Various Diseases and Clinical Heterogeneity Are Associated With "Hot Cross Bun".

Shuzhen Zhu,Jialing Zheng,Qing Wang,Mengjue Yu,Ling-Ling Chan,Bin Deng,Zifeng Huang,Eng-King Tan,Yanran Liang,Zhibo Wen,Yanjun Huang,Hualing Li,Zihan Chang

doi:10.3389/fnagi.2020.592212

Abstract

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.

Highlights

Parkinson’s disease (PD) is the second common age-related neurodegenerative disease with the motor and non-motor dysfunctions following Alzheimer’s disease (AD) due to the progressive loss of dopaminergic neurons in the substantia nigra pars compacta (SNpc) (Qian and Huang, 2019; Xu et al, 2019; Beheshti et al, 2020; Yang et al, 2020)
We highlight for the first time inflammation (NMOSD, multiple sclerosis (MS), and acute disseminated encephalomyelitis (ADEM)) and toxic damage [toxic encephalopathy caused by phenytoin sodium (TEPS)] as new disease association with hot cross bun” sign (HCBs) (Figures 1, 3 and Table 1)
‘Hot cross bun’ sign (HCBS), “hot cross bun” sign; RIS, radiologic information system; MSA, multiple system atrophy; NMOSD, neuromyelitis optica spectrum disorders; MS, multiple sclerosis; ADEM, acute disseminated encephalomyelitis; BE, brainstem encephalitis; LCUC, lung cancer with undefined cause; BPI, bilateral middle cerebral peduncle infarction; SCA, spinocerebellar ataxia; TEPS, toxic encephalopathy caused by phenytoin sodium

Summary

Introduction

Parkinson’s disease (PD) is the second common age-related neurodegenerative disease with the motor and non-motor dysfunctions following Alzheimer’s disease (AD) due to the progressive loss of dopaminergic neurons in the substantia nigra pars compacta (SNpc) (Qian and Huang, 2019; Xu et al, 2019; Beheshti et al, 2020; Yang et al, 2020). Conventional magnetic resonance imaging (cMRI) is often used to aid the diagnosis of some neurodegenerative diseases such as dementia, spinocerebellar ataxia (SCA), and multiple system atrophy (MSA), but its sensitivity is usually relative low (Massey et al, 2012; Goldman et al, 2014; Ozaki et al, 2015, 2019; Filippi et al, 2017; Agosta et al, 2018; Shi et al, 2018; Mazzucchi et al, 2019; Yang et al, 2019). 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)

Methods

Results

Discussion

Conclusion