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Brain, Vol. 110, No. 1, 149-165, 1987
© 1987 Oxford University Press

research-article

STUDIES ON THE AETIOLOGY AND PATHOGENESIS OF MOTOR NEURON DISEASES

1. LATHYRISM: CLINICAL FINDINGS IN ESTABLISHED CASES

A. C. LUDOLPH1,5, J. HUGON1,6, M. P. DWIVED1,7, H. H. SCHAUMBURG1,3,4 and P. S. SPENCER1,2,4,

1From the The Institute of Neurotoxicology, Albert Einstein College of Medicine New York, USA 2From the Department of Neuroscience, Albert Einstein College of Medicine New York, USA 3From the Department of Neurology, Albert Einstein College of Medicine New York, USA 4From the Department of Pathology, Albert Einstein College of Medicine New York, USA 5From the Department of Neurology, University of Münster Minster, W. Germany 6From the Department of Neurology, Limoges University Hospital Limoges, France 7From the Department of Community Medicine, Mahatma Gandhi Institute of Medical Sciences Sewagram, Nagpur, India

Correspondence to: Correspondence to: Professor P. S. Spencer, Institute of Neurotoxicology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA.

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.

Received November 26, 1985. Revised March 20, 1986. Accepted April 10, 1986.


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