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‘Hot‑cross bun’ and ‘inverse trident sign’ in progressive multifocal leukoencephalopathy with HIV seropositivity Rajendra S. Jain, Kadam Nagpal, Shankar Tejwani Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India A 42‑year‑old male human immunodeficiency virus (HIV) positive, presented with 2‑month history of subacute onset, progressive ataxia of gait and limbs, dysarthria and dysphagia for both solids and liquids with nasal regurgitation. Otherwise history was negative. There was history of multiple sexual contacts in the past. On examination, patient was hemodynamically stable, conscious and well oriented. Neurological examination revealed mini‑mental state examination score of 24/30, bilateral gaze evoked nystagmus, diminished palatal movements with absent gag reflex on right side, normal tone and motor power, brisk deep tendon reflexes, and bilateral cerebellar signs (gait and limb ataxia). CD‑4 cell count was 160 cells/µl. Cerebrospinal fluid examination revealed normal cell count, elevated protein (100 mg/dl) and positive cerebrospinal fluid‑polymerase chain reaction (CSF‑ PCR) for JC virus. Tests for other opportunistic infections were negative. Magnetic resonance imaging (MRI) brain showed an ‘inverse trident sign’ in upper pons and a ‘hot‑cross bun’ sign in middle and lower pons [Figure 1]. In addition, there were associated asymmetric T2‑weighted hyperintensities in middle and inferior cerebellar peduncles and bilateral parieto‑occipital regions without any contrast enhancement [Figure 2]. MR spectroscopy showed a reduced N‑acetyl aspartate (NAA) and slightly elevated choline/creatine ratio [Figure 3].
Hot‑cross bun sign is frequently seen in cerebellar type of multiple system atrophy (MSA) and spinocerebellar ataxia (SCA), due to selective loss of myelinated transverse pontocerebellar fibers and neurons in pontine raphe with preservation of pontine tegmentum and corticospinal tracts. Other clinical conditions where hot‑cross bun sign is rarely reported include progressive multifocal leukoencephalopathy (PML) in HIV seropositive, variant‑Creutzfeld-Jakob disease (CJD), secondary Parkinsonism and cerebrotendinous xanthomatosis. [1] Trident sign has been reported in Wilson’s disease and central pontine myelinolysis (CPM), but never in PML to the best of our knowledge. The plausible mechanism for a ‘trident sign’ has been preferential involvement of transverse pontine fibers.[2] We postulate similar mechanism for an ‘inverse trident sign’ as involvement of transverse pontine fibers is clearly evident. Moreover, our patient had predominant involvement of posterior fossa structures similar to the findings reported by others. [3,4] No specific treatment is available for PML in HIV patients currently; however, early administration of highly active anti‑retroviral therapy (HAART) is recommended to prevent this dreaded complication.
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Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.137032
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Figure 1: (a) MRI brain T2 axial section showing hyperintense signals predominantly involving transverse pontine fibres at upper pons resulting in an ‘Inverse trident’ sign (b) MRI brain T2 axial section showing ‘Hot-cross bun’ sign at lower pons and asymmetrical hyperintense signals in left inferior cerebellar peduncle
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Jain, et al.: Hot cross bun and inverse trident sign in HIV-PML
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Figure 2: (a and b) MRI brain FLAIR images showing hyperintensities in pons and middle cerebellar peduncles with encephalomalacia (c) Post contrast MR images showing no contrast enhancement. (d) MRI brainFLAIR images showing asymmetrical parieto-occipital hyperintensities
Figure 3: MR spectroscopy showing reduced NAA and slightly elevated choline/creatine ratio
Address for correspondence:
3.
Dr. Rajendra Singh Jain,126, Janakpuri II, Imli Ka Phatak, Jaipur ‑ 302 005, Rajasthan, India. E‑mail: [email protected]
4.
References 1. 2.
342
Jain RS, Sannegowda RB, Agrawal A, Hemrajani D, Jain R, Mathur T. ‘Hot cross bun’ sign in a case of cerebrotendinous xanthomatosis: A rare neuroimaging observation. BMJ Case Rep 2013;2013. Biotti D, Durupt S. A trident in the brain, central pontine myelinolysis. Pract Neurol 2009;9:231‑2.
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. 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 article: Jain RS, Nagpal K, Tejwani S. 'Hotcross bun' and 'inverse trident sign' in progressive multifocal leukoencephalopathy with HIV seropositivity. Neurol India 2014;62:341-2. Source of Support: Nil, Conflict of Interest: None declared.
Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
‘Hot‑cross bun’ and ‘inverse trident sign’ in progressive multifocal leukoencephalopathy with HIV seropositivity Rajendra S. Jain, Kadam Nagpal, Shankar Tejwani Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India A 42‑year‑old male human immunodeficiency virus (HIV) positive, presented with 2‑month history of subacute onset, progressive ataxia of gait and limbs, dysarthria and dysphagia for both solids and liquids with nasal regurgitation. Otherwise history was negative. There was history of multiple sexual contacts in the past. On examination, patient was hemodynamically stable, conscious and well oriented. Neurological examination revealed mini‑mental state examination score of 24/30, bilateral gaze evoked nystagmus, diminished palatal movements with absent gag reflex on right side, normal tone and motor power, brisk deep tendon reflexes, and bilateral cerebellar signs (gait and limb ataxia). CD‑4 cell count was 160 cells/µl. Cerebrospinal fluid examination revealed normal cell count, elevated protein (100 mg/dl) and positive cerebrospinal fluid‑polymerase chain reaction (CSF‑ PCR) for JC virus. Tests for other opportunistic infections were negative. Magnetic resonance imaging (MRI) brain showed an ‘inverse trident sign’ in upper pons and a ‘hot‑cross bun’ sign in middle and lower pons [Figure 1]. In addition, there were associated asymmetric T2‑weighted hyperintensities in middle and inferior cerebellar peduncles and bilateral parieto‑occipital regions without any contrast enhancement [Figure 2]. MR spectroscopy showed a reduced N‑acetyl aspartate (NAA) and slightly elevated choline/creatine ratio [Figure 3].
Hot‑cross bun sign is frequently seen in cerebellar type of multiple system atrophy (MSA) and spinocerebellar ataxia (SCA), due to selective loss of myelinated transverse pontocerebellar fibers and neurons in pontine raphe with preservation of pontine tegmentum and corticospinal tracts. Other clinical conditions where hot‑cross bun sign is rarely reported include progressive multifocal leukoencephalopathy (PML) in HIV seropositive, variant‑Creutzfeld-Jakob disease (CJD), secondary Parkinsonism and cerebrotendinous xanthomatosis. [1] Trident sign has been reported in Wilson’s disease and central pontine myelinolysis (CPM), but never in PML to the best of our knowledge. The plausible mechanism for a ‘trident sign’ has been preferential involvement of transverse pontine fibers.[2] We postulate similar mechanism for an ‘inverse trident sign’ as involvement of transverse pontine fibers is clearly evident. Moreover, our patient had predominant involvement of posterior fossa structures similar to the findings reported by others. [3,4] No specific treatment is available for PML in HIV patients currently; however, early administration of highly active anti‑retroviral therapy (HAART) is recommended to prevent this dreaded complication.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.137032
Neurology India | May-Jun 2014 | Vol 62 | Issue 3
a
b
Figure 1: (a) MRI brain T2 axial section showing hyperintense signals predominantly involving transverse pontine fibres at upper pons resulting in an ‘Inverse trident’ sign (b) MRI brain T2 axial section showing ‘Hot-cross bun’ sign at lower pons and asymmetrical hyperintense signals in left inferior cerebellar peduncle
341
Jain, et al.: Hot cross bun and inverse trident sign in HIV-PML
a
b
c
d
Figure 2: (a and b) MRI brain FLAIR images showing hyperintensities in pons and middle cerebellar peduncles with encephalomalacia (c) Post contrast MR images showing no contrast enhancement. (d) MRI brainFLAIR images showing asymmetrical parieto-occipital hyperintensities
Figure 3: MR spectroscopy showing reduced NAA and slightly elevated choline/creatine ratio
Address for correspondence:
3.
Dr. Rajendra Singh Jain,126, Janakpuri II, Imli Ka Phatak, Jaipur ‑ 302 005, Rajasthan, India. E‑mail: [email protected]
4.
References 1. 2.
342
Jain RS, Sannegowda RB, Agrawal A, Hemrajani D, Jain R, Mathur T. ‘Hot cross bun’ sign in a case of cerebrotendinous xanthomatosis: A rare neuroimaging observation. BMJ Case Rep 2013;2013. Biotti D, Durupt S. A trident in the brain, central pontine myelinolysis. Pract Neurol 2009;9:231‑2.
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. 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 article: Jain RS, Nagpal K, Tejwani S. 'Hotcross bun' and 'inverse trident sign' in progressive multifocal leukoencephalopathy with HIV seropositivity. Neurol India 2014;62:341-2. Source of Support: Nil, Conflict of Interest: None declared.
Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
