Cortical atrophy more likely in those with motor onset in Huntington’s disease

The following article is part of the conference coverage of the virtual annual meeting of the International Congress on Parkinson’s and Movement Disorders (MDS). Neurology Advisor staff will share the latest news related to research from leading neurology experts. Look back for the latest news from the MDS 2021 Annual Virtual Meeting.

Cortical atrophy is more likely to occur in patients with Huntington’s disease (HD) with motor onset than in those who have a combination of motor and psychiatric symptoms in the first year of clinical onset, according to data presented at the International Parkinson’s Congress became the Virtual Congress 2021 of the Disease and Movement Disorders Society (MDS) from September 17th to 22nd, 2021.

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The main symptom in Huntington’s disease clinical onset can range from motor, psychiatric to motor and psychiatric symptoms. However, it is not clear why these phenotypic differences occur within this patient population.

The current study analyzed the MRI characteristics of the brain in patients with Huntington’s disease and the association with the main symptoms at the onset of the disease.

Huntington’s disease patients were enrolled in the study by the National Institute of Neurology and Neurosurgery in Mexico. Patients were screened for features of Huntington’s disease during the first year of onset of main symptoms, MRI features of the brain, and the size of their trinucleotide-cytosine-adenine-guanine (CAG) expansion determined by triple-prime polymerase Chain reaction (PCR) has been quantified. Patterns of disease progression based on the initial symptoms were elucidated.

A total of 68 patients with HD (57.4% women; mean age 49 ± 12.7 years) were included in the study, with HD diagnosed prior to 9.2 ± 4.6 years and a CAG size of 46.4 ± Was 5.1 repetitions in length.

The main symptoms at clinical onset were motor (32.4%; n = 22), psychiatric (30.9%; n = 21) or mixed motor and psychiatric (36.8%; n = 25).

During the comparative analysis, 69.1% (n = 47) of the patients presented with cortical atrophy. Stratified according to the status of cortical atrophy, patients with vs. without atrophy had a longer duration of HD (9.4 vs. 8.9 years; p <0.001), exhibited more motor symptoms alone than motor symptoms with psychiatric symptoms (90.4% vs. 56.0% and P = 0.02) and had shorter CAG expansions (45.8 vs 47.8 repetitions; P = 0.03).

The study authors emphasized that these results were inconsistent with previous studies that attributed the occurrence of motor symptoms to progression of atrophy from subcortical regions within the striatum to the cortical areas.

Additional research is needed to correlate features of MRI brain atrophy with overt symptoms within the first year of clinical onset in patients with HD.


Estrada-Rodriguez H, Garcia-Romero JD, Muñuzuri-Camacho MA, et al. Correlation of cortical atrophy on brain MRI with clinical onset of Huntington’s disease. Presented at: MDS Virtual Congress 2021; 17.-22. September 2021. Executive summary 228.

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