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. 2018 Jun;8(6):e00999.
doi: 10.1002/brb3.999. Epub 2018 May 21.

Oral phase dysphagia in facial onset sensory and motor neuronopathy

Mitsuru Watanabe  1 , Wataru Shiraishi  1 , Ryo Yamasaki  1 , Noriko Isobe  2 , Motohiro Sawatsubashi  3 , Ryuji Yasumatsu  3 , Takashi Nakagawa  3 , Jun-Ichi Kira  1
Affiliations

Oral phase dysphagia in facial onset sensory and motor neuronopathy

Mitsuru Watanabe et al. Brain Behav. 2018 Jun.

Abstract

Introduction: Facial onset motor and sensory neuronopathy (FOSMN) is a rare disease whose cardinal features are initial asymmetrical facial sensory deficits followed by bulbar symptoms and spreading of sensory and motor deficits from face to scalp, neck, upper trunk, and upper extremities in a rostral-caudal direction. Although bulbar involvement is frequently observed in FOSMN, dysphagia in these patients has not been fully described. In this study, we aimed to characterize dysphagia as a prognostic factor in FOSMN by investigating our institutional case series.

Methods: We retrospectively reviewed the medical records, including swallowing function tests, of six patients with FOSMN (three men and three women) who were thoroughly examined at Kyushu University Hospital between 1 January 2005 and 30 November 2017.

Results: Average age at onset was 58.5 years; average disease duration was 5.7 years. All patients developed bulbar dysfunction and dysphagia (at an average of 1.8 and 2.6 years from onset, respectively), resulting in choking episodes in three patients, percutaneous endoscopic gastrostomy placement in three, and recurrent aspiration pneumonia in one. Four of five patients evaluated with videofluoroscopic swallowing studies had poor oral retention, leading to bolus flowing into the pharynx before swallowing; the fifth patient showed poor lingual transfer. Fiberoptic endoscopic evaluation of swallowing revealed leakage of blue-dyed water from the mouth to the pharynx in three patients because of poor oral retention, but only mild pharyngeal phase dysphagia in all four cases evaluated.

Conclusions: Oral phase dysphagia predominates in the early stage of FOSMN.

Keywords: dysphagia; facial onset sensory and motor neuronopathy; neurodegeneration; neuroinflammation; oral phase; prognostic factor.

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Figures

Figure 1
Figure 1
Reduced 123I‐ioflupane binding in the bilateral striata of Patient 3. Dopamine transporter SPECT with 123I‐ioflupane was performed in Patient 3. 123I‐ioflupane binding was reduced in the bilateral striata, with left‐sided predominance. The specific binding ratio was 3.68 on the right and 2.66 on the left side; the asymmetry index was 32.2%
Figure 2
Figure 2
Blink reflex findings before and after immunotherapy in Patient 4. Blink reflex findings with left‐sided stimulation in Patient 4 are shown. Delayed ipsi‐ and contralateral R2 responses (iR2 and cR2, respectively) to left‐sided stimulation improved after intravenous immunoglobulin (IVIg) therapy. The latencies of iR2 and cR2 before treatment were 45.7 and 46.0 ms, respectively; those after treatment were 36.6 and 34.5 ms, respectively
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