Atypical case of supranuclear ophtalmoplegia and parkinsonism in combination with amyotrophic lateral sclerosis
2008
Abstract
Amyotrophic lateral sclerosis (ALS) may be accompanied by frontotemporal cognitive impairment. However, severe parkinsonism and supranuclear ophtalmoplegy are unusual in ALS.
We report two cases with coincident parkinsonism and clinically defined ALS together with frontotemporal cognitive impairment. Neuropathological examination revealed ubiquitine immunoreactive neuronal changes in cerebral cortex and anterior horns of the spinal cord, but interestigly no pathological changes in the brainstem nor basal ganglia.
Our observation demonstrates that parkinsonism early in onset of the disease course may be a clinical feature of FTLD-MND, although majority of histopatological changes is localized in the cerebral cortex.