Vestibular and Ocular Motor Phenotype in a Case of Paraneoplastic Autoimmune Kelch-Like Protein-11 Encephalitis

OBJECTIVES: KLHL11 encephalitis is often associated with vestibular symptoms and ataxia. However, the specific disorders of the vestibular and oculomotor systems have only been partially investigated so far. We present a case of paraneoplastic KLHL11 encephalitis in which vestibulo-oculomotor function was quantitatively assessed. METHODS: A 1.5T MRI scan of the brain and cervical spinal cord was performed. KLHL11 IgG in serum and CSF was detected using a fixed cell-based assay. Vestibulo-oculomotor function was assessed by videooculography with rotational chair tests. RESULTS: MRI scans of the brain and cervical spine were normal. Serum and CSF were strongly positive for KLHL11 antibodies, confirming the diagnosis of KLHL11 encephalitis. Videooculography revealed spontaneous downbeat nystagmus and horizontal gaze-evoked nystagmus bidirectionally, pronounced on left gaze. Smooth pursuit eye movements were saccadic in both directions. Horizontal and vertical saccades showed prolonged latencies, mild dysmetria, and normal saccadic velocities. In the rotational tests, the vestibulo-ocular reflex (VOR) was symmetrical at all frequencies and exhibited a normal to high-normal gain. The VOR fixation-suppression during sinusoidal rotation was almost abolished. DISCUSSION: Our clinical neurophysiologic data imply that KLHL11 encephalitis is characterized by a pronounced vestibulo-cerebellar syndrome, which places it among the few autoimmune encephalitides with this specific phenotype.