肥大性下橄榄变性的临床及影像学特征

doi:10.13418/j.issn.1001-165x.2016.06.021

Abstract

Abstract:

Objective　Hypertrophic olivary degeneration (HOD) is a unique transsynaptic degeneration with special clinical manifestations, occurringdelayed damage after the primary damage to the dentato-rubro-olivary pathway(DROP). This paper aims to investigate the clinical and neuroimaging characteristics of HOD.　Methods　We retrospectively analyzed the clinical data of 11 patients with HOD. Results　Of the 11 patients, 10 were male and 1 female, with an average age of 53.5 years old. Primary diseases included midbrain hemorrhage (n=1), pontine hemorrhage (n=8) ，pontine infarction(n=1) and pontine gliomas (n=1). The onset of HOD symptoms，which included dizziness, nystagmus, palatal myoclonus, hypermyotonia, involuntary movement, abnormal posture, ataxia，was meanly 3.8 months later than that of the primary diseases. Enlargement and increased T2 signal intensity of the inferior olive distant from the primary damage were MRI features of HOD. When the primary lesion only affected the unilateral central tegmental tract or red nucleus, HOD was ipsilateral and symptoms occurred in the contralateral side. If the lesion involved both the central tegmental tracts or superior cerebellar pedunculus or red nuclei, HOD and symptoms were bilateral. Baclofen, clonazepam and carbamazepine could be used to alleviatethe clinical symptoms. Conclusion　We should be vigilant about the occurrence of HOD secondary to lesions involved the DROP, in which delayed symptoms could be vital clues.ataxia and involuntary movement are the most common clinical manifestation,and brain MRI is of great help for further diagnosis.

Key words: Hypertrophic olivary degeneration, Dentato-rubro-olivary pathway, Magnetic resonance imaging

WU Lei, AN Hong-wei，SUN Xuan, JIN Di，DONG Zhao，HUANG De-hui, WU Wei-ping. Clinical and neuroimaging characteristics of hypertrophic olivary degeneration[J]. Chinese Journal Of Clinical Anatomy, 2016, 34(6): 700-705.

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Clinical and neuroimaging characteristics of hypertrophic olivary degeneration

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