Cross-Sectional Area Analysis of the Head of the Caudate Nucleus in Huntington’s Disease

Abstract

Background: Caudate nucleus atrophy is a well-known neuroimaging feature of Huntington’s disease (HD). Some researchers have reported a decrease in the volume of the striatum on magnetic resonance images (MRIs) even in the presymptomatic stage of the disease. Despite the many neuroimaging studies on HD, the optimal method for measuring the caudate nucleus area on MRIs and the most effective cutoff values for diagnosing HD remain unclear. Objectives and Methods: To define suitable imaging sequences and cutoff values for HD, we measured the area of the head of the caudate nucleus (HCN) in 11 patients with HD, 22 age- and sex-matched individuals without neurodegenerative disorders in the central nervous system, 22 sex-matched patients with Alzheimer’s disease, 22 sex-matched patients with Parkinson’s disease, and 7 patients with dentatorubral-pallidoluysian atrophy. Results: On T2-weighted images (T2WIs), we found significantly reduced HCN area at the rostral level in individuals with HD relative to those of the individuals in the other groups. A significant inverse correlation (ρ = –0.61, p = 0.046) was observed between the HD duration and HCN area at the rostral slice level on T2WIs. The cutoff value for distinguishing patients with HD from healthy individuals and those with other neurodegenerative diseases was 85 mm² at the rostral level on T2WIs (100% sensitivity and specificity). Conclusions: This cutoff value can be applied clinically to evaluate brain atrophy in HD. Our method is advantageous because it is simple and can be implemented easily in daily clinical practice.

Diagnosis of Huntington’s disease via sequential 18F-DOPA and 18F-FDG PET/MRI

“…However, physiological volume of the caudate nucleus in both MR tomography and VBM, as seen in this patient, is a very uncommon finding in HD and stands in contrast to the most recent report of Furukawa et al, who reported a fundamental reduction in the head of the caudate nucleus in patients with HD compared to sex-and age-matched healthy individuals [6]. Possible reasons for those discrepancies include the small sample size and the considerable range of symptom duration prior to scanning in Furukawa's study, the atypical clinical symptoms of our patient, low amount of CAG repeats and relatively short duration of illness, resulting in preserved caudate nucleus volume.…”

Orofacial Dyskinesia in a Young Man

“…1 The prominence of caudate head atrophy indicated several extrapyramidal diseases, including Huntington disease, Huntington disease-like 2 (HDL-2), chorea-acanthocytosis (ChAc), and McLeod syndrome. [1][2][3] Although his low-density lipoprotein and apolipoprotein B levels decreased, his vitamin E levels were normal; thus, hypobetalipoproteinemia was unlikely. Acanthocytes are not present in Huntington disease, therefore it was excluded.…”

“…This led to the consideration of neuroacanthocytosis and inherited disorders of lipoprotein metabolism (eg, abetalipoproteinemia and hypobetalipoproteinemia); the latter leads to vitamin E malabsorption . The prominence of caudate head atrophy indicated several extrapyramidal diseases, including Huntington disease, Huntington disease–like 2 (HDL-2), chorea-acanthocytosis (ChAc), and McLeod syndrome . Although his low-density lipoprotein and apolipoprotein B levels decreased, his vitamin E levels were normal; thus, hypobetalipoproteinemia was unlikely.…”