In 1922, we presented before the American Neurological Association1 the clinical and pathologic observations in a case which we termed progressive subcortical encephalopathy. Clinically, the case (that of a child) was characterized by an acute onset and progressive development of generalized spasticity and mental deterioration, followed by convulsive attacks and other features of a decerebrate rigidity as terminal events. The anatomic alterations were striking. They were degenerative and, although diffuse and widely distributed, were nevertheless distinctly limited to the white substance (subcortex) of the cerebral and cerebellar hemispheres. The gray matter was undisturbed and was without the slightest suggestion of disease.
Since that time, three more cases of similar character have come under observation. They are all so strikingly alike anatomically and have so much in common clinically that we are presenting them as members of a single group. In describing these cases, it is our aim to find
GLOBUS JH, STRAUSS I. PROGRESSIVE DEGENERATIVE SUBCORTICAL ENCEPHALOPATHY(SCHILDER'S DISEASE). Arch NeurPsych. 1928;20(6):1190-1228. doi:10.1001/archneurpsyc.1928.02210180041003