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H7 The history of huntington’s nutrition
  1. Susan S Sandler1,2,3
  1. 1EHDN Dietitians Committee, Member, USA
  2. 2Academy of Nutrition and Dietetics, Registered, USA
  3. 3Dietitian-Nutritionist, Certified, USA


Background The national diets of the first HD population have something in common: they were leucine intensive.

As the nutritionist on a dedicated 48-bed HD residential unit for 15 years, I questioned whether we were unknowingly feeding clients the wrong distribution of the major food groups – protein, carbohydrate, fat – and thus exacerbating weight loss. In particular, the condition of wasting, which is muscle loss, is leucine dependent. Further, were the dietary proportions given to HD clients causing more muscle loss than fat loss?

Aims This poster highlights the common nutritional history of the countries where Huntington’s disease (HD) is prevalent.

Methods This is an historical survey of the diet of the first HD populations, as seen by a nutritionist.

  1. HD is found mostly in countries with a high dairy intake, i.e., a leucine-based diet. In dairy both the curd and whey proteins are entirely leucine.

  2. Fish, added later to that dairy base, and its bones are entirely leucine—especially cured fish with its edible bones. Specifically, salt-water fish with edible bones, such as cod/salt cod, herring, sardines, and salmon, were available to sailors.

  3. Could the people in whom the HD gene mutated have survived the Black Plague in 1659 because they were sailors at sea on extended fishing expeditions for cod and other above-mentioned fish species?

  4. Fishing fleets were staffed by “lads,” teenage boys whose bodies were in anabolism, simultaneous with the catabolic conditions of marginal starvation from limited rations of salt cod and marginal dehydration from a ration of only 8 ounces of fluid per person per day.

  5. Until 1770, when Captain Cook proved the need for dietary acid, vitamin B6 absorption – which is acid dependent – might have been inadequate for the metabolic load of leucine in the diet of sailors. (B6 is the gatekeeper for leucine metabolism.)

  6. The Japanese prevalence of HD is only 0.5, despite primary reliance on fish, and especially cured fish, in the diet. The Japanese diet is dissimilar in its abundance of acid, present in fermented foods, and in its limited sources of dairy.

Conclusion Redistribution of the proportions of the major foods groups in the diet given to HD clients should continue to be investigated.

  • nutrition
  • wasting
  • leucine

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