Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias.

Abstract:

OBJECTIVES:Hereditary spastic paraplegias (HSP) are a group of genetic diseases characterized by lower limb spasticity with or without additional neurological features. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications. The aim of this study was to evaluate the frequency and clinical characteristics of dysphagia in different types of HSP. METHODS:A two-center cross-sectional prevalence study was performed. Genetically confirmed HSP patients were evaluated using the Northwestern Dysphagia Patient Check Sheet and the Functional Oral Intake Scale. In addition, self-perception of dysphagia was assessed by the Eat Assessment Tool-10 and the Swallowing Disturbance Questionnaire. RESULTS:Thirty-six patients with spastic paraplegia type 4 (SPG4), five with SPG11, four with SPG5, four with cerebrotendinous xanthomatosis (CTX), three with SPG7, and two with SPG3A were evaluated. Mild to moderate oropharyngeal dysphagia was present in 3/5 (60%) of SPG11 and 2/4 (50%) of CTX patients. A single SPG4 (2%) and a single SPG7 (33%) patient had mild oropharyngeal dysphagia. All other evaluated patients presented with normal or functional swallowing. CONCLUSIONS:Clinically significant oropharyngeal dysphagia was only present in complicated forms of HSP Patients with SPG11 and CTX had the highest risks for dysphagia, suggesting that surveillance of swallowing function should be part of the management of patients with these disorders.

journal_name

Arq Neuropsiquiatr

authors

Jacinto-Scudeiro LA,Machado GD,Ayres A,Burguêz D,Polese-Bonatto M,González-Salazar C,Siebert M,França Junior MC,Olchik MR,Saute JAM

doi

10.1590/0004-282X20190180

subject

Has Abstract

pub_date

2019-12-01 00:00:00

pages

843-847

issue

12

eissn

0004-282X

issn

1678-4227

pii

S0004-282X2019001200843

journal_volume

77

pub_type

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