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Spinocerebellar ataxia — SCE Neurology MCQ

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HardMovement DisordersSpinocerebellar ataxiaSCE Neurology

A 79-year-old woman presented with adult-onset progressive ataxia with autosomal dominant family history. On examination, there was dysarthria, gaze-evoked nystagmus and pyramidal signs. Initial investigations showed: MRI showed cerebellar atrophy and genetic panel identified CAG expansion. What is the most appropriate investigation?

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Correct answer: D — Clinical movement-disorder assessment with targeted DaTSCAN when uncertain

Clinical movement-disorder assessment with targeted DaTSCAN when uncertain is the best answer because the vignette describes Spinocerebellar ataxia: dominant cerebellar syndrome with CAG expansion indicates spinocerebellar ataxia. MRI brain with diffusion-weighted imaging is plausible in a neighbouring presentation, but the chronology, examination or investigation pattern does not match the key discriminator here. Carotid duplex ultrasound and EEG with sleep-deprived recording are less appropriate because they would require different localisation, timing or test findings; MR venography would fit a different syndrome. Clinical pearl: alcoholic cerebellar degeneration lacks Mendelian inheritance.

Reference: NICE NG71; Movement Disorder Society consensus criteria