Huntington's disease

Case contributed by A.Prof Frank Gaillard

Presentation

Gene positive for Huntington's disease. Early symptoms.

Patient Data

Age: 55 years
Gender: Female
MRI

The caudate nuclei and the putamen, are volume-depleted as demonstrated on segmented volumetric scans as well as the with a frontal horn to intercaudate ratio of 1.57 (normal 2.2-2.6), and intercaudate to inner table ratio 0.19 (normal 0.09-0.12).

The remainder of the brain is unremarkable in appearance. No intra-axial or extra-axial collection, mass or focal abnormality. No chronic small vessel ischaemic change or susceptibility induced signal loss.

Conclusion: Volume loss of the caudate nuclei and putamen is consistent Huntington's disease.

Case Discussion

I'm not generally a big believer in measurements, however, in Huntington's disease, the frontal horn to intercaudate ratio (FH:CC) and intercaudate to inner table ratio (CC:IT) are useful. 

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Case information

rID: 41811
Case created: 21st Dec 2015
Last edited: 22nd May 2017
Inclusion in quiz mode: Excluded

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