Ventricular sizes on computerized tomographic (CT) scans were compared in seven patients with Huntington disease, 20 patients with cerebral atrophy, and 20 normal controls. The bicaudate index--the ratio of the width of both lateral ventricles at the level of the heads of the caudate nuclei to the distance between the outer tables of the skull at the same level--significantly discriminated among the three groups. The bicaudate indices were: Huntington disease 0.209 +/- 0.007, cerebral atrophy 0.121 +/- 0.006, and controls 0.092 +/- 0.003.
Decreased levels of gamma aminobutyric acid (GABA) and its synthetic enzyme, glutamic acid decarboxylase, have been found in the brains of patients with Huntington disease. In an attempt to augment GABA-mediated neurotransmission, daily doses of 25 gm of L-glutamate (the substrate for glutamic acid decarboxylase) and 500 mg of pyridoxine, its cofactor, were given to five patients with Huntington disease. This regimen was continued for 2 years. Assessment of motor and behavioral function indicated no improvement on this regimen.
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