Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy

Padmanabhan, Sandeep; Cherian, Ajith; Iype, Thomas; Mathew, Mini; Smitha, Sony

doi:10.4103/0972-2327.120479

Cited by 10 publications

(5 citation statements)

References 7 publications

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“…were analyzed (Muqit et al, 2001;Soares-Fernandes et al, 2009;Yadav et al, 2011;Brooks, 2012;Jain et al, 2013Jain et al, , 2014Padmanabhan et al, 2013;Pedroso et al, 2013;Roh et al, 2013;Goldman et al, 2014;Deguchi et al, 2015;Ozaki et al, 2015Ozaki et al, , 2019Pan et al, 2015;Ishikawa et al, 2016;Wang et al, 2016;Nagpal and Agarwal, 2017;Way et al, 2019;Carre et al, 2020), and 39 reporting cases were reviewed. For published cases resulting from the systematic review, we collected data on disease diagnosis, age, gender, disease duration, symptoms (parkinsonism or cerebellar ataxia or urination dysfunction), imaging features, treatment, and the response of the HCBs to treatment.…”

Section: Search Strategy Selection Criteria and Standard Patient Comentioning

confidence: 99%

“…Demographic and clinical characteristics for each case are listed in Table 1 (Muqit et al, 2001;Srivastava et al, 2005;Takao et al, 2007;Massano et al, 2008;Suresh Chandran et al, 2008;Soares-Fernandes et al, 2009;Yadav et al, 2011;Recio Bermejo et al, 2012;Gooneratne et al, 2013;Jain et al, 2013Jain et al, , 2014Padmanabhan et al, 2013;Pedroso et al, 2013;Roh et al, 2013;Zhang et al, 2013;Namekawa et al, 2015;Pan et al, 2015;Das et al, 2016;Ishikawa et al, 2016;Lin et al, 2016;Wang et al, 2016;Nagpal and Agarwal, 2017;Gan et al, 2018;Way et al, 2019); the published and newly screened MSA patients with HCBs in our center are listed in Supplementary Table 1.…”

Section: Demographic Characteristics and Clinical Phenotypes In Hcbs-mentioning

confidence: 99%

“…The signal is typically seen in MSA and SCA usually thought to result from the gliosis of pontocerebellar fibers (Gulati et al, 2007). However, recent evidence suggests that HCBs is not a specific sign for MSA and SCA, but it can be observed in other diseases along with various clinical phenotypes (Muqit et al, 2001;Soares-Fernandes et al, 2009;Yadav et al, 2011;Jain et al, 2013Jain et al, , 2014Padmanabhan et al, 2013;Pedroso et al, 2013;Roh et al, 2013;Zhang et al, 2013;Pan et al, 2015;Ishikawa et al, 2016;Wang et al, 2016). To our knowledge, few studies have systematically depicted the disease spectrums with HCBs, analyzed the clinical heterogeneity, and evaluated the disease progression and clinical outcomes (van Eimeren et al, 2019).…”

Section: Introductionmentioning

confidence: 99%

“…The main differential diagnoses of the HCBs-related diseases are depicted. Discriminating features are highlighted.seropositivity(Padmanabhan et al, 2013;Jain et al, 2014), variant Creutzfeldt-Jakob disease(Soares-Fernandes et al, 2009)], hereditary [hereditary cerebellar ataxia including SCA(Pedroso et al, 2013;Wang et al, 2016)], and stroke [bilateral pons infarction(Roh et al, 2013)]. Among these, MSA and SCA were the most common etiologies.…”

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confidence: 99%

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Various Diseases and Clinical Heterogeneity Are Associated With “Hot Cross Bun”

Zhu

Deng

et al. 2020

Front. Aging Neurosci.

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Objective: To characterize the clinical phenotypes associated with the “hot cross bun” sign (HCBs) on MRI and identify correlations between neuroimaging and clinical characteristics.Methods: Firstly, we screened a cohort of patients with HCBs from our radiologic information system (RIS) in our center. Secondly, we systematically reviewed published cases on HCBs and classified all these cases according to their etiologies. Finally, we characterized all HCBs cases in detail and classified the disease spectra and their clinical heterogeneity.Results: Out of a total of 3,546 patients who were screened, we identified 40 patients with HCBs imaging sign in our cohort; systemic literature review identified 39 cases, which were associated with 14 diseases. In our cohort, inflammation [neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS), and acute disseminated encephalomyelitis (ADEM)] and toxicants [toxic encephalopathy caused by phenytoin sodium (TEPS)] were some of the underlying etiologies. Published cases by systemic literature review were linked to metabolic abnormality, degeneration, neoplasm, infection, and stroke. We demonstrated that the clinical phenotype, neuroimaging characteristics, and HCBs response to therapy varied greatly depending on underlying etiologies.Conclusion: This is the first to report HCBs spectra in inflammatory and toxication diseases. Our study and systemic literature review demonstrated that the underpinning disease spectrum may be broader than previously recognized.

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Section: Search Strategy Selection Criteria and Standard Patient Comentioning

confidence: 99%

Section: Demographic Characteristics and Clinical Phenotypes In Hcbs-mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

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Various Diseases and Clinical Heterogeneity Are Associated With “Hot Cross Bun”

Zhu

Deng

et al. 2020

Front. Aging Neurosci.

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“…Hot cross bun sign, a cruciate hyperintensity in the pons, best seen on axial T2-weighted and FLAIR sequences of MRI has been described.This sign is classically described in degenerative diseases like multiple system atrophy (MSA) , Spinocerebellar Ataxia 2 and 3 , variant Creutzfeldt-Jacob disease and rarely with infectious diseases of the CNS. Treatment is by anti retroviral therapy [ 46 ].…”

Section: Infectious and Toxin Mediated Causes Of Treatable Ataxiasmentioning

confidence: 99%

Treatable cerebellar ataxias

Kajaria

Kishore

2020

Clinical Parkinsonism & Related Disorders

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Cerebellar ataxic syndrome is a heterogenous class of disorders which can result from a miscellany of causes- genetic or acquired. There are a few metabolic, immune mediated, inflammatory and hereditary causes of ataxia which can be diagnosed from the gamut of possibilities, offering great relief to the ailing patient, their family and the treating physician. A pragmatic algorithm for diagnosing treatable causes of ataxia includes a thorough clinical history, meticulous examination for associated signs and an investigative mind to clinch the diagnosis. With novel diagnostic techniques and targeted therapies, early diagnosis and treatment can lead to favourable outcomes. In this review, diseases presenting predominantly as cerebellar ataxia and are treatable by targeted therapies are discussed.

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The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature

Naidoo

Wells

Rugbeer

et al. 2022

Movement Disord Clin Pract

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Background The “hot cross bun” sign is a cruciform hyperintensity is seen on T2 weighted imaging within the pons. The sign is considered to be pathognomic for Multiple system atrophy type C. The clinical and radiological features of Multiple system atrophy type C overlap with the autosomal dominant inherited ataxias. We present a case series of 3 African patients with genetically proven Spinocerebellar Ataxia presenting with the Hot cross bun sign and a scoping review of similar studies. Cases We described the phenotypic and radiological presentation of genetically confirmed SCA‐2 in two, and SCA‐7 in one patient, with the “hot cross bun” sign. Literature Review We performed a scoping review on the Hot Cross Bun Sign. A total of 66 articles were retrieved. We describe the diverse aetiologies of the sign and associated phenotypic and radiological features. We review the Spinocerebellar Ataxias described with a Hot cross bun sign and make comparisons to Multiple System Atrophy Type C [Ref. 1,2]. Conclusions To our knowledge this is the first description of an African cohort presenting with the Hot Cross Bun Sign. We expand the differential diagnosis of the Hot Cross Bun Sign.

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Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy

Cited by 10 publications

References 7 publications

Various Diseases and Clinical Heterogeneity Are Associated With “Hot Cross Bun”

Various Diseases and Clinical Heterogeneity Are Associated With “Hot Cross Bun”

Treatable cerebellar ataxias

The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature

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