TY  - JOUR
AU  - Schlapakow, Elena
AU  - Keil, Vera C.
AU  - Paus, Marie
AU  - Kornblum, Cornelia
AU  - Hattingen, Elke
AU  - Klockgether, Thomas
TI  - Multiple system atrophy mimicry in MRI: Watch out for paraneoplastic rhombencephalitis
JO  - Journal of clinical neuroscience
VL  - 76
SN  - 0967-5868
CY  - Burlington, Mass.
PB  - Harcourt
M1  - DZNE-2020-01124
SP  - 238-240
PY  - 2020
AB  - 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.
KW  - Breast Neoplasms
KW  - Cerebellar Ataxia: diagnosis
KW  - Cerebellar Ataxia: diagnostic imaging
KW  - Cerebellar Diseases: diagnostic imaging
KW  - Cerebellum: diagnostic imaging
KW  - Diagnosis, Differential
KW  - Female
KW  - Humans
KW  - Magnetic Resonance Imaging
KW  - Male
KW  - Middle Aged
KW  - Middle Cerebellar Peduncle: diagnostic imaging
KW  - Multiple System Atrophy: diagnosis
KW  - Multiple System Atrophy: diagnostic imaging
KW  - Spinocerebellar Degenerations: diagnostic imaging
LB  - PUB:(DE-HGF)16
C6  - pmid:32307300
DO  - DOI:10.1016/j.jocn.2020.04.052
UR  - https://pub.dzne.de/record/151540
ER  -