The Hot Cross Bun Sign: A Journey Across Etiologies

Abstract

When the hot cross bun (HCB) was created in the 12th century, little did its makers know that the buns’ rather innocuous existence would extend far beyond the realms of the culinary world and that it would play a significant role in the field of neurology. This sudden shot to neurological fame occurred in 1998 when Schrag et at,1 christened the imaging observation with the statement “Infratentorial signal changes had characteristic appearances. The pontine hyperintensity was cruciform resembling a ‘hot cross bun’” and suggested that this sign was specific to multiple system atrophy (MSA). In fact, the association is so well established that a general survey among neurologists regarding conditions associated with the HCB sign (HCBS) will probably always lead to the response of MSA-C and perhaps a spinocerebellar ataxia (SCA). However, it is time to cross out this specificity and understand that the HCB is in no way a sign specific to an etiology.2 The current issue features two articles which strongly reinforce the above concepts. A case of HCBS in ATX-ELOVL4 (SCA34) by Moreno-Escobar and Tripathi3 and the article by Naidoo et al4 which is a case series of HCBS in ATX-ATXN2 (SCA2) and ATX-ATXN7 (SCA7) accompanied by a highly comprehensive review of the HCBS...