Regular Paper

Acta Neuropathologica

, Volume 88, Issue 2, pp 113-121

First online:

Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofibrillary tangles, and its relation to progressive supranuclear palsy

  • H. MoriAffiliated withDepartment of Neurology, Tokyo Metropolitan Neurological Hospital
  • , M. NishimuraAffiliated withDepartment of Neurology, Tokyo Metropolitan Neurological Hospital
  • , Y. NambaAffiliated withDepartment of Pathology, Tokyo Metropolitan Neurological Hospital
  • , M. OdaAffiliated withDepartment of Pathology, Tokyo Metropolitan Neurological Hospital

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Abstract

The neuropathological findings, including immunohistochemistry and electron microscopy, of two patients with clinical findings consistent with corticobasal degeneration (CBD) are reported. Both patients showed degeneration of the precentral cortex, the substantia nigra, the pallidum, and the thalamus. Many ballooned neurons were seen in the cerebral cortex, and argentophilic, skein-like inclusions suggesting neurofibrillary tangles (NFTs) were found in the brain stem and precentral cortex in patient 1. In contrast, patient 2 clearly showed NFTs in the brain stem and dentate nucleus which were indistinguishable from those seen in progressive supranuclear palsy (PSP), while only a few ballooned neurons were found in the cerebral cortex. Gallyas silver stain showed many argentophilic inclusions suggesting NFTs in the brain stem, subcortical nuclei, and cerebral cortex in both patients. Immunohistochemistry for tau showed tau-positive neurons in the cerebral cortex, brain stem, subcortical nuclei and spinal cord, and tau-positive glial cells were seen in the cerebral cortex, white matter and subcortical nuclei, and thread-like structures were seen in the cerebral cortex and white matter. Electron microscopy of the brain stem showed NFTs consisting of paired helical filaments in patient 1, and paired helical filaments and straight tubules in patient 2. Immunoelectron microscopy revealed parallel tau-positive filaments in the cerebral cortex in patent 1. From the two patients, the widespread appearance of abnormal tau and NFTs is one of the essential pathological features in CBD, and it also appears that CBD and PSP have some common underlying pathological processes. Patient 2 is closer to PSP than patient 1 and suggests CBD would link to PSP.

Key words

Corticobasal degeneration Progressive supranuclear palsy Neurofibrillary tangles Abnormal tau