ABNORMALITIES OF NEURAL FUNCTION IN THE PRESENCE OF INADEQUATE NUTRITION 



!9°5 



legs with high consumption of certain leguminous 

 seeds. The association with the consumption of 

 lathyrus was clearly established in Spain after the 

 Civil War (i ig). 



Lathyrism is seen predominately in young adult 

 males. The prodromal period, which may be only 

 one night, is characterized by symptoms of coldness 

 of the feet, muscle pains and cramps. The patient 

 notices increasing stiffness and weakness of the legs 

 and becomes unable to walk. Spastic paraplegia and 

 rigidity of the legs develop and voluntary movements 

 disappear. Once this state develops there is usually 

 very little or no change in the neurologic picture. 

 Sensations are normal, extensor plantar reflexes are 

 present, and abdominal reflexes may be lost. Tendon 

 reflexes are normal in the arms and hyperactive in 

 the legs. No other manifestations are observed. In 

 other respects, the patients appear healthy, and are 

 free of signs of malnutrition and without noticeable 

 psychic change. 



The pathology of the disease in man is not well 

 known. The best descriptions are those by Stock- 

 mann (262) and by Filimonoff (71 ). The latter found 

 degeneration of GolPs tract and the cerebellar path- 

 way at the level of the cervical spinal cord, as well 

 as lesions of the cortical Betz cells. The pathology 

 seems to correspond to the clinical manifestations of 

 spastic paraplegia. 



A pathological condition, later termed 'odoratism' 

 (279), was produced in the rat fed another variety of 

 lathyrus seed, the common garden pea {Lathyrus 

 odoratus) (85, 144). The stud) culminated in the 

 isolation of beta-aminopropionitrile which, when 

 administered to rats, produces the disease (265). 

 This substance is not contained in the seeds of all 

 lathyrus varieties. The disease produced by /.. 

 odoratus is clearly different from human lathyrism, 

 the primary lesions in odoratism affecting not the 

 nervous system but tissues of mesenchymal origin. 



Cicerism 



Another disorder produced by ingestion of legumi- 

 nous seeds is 'cicerism.' Jimenez-Diaz & Yivanco 

 (120, 121) found that rats fed cooked chick-peas 

 (Cicer arietinum) or protein extracted from these peas 

 display nervousness, irritability and continuous 

 movement, followed by abnormal gait and hind-leu 

 hypercxtcnsion. Rapid deterioration follows with 

 gross incoordination of movements, loss of righting 

 and convulsive fits. Death occurs in a few days. The 

 disorder is prevented by administration of methionine 

 (92, 280) but not vitamins A, D, Bi, B», B 6 , nicotinic 

 acid or pantothenic acid. Since the effect is not pro- 

 duced by eating of the whole fresh pea, a protective 

 factor apparently is present which is destroyed by 

 cooking. 



Pin nylpyruvic Oligophrenia 



This rare form of mental deficiency is due not to a 

 lack of any essential nutrient but to a faulty metabol- 

 ism. Specifically, there is an enzyme blockage of the 

 transformation of phenylalanine to tyrosine. Phenyl- 

 alanine accumulates in the blood and cerebrospinal 

 fluid and is partly broken down to phenylpyruvic as 

 well as phenyllactic and phenylacetic acids which 

 appear in abnormal amounts in the urine. Mental 

 deficiency in phenylketonuria is considered to be 

 due to "intoxication' by phenylalanine or one of its 

 metabolites. Several reports indicate that feeding 

 diets low in phenylalanine (7, 18, 297) results in 

 behavioral improvement, but the identity of the 

 neurotoxic factor was not definitely established. 

 Clearly, the special diets should be initiated at an 

 earlv age in order to prevent irreversible damage to 

 the central nervous system. 



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