Objective: We aimed to assess the prevalence of swallowing disorder and to characterize the main clinical signs of dysphagia in genetically confirmed HSP patients.

Background: The core feature of hereditary spastic paraplegias (HSP) is lower limb spasticity, which might be accompanied or not by additional neurological features1. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications.

Method: A two-center cross-sectional prevalence study was performed. Genetically confirmed HSP patients were evaluated by the Northwestern Dysphagia Patient Check Sheet (NDPCS)2 and the Functional Oral Intake Scale3. In addition, self-perception of dysphagia was assessed by the Eat Assessment Tool (EAT-10)4 and the Swallowing Disturbance Questionnaire (SDQ)5.

Results: We evaluated thirty-six patients with spastic paraplegia type 4 (SPG4), five with SPG11, four with both SPG5 and cerebrotendinous xanthomatosis (CTX), three with SPG7, and two with SPG3A. Regarding the clinical evaluation of swallowing, 3/5 (60%) and 2/4 (50%) of SPG11 and CTX patients, respectively, presented mild to moderate oropharyngeal dysphagia, which was the most serious clinical finding of this study. The prevalence of dysphagia in the other groups was much lower, with the majority of patients being diagnosed with normal or functional swallowing. Dysphagia prevalence and description of clinical signs for each HSP forms are described in Table 1 (insert table 1).

Conclusion: Oropharyngeal dysphagia of clinical relevance was only present in complicated forms of HSP and it was not perceived by patients. SPG11 and CTX presented highest risks for dysphagia, suggesting that surveillance of swallowing function should be part of the management of patients with these disorders.

References: References 1. Depienne C, Stevanin G, Brice A, Durr A. Hereditary spastic paraplegias: an update. Curr Opin Neurol 2007;20: 674–80. 2. Magalhães Junir, HV; Pernambuco, LA; Souza, LBR; Ferreira, MAF; Lima, KC. Translation and cross-cultural adaptation of the Northwestern Dysphagia Patient Check Sheet to Brazilian Portuguese. CoDAS 2013;25: 369-74. 3. Crary MA, Groher ME. Initial Psychometric Assessment of a Functional Oral Intake Scale for dysphagia in stroke patients. Arch Phys Med Rehabil. 2005; 86:1516-20. 4. Gonçalves, MIR; Remaili, CB; Bhelau, M. Cross-cultural adaptation of the Brazilian version of the Eating Assessment Tool – EAT-10.CoDAS 2013;2:601-4. 5. Ayres A, Ghisi M, Rieder CRM, Manor Y, Olchik MR Rev. Translation and Cultural Adaptation of Swallowing Disturbance questionnaire for Brazilian Ppopulation. CEFAC. 2016; 18: 828-834.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/prevalence-of-oropharyngeal-dysphagia-in-hereditary-spastic-paraplegias/