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Childhood- and Adult-Onset ET Share Pathologies
Neurosci Lett; ePub 2017 Sep 1; Louis, Kuo, et al
Pathological changes observed in the cerebellum in essential tremor (ET) are a part of the pathophysiological cascade of events in both childhood-onset and adult-onset forms of the disease and both groups seem to reach the same pathological endpoints at a similar age of death, a recent study found. Researchers quantified a broad range of postmortem features in 60 ET cases (11 childhood-onset and 49 adult-onset) and 30 controls. They found:
- Compared to controls, childhood-onset ET cases had lower Purkinje cell (PC) counts, higher torpedo counts, higher heterotopic PC counts, higher basket cell plexus rating, and marginally higher PC axonal recurrent collateral counts.
- The median PC thickened axonal profile count and median PC axonal branching count were 2 to 5 times higher in childhood-onset ET than controls, but the differences did not reach statistical significance.
- Childhood-onset and adult-onset ET had similar PC counts, torpedo counts, heterotopic PC counts, basket cell plexus rating, PC axonal recurrent collateral counts, PC thickened axonal profile count and PC axonal branching count.
- In conclusion, childhood-onset and adult-onset ET shared similar pathological changes in the cerebellum.
Louis ED, Kuo S-H, Tate WJ, Kelly GC, Faust PL. Cerebellar pathology in childhood-onset vs. adult-onset essential tremor. [Published online ahead of print September 1, 2017]. Neurosci Lett. doi:10.1016/j.neulet.2017.08.072.