Describe three diverse cases of immune-mediated movement disorders, including clinical features, diagnostic approach and outcomes.

Case 1: A 44-year-old male presented with right upper extremity and face dystonic tremor in setting of biopsy-confirmed common variable immunodeficiency (CVID)-associated granulomatous disease. Investigations demonstrated elevated CSF protein, with normal MRI and FDG-PET brain imaging. Neurological symptoms progressed despite corticosteroids and subsequent monthly IVIG. Subsequent infliximab treatment (with ongoing IVIG) did not improve the tremor. 

Case 2: A 61-year-old female presented with progressive, constant chorea of the upper extremities, jaw and head. Laboratory results supportive of autoimmunity included positive ANA by IFA 1:2560, mitochondrial antibody 79.4 (nl <20), thyroid peroxidase antibody 68.5 (nl <9), and gastric parietal cell antibody 52.6 (nl <24.9). CSF was notable only for mildly elevated myelin basic protein. Brain imaging was normal. She had no clinical response to corticosteroids or plasma exchange.

Case 3: A 48-year old male with Sjögren’s Syndrome (SS) presented with spastic dysarthria, left-sided choreiform movements and ataxic gait that started after aseptic meningitis. Investigations supportive of autoimmunity included ANA by IFA 1:160 and a low positive SSA antibody of 70 (nl <40), with a labial salivary gland biopsy consistent with SS. Unique oligoclonal bands were present on serial CSF.  IV methylprednisolone resulted in mild improvement in dysarthria; there was no further improvement with IVIG treatment.  

There is clinical diversity and variable treatment response of movement disorders associated with systemic autoimmunity.  Because movement disorders may be the presenting symptom of systemic autoimmunity, neurologists should be familiar with the range of manifestations.