Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy

Mano, Tomoo; Katsuno, Masahisa; Banno, Haruhiko; Suzuki, Keisuke; Suga, Noriaki; Hashizume, Akira; Araki, Atsuko; Hijikata, Yasuki; Tanaka, Seiya; Takatsu, Jun; Watanabe, Haruo; Yamamoto, Masahiko; Sobue, Gen

doi:10.1159/000431088

Cited by 10 publications

(7 citation statements)

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“…Given that the presence of pharyngeal residue is the most common finding in SBMA patients and a laryngeal penetration risk exists even in those without subjective dysphagia, we recommend the chin‐tuck maneuver and the effortful swallow as safe methods of swallowing for all SBMA patients with dysphagia 36, 37. We also reported that a 6‐week head lift exercise may improve swallowing dysfunction as measured by tongue pressure 38. Reinforcement of compensatory swallowing mechanisms may lead to effective rehabilitation in dysphagia patients.…”

Section: Discussionmentioning

confidence: 89%

Swallowing markers in spinal and bulbar muscular atrophy

Banno

Katsuno

Suzuki

et al. 2017

Ann Clin Transl Neurol

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ObjectiveWe examined the characteristics of dysphagia in spinal and bulbar muscular atrophy, a hereditary neuromuscular disease causing weakness of limb, facial, and oropharyngeal muscles via a videofluoroscopic swallowing study, and investigated the plausibility of using these outcome measures for quantitative analysis.MethodsA videofluoroscopic swallowing study was performed on 111 consecutive patients with genetically confirmed spinal and bulbar muscular atrophy and 53 age‐ and sex‐matched healthy controls. Swallowing of 3‐mL liquid barium was analyzed by the Logemann's Videofluorographic Examination of Swallowing worksheet.ResultsOf more than 40 radiographic findings, the most pertinent abnormal findings in patients with spinal and bulbar muscular atrophy, included vallecular residue after swallow (residue just behind the tongue base), nasal penetration, and insufficient tongue movement (P < 0.001 for each) compared with healthy controls. Quantitative analyses showed that pharyngeal residue after initial swallowing, oral residue after initial swallowing, multiple swallowing sessions, and the penetration–aspiration scale were significantly worse in these patients (P ≤ 0.005 for each) than in controls. In patients with spinal and bulbar muscular atrophy, laryngeal penetration was observed more frequently in those without subjective dysphagia.InterpretationDysphagia of spinal and bulbar muscular atrophy was characterized by impaired tongue movement in the oral phase and nasal penetration followed by pharyngeal residues, which resulted in multiple swallowing sessions and laryngeal penetration. Although major limitations of reproducibility and radiation exposure still exist with videofluoroscopy, pharyngeal residue after initial swallowing and the penetration–aspiration scale might serve as potential outcome measures in clinical studies.

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Section: Discussionmentioning

confidence: 89%

Swallowing markers in spinal and bulbar muscular atrophy

Banno

Katsuno

Suzuki

et al. 2017

Ann Clin Transl Neurol

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“…It is increasingly recognized that non-neurological features of the disease have an equally important impact on the patients' quality of life ( 3 , 31 – 34 , 87 , 88 , 91 , 92 ). Until now, clinical trials on SBMA focused almost exclusively on the treatment of motor symptoms ( 14 , 45 , 47 , 51 – 53 , 55 , 57 , 68 , 92 , 93 ), but a shift to targeted molecular therapies ( 94 ) and focus on systemic processes are likely to be witnessed in the near future. From a clinical trial perspective, ideal biomarkers should undergo robust validation, sensitivity and specificity profiling, and sampling and measurement harmonization across different centers.…”

Section: Discussion and Future Perspectivesmentioning

confidence: 99%

“…In SBMA, VF can reliably detect the impairment of the oral phase of deglutition confirming large amount of oral barium residue ( 56 ). VF has been previously used in clinical trials ( 51 , 55 , 68 ), but the lack of standardization makes it less suitable for robust multicenter studies.…”

Section: Biomarkers Of Neurological Involvement In Sbmamentioning

confidence: 99%

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Biomarkers of Spinal and Bulbar Muscle Atrophy (SBMA): A Comprehensive Review

et al. 2018

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Spinal and bulbar muscular atrophy (SBMA), also known as Kennedy's disease, is a rare, X-linked, late onset neuromuscular disorder. The disease is caused by a CAG trinucleotide repeat expansion in the first exon of the androgen receptor gene. It is characterized by slowly progressive lower motor neurons degeneration, primary myopathy and widespread multisystem involvement. Respiratory involvement is rare, and the condition is associated with a normal life expectancy. Despite a plethora of therapeutic studies in mouse models, no effective disease-modifying therapy has been licensed for clinical use to date. The development of sensitive monitoring markers for the particularly slowly progressing pathology of SBMA is urgently required to aid future clinical trials. A small number of outcome measures have been proposed recently, including promising biochemical markers, which show correlation with clinical disability and disease-stage and progression. Nevertheless, a paucity of SBMA-specific biomarker studies persists, delaying the development of monitoring markers for pharmaceutical trials. Collaborative efforts through international consortia and multicenter registries are likely to contribute to the characterization of the natural history of the condition, the establishment of disease-specific biomarker panels and ultimately contribute to the development of disease-modifying drugs.

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“…Recently, a sequence of head-lift exercises has been proposed as it seems to ameliorate swallowing dysfunction [99]. This hypothesis has been tested in six patients with good results but needs to be confirmed in larger and longitudinal trials.…”

Section: Experimental Treatmentsmentioning

confidence: 99%

Kennedy disease (X-linked recessive bulbospinal neuronopathy): A comprehensive review from pathophysiology to therapy

2017

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Acknowledgments: the authors thank AFM-Téléthon, Téléthon Biobank, Institut pour la Recherche sur la Moelle épinière et l'Encéphale (IRME), Association pour la Recherche sur la SLA (ARSla) and the University of Padova for their research support.Kennedy disease (X-Linked recessive Bulbospinal Neuronopathy): a comprehensive review from pathophysiology to therapy. AbstractKennedy's disease, also known as spinal and bulbar muscular atrophy (SBMA), is a rare, adult-onset, Xlinked recessive neuromuscular disease. It is caused by expansion of a CAG repeat sequence in exon 1 of the androgen-receptor (AR) gene, encoding a poly-glutamine (polyQ) tract. Poly-Q-expanded AR accumulates in nuclei and initiates degeneration and loss of motor neurons and dorsal root ganglia. The disease has been retained to be a pure lower motor neuron disease for a long time, but recently the presence of important hyper-Creatine-Kinase-emia and of myopathic alterations on muscle biopsy has suggested the presence of a primary myopathy underlying a wide proportion of clinical manifestations. The disease, that affects male adults, is characterized by muscle weakness and atrophy localized proximally in the limbs and by bulbar involvement. Sensory disturbances are associated to the motor phenotype but may be subclinical. The most frequent systemic symptom is gynecomastia related to androgen insensitivity, but other abnormalities, such as heart rhythm and urinary disturbances, have also been reported. The course of the disease is slowly progressive with normal life expectancy. The diagnosis of SBMA is based on genetic testing, with 38 CAG repeats retained to be pathogenic. Even though several therapeutic attempts have been made in mouse models, no effective disease modifying therapy is available but symptomatic therapy is beneficial for the management of weakness, fatigability and bulbar symptoms.

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Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy

Cited by 10 publications

References 36 publications

Swallowing markers in spinal and bulbar muscular atrophy

Swallowing markers in spinal and bulbar muscular atrophy

Biomarkers of Spinal and Bulbar Muscle Atrophy (SBMA): A Comprehensive Review

Kennedy disease (X-linked recessive bulbospinal neuronopathy): A comprehensive review from pathophysiology to therapy

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