Video NeuroImages: Head titubation in anti-mGluR1 autoantibody-associated cerebellitis
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A 39-year-old woman presented with apathy, catatonia, and continuous large-amplitude oscillatory head movements (head titubation) evolving over 1 month (video, links.lww.com/WNL/A353). Severe ataxia developed weeks later. Brain MRI, EEG, and serologies were normal. Cell- and tissue-based assays were positive for anti–metabotropic glutamate receptor 1 (mGluR1) antibodies (serum 1:12,800, CSF 1:512; figure). Head titubation, a slow-frequency cerebellar outflow tremor of the head associated with axial hypotonia, may appear in cerebellar abnormalities (Joubert syndrome, Dandy-Walker syndrome) or disorders affecting the anterior lobe of the cerebellum.1 Subacute head titubation should raise suspicion for an mGluR1-associated autoimmune cerebellitis, especially if associated with psychiatric changes.2
[1] S. Pittock,et al. Metabotropic glutamate receptor type 1 autoimmunity , 2016, Neurology.
[2] G. Zanni. Head titubation: an unrecognized manifestation of Joubert syndrome and other (midline) cerebellar disorders? , 2014, Developmental medicine and child neurology.