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Disentangling the Phenotypes of Anti‐IgLON5 Disease: When Clinical Suspicion Matters

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restriction in those with a PSP-like phenotype. These fi ndings are in line with the previously reported absence of square wave jerks among the oculomotor abnormalities of anti-IgLON5 patients compared… Click to show full abstract

restriction in those with a PSP-like phenotype. These fi ndings are in line with the previously reported absence of square wave jerks among the oculomotor abnormalities of anti-IgLON5 patients compared to PSP. 6 The presence of chorea, prominent bulbar symptoms, or cognitive impairment in a patient with gait distur-bance may also guide the diagnosis. Noticeably, IgLON5 has been proposed as the most common cause of autoimmune chorea in the elderly rising as an important differential diagnosis with Huntington ’ s disease (HD). The integrity of the caudate nucleus in neuroimaging, and the presence of sleep disorders should lead clinicians to suspect it. A relatively poor response to immunotherapy was reported in these patients. Nonetheless, a different outcome, if patients were treated early in the disease course, cannot be excluded. Indeed, a systematic review of treatment response of previously reported anti-IgLON5 cases suggested that early treatment and combination therapy are associated with better outcomes. 7

Keywords: disease clinical; disease; anti iglon5; phenotypes anti; disentangling phenotypes; iglon5 disease

Journal Title: Movement Disorders Clinical Practice
Year Published: 2022

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