The neuropathological findings of the cases 5 and 6
| Anatomical site | Case number | |
|---|---|---|
| 1Ubiquitin positive, tau and α-synuclein negative inclusions. | ||
| (+), very mild pathology or uncertain finding; +, mild pathology; ++, moderate pathology; +++, severe pathology. | ||
| 5 | 6 | |
| Cerebral cortex | ||
| Microvacuolar change | + | |
| Intraneuronal inclusions1 | ||
| Temporal cortex | ++ | ++ |
| Dentate gyrus | +++ | +++ |
| Ubiquitinated neuritis | ++ | ++ |
| Substantia nigra | ||
| Neurone loss | (+) | (+) |
| Intraneuronal inclusions1 | – | – |
| Cerebellum | ||
| Purkinje cell loss | ++ (focal) | ++ (focal) |
| Intraneuronal inclusions1 | + | + |
| Axonal swellings (torpedoes) | + | + |
| Hypoglossal nucleus | ||
| Intraneuronal inclusions1 | ++ | + |
| Spinal cord | ||
| Anterior horn neurone loss | +++ | – |
| Intraneuronal inclusions1 | + | (+) |
| Loss of myelin staining | ++ | – |