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LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 2  |  Page : 293-294

"Hot cross bun" sign in HIV-related progressive multifocal leukoencephalopathy


1 Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India
2 Department of Neuroradiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India

Date of Submission10-Apr-2010
Date of Decision10-Apr-2010
Date of Acceptance22-Jun-2010
Date of Web Publication7-Apr-2011

Correspondence Address:
Ravi Yadav
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.79149

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How to cite this article:
Yadav R, Ramdas M, Karthik N, Kulkarni GB, Dawn R, Kumar M V, Nagaraja D. "Hot cross bun" sign in HIV-related progressive multifocal leukoencephalopathy. Neurol India 2011;59:293-4

How to cite this URL:
Yadav R, Ramdas M, Karthik N, Kulkarni GB, Dawn R, Kumar M V, Nagaraja D. "Hot cross bun" sign in HIV-related progressive multifocal leukoencephalopathy. Neurol India [serial online] 2011 [cited 2019 Oct 22];59:293-4. Available from: http://www.neurologyindia.com/text.asp?2011/59/2/293/79149


Sir,

Hot cross bun (HCB) sign is a cruciform 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 cerebellar type of multiple system atrophy (MSA-c). However, this sign has also been described in other conditions. [1],[2],[3],[4],[5] We report here, HCB in 2 patients of human immunodeficiency virus (HIV)-related progressive multifocal leukoencephalopathy (PML).

A 29-year-old woman and a 32-year-old man presented to us with asymmetrical cerebellar syndrome with pyramidal and bulbar dysfunction of subacute onset of 2 and 3 months duration, respectively. Cerebrospinal fluid (CSF) analysis showed elevated proteins, 600 mg/dL and 550 mg/dL, respectively, and positive polymerase chain reaction (PCR) for JC virus. HIV serology was positive in both the patients with a CD4+ T-cell count of 136/μL and 156/μL, respectively.

MRI of brain, multiplanar T1, T2, FLAIR, diffusion, and postcontrast imaging done on 3T MRI system showed the classical HCB sign in both the patients [Figure 1] and [Figure 2]. Magnetic resonance spectroscopy (MRS) revealed gross reduction of N-acetyl aspartic acid (NAA) with NAA/creatine (Cr) ratio of 0.33 and 0.56 in the cerebellar hemisphere bilaterally. Choline (Cho) was mildly elevated with Cho/Cr ratio of approximately 1.1 and 1.5, respectively.
Figure 1: FLAIR (a), Axial T2 (b), and contrast T1 axial (c) MRI images of the patient showing cross-shaped T2 signal hyperintensity within the pons ("hot cross bun" sign). No enhancement was identifiable in contrast scan

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Figure 2: The lesion appears hyperintense on FLAIR (a) and T2-weighted images (b). It is hypointense and nonenhancing on postcontrast T1-weighted images (c). "Hot cross bun" is evident in all the images

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HIV-related PML presents with usually clinical features of limb weakness, cognitive deficits, speech or visual deficits, ataxia, and less commonly with seizures and headache. [6] The chances of development of HIV-related PML correlates with low CD4 count. [7] Both our patients had a presentation with predominantly asymmetrical cerebellar ataxia, speech deficits, limb weakness, low CD4 count (less than 200/μL) and nonenhancing lesions in MRI scan. They were detected to be positive for JC virus PCR, thus confirming the diagnosis of HIV-related PML. Usually, PML affects periventricular and subcortical frontal and parieto-occipital white matter followed by brainstem, cerebellum, thalamus, basal ganglia, corpus callosum, and rarely cervical and thoracic spinal cord. [7] MRS supported the PML diagnosis as the lesions showed a decreased NAA and Cr content, whereas choline-containing substances (Cho) and lactate were elevated. [7] Our patients had asymmetrical involvement of posterior fossa structures (cerebellum, pons, and midbrain) with signal change and atrophy with the HCB sign. In MSA-c, the degeneration of pontine neurons and loss of myelinated transverse pontocerebellar fibers with preserved tegmentum and corticospinal tracts has been cited as the main cause for this sign. [1] It was proposed that HCB sign is due to gliosis in addition to the loss of neurons. [2] HCB sign has also been observed in spinocerebellar ataxia (SCA) type 2 and 3. In a study of T2-weighted axial MRIs from 138 SCA patients, an overall frequency of HCB sign was 8.7%. Of these, 25.7% were SCA 2 and 1.3% were SCA 3. [3] The sign has also been described in a variant of Creutzfeldt Jacob disease and in a case of severe parkinsonian syndrome associated with cerebellar and brain stem dysfunction due to presumed vasculitis. [4],[5] We consider the HCB sign in our patients was also an end result of dying back phenomenon in the axons of various cerebellar connections along with degeneration of pontine neurons and pontocerebellar fibers with gliosis over months. Thus we conclude that PML is also one of the rare causes of HCB sign.

 
 » 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
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2.Takao M, Kadowaki T, Tomita Y, Yoshida Y, Mihara B. 'Hot-cross bun sign' of multiple system atrophy. Intern Med 2007;46:1883.   Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
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
[PUBMED]  [FULLTEXT]  
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
[PUBMED]  [FULLTEXT]  
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
[PUBMED]  [FULLTEXT]  
6.Berenguer J, Miralles P, Arrizabalaga J, Ribera E, Dronda F, Baraia-Etxaburu J, et al. Clinical course and prognostic factors of progressive multifocal leukoencephalopathy in patients treated with highly active antiretroviral therapy. Clin Infect Dis 2003;36:1047-52.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Post MJ, Yiannoutsos C, Simpson D, Booss J, Clifford DB, Cohen B, et al. Progressive multifocal leukoencephalopathy in AIDS: are there any MR findings useful to patient management and predictive of patient survival? AIDS Clinical Trials Group, 243Team. AJNR Am J Neuroradiol 1999;20:1896-906.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  


    Figures

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