Immunohistochemical evidence for the selective involvement of dorsal root fibres in Friedreich's ataxia.

An immunohistochemical study was carried out in order to elucidate the selective involvement of the dorsal root fibres from two patients with Friedreich's ataxia in comparison with those of 10 neurologically normal control individuals. For this purpose, antibodies to substance P and to synaptophysin were used. Substance P-immunoreactive unmyelinated fibres forming a dense network in the normal substantia gelatinosa of the spinal dorsal horn predominantly originate from a subpopulation of small cells of the dorsal root ganglia, while synaptophysin is present in virtually all nerve cell axon terminals and is useful for visualizing axon terminals in the nervous system. Strong substance P-like immunoreactivity was seen in the substantia gelatinosa of patients with Friedreich's ataxia. By contrast, there was marked depletion of synaptophysin immunoreactivity in the posterior column nuclei, with the gracile nucleus showing greater loss of positive puncta than the cuneate nucleus.