Huntington disease

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Abnormal movements for 5 years.

Patient Data

Age: 57
Gender: Female
mri

The caudate nuclei are atrophied with a mildly widened inter caudate distance (21 mm). The intercaudate distance to inner table ratio (CC:IT) is increased (0.185; N = 0.09-0.12) and frontal horn width to intercaudate ratio (FH:CC) is low (1.6; N = 2.2-2.6). There is associated putamen atrophy. The sulci are prominant for age, particularly in the frontal lobes.

Conclusion:

Findings are consistent with the patient's known Huntington's disease.

Case Discussion

This patient is has been tested and is gene positive for Huntington disease. This case also highlights the utility, in some settings, of measurements. Merely 'eye balling' the caudate heads does not clearly show them to be abnormal, when in fact the CC:IT and FH:CC ratios are quite abnormal. 

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