[...]... lateral third of the peduncle As they enter the base of the pons, the descending fibers of the cerebral peduncle become intermingled with the somata and crossing axons of the neurons of the pontine nuclei The bulk of corticofugal fibers that enter the pons from the cerebral peduncle terminate here in the pontine nuclei Fibers destined for the spinal cord emerge on the ventral aspect of the medulla... writing, is in the terminal stages of ALS It is impossible to imagine the nightmare of a neurologist, dedicated to ALS developing the disease that has occupied his career Richard, a personal friend of mine and many of the contributors of this volume, started the ALS Clinic at the University of California (San Francisco) in 1993 He was dedicated to the disease and care of patients suffering from it He... first case of ALS was in fact a case of PLS Konzo was first identified in 1936 by Tessitore (Trolli, 1938), a district medical officer in the Kahemba District in the south-eastern part of the Bandundu Province of the Democratic Republic of Congo (DRC) There, konzo is known to be endemic with a prevalence as high as 5% in certain villages However, amongst 146 identified cases there were reports of some... Origins and early descriptions of Duchenne muscular dystrophy Muscle Nerve 28: 402422 Van den Berg-Vos RM, Visser J, Franssen H, de Visser M, de Jong JMBV, Kakmijn S, Wokke JHJ, Van den Berg LH (2003) Sporadic lower motor neuron disease with adult onset: classification of subtypes Brain 126: 10361047 Whiting MG (1963) Toxicity of cycads Econ Bot 17: 271302 Handbook of Clinical Neurology, Vol 82 (3rd series)... and physiology of the corticospinal system MARC H SCHIEBER* Departments of Neurology and Neurobiology & Anatomy and the Brain Injury Rehabilitation Program at St Marys Hospital, University of Rochester School of Medicine and Dentistry, Rochester, New York, USA 2.1 Introduction In 1905, Campbell compiled his histologic studies of the cerebral cortex in normal apes and humans, in a number of human amputees... in which the laws of physics seem to break down After receiving his PhD, he stayed at Cambridge, becoming known even in his 20s for his pioneering ideas and use of Einsteins formulae, as well as his questioning of older, established physicists In 1968 he joined the staff of the Institute of Astronomy in Cambridge and began to apply the laws of thermodynamics to black holes by means of very complicated... (2) Remarks on Paralysis of the Movements of the Trunk in Hemiplegia The MacMillan Company, New York Belhaj-Saùf A, Cheney PD (2000) Plasticity in the distribution of the red nucleus output to forearm muscles after unilateral lesions of the pyramidal tract J Neurophysiol 83: 31473153 Beloozerova IN, Sirota MG (1993a) The role of the motor cortex in the control of accuracy of locomotor movements in... sign of Babinski: A Study of the Evolution of Cortical Dominance in Primates Charles C Thomas, Springfield, IL Fulton JF, Kennard MA (1932) A study of flaccid and spastic paralyses produced by lesions of the cerebral cortex in primates Res Pub Ass Neur Ment Dis 13: 158210 Futami T, Shinoda Y, Yokota J (1979) Spinal axon collaterals of corticospinal neurons identified by intracellular injection of horseradish... Porter R (1962) Electrical thresholds of unimpaled corticospinal cells in the cat Q J Exp Physiol Cogn Med Sci 47: 134140 Hines M (1936) The anterior border of the monkeys (Macaca mulatta) motor cortex and the production of spasticity Am J Physiol 116: 76 34 M H SCHIEBER Hoff EC, Hoff HE (1934) Spinal terminations of the projection fibers from the motor cortex of primates Brain 57: 454474 Houk JC,... years of onset of his disease and most likely had ALS Aran reports that one of the patients three sisters and two maternal uncles had died of a similar disease It seems that this was the first hereditary case of ALS It took another 143 years before the superoxide dismutase gene (SOD1) was discovered to be associated with familial ALS (Rosen et al., 1993) Eleven missence mutations were found in 13 of 18 . of subtypes. Brain 12 6: 10 36 10 47. Whiting MG (19 63). Toxicity of cycads. Econ Bot 17 : 2 71 302. HISTORICAL ASPECTS OF MOTOR NEURON DISEASES 11 Ch 01- N 518 94. René- Théophile-Hyacinthe Laënnec (17 81 18 26 ), Baron Guillaume Dupuytren (17 77 18 35), François Magendie (17 83 18 55) and Léon Cruveilhier (17 91 18 74). He graduated in