Spastic pseudosclerosis (cortico-pallido-spinal degeneration), by Charles Davison (New York), Brain 1932: 55; 247-264 and Severe dementia associated with bilateral symmetrical degeneration of the thalamus, by K. Stern (From the National Hospital, Queen Square, London), Brain 1939: 62; 157-171.

Abstract

Dr Davison has recently seen two cases in adults—running a rapid course characterized by mental symptoms, pyramidal and extrapyramidal signs, and muscle atrophy—that ‘do not conform to any disorder recognized hitherto’. J.R. has a 3-year history of weakness in the left leg that does not change until 1 year before presentation, when his speech deteriorates and the upper limbs become involved. On admission he is bedridden, drooling, with spasmodic laughter and crying and severe dysarthria. Examination shows rigidity, dystonia in a ‘striatal attitude’, increased tendon reflexes and bilateral Babinski signs, as well as muscle atrophy with generalized ‘fibrillary twitchings’. He is dishevelled, with impaired memory and responds only to simple commands. Bulbar function deteriorates thereafter and within a few months he is dead from aspiration pneumonia. At autopsy, the brain and spinal cord are obviously atrophic. Microscopic examination shows proliferated and occluded small vessels, some with calcification, in the cortex and basal ganglia. However, the most conspicuous histological findings are destruction of ganglion cells in the third, fifth and sixth layers of the cortex, with vacuolation; loss of the giant pyramidal Betz cells; ‘enormous’ proliferation of microglia and protoplasmic astrocytes in white and grey matter; marked neuronal loss and demyelination of surviving axons, in the rostral and middle portions of the globus pallidus, corpus Luysii and paraventricular nuclei; and degeneration both of the pyramidal tracts and anterior horn cells (Fig. 1). Taken together, these pathologies are not those of a primary inflammatory or vascular disorder; nor are the plaques and other pathological hallmarks of Alzheimer's disease present; and in the spinal cord, the appearances are typical of amyotrophic lateral sclerosis. Figure 1 Case 1 (J.R.): two ganglion cells from the globus pallidus; one showing satellitosis and the other poor chromatin substance. Cresyl violet stain, ×700 (reproduced from Davison, 1932). E.G. dies the …