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No patient had past or existing signs and symptoms of erythrokeratodermia variabilis, which had previously already been reported. MRI unveiled cerebellar atrophy, with pontine atrophy (4 of 6 customers), and cruciform hypersignal when you look at the pons (2 of 6 patients). Fluorodeoxyglucose-PET showed diffuse cerebellar hypometabolism in most 5 tested customers with refined parietal hypometabolism in 3. Significant cognitive deficits were present in executive perf
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