Annals of Indian Academy of Neurology
IMAGES IN NEUROLOGY
Year
: 2013  |  Volume : 16  |  Issue : 3  |  Page : 406-

Hot cross bun sign in a patient with cerebellar ataxia


Inuka Kishara Gooneratne, Manjula Chandragomi Caldera, Sujith Priyankara Perera, Ranjanie Gamage 
 Institute of Neurology, National Hospital of Sri Lanka, Sri Lanka

Correspondence Address:
Inuka Kishara Gooneratne
10/1 Borella Cross Road, Colombo 8
Sri Lanka




How to cite this article:
Gooneratne IK, Caldera MC, Perera SP, Gamage R. Hot cross bun sign in a patient with cerebellar ataxia.Ann Indian Acad Neurol 2013;16:406-406


How to cite this URL:
Gooneratne IK, Caldera MC, Perera SP, Gamage R. Hot cross bun sign in a patient with cerebellar ataxia. Ann Indian Acad Neurol [serial online] 2013 [cited 2019 Sep 18 ];16:406-406
Available from: http://www.annalsofian.org/text.asp?2013/16/3/406/116973


Full Text

A 20-year-old man presented with slowly progressive coordination problems in his trunk and legs for the last 6 years. Examination showed limb, truncal, and gait ataxia. There was impaired deep sensation, proprioceptive deficit, and areflexia of the legs.

T2-weighted Magnetic Resonance Images of the brain showed the "hot cross bun" sign at the level of the pons. A DNA analysis showed increased CAG trinucleotide repeats in the spinocerebellar ataxia (SCA-2) gene on chromosome 12q24.1.

 Commentary



The hot cross bun sign has mostly been described in patients with multiple system atrophy of the cerebellar type called olivo-ponto-cerebellar atrophy. This finding is related to degeneration of the transverse ponto-cerebellar fibers at the base of the pons, the middle part of the formatio reticularis and the ponto-cerebellar fibers between the lemniscus medialis and the tractus pyramidalis. [1] This case shows that the hot cross bun sign can also be seen in other neurodegenerative cerebellar disorders. [1],[2]

SCA is a clinically, pathologically, and genetically heterogeneous group of dominantly inherited neurodegenerative disorders characterized by adult-onset progressive cerebellar ataxia. [3] A recent study suggested an overall prevalence of hot cross bun sign in SCA patients to be 8.7%. [2] The majority of patients with the hot cross bun sign were of the SCA2 subtype. A minority of patients with SCA3 were also positive for the hot cross bun sign. [2] In another study in Western India, a single patient with SCA6 was also positive for the hot cross bun sign. [4] Thus the hot cross bun sign may be present in a spectrum of SCA subtypes.[Figure 1]{Figure 1}

References

1Marrannes J, Mulleners E. Hot cross bun sign in a patient with SCA-2. JBR-BTR 2009;92:263.
2Lee YC, Liu CS, Wu HM, Wang PS, Chang MH, Soong BW. The 'hot cross bun' sign in the patients with spinocerebellar ataxia. Eur J Neurol 2009;16:513-6.
3Soong BW, Paulson HL. Spinocerebellar ataxias: An update. Curr Opin Neurol 2007;20:438-46.
4Khadilkar SV, Dabi R, Dhonde P, Nadkarni N, Kulkarni S, Sarnath D. Trinucleotide repeat spinocerebellar ataxias: experience of a tertiary care centre in Western India with review of Indian literature. Neurol Asia 2012;17:213-7.