Cerebellar atrophy with Chiari malformation: An example of trans-synaptic degeneration?

James Peters; Catherine Ding; Shalini Amukotuwa; Peter Kempster

doi:10.1016/j.jocn.2019.08.005

Cerebellar atrophy with Chiari malformation: An example of trans-synaptic degeneration?

J Clin Neurosci. 2019 Nov:69:279-280. doi: 10.1016/j.jocn.2019.08.005. Epub 2019 Aug 22.

Authors

James Peters¹, Catherine Ding², Shalini Amukotuwa³, Peter Kempster⁴

Affiliations

¹ Neurosciences Department, Monash Medical Centre, Clayton, Australia. Electronic address: James.Peters@monashhealth.org.
² Neurosciences Department, Monash Medical Centre, Clayton, Australia.
³ Department of Imaging, Monash Medical Centre, Clayton, Australia.
⁴ Neurosciences Department, Monash Medical Centre, Clayton, Australia; Department of Medicine, School of Clinical Sciences, Monash University, Clayton, Australia.

PMID: 31447362
DOI: 10.1016/j.jocn.2019.08.005

Abstract

A 23-year old man was found to have a Chiari Type 1 malformation and cerebellar atrophy. While this association has previously been described, the remote cerebellar atrophy is difficult to explain. We believe the answer lies with our finding of signal hyperintensity on MR imaging at the level of the inferior olives. This suggest hypertrophic olivary degeneration, caused by trans-synaptic degeneration following disruption to the Guillain-Mollaret triangle. Propagation of this process to the cerebellar Purkinje cells occurs in some cases. We describe a case in support of this hypothesis and review previously published evidence.

Keywords: Cerebellar atrophy; Chiari malformation; Hypertrophic olivary degeneration; Trans-synaptic degeneration.

Publication types

Case Reports

MeSH terms

Arnold-Chiari Malformation / complications
Arnold-Chiari Malformation / pathology*
Atrophy / pathology
Cerebellar Diseases / etiology
Cerebellar Diseases / pathology*
Humans
Magnetic Resonance Imaging / methods
Male
Olivary Nucleus / pathology*
Retrograde Degeneration / pathology*
Young Adult