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IMAGES IN NEUROLOGY
Year : 2013  |  Volume : 16  |  Issue : 4  |  Page : 672-673
 

Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy


1 Department of Neurology, Government Medical College, Trivandrum, Kerala, India
2 Consultant Ophthalmologist, Rose Eye Clinic, Vellayambalam, Trivandrum, Kerala, India

Date of Submission10-May-2013
Date of Decision23-Jun-2013
Date of Acceptance05-Jul-2013
Date of Web Publication25-Oct-2013

Correspondence Address:
Ajith Cherian
Department of Neurology, Government Medical College, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.120479

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How to cite this article:
Padmanabhan S, Cherian A, Iype T, Mathew M, Smitha S. Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy. Ann Indian Acad Neurol 2013;16:672-3

How to cite this URL:
Padmanabhan S, Cherian A, Iype T, Mathew M, Smitha S. Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy. Ann Indian Acad Neurol [serial online] 2013 [cited 2020 Jan 27];16:672-3. Available from: http://www.annalsofian.org/text.asp?2013/16/4/672/120479



   Introduction Top


Hot cross bun (HCB) sign is a cruciate hyperintensity in the pons best seen on axial T2-weighted and fluid attenuation inversion recovery (FLAIR) sequences of magnetic resonance imaging (MRI) of the brain. This sign is classically described in degenerative diseases like multiple system atrophy (MSA) and rarely with infectious diseases of the central nervous system. We report here, HCB sign in a patient of human immunodeficiency virus (HIV)-related progressive multifocal leukoencephalopathy (PML).


   Case Top


A 37-year-old lady presented to us with subacute onset progressive ataxia, with tendency to sway toward the left, of 2 month duration. She had significant weight loss, anorexia, and recurrent fevers for 6 months. She was emaciated, had oral candidiasis, and hepatomegaly. Neurological examination revealed left gaze evoked nystagmus, incoordination involving the left extremities, and slurred speech. HIV serology done was positive with a CD4 count of 28/μL. Cerebrospinal fluid study revealed normal opening pressure, mild elevation of protein (65 mg%) with no cells and normal glucose.

MRI of the brain multiplanar T1, T2, FLAIR, diffusion and postcontrast done on a 1.5 Tesla system (Avanto-SQ Engine, Siemens Medical Systems, Erlanger, Germany) revealed the classical HCB sign in pons. In addition, there were asymmetrical T2 hyperintense lesions involving the left middle and inferior cerebellar peducle extending into left cerebellar hemisphere with atrophy of the same structures [Figure 1]. The left cerebellar hemispheric hyperintensities were partially suppressible on FLAIR and not enhancing on contrast [Figure 2]. Magnetic resonance spectroscopy revealed reduction of N-Acetylaspartic acid peak with mild elevation of choline. Imaging features suggested a possible necrotic demyelinating process asymmetrically, involving the left cerebellar white matter and transverse pontine fibers consistent with PML.
Figure 1: Axial T2 (a) and fluid attenuation inversion recovery (b) showing "hot cross bun" appearance with cruciate T2 hyperintensity in the pons (arrow). Postcontrast T1 axial (c) showing no enhancement. Inset shows top view of hot cross bun

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Figure 2: Axial T2 (a) showing asymmetrical T2 hyperintensity involving the middle cerebellar peduncle extending to the left cerebellar hemisphere, partially suppressed on fluid attenuation inversion recovery (b) with no contrast enhancement (c) Note the asymmetric atrophy involving the left cerebellar hemisphere

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   Discussion Top


The cruciform hyperintensity seen in pons resembles a HCB baked for the last Thursday before Easter and hence called so. The sign is due to a selective loss of myelinated transverse pontocerebellar fibers and neurons in the pontine raphe with preservation of the pontine tegmentum and corticospinal tracts. [1] It is most often seen in cerebellar type of MSA. [2] HCB sign has also been reported in spinocerebellar ataxia (SCA) and variant  Creutzfeldt-Jakob disease More Details. [3],[4] Muqit et al., [5] in 2001 described a patient with  Parkinsonism More Details due to presumed vasculitis with HCB sign on MRI. HCB sign has been reported in two patients with HIV-related PML from India by Yadav et al.,[6] [Table 1]. The HCB appearance in PML is probably due to the damage and subsequent gliosis of the cerebellar connections and pontocerebellar fibers in the pons due to viral infection [Table 2] (Lists imaging differentiating features of PML from MSA and SCA). To date, ours is the world's second report of a patient with HIV-related PML with HCB sign on MRI.
Table 1: Conditions where ''hot cross bun sign'' in pons is described in literature

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Table 2: Points to differentiate progressive multifocal leukoencephalopathy from multiple system atrophy and spinocerebellar ataxias (SCA 2 and 3) on magnetic resonance imaging despite the presence of "Hot cross bun" sign

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[7]

 
   References Top

1.Schrag A, Kingsley D, Phatouros C, Mathias CJ, Lees AJ, Daniel SE, et al. Clinical usefulness of magnetic resonance imaging in multiple system atrophy. J Neurol Neurosurg Psychiatry 1998;65:65-71.  Back to cited text no. 1
    
2.Savoiardo M, Strada L, Girotti F, Zimmerman RA, Grisoli M, Testa D, et al. Olivopontocerebellar atrophy: MR diagnosis and relationship to multiple system atrophy. Radiology 1990;174:693-6.  Back to cited text no. 2
    
3.Lee 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.  Back to cited text no. 3
    
4.Soares-Fernandes JP, Ribeiro M, Machado A. "Hot cross bun" sign in variant Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol 2009;30:E37.  Back to cited text no. 4
    
5.Muqit MM, Mort D, Miskiel KA, Shakir RA. "Hot cross bun" sign in a patient with parkinsonism secondary to presumed vasculitis. J Neurol Neurosurg Psychiatry 2001;71:565-6.  Back to cited text no. 5
    
6.Yadav R, Ramdas M, Karthik N, Kulkarni GB, Dawn R, Kumar MV, et al. "Hot cross bun" sign in HIV-related progressive multifocal leukoencephalopathy. Neurol India 2011;59:293-4.  Back to cited text no. 6
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7.Klockgether T, Skalej M, Wedekind D, Luft AR, Welte D, Schulz JB, et al. Autosomal dominant cerebellar ataxia type I. MRI-based volumetry of posterior fossa structures and basal ganglia in spinocerebellar ataxia types 1, 2 and 3. Brain 1998;121:1687-93.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]


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