Neuroimaging features of progressive ataxia and palatal tremor

Achados de neuroimagem da ataxia progressiva com tremor palatal

A 56-year-old man complained about a 6-year-history of gait disturbance and bilateral tinnitus. His neurological family history was unremarkable. Physical examination disclosed gait ataxia, slowly horizontal saccadic pursuit and a 2Hz frequency palatal tremor. Neuroimaging unveiled inferior olivary complex hypertrophy bilaterally and mild cerebellar atrophy (Figure), highly suggestive of progressive ataxia and palatal tremor (PAPT). PAPT represents a rare idiopathic neurodegenerative disease characterized mainly by progressive cerebellar ataxia, variable bulbar dysfunction and symptomatic palatal tremor¹ with the peculiar neuroimaging finding of hypertrophy and variable hyperintensity of the inferior olivary complex².

Figure
Different brain MRI sequences (A-E) showing cerebellar atrophy and bilateral hypertrophy with hyperintensities of the inferior olivary complex (white arrows), a frequently described finding in sporadic tremor ataxia and palatal tremor (PAPT).

References

• ¹
  Brinar VV, Barun B, Zadro I, Ozretic D, Habek M. Progressive ataxia and palatal tremor. Arch Neurol. 2008;65(9):1248-9. http://dx.doi.org/10.1001/archneur.65.9.1248
  » https://doi.org/10.1001/archneur.65.9.1248
• ²
  Samuel M, Torun N, Tuite PJ, Sharpe JA, Lang AE. Progressive ataxia and palatal tremor (PAPT): clinical and MRI assessment with review of palatal tremors. Brain. 2004;127(6):1252-68. http://dx.doi.org/10.1093/brain/awh137
  » https://doi.org/10.1093/brain/awh137

Publication Dates

• Publication in this collection
  July 2015

History

• Received
  05 July 2014
• Reviewed
  19 Jan 2015
• Accepted
  06 Feb 2015