Striatal hypometabolism in premanifest and manifest Huntington’s disease patients

Abstract

F-FDG PET/CT might be a helpful tool to identify patterns of glucose metabolism in the striatum across the stages of HD and might be relevant in assessing the clinical status of gene-expanded HD carriers due to the fact that dysfunctional glucose metabolism begins at early preHD stages of the disease. F-FDG PET/CT appears as a promising method to monitor the response to disease-modifying therapies even if applied in premanifest subjects.

Spatial distribution of flow and oxygenation in the cerebral venous drainage system

“…The reliability of noninvasive regional venous oxygenation measurements shows promise for the investigation of disease states. For instance, metabolism in the deep gray matter of patients with Huntington's disease was shown to decrease in the presymptomatic stage, and time to disease onset could be predicted based on those measurements . However, for this invasive PET measurements are necessary, which cannot be repeated frequently due to the associated radiation exposure.…”

“…For instance, metabolism in the deep gray matter of patients with Huntington's disease was shown to decrease in the presymptomatic stage, and time to disease onset could be predicted based on those measurements. 8 However, for this invasive PET measurements are necessary, which cannot be repeated frequently due to the associated radiation exposure. In addition, further research is hampered due to the fact that invasive measurements are ethically more difficult to perform on healthy control subjects.…”

“…Knowledge of blood oxygenation in major venous branches is also of significant clinical value. In Huntington's disease (HD), decreased striatal metabolism, as evaluated with positron emission tomography (PET), was shown to occur even in the presymptomatic phase and to precede the loss of striatal volume and the onset of symptoms . Therefore, striatal venous oxygenation measurement may serve as a progression biomarker in HD and could be valuable for the development of disease‐modifying therapies.…”

“…In Huntington's disease (HD), decreased striatal metabolism, 7 as evaluated with positron emission tomography (PET), was shown to occur even in the presymptomatic phase and to precede the loss of striatal volume and the onset of symptoms. 8 Therefore, striatal venous oxygenation measurement may serve as a progression biomarker in HD and could be valuable for the development of disease-modifying therapies. In neonatal hypoxic-ischemic encephalopathy, brain metabolism was found to be decreased 9,10 and could thus be used to evaluate the effect of newly developed neuroprotective therapies.…”

Novel Imaging Biomarkers for Huntington’s Disease and Other Hereditary Choreas

“…Different PET imaging studies investigating the glucose metabolism in HD mutation carriers were able to define subcortical and cortical metabolic patterns across the entire disease spectrum of the neurodegenerative process with a progressive reduction of subcortical and cortical glucose metabolism [ 115 – 117 ]. The reduction in striatal metabolism is an early feature that can be observed in the premanifest phase of the disease (before the motor onset of the disease).…”