We report a patient with cerebrotendinous xanthomatosis (CTX) presenting with dementia, spastic tetraparesis and an unreported akinetic-rigid syndrome. Computed tomography (CT) showed only cerebellar abnormalities while magnetic resonance imaging (MRI) detected additional pallidal and mesencephalic focal alterations. MRI findings, but not CT, correlated with the clinical picture.

Cerebrotendinous xanthomatosis is one of the rare forms of treatable hereditary neurodegenerative disorders. It is due to a defect in hydroxylation of cholesterol side chain that impairs oxidative cleavage of cholesterol leading to excess accumulation of cholesterol. Here we present such a case which presented to us with recurrent generalized tonic clonic seizures. He is under treatment for the...

The authors reanalyzed data from Scherer and Wallbott's (Scherer, 1997b; Scherer & Wallbott, 1994) International Study of Emotion Antecedents and Reactions to examine how phenomenological reports of emotional experience, expression, and physiological sensations were related to each other within cultures and to determine if these relationships were moderated by cultural differences, which were o...