Association between paraneoplastic rhombencephalitis and hypertrophic olivary degeneration


Hypertrophic olivary degeneration (HOD) is a condition caused by interruption of white matter tracts within the dentato-rubro-olivary pathway (DROP), resulting in inferior olivary T2 hyperintensity and enlargement on MRI. Olivary atrophy may be seen as a sequela of HOD.1 HOD has been described in association with brainstem infarcts, tumours, demyelination and vascular lesions such as cavernous malformations.2 However, HOD can also be seen in the absence of any lesion in the DROP and is sometimes idiopathic.3

Rhombencephalitis refers to inflammation involving the brainstem and/or cerebellum and can rarely be caused by paraneoplastic syndromes. Several neural specific antibodies are classically associated with paraneoplastic rhombencephalitis, including anti-Ma2 and antineuronal nuclear antibody type 2 (ANNA2 or anti-Ri). Kelch-like protein 11 (KLHL11) IgG is another recently described serological biomarker that has a strong association with paraneoplastic rhombencephalitis.4 The tumours frequently associated with paraneoplastic rhombencephalitis include testicular…

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