Best Post of the April 2012: Photomicrographs of spinocerebellar ataxia type 6 case

The next in our "Best of the Month" series is from April 26, 2012. It's a follow-up from a prior post in which I showed the gross finding from the same case of spinocerebellar ataxia type 6:

A month ago, I posted gross photographs from an autopsy case of mine of a patient with spinocerebellar ataxia type 6. I am now following up with histologic images for this case. The most striking feature was the marked loss of Purkinje cells, which makes sense because SCA6 involves mutation of a calcium channel that is highly expressed on Purkinje neurons:

Many remaining Purkinje neurons demonstrated degenerative changes, including mis-shapened nuclei

Attenuated cerebellar granule cell layer

More Purkinje cells in bad shape

Cerebellar dentate neurons also dengenerated, but not as extensively as Purkinje cells

Mild involvement of inferior olives. Note the mis-shapen and darkened nuclei of neurons in this location.

Pons minimally effected, with only occasional degenerating neurons as seen in the center of this photomicrograph next to a normal neuron