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 tremor11 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.65.9.12...
with the peculiar
neuroimaging finding of hypertrophy and variable hyperintensity of the inferior olivary
complex22 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...
.
References
-
1Brinar 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 -
2Samuel 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
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Received
05 July 2014 -
Reviewed
19 Jan 2015 -
Accepted
06 Feb 2015