Dysphagia in Huntington's Disease

Dysphagia in Huntington's Disease Studies

One study examined the diagnosed occurrence of dysphagia in Huntington's disease; Kagel and Leopold (1992) examined 35 patients with Huntington's disease (mean age 45.5) using a clinical questionnaire, assessment of feeding, and videofluoroscopic exam.

Twenty-seven patients (80 percent) coughed or choked on both solids and liquids; six were symptomatic with food only, and two with liquid only. Thus, 100 percent of patients with Huntington's disease showed dysphagia of one kind or another.

In the hyperkinetic variant of Huntington's disease (30 of 35 patients), 29 (96.7 percent) demonstrated oral dysphagia, 27 (90 percent) pharyngeal dysphagia, and 11 (36.6 percent) esophageal dysphagia during clinical assessment.

A total of 100 percent of hypokinetic-Huntington's disease patients (five patients) demonstrated oral and pharyngeal swallowing dysfunction; none demonstrated esophageal dysphagia (Kagel and Leopold, 1992).

Selection methodology of these patients was not described; therefore, there is a possibility that the researchers specifically selected patients with dysphagia for study.

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Arch Neurol. 1985 Jan;42(1):57-60

Dysphagia in Huntington's disease.
Leopold NA, Kagel MC.

Dysphagia is a common complication of Huntington's disease (HD) that is frequently responsible for the potentially lethal respiratory events of aspiration or asphyxiation.

Twelve patients who had HD and a history of dysphagia underwent extensive multi-disciplinary clinical examinations. All of the patients, regardless of the clinical severity of their disease, demonstrated impaired control of many voluntary aspects of food intake that affected swallowing efficiency.

Abnormalities of the rate of food consumption, mastication, bolus transfer, respiration, and swallow initiation seem to be responsible for most dysphagic symptoms in HD. Less prominent abnormalities of the pharyngoesophageal phases of ingestion were also noted.

Dysphagia therapy was initiated in 11 of 12 patients. All of the patients' conditions improved; a majority (8/11) of the patients returned to an unrestricted diet. This improvement persisted for as long as three years, while other clinical features of HD intensified.

PMID: 3155611 [PubMed - indexed for MEDLINE

Dysphagia. 1992;7(2):106-14.

Dysphagia in Huntington's disease: a 16-year retrospective.
Kagel MC, Leopold NA.
Department of Medicine, Crozer-Chester Medical Center, Upland, Pennsylvania.

Degenerative diseases of the basal ganglia are commonly complicated by dysphagia. In 35 patients with Huntington's disease (HD), a hereditary neurodegenerative basal ganglia disease characterized by chorea, dementia, and emotional changes, an extensive battery of clinical and radiologic procedures helped to identify numerous abnormalities of deglutition.

The results permitted the classification of our patients with HD into hyperkinetic (HD-h) or rigid-bradykinetic (HD-rb) groups. Although the two groups share multiple abnormalities, statistically significant intergroup differences were observed.

Clinical assessment of the HD-h cohort (30 patients) demonstrated rapid lingual chorea, swallow incoordination, repetitive swallows, prolonged laryngeal elevation, inability to stop respiration, and frequent eructations.

In the HD-rb group (five patients), frequently observed abnormalities included mandibular rigidity, slow lingual chorea, coughing on foods, and choking on liquids.

Videofluoroscopic swallowing studies (VFSS) using a variety of barium-impregnated foods and liquids confirmed the abnormalities noted on the clinical assessment. Respiratory and laryngeal chorea, pharyngeal space retention, and aspiration were also identified.

Numerous compensatory techniques introduced during videofluoroscopy benefited all patients.

PMID: 1533361 [PubMed - indexed for MEDLINE]

Summary

Dysphagia is quite commonly diagnosed in all of these neurological patients. Estimates have been very high for stroke, Parkinson's disease, Alzheimer's disease, and Huntington's disease; however, fairly low estimates have also been reported for those diseases on which there was more than one study published.

These variations indicate interstudy differences in diagnostic criteria and methodology. It is therefore impossible to determine if dysphagia is prevalent enough in any one of these diseases that 100 percent of these patients should be tested for dysphagia; a determination whether or not to do this would be based on clinical judgment.

It must also be pointed out that dysphagia, per se, is not a risk factor for pneumonia; it is the aspiration of certain substances that leads to pneumonia. Dysphagia, on the other hand, may put a patient at risk for mal-nutrition if the patient is unable to safely swallow adequate amounts of food.