AUTHOR=Zhou Chenguang , He Yuanhong , Chao Zhiwen , Zhu Yinghui , Wang Peng , Wang Xingping , Liu Shanshan , Han Wei , Wang Jianping 

TITLE=Wernekink Commissure Syndrome Secondary to Bilateral Caudal Paramedian Midbrain Infarction Presenting with a Unique “Heart or V” Appearance Sign: Case Report and Review of the Literature

JOURNAL=Frontiers in Neurology

VOLUME=Volume 8 - 2017

YEAR=2017

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2017.00376

DOI=10.3389/fneur.2017.00376

ISSN=1664-2295

ABSTRACT=Wernekink Commissure Syndrome secondary to caudal paramedian midbrain infarction (CPMI) is a rare midbrain syndrome involving the decussation of the superior cerebellar peduncle in the caudal paramedian midbrain tegmentum. The central characteristics are constant bilateral cerebellar dysfunction, variable eye-movement disorders and rare delayed palatal myoclonus. Following is a description of the case of a 60-year-old man who presented with dizziness, slurred speech and difficulty walking. Neurological examination revealed bilateral cerebellar dysfunction and bilateral internuclear ophthalmoplegia (bilateral INO). Serial magnetic resonance imaging (MRI) revealed a lesion in the caudal paramedian midbrain with a “heart-shaped” sign on fluid-attenuation inversion recovery images and a “V-shaped” appearance on diffusion-weighted imaging (DWI). An acute CPMI with a “heart or V” appearance sign was diagnosed. Upon follow-up evaluation three months later, a palatal tremor accompanied by involuntary head tremor was discovered.  Hypertrophy and increased signal of the bilateral inferior olivary nucleus, compatible with hypertrophy olivary degeneration (HOD) were revealed during a subsequent MRI study.