Literature DB >> 23417372

'Hot cross bun' sign in a case of cerebrotendinous xanthomatosis: a rare neuroimaging observation.

Rajendra Singh Jain1, Raghavendra Bakki Sannegowda, Amit Agrawal, Deepika Hemrajani, Rahul Jain, Tarun Mathur.   

Abstract

We report a 25-year-old young man presenting with cognitive decline, pancerebellar features, spastic quadriparesis, bilateral cataract (operated) and tendo-Achilles swelling (xanthoma). The CT of the head showed bilateral cerebellar hypodensities. There were bilateral cerebellar hypointensities involving dentate nuclei on T1-weighted images with corresponding hyperintensities on T2-weighted MRI. Additionally, an interesting MRI finding-'hot cross bun' appearance was seen in pons which has not been reported in the literature so far. Biopsy from tendo-Achilles confirmed xanthoma. He was treated with chenodeoxycholic acid following which he showed improvement in cognition and weakness.

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Mesh：

Year:  2013        PMID： 23417372      PMCID： PMC3604450          DOI： 10.1136/bcr-2012-006641

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN： 1757-790X


  10 in total

1.  "Hot cross bun" sign in a patient with parkinsonism secondary to presumed vasculitis.

Authors:  M M Muqit; D Mort; K A Miskiel; R A Shakir
Journal:  J Neurol Neurosurg Psychiatry       Date:  2001-10       Impact factor: 10.154

2.  "Hot cross bun" sign in variant Creutzfeldt-Jakob disease.

Authors:  J P Soares-Fernandes; M Ribeiro; A Machado
Journal:  AJNR Am J Neuroradiol       Date:  2009-03       Impact factor: 3.825

3.  Cerebrotendinous cholestanolosis in relation to other cerebral xanthomatoses.

Authors:  J G de Jong; C M van Gent; J W Delleman
Journal:  Clin Neurol Neurosurg       Date:  1977       Impact factor: 1.876

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

Authors:  Ravi Yadav; Mahendra Ramdas; N Karthik; Girish Baburao Kulkarni; Rose Dawn; M Veerendra Kumar; D Nagaraja
Journal:  Neurol India       Date:  2011 Mar-Apr       Impact factor: 2.117

5.  Cerebrotendinous xanthomatosis without tendon xanthomas mimicking Marinesco-Sjoegren syndrome: a case report.

Authors:  H R Siebner; S Berndt; B Conrad
Journal:  J Neurol Neurosurg Psychiatry       Date:  1996-05       Impact factor: 10.154

6.  Increased concentrations of cholestanol and apolipoprotein B in the cerebrospinal fluid of patients with cerebrotendinous xanthomatosis. Effect of chenodeoxycholic acid.

Authors:  G Salen; V Berginer; V Shore; I Horak; E Horak; G S Tint; S Shefer
Journal:  N Engl J Med       Date:  1987-05-14       Impact factor: 91.245

7.  The 'hot cross bun' sign in the patients with spinocerebellar ataxia.

Authors:  Y-C Lee; C-S Liu; H-M Wu; P-S Wang; M-H Chang; B-W Soong
Journal:  Eur J Neurol       Date:  2009-04       Impact factor: 6.089

8.  Long-term treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid.

Authors:  V M Berginer; G Salen; S Shefer
Journal:  N Engl J Med       Date:  1984-12-27       Impact factor: 91.245

9.  Cerebrotendinous xanthomatosis: clinical and MRI study (a case report).

Authors:  M Fiorelli; V Di Piero; S Bastianello; L Bozzao; A Federico
Journal:  J Neurol Neurosurg Psychiatry       Date:  1990-01       Impact factor: 10.154

10.  Clinical usefulness of magnetic resonance imaging in multiple system atrophy.

Authors:  A Schrag; D Kingsley; C Phatouros; C J Mathias; A J Lees; S E Daniel; N P Quinn
Journal:  J Neurol Neurosurg Psychiatry       Date:  1998-07       Impact factor: 10.154
  10 in total
  9 in total

Review 1.  Diagnosis and differential diagnosis of MSA: boundary issues.

Authors:  Han-Joon Kim; Beom S Jeon; Kurt A Jellinger
Journal:  J Neurol       Date:  2015-02-07       Impact factor: 4.849

2.  Reverse 'hot cross bun', 'Mercedes-Benz', 'face of the giant panda and her cub' signs with pontine infarcts: a radiological pandora.

Authors:  Rajendra Singh Jain; Raghavendra Bakki Sannegowda; Rahul Jain; Swayam Prakash
Journal:  BMJ Case Rep       Date:  2014-09-15

Review 3.  Natural history of neurological abnormalities in cerebrotendinous xanthomatosis.

Authors:  Janice C Wong; Kailey Walsh; Douglas Hayden; Florian S Eichler
Journal:  J Inherit Metab Dis       Date:  2018-02-26       Impact factor: 4.982

Review 4.  'Hot cross bun' sign with leptomeningeal metastases of breast cancer: a case report and review of the literature.

Authors:  Zhenyu Pan; Guozi Yang; Tingting Yuan; Yongxiang Wang; Xiaochuan Pang; Yan Gao; Lihua Dong
Journal:  World J Surg Oncol       Date:  2015-02-12       Impact factor: 2.754

5.  The 'Hot Cross Bun' Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases.

Authors:  Christopher Way; David Pettersson; Amie Hiller
Journal:  J Mov Disord       Date:  2018-12-19

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

Authors:  Shuzhen Zhu; Hualing Li; Bin Deng; Jialing Zheng; Zifeng Huang; Zihan Chang; Yanjun Huang; Zhibo Wen; Yanran Liang; Mengjue Yu; Ling-Ling Chan; Eng-King Tan; Qing Wang
Journal:  Front Aging Neurosci       Date:  2020-11-20       Impact factor: 5.750

7.  The "hot cross bun sign" in patients with autoimmune cerebellar ataxia: A case report and literature review.

Authors:  Mange Liu; Haitao Ren; Nan Lin; Ying Tan; Siyuan Fan; Hongzhi Guan
Journal:  Front Neurol       Date:  2022-08-19       Impact factor: 4.086

8.  Xanthomatosis in bilateral hands mimicking rheumatoid arthritis: A case report.

Authors:  Dan Li; Longfei You; Songqing Fan; Lihua Tan
Journal:  Medicine (Baltimore)       Date:  2017-12       Impact factor: 1.817

9.  Cerebrotendinous Xanthomatosis: Molecular Pathogenesis, Clinical Spectrum, Diagnosis, and Disease-Modifying Treatments.

Authors:  Shingo Koyama; Yoshiki Sekijima; Masatsune Ogura; Mika Hori; Kota Matsuki; Takashi Miida; Mariko Harada-Shiba
Journal:  J Atheroscler Thromb       Date:  2021-05-08       Impact factor: 4.928
  9 in total

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