Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia☆,☆☆,★
Section snippets
Subjects
In 1995, the A-T Children’s Project established a national clinical center for A-T at Johns Hopkins Medical Institutions. Between February 1996 and March 1998, 75 consecutive patients participated in multidisciplinary evaluations including a physical examination, a clinical feeding/swallowing evaluation, and a nutritional assessment. Criteria for a diagnosis of A-T included the presence of characteristic neurologic features (gait ataxia, oculomotor dysfunction, dysarthria, and a movement
Patient Demographics and Growth Parameters
Of the 70 patients completing a clinical feeding/swallowing evaluation, 51 also participated in a VFSS procedure. The mean age of patients participating in only the clinical evaluation was significantly younger than the mean age of patients who participated in both the clinical evaluation and VFSS procedure (Table I). At all ages, patients with A-T demonstrated growth parameters that were lower than normative values for age. Height, weight, and weight for height were significantly lower for the
DISCUSSION
In this investigation we examined the oropharyngeal swallow and identified the presence of dysphagia in patients with A-T. This problem appears to be progressive because dysphagia with concomitant airway contamination and complaints of swallowing problems occur more frequently in older patients with A-T. The assumption that swallowing function worsens with age is consistent with the neurodegeneration progression and the bulbar impairments associated with A-T.2, 3
Although this was a
Acknowledgements
We acknowledge the assistance of Ms Donna Dieterich, patient coordinator for the A-T Clinical Center.
References (26)
Ataxia telangiectasia
Semin Pediatr Neurol
(1998)- et al.
Silent aspiration prominent in children with dysphagia
Int J Pediatr Otorhinolaryngol
(1994) - et al.
Physical growth: National Center for Health Statistics percentiles
Am J Clin Nutr
(1979) - et al.
The incidence and gene frequency of ataxia-telangiectasia in the United States
Am J Hum Genet
(1986) Ataxia-telangiectasia: an overview
- et al.
Ataxia-telangiectasia: an interdisciplinary approach to pathogenesis
Medicine
(1991) - et al.
The genetic defect in ataxia-telangiectasia
Annu Rev Immunol
(1997) - et al.
Ataxia telangiectasia in the British Isles: the clinical and laboratory features of 70 affected individuals
Q J Med
(1992) - et al.
Videofluoroscopic investigation of feeding disorders of children with multiple handicaps
Dev Med Child Neurol
(1989) - et al.
Feeding ability in Rett syndrome
Dev Med Child Neurol
(1997)
Dysphagia: its treatment in the profoundly retarded patient with cerebral palsy
Arch Phys Med Rehabil
Consequences of the delayed diagnosis of ataxia-telangiectasia
Pediatrics
Oral-motor and feeding assessment
Cited by (0)
- ☆
Supported in part by the Ataxia-Telangiectasia Children’s Project, Boca Raton, Florida; the Pediatric General Clinical Research Center, the Johns Hopkins Hospital, Baltimore, Maryland; Grant RR00052, Division of Research Resources National Institutes of Health; and the Hospital for Consumptives of Maryland Eudowood Board.
- ☆☆
Reprint requests: Maureen A. Lefton-Greif, PhD, The Ataxia-Telangiectasia Clinical Center, Johns Hopkins Children’s Center, CMSC 1102, 600 N Wolfe St, Baltimore, MD 21287-3923.
- ★
0022-3476/2000/$12.00 + 0 9/21/103567