Familial progressive aphasia:Its relationship to other forms of lobar atrophy

Two brothers presented with slowly progressive aphasia. One brother, who became behaviourally disturbed only at the end of his illness, was found at necropsy to have predominant left frontotemporal atrophy. The other brother developed severe behavioural disturbances shortly after the onset of language impairment. His brain revealed bilateral frontotemporal atrophy. In both there was non-Alzheimer's disease pathology with the histological features of loss of large cortical nerve cells, spongiform change and mild gliosis. The differential anatomical atrophy supports the view that clinical manifestations of lobar atrophy are dictated by the topographical distribution of a common underlying pathology, linking the syndromes of progressive aphasia to dementia of frontal lobe type (DFT) and DFT with motor neuron disease

Abnormal RNA metabolism in spinal motor neurons in the wobbler mouse

RNA metabolism was studied in anterior horn neurons in wobbler mice. In the wobbler mice, RNA content was reduced in histologically normal neurons but not consistently reduced in vaculated neurons, as compared with normal littermates. Nuclear and nucleolar incorporation of [5–3H]uridine was reduced both in vacuolated and in histologically normal neurons of wobbler mice, while cytoplasmic counts were higher in histologically normal neurons of wobbler mice than in vacuolated neurons or the neurons of normal littermates. These findings point to an abnormality of RNA synthesis which precedes the earliest morphological changes seen in nerve cells with the light microscope. Nuclear [5–3H]uridine uptake was reduced both in small and in large neurons, a finding suggesting that the metabolic abnormality is probably not confined to α-motoneurons