156 TEIJI HOSHINO 



Under the name of hereditary ataxia, we understand a condi- 

 tion of congenital disturbance of coordination of movements 

 or even in standing, static ataxia. This disease is generally 

 classified into two types: one is the Friedreich's ataxia and 

 the other is the L'heredo-ataxia cerebelleuse. The former 

 was first described by Friedreich in 1863, who noticed a 

 typical hereditary form of a chronic degenerative atrophy 

 in the posterior and lateral funiculi of the spinal cord. The 

 affection is characterized clinically by a disturbance in the 

 coordination of movements, as he says: "Die Krankheiten 

 diirfte vom klinischen Gesichtspunkte aus als chronische pro- 

 gressive Lahmung der Combination der Bewegungen, von pathol- 

 ogist anatomischen Standpunkte aus als chronische degener- 

 ative Atropine der spinalen Hinterstrange zu bezeichnen sein." 

 After his discovery of this affection, similar cases were reported 

 by Carpenter (71) and Gowers ('80) in England, and Brousse in 

 France ('82), who had proposed to call the affection by the name 

 of Friedreich's disease (Marie, '92). The chief changes in this 

 disease are usually a thin small cord, with degeneration or atro- 

 phy, and, consequently secondary sclerosis of the lateral and 

 posterior columns, and thickening of the pia mater. The fre- 

 quently affected tracts are in the proprioceptive systems in the 

 cord, namely, the direct spinocerebellar tracts and the columns 

 of Goll and Burdach, but sometimes the lateral pyramidal tract 

 is also involved. Occasionally there is more or less diminution 

 in the number of fibers in the anterolateral column, and also 

 atrophy of the cells in both the anterior and posterior horns 

 (Blocq and Marinesco, '90; Barker, '03). The cells of the col- 

 umn of Clarke are notably degenerated or else very poor in devel- 

 opment. All authors agree in that this disease is due to an ar- 

 rest of development or growth of the various systems of fibers in 

 the spinal cord (Menzel, '91). 



Under the title of L'heredo-ataxia c6rebelleuse, Marie ('93) 

 reported two cases of a familial ataxia, and collected from the 

 literature a series of similar cases, in which atrophy of the cere- 

 bellum was found. He has established a symptom-complex dis- 

 tinct from Friedreich's ataxia. Marie's disease is believed to be 



