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CLINICAL VIGNETTES

Progressive Ataxia with Palatal Tremor Due to Gluten Sensitivity


Ammar Kheder, MRCP,1 Stuart Currie, FRCR,2 Charles Romanowski, MD,2 and Marios Hadjivassiliou, MD1*

1
Department of Neurology, Royal Hallamshire Hospital, Sheffield, United Kingdom
2
Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield, United Kingdom

Palatal tremor is characterized by involuntary and During the ensuing years she remained clinically
continuous rhythmic movement of the soft palate. It is stable from the ataxia point of view. The patient and
divided into symptomatic palatal tremor (SPT) and her husband spontaneously reported improvement of
essential palatal tremor (EPT).1 In EPT the patient the palatal tremor 2 years after the introduction of the
complains of ear discomfort and a clicking noise gen- gluten-free diet. Repeat MR imaging (Fig. 1) showed
erated by the contraction of the tensor veli palatini
muscle, responsible for opening of the eustachian
tube. In SPT there is contraction of the levator veli
palatini muscle, but the patient is usually unaware of
it. A subgroup of SPT is associated with ataxia,
known as progressive ataxia with palatal tremor
(PAPT).2 SPT and PAPT can result from a lesion in
the Mollaret triangle (inferior olive, red nucleus, con-
tralateral dentate nucleus). In some cases no lesion is
demonstrable, and the etiology remains obscure. We
describe here a patient with PAPT due to gluten
sensitivity.
A 66-year-old woman with a history of rheumatoid
arthritis was first referred to neurology 24 years ago
with a 1-year history of slurred speech and unsteadi-
ness of gait. Examination revealed gait ataxia, slurred
speech, and palatal tremor. There was no family his-
tory of ataxia. Investigations including CSF analysis
and genetic, paraneoplastic antibody, and vitamin E
testing were negative. MRI demonstrated cerebellar at-
rophy involving both the vermis and the hemispheres
and revealed hypertrophic inferior olives without any
structural lesion. Antigliadin antibodies were positive,
and her HLA was DQ2 in keeping with gluten sensi-
tivity. She refused a duodenal biopsy but commenced
a gluten-free diet.

------------------------------------------------------------
Additional Supporting Information may be found in the online version of
this article.
*Correspondence to: Marios Hadjivassiliou, Department of Neurology,
Royal Hallamshire Hospital, Sheffield, United Kingdom; m.hadjivassiliou@
sheffield.ac.uk
Relevant conflicts of interest/financial disclosures: Nothing to
disclose.
FIG. 1. A: Axial T2-weighted image through the posterior fossa dem-
Full financial disclosures and author roles may be found in the online onstrating olivary hypertrophy associated with abnormal high signal
version of this article. (arrows). B: Axial T2-weighted image acquired while the patient
Received: 15 June 2011; Revised: 17 August 2011; Accepted: adhered to a strict gluten-free diet (approximately 12 years after image
29 August 2011 in A) demonstrating radiological improvement with a reduction in oli-
Published online in Wiley Online Library (wileyonlinelibrary.com). vary hypertrophy (arrows) and less diffuse olivary abnormal T2
DOI: 10.1002/mds.23987 hyperintensity.

62 Movement Disorders, Vol. 27, No. 1, 2012


C L I N I C A L V I G N E T T E S

reduction of the hypertrophy of the olives. The patient suggest a more dynamic process than just degenera-
remains stable on a gluten-free diet with no progres- tion. We can only postulate that an ongoing inflamma-
sion of the ataxia. tory process (seen in the context of gluten ataxia) may
There have been 2 case reports of PAPT in the con- be interfering with neural pathways in the triangle of
text of celiac disease.3,4 Ataxia is the commonest neu- Mollaret. Resolution of such inflammation following
rological manifestation among gluten-related diseases the introduction of the gluten-free diet may have
and has been shown to improve or stabilize with a resulted in improvement of the palatal tremor.
strict gluten-free diet. Palatal tremor, however, is an Gluten sensitivity should be added to the differential
uncommon finding in gluten ataxia. In this case, not diagnosis of PAPT.
only did the ataxia stabilize, but the patient reported
reduction in the frequency and severity of the palatal
tremor, associated with less prominent olivary hyper-
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Movement Disorders, Vol. 27, No. 1, 2012 63

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