Abstract
Background
We report on a unique complication of cerebellar ataxia and motor neuron disease named
Asidan/SCA36 with a high frequency of tongue atrophy. We aimed to elucidate dysphagia
in amyotrophic lateral sclerosis (ALS) and spinal, bulbar muscular atrophy (SBMA),
and Asidan/SCA36 patients with new methods.
Methods
Patients diagnosed with ALS (n=20), SBMA (n=6), and Asidan (n=12) were included. A videofluoroscopic swallow study (VFS), an assessment of maximal
tongue pressure (MTP), and impedance pharyngography (IPG) were applied.
Results
The frequencies of VFS abnormalities were 70%, 50%, and 33% in ALS, SBMA, and Asidan/SCA36,
respectively. Compared with control subjects (31.6±6.3 kPa, mean±SD), MTP was significantly decreased in ALS patients and SBMA patients, but was relatively
preserved in Asidan patients. ALS patients performed more swallowing actions (Ns)
detected by IPG than did control subjects, but SBMA and Asidan/SCA36 patients performed
similar Ns to control subjects.
Conclusions
VFS showed a higher frequency of swallowing abnormalities in ALS patients. MTP and
IPG measurements showed the most severe involvement in ALS patients and a relatively
preserved swallowing function in SBMA and Asidan/SCA36 patients.
Keywords
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Article info
Publication history
Published online: November 12, 2012
Accepted:
October 24,
2012
Received in revised form:
October 18,
2012
Received:
September 12,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
