Applied nutritional investigation
Use of oral nutritional supplements in patients with Huntington’s
disease
Araceli Trejo, B.S.
a,
*, Marie-Catherine Boll, M.D., M.Sc.
b
, Ma. Elisa Alonso, M.D.
c
,
Adriana Ochoa, B.S.
c
, and Leora Velásquez, M.D., M.Sc.
d
a
Nutrition Laboratory, Instituto Nacional de Neurología y Neurocirugía “MVS”, Mexico D.F., Mexico
b
Department of Neurology, Instituto Nacional de Neurología y Neurocirugía “MVS”, Mexico D.F., Mexico
c
Department of Neurogenetics and Molecular Biology, Instituto Nacional de Neurología y Neurocirugía “MVS”, Mexico D.F., Mexico
d
Department of Neuroepidemiology, Instituto Nacional de Neurología y Neurocirugía “MVS”, Mexico D.F., Mexico
Manuscript received May 11, 2004; accepted December 1, 2004.
Abstract Objective: This study assessed the effect of oral nutritional supplements on the nutritional status
of patients with Huntington’s disease.
Methods: This was an experimental, longitudinal, prospective study of 30 patients with Hunting-
ton’s disease. We performed neurologic evaluation and dietary assessment and measured anthro-
pometric indexes and biochemical indicators; in addition, patients were questioned about their
weight, appetite, chewing difficulty, and dysphagia. Patients consumed two cans daily of a nutri-
tional supplement that contributed an extra 473 kcal to their diet for a 90-d period. At the study’s
end, the supplement was suspended and the same variables were reassessed.
Results: After 90 d, 68.7% of patients had increased body weight, 68.7% had ideal body weight
percentages and body mass indexes, 53.3% had increased midarm circumferences, and 60.0% had
increased arm muscle circumferences and body fat percentages; these changes were statistically
significant (P 0.05). The neurologic evaluation subscales and the biochemical indicators did not
change significantly. With regard to subjective variables, patients who reported losing weight during
the 3 mo before the study did not lose more weight and patients who reported having an increased
appetite before the study remained stable during the study.
Conclusions: The nutritional intervention stabilized or slightly improved the anthropometric
variables assessed; however, no significant change in body mass index occurred in 87% of patients.
For the purpose of maintaining an acceptable nutritional status in patients who have Huntington’s
disease and normal nutritional status, we suggest oral nutritional supplements that contribute an
average of 473 kcal/d in addition to a normal diet. © 2005 Elsevier Inc. All rights reserved.
Keywords: Oral nutritional supplements; Huntington’s disease; Nutritional status; Undernourishment
Introduction
Huntington’s disease (HD) is a neurodegenerative dis-
ease with dominant autosomal inheritance that is character-
ized by neuronal degeneration in the caudated nucleus; its
main clinical manifestations are involuntary movements
(chorea), psychiatric disturbances, and cognitive impair-
ment. HD results from a mutation in the huntingtin gene,
which is located on the short arm of chromosome 4 (4p16.3)
[1]. The mutated gene is characterized by a CAG (cytosine,
adenine, guanine) trinucleotide expansion within the first
exon [2]. The clinical diagnosis of HD is based on pedigree
and neurologic examination and is confirmed by the length
of the CAG repeat [3].
Symptoms of HD can modify the nutritional status of
patients, and weight loss is frequently observed [4]. In the
evaluation of the nutritional status of patients with HD,
body weight, body mass index (BMI), and arm circumfer-
ence have been reported to be significantly decreased in
This study was supported by Fresenius Kabi, Mexico.
* Corresponding author. Tel.: +011-5255-5606-4120, ext. 2052; fax:
+011-5255-5424-0808.
E-mail address: aratrejoc@hotmail.com (A. Trejo).
Nutrition 21 (2005) 889 – 894
www.elsevier.com/locate/nut
0899-9007/05/$ – see front matter © 2005 Elsevier Inc. All rights reserved.
doi:10.1016/j.nut.2004.12.012