURES.

The presenting complaint was inability to use the lower limbs. Thelimbs were reported to be weak and stiff. The disability was so great in somepalierts that they were confined to bed. Others managed to get about with theaid of sticks. The patients were markedly ataxic while attempting to stand orwalk and the gait was spastic.

On examination, the tone of the muscles of the lower limbs was found tobe considerably increased and their power very much diminished. The limbswere spastic. There was no fibrillation ; wasting was not observed except ina few cases of long duration where there was a moderate degree of wastingpresumably due to disuse. The knee-jerks were exaggerated in all cases andpstellar clonus could be elicited in some cases. The ankle jerks were alsoexaggerated and ankle clonus was frequently present. The plantar responsewas extensor on both sides in all but three cases in which it could not be elicited.

C. GOPALAN 335

„, abdominal reflexes were variable ; in 28 cases they were normal in all•quadrants, in 12 others they were brisk, in 11 cases they were sluggish, in oneSOT the other of the upper quadrants and in nine others they were absent in all¡'quadrants.fe The deep reflexes in the upper limbs were normal in 52 cases and unusually'"brisk in nine cases. There was no spasticity of the upper limbs in any case.Çt There was a striking absence of sensory changes. Touch, temperature,pressure, pain, position, muscle, joint and vibration sensations were all fullyretained. Patients did not complain of girdle pain and there was no zone ofhyperaesthesia.

Sphincteric disturbances were completely absent in 57 cases. Four patients,however, suffered from precipitancy of micturition. The cranial nerves wereall normal. There were no mental changes.

No local disease of the spine could be detected on clinical examinationand X-ray of the spine carried out in 19 cases did not reveal any abnormality.

The cerebrospinal fluid was clear and under normal pressure. There wasno evidence of obstruction to the flow of cerebrospinal fluid. Quickenstedt'ssign was positive in all cases. Myelography carried out in three cases alsorevealed no obstruction. The protein, chloride and sugar content of the fluidwere within normal range in all cases and cells were either few or absent.Lange's test was negative and so were the Wassermann and Kahn reactions.

The blood also gave a negative Wassermann reaction.Haematological examination.—In all the 11 cases investigated by the writer

there was hypochromic microcytic anaemia of moderate severity. In 12 othercases in which the results of haematological examination were recorded, therewas also evidence of moderate anaemia of the microcytic hypochromic type.Sternal marrow in all nine cases in which it was examined showed normoblasticreaction.

Fractional gastric analysis conducted in 13 cases showed normal gastricacidity in three cases, hypochlorhydria in five cases and achlorhydria in fivecases. In two of the latter, the achlorhydria was histamine-fast. (Sternalmarrow was examined in all cases showing achlorhydria and showed normoblasticreaction.)

Clinical signs of dietary deficiency were not recorded in the cases collectedfrom the hospital records. In the cases examined by the writer the state ofnutrition was not worse than that obtaining among other patients in the generalwards of the hospital. None of these cases showed angular stomatitis, glossitisor ocular signs of B complex deficiency. Phrynoderma was not seen in anycase. Night blindness was present in one case. All the cases, however, showedconjunctival discoloration and patchy thickening of subconjunctival tissue.Mosaic dermatosis affecting the anterolateral aspects of the legs was presentin four cases. Two patients who were past 40 years exhibited cataract.

THE LATHYRISM SYNDROME

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xnee jerk. A.J. ankle jerk. B.J. biceps jerk. S J . supinator jerk. A.R. abdomenP.R. plantar reflex. • present. • • exaggerated. — negative. M. male. F. female.

H.C. hypochromic anaemia. Hypo, hypochlorhydria. Achl. achlorhydria. P.M. precipitancy of i

DISCUSSION.

The condition described here is thus a pure upper motor neurone lesionconfined to the lower extremities. General nervous diseases like subacutecombined degeneration, amyotrophic lateral sclerosis, disseminated sclerosis,syringomyelia, and neurosyphilis on the one hand, and local lesions affectingthe spinal cord like tumours, tuberculous disease of the spine, etc., on the other,could be easily excluded in these cases. Indeed, the condition does not fit ininto any of the clinical entities known at present except that it may be classedjndcr primary lateral sclerosis which, however, is no more than a convenient label.

The disease, however, has several features in common with lathyrism andis, in fa"t, clinically indistinguishable from it. Thus, lathyrism is also charac-terised by spastic paraplegia affecting the lower limbs, normal cerebro-spinalfluid, absence of sensory changes and absence of involvement of other partsof the body. The age and sex incidence observed in these cases is also inconformity with that reported in lathyrism. Thus BUCHANAN (1902), YOUNG(1926), SHAH (1939), and SHOURIE (1945) all observed that the majority ofcases of lathyrism were in the younger age groups and that males were predomi-nantly affected by the disease. The striking discrepancy, however, was thatnone of the cases reported here had ever subsisted on lathyrus.

The relationship of this syndrome to lathyrism and the role of malnutritionin its development require elucidation. It is noteworthy that all the subjectssuffering from the disease belonged to the poorest section of the community

337

roipinal fluid.

| Lange'sChemistry. ¡ test.

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analysis.

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Ind. indifferent. *4 present in all four quadrants. *3 present in three quadrants.•2 present in two quadrants. Ab. abrupt. Su. subacute. Gr. gradual.

subsisting on grossly inadequate diets. SHOURIE (1946) also found that thevictims of lathyrism were the poorest class of people. However, clinical signsof deficiency diseases were lacking and the same was true of lathyrism (SHOURIE,1946). MELLANBY (1931), from his work on puppies, believed that vitamin Adeficiency was mainly responsible for the development of lathyrism and wasinclined to class lathyrism as a food deficiency disease. YOUNG (1927) foundnight blindness common in a village affected by lathyrism, and also observedthat the disease did not occur in neighbouring villages where the diet containedas much lathyrus but more vitamin A, fish and meat. SHAH (1939) reportedgreat improvement in his cases of lathyrism when vitamins A and D weregiven. On the other hand, BASU and others (1936) pointed out that Lathyrussativus, which formed the staple food in famine conditions, was a poor sourceof tryptophane.

The occurrence of spastic paraplegia in experimental animals fed ondifferent deficient diets has been reported by several workers. MELLANBY(1931) demonstrated lesions in the spinal cord in puppies on a diet deficient invitamin A. Paralysis of the hind limbs and posterior third of the body has beenreported in mice fed on diets deficient in pantothenic acid (MORRIS andLIPPINCOTT, 1941). Spastic paralysis of the hind limbs was observed in rats(BOAS, 1927) and dogs on a biotin deficient diet. Degeneration of the lumbarcord has been reported in rats on a vitamin E deficient diet (RINGSTED, 1935),while WINTROBE and others (1940) demonstrated degeneration of the spinal cordin pigs on a diet deficient in some unknown factor.

338THE LATIIVRISM SYNDROME

Í/

1The salient fact that emerges from these diverse claims is that the integrity 1

of the spinal cord is dependent on the supply of an adequate diet and that«probably more than one essential nutrient is concerned in the process. Aconvincing proof of this in the human subject is the reported occurrence ofspastic paraplegia among malnourished prisoners of war (DENNY-BROWN, 1945 ;SPILLANE, 1947). SPILLANE has remarked on the similarity of these cases ofspastic paraplegia to cases of lathyrism. There was obviously no question oflathyrus playing any part in these cases.

The one striking feature common to cases reported here, to cases of spasticparaplegia among prisoners of war, and to cases of lathyrism is the backgroundof malnutrition. MELLANBY (1934) postulated " that Lathyrism was lack ofsome protective food which could normally prevent the detrimental effects of

/ toxins which are always present in the germ of grain." The toxin may act bybinding, an essential nutrient and thus rendering it unavailable to the subject.The occurrence of the lathyrism syndrome among non-lathyrus eating popula-tions suggests that the toxic agent, may not always be the same and sometimesmay be other than that contained in the seeds of Vicia sativa or variouskinds of lathyrus. Lathyrism may thus be looked upon as a syndrome causedby nutritional deficiency which apparently weakens the resistance of the lowersegments of the spinal cord to various toxic agents.

SUMMARY OF CONTENTS.

A syndrome of spastic paraplegia among poor South Indians subsisting ongrossly inadequate diets, not containing lathyrus, has been described. Thesimilarities between this syndrome and lathyrism have been pointed out. Therole of malnutrition in the causation of the syndrome has been discussed.

REFERENCES.ACTON, H. W. (1922). Indian vied. Gaz., 57, 241.BASU, K. P., et al. (1936-37). Indian J. med. Res., 24, 1001 and 1027.BOAS, M. A. (1927). Biochem. J., 21, 712.BUCHANAN, \V. J. (1904). Nagpur Civil Administration Report.DENNY-BROWN, D. (1945). Report on Neurological Results of Internment. London :H.M.S.O.MCCAHRISON, R. (1926). Indian 7. med. Res., 14, 379.

. (1928). ¡bid., 15, 797.MACKENZIE, L. H. L. (1927). Indian med Gaz., 62, 201.MELLANBY, E. (1931). Brain, 54, 247.

. (1934). Nutrition and Disease. London: Oliver and Boyd.MiNCHIN, R. L. H. (1940). Brit. med. J., 1, 253.MORRIS, H. P. & LIPPINCOTT, S. W. (1941). J. nat. Cancer Inst., 2, 29 and 39.RINCSTED, A. (1935). Biochem. J., 27,788.SHAH, S. R. A. (1939). Indian med. Gas., 74, 385.SHOURIE, L. (1945). Indian J. med. Res., 33, 247.SPILLANE, J. D. (1947). Nutritional Disorders of the Nervous System. Edinburgh :

Livingstone Publication, p. 187.STOTT, H. (1930). Indian J. med. Res., 18, 51.WINTROBE, M. M., et al. (1940). Johns Hopk. Hosp. Bull., 67, 377.VOUNG, M. T. C. (1927). Indian J. med. Res., 15, 453.

339

ANSACTIONS OF THE ROYAL SOCIETY OF.TROPICAL MEDICINE AND HYGIENE.Vol. 44. No. 3. December, 1950.

NUTRITIONAL ILL HEALTH AMONG MALAY CHILDRENIN THE IPOH DISTRICT OF PERAK, MALAYA*

A CASE DESCRIBED OF SEVERE MALNUTRITION WITHENLARGED LIVER.

BY

FLORENCE ADAM THOMSON,Medical Officer, Malaya.

Records of a small series (1,703) of Malay children in the Ipoh districtof Perak have been collected during the past 9 months. The area covered isabout 500 square miles and is mainly rural in character although the nearnessof Ipoh, a large town, the capital of the State of Perak (population, 1947 :Chinese, 115,089; Malay, 15,078; Malaysians, 1,354; Indian, 21,867;others, 2,674) has influenced these rural Malays to a great extent. Apart fromrice, they rarely grow their own food and much prefer an occasional trip onthe bus to town where they buy mainly Chinese grown vegetables, salt fish,sugar and tins of sweetened condensed milk. Meat is rarely eaten, and asnearly all Malays are Mohammedan, pork, the most easily available meat, bothdomestic and wild, never. Malays are not milk drinkers, and although buffaloesand goats are sometimes kept, the milk in the case of the buffalo is very rarelyused, and goats' milk not at all.

Careful questioning of mothers both at the town clinic and at the clinicsheld in the rural areas has revealed these dietetic habits, and as BURGESS (1948)has already noted, diets are grossly deficient in protein, minerals, vitamins and,

• Published by kind permission of Dr. R. B. MACGREGOR, C.M.G., Director of MedicalServices, Federation of Malaya.