Multiple system atrophy mimicry in MRI: Watch out for paraneoplastic rhombencephalitis

Schlapakow, Elena; Paus, Marie; Keil, Vera C.; Klockgether, Thomas; Kornblum, Cornelia; Hattingen, Elke

doi:10.1016/j.jocn.2020.04.052

Journal Article

DZNE-2020-01124

Multiple system atrophy mimicry in MRI: Watch out for paraneoplastic rhombencephalitis

Schlapakow, E. ; Keil, V. C. ; Paus, M. ; Kornblum, C. ; Hattingen, E. ; Klockgether, T. (Last author)DZNE*

2020
Harcourt Burlington, Mass.

Journal of clinical neuroscience 76, 238-240 (2020) [10.1016/j.jocn.2020.04.052]

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Abstract: The hot cross bun (HCB) sign describes cruciform-shaped T2-weighted hyperintensities of pontocerebellar fibers within the pons and is a typical, but not specific imaging hallmark of the cerebellar variant of multiple system atrophy (MSA-C). We report a case of a 51-year-old woman who was first diagnosed with MSA-C based on progressive cerebellar ataxia, the HCB sign and T2-weighted hyperintensities in middle cerebellar peduncles on MRI. However, further diagnostic work-up revealed positive anti-amphiphysin antibodies in blood and cerebrospinal fluid and subsequently breast cancer. This report of a paraneoplastic rhombencephalitis which initially mimicked MSA-C imaging features stresses the importance of considering immune-mediated rhombencephalitis as differential diagnosis in cases of progressive cerebellar ataxia and the HCB sign on T2-weighted MRI, especially in the absence of pontocerebellar atrophy.

Keyword(s): Breast Neoplasms (MeSH) ; Cerebellar Ataxia: diagnosis (MeSH) ; Cerebellar Ataxia: diagnostic imaging (MeSH) ; Cerebellar Diseases: diagnostic imaging (MeSH) ; Cerebellum: diagnostic imaging (MeSH) ; Diagnosis, Differential (MeSH) ; Female (MeSH) ; Humans (MeSH) ; Magnetic Resonance Imaging (MeSH) ; Male (MeSH) ; Middle Aged (MeSH) ; Middle Cerebellar Peduncle: diagnostic imaging (MeSH) ; Multiple System Atrophy: diagnosis (MeSH) ; Multiple System Atrophy: diagnostic imaging (MeSH) ; Spinocerebellar Degenerations: diagnostic imaging (MeSH)