Temporal Lobe Lesions in Amyotrophic Lateral Sclerosis with or without Dementia: A Neuropathological Study

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<jats:p>A neuropathological study of cases of amyotrophic lateral sclerosis with dementia [ALS‐D (+)] revealed, in addition to changes of the motor neuron system, temporal lobe (TL) lesions that had a characteristic distribution. The alterations include neuronal loss emphasized in the medial cortex of the temporal tip and focal depletion of pyramidal neurons in the pes hippocampi. These observations indicate that these regions may be the first non‐motor cerebral cortex structures to be involved in this condition, and that the neuronal loss in these TL areas may play a role in the mental symptoms observed in ALS‐D(+). Moreover, the TL of approxiamtely 10% of ALS cases without dementia [ALS‐D (‐)] had similar alterations. This poses the question of whether these cases may represent an intermediate subgroup between ALS‐D (‐) and ALS‐D (+), or whether the ALS‐D (‐) patients with TL lesions were indeed demented, but in whom the proper the proper diagnoses were overlooked. Answers to these points can only be provided by detailed clinicopathological studies on appropriately matched ALS patients</jats:p>

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