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Acute generalised chorea can be attributed to multiple causes, including non-ketotic hyperglycaemia. This cause has been associated with characteristic image signs of striatal hyperdensity on CT scan and hyperintensity on T1 weighted (T1W) MRI.
We report a patient presenting with this syndrome in which a postmortem study was conducted. The findings are discussed together with the neuropathological data available in the literature, contributing towards an explanation of the nature of the imaging signs that has remained elusive.
A 73-year-old woman was admitted to our neurological department for acute generalised chorea of 8 days’ duration. There was no relevant personal background or family history.
On admission, the patient presented with orofacial dyskinesias and choreic movements in the neck, trunk, upper and lower limbs. The aetiological diagnostic work-up for acute chorea revealed severe hyperglycaemia on admission (>27.8 mmol/l), bicytopenia with anaemia (erythrocyte count 2.8×106/mm3, haemoglobin 8.1 g/dl) and thrombocytopenia (104 000/μl), and an isolated antiphospholipid antibody positive titre. The remaining investigation for acute chorea was normal. The imaging studies revealed …