1/7. skin and bone lesions (dermato-osteolathyrism), possible side effects of D-penicillamine treatment, in a boy with cystinuria. A 2 1/4 year-old boy was treated for cystinuria and urolithiasis with high fluid intake, sodium bicarbonate, and D-penicillamine, over a period of 5 3/4 years, unauthorized interruptions and prescribed pauses included. Therapy was partially sucessful but regrowth of calculi coincided with interruptions of D-penicillamine administration and also with the institution of a low-dose D-penicillamine regime. Flat feet, scoliosis, pectus carinatum, hypermobility of joints, molluscoid pseudotumors and atrophic scars were alarming side effects of D-penicillamine. However, the possibility was not excluded that a forme fruste of an ehlers-danlos syndrome preexisted in this boy and was effected by D-penicillamine. Only the molluscoid pseudotumors regressed when D-penicillamine was reduced or omitted temporarily. Osteolathyrism caused by D-penicillamine has hitherto not been reported in man. ( info) |
2/7. Anterior horn cell changes in a case of neurolathyrism. light and electron microscopic studies were made of the anterior horn cells in a case of neurolathyrism. Eosinophilic inclusions were observed at the light microscopic level. Fine structural study revealed the presence of two types of cytoplasmic inclusions. The first were aggregates of filaments, approximately 80-100 A in width, arranged in small bundles. The second were crystalloid structures composed of elongated elements, about 800 A wide, with hexagonal cross sections. The relationship to each other and the significance of these inclusions are unknown. ( info) |
| A 10-year-old child with asthmatic attacks related to lathyrus sativus flour inhalation was studied in our department. skin test and specific bronchial provocation challenge were positive. Specific IgE antibodies to lathyrus sativus flour was demonstrated by indirect enzyme immunoassay. We suggest that our patient's allergic symptoms were due to the development of Type I allergic reactivity to L. sativus antigens. ( info) |
4/7. Studies on the aetiology and pathogenesis of motor neuron diseases. 1. lathyrism: clinical findings in established cases. lathyrism is a toxic disease of the motor system constantly associated with primary consumption of the seed of lathyrus sativus (chickling or grass pea). Neurological examination was performed on 38 affected subjects drawn from four regions of the Indian subcontinent endemic for lathyrism. All showed a consistent and largely symmetric pattern of neurological deficit similar to that displayed by 5 Europeans with longstanding lathyrism now living in israel. Hallmarks of lathyrism include a pyramidal pattern of motor weakness combined with greatly increased tone in the thigh extensors and adductors and in the gastrocnemius muscles so that the more severely affected walk on the balls of their feet with a lurching scissoring gait. Extensor plantar responses are uniformly present in such cases, and the knee and ankle tendon reflexes are exaggerated and often clonic. Hoffmann signs and exaggerated biceps and/or triceps tendon jerks are also found in the most severely affected. Sensory signs are absent, although perverse sensations in the legs are frequently reported at the onset. walking difficulties commonly begin suddenly but may also appear subacutely or insidiously. Some individuals experience partly reversible symptoms suggestive of a diffuse CNS excitation of somatic, motor and autonomic function. ( info) |
| We examined the central nervous system of a 67-year-old man who showed symptoms of lathyrism after being imprisoned at age 35 and fed on a diet of chick peas, lathyrus sativus. The most obvious changes were loss of axons and myelin in the pyramidal tract in the lumbar spinal cord. These alterations correlated with spastic paraparesis. Other changes at the same level were a mild degree of degeneration of anterior horn cells. pallor of Goll's tracts and axonal swelling in Goll's nuclei were also observed. A few examples of senile plaques and neurofibrillary tangles were observed in Ammon's horn. ( info) |
6/7. Peripheral and central conduction studies in neurolathyrism. To study the involvement of motor and sensory pathways in neurolathyrism, 19 patients with lathyrism from Unnao, india, where lathyrism is endemic, were studied. The mean age of the patients at the time of the onset of illness was 35.8 (range 18-70) years. The mean duration of illness was 15.6 (range 2-30) years. The clinical picture comprised walking difficulty due to stiffness and mild weakness in all 19 patients, cramps in the legs in five, frequency or urgency of micturition in five, and flexor spasms in three. There was pronounced leg spasticity with a mean Ashworth score of 4.1 (range 2.9-5). Central motor conduction to the tibialis anterior muscle (CMCT-TA) was slow in 14 of the 17 patients (21 sides). Slowing of peripheral motor nerve conduction, although less pronounced, was significant in the upper limb in four and the lower limb in seven sides. The tibial somatosensory evoked potentials were normal and peroneal nerve conduction was marginally impaired. Values for CMCT-TA correlated with the degree of spasticity (p < 0.02) whereas weakness, crossed adductor reflexes, and clonus did not. The wide variability of CMCT-TA in lathyrism may be due to involvement of different types of fibres. Large diameter fibre involvement may cause pronounced slowing. Small diameter fibre involvement could produce appreciable spasticity and mild weakness but a lesser degree of slowing or even normal conduction. ( info) |
7/7. Evidence of osteolathyrism among patients suffering from neurolathyrism in bangladesh. In a study of 500 patients suffering from neurolathyrism in bangladesh it was found that 60 (all male) complained of bone pain and showed skeletal deformities suggestive of osteolathyrism. On X-ray examination a failure of fusion in both vertebral and iliac epiphyses was found in two patients. At the age of these patients (30 and 37 years) such failure was considered a clear evidence of osteolathyrism. All 60 patients were accustomed to eating the green parts of lathyrus sativus, which contain 2-cyanoethyl-isoxazolin-5-one, a compound that chemically and metabolically can produce the osteolathyrogen beta-aminopropionitrile (BAPN), as well as foods made from the seeds of the same plant which contain the neurotoxin 3-N-oxalyl-2,3-diaminopropanoic acid (beta-ODAP). ( info) |