FRONTOTEMPORAL LOBAR DEGENERATION WITH UPPER MOTOR NEURON DISEASE/ PRIMARY LATERAL SCLEROSIS

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Abstract
Frontotemporal lobar degeneration (FTLD) is a pathologic entity characterized by the presence of neuronal loss and gliosis affecting mainly cortical lamina II of the frontal and temporal neocortices.1 In some cases the pathologic findings of FTLD are accompanied by motor neuron disease (FTLD-MND), with degeneration of the descending corticospinal tracts and loss of cranial nerve and anterior horn motor neurons.2 Intracytoplasmic inclusions in motor neurons occur and can be demonstrated with ubiquitin and TDP-43 antibodies.3 In a recent study of 17 cases of pathologically confirmed FTLD-MND, we demonstrated that degeneration of the corticospinal tract and loss of motor neurons are variable.2 In all but one case, we found evidence of lower MND with neuronal loss and gliosis affecting cranial nerve XII nuclei or anterior horn cell loss. It therefore seems to be the case that when MND accompanies FTLD, lower MND is almost always present. Therefore, we set out to determine if FTLD-MND truly occurs with isolated upper MND, a feature of so-called primary lateral sclerosis,4 hence FTLD-PLS. ### Methods. We identified 25 cases from Mayo Clinic Rochester and Jacksonville that had been studied …