The Evidence for ALS as a Multisystems Disorder of Limited Phenotypic Expression

Can. J. Neurol. Sci. 2001; 28: 293-308

Figure 1: Neuropathological features of cognitive impairment in sporadic ALS. Consistent with the imaging findings, prominent frontal atrophy can be observed in cognitively impaired patients with ALS. In Figure A, striking atrophy is evident throughout the frontal lobe, including the anterior cingulate gyrus (photograph courtesy of Dr. David Munoz). The only histological feature identified as a consistent feature of cognitive impairment is the presence of superficial linear spongiosis (B, 10x mag.) not observed in cognitively intact patients (C, 10x mag.). In the majority of patients, this was also accompanied by a transcortical microglia activation (D, 20x mag.; HLA-DR immunostaining of microglia with the antigen:antibody conjugate detected using 3,4-diaminobenzidine, giving rise to a brown coloration.). The later finding was not, however, restricted to the first and second cortical layers, those that are most affected by the superficial linear spongiosus.

 

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