Case Study Indicates That CD8+, Perforin+ and Granzyme B+ Infiltrate the Cerrebullum in Gluten Ataxia

Celiac.com 08/29/2018 - Up to one in twelve patients with gluten sensitivity develops neurological symptoms such as ataxia, dementia, seizures or peripheral neuropathy, though the reasons for this are still poorly understood.

As a means of better understanding the immunological mechanisms behind this reality, a team of researchers recently reported the case of a 68‐year‐old male patient suffering from progressive ataxia and dementia associated with chronic diarrhea, and both elevated IgG and IgA antigliadin‐antibodies. 

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The research team included Michel Mittelbronn, Jens Schittenhelm, Gellert Bakos, Rob A. De Vos, Manfred Wehrmann, Richard Meyermann, and Katrin Bürk. They are variously affiliated with the Institute of Brain Research at the University of Tübingen, and the Institute for Cell Biology, Department of Immunology at the University of Tübingen, Tübingen, Germany, the Neurological Institute/Edinger Institute, Goethe University Medical School, Frankfurt, the Department of Pathology, St. Georg Hospital, Leipzig, Germany, and with the Laboratory for Pathology, Enschede, the Netherlands.

Autopsy indicated that frequent argyrophilic glial and neuronal inclusions within the basal nucleus of Meynert were the structural markers of the cognitive decline.  The patient showed substantial neuronal loss in the cerebellar cortex and the inferior olives, along with infiltrating CD8+/perforin+/granzyme B+ cells, and reactive astrogliosis and microglial activation. 

In patients with gluten sensitivity and neurological disease, it is likely that CD8+ cytotoxic T and NK cells function as effector cells that trigger neuronal cell death, and thus might play some role in triggering cerebellar symptoms in gluten ataxia cases. The team concludes by noting that an absence of B‐ or plasma cells, along with multiple CD8+, granzyme B and perforin expressing cells in ataxia‐associated brain areas, indicates pronounced cytotoxic effects in neuro-pathogenesis of gluten sensitivity.

This is one of the first reports to indicate that CD8+, perforin+, and granzyme B+ effector cells infiltrate the cerebellum and inferior olives in cases of gluten ataxia.

Read more in: Neuropathology