Objective:Anti-IgLON5 disease is a recently described neurological disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are under-reported. Here we describe the frequency and types of movement disorders in a series of consecutive patients with this disease.Methods:In this retrospective, observational study, the presence and phenomenology of movement disorders were assessed with a standardized clinical questionnaire. Available videos were centrally reviewed by three experts in movement disorders.Results:Seventy two patients were included. In 41 (57%) the main reason for initial consultation was difficulty walking along with one or several concurrent movement disorders. At the time of anti-IgLON5 diagnosis, 63 (87%) patients had at least one movement disorder with a median of three per patient. The most frequent abnormal movements were gait and balance disturbances (52 patients, 72%), chorea (24, 33%), bradykinesia (20, 28%), dystonia (19, 26%), abnormal body postures or rigidity (18, 25%), and tremor (15, 21%). Other hyperkinetic movements (myoclonus, akathisia, myorhythmia, myokymia, or abdominal dyskinesias) occurred in 26 (36%) patients. The craniofacial region was one of the most frequently affected by multiple concurrent movement disorders (23 patients, 32%) including dystonia (13), myorhythmia (6), chorea (4) or myokymia (4). Considering any body region, the most frequent combination of multiple movement disorders consisted of gait instability or ataxia associated with craniofacial dyskinesias or generalized chorea observed in 31(43%) of patients. In addition to abnormal movements, 87% of patients had sleep alterations, 74% bulbar dysfunction, and 53% cognitive impairment. Fifty-five (76%) patients were treated with immunotherapy, resulting in important and sustained improvement of the movement disorders in only seven (13%) cases.Conclusions:Movement disorders are a frequent and leading cause of initial neurological consultation in patients with anti-IgLON5 disease. Although multiple types of abnormal movements can occur, the most prevalent are disorders of gait, generalized chorea, and dystonia and other dyskinesias that frequently affect craniofacial muscles. Overall, anti-IgLON5 disease should be considered in patients with multiple movement disorders, particularly if they occur in association with sleep alterations, bulbar dysfunction, or cognitive impairment.

Since COVID-19 was first reported, different neurological complications have been acknowledged, but their description is constantly evolving. We report a case of concurrent tonic pupil and trochlear nerve palsy in this context. A 62-year-old man reported a 5-day history of binocular vertical diplopia and blurred vision in his left eye, noticing that his left pupil was dilated. He had suffered a flu-like syndrome 2 weeks before. Clinical exam showed a right trochlear nerve palsy and a left mydriatic pupil. MRI, X chest ray, and analytical results were normal. Antibodies for SARS-CoV-2 were positive (low IgM and high IgG titers). Antiganglioside antibodies were negative. A 0.125% pilocarpine test confirmed Adie's pupil diagnosis. The patient was treated with a tapered prednisone dose with resolution of his diplopia but no change in Adie's pupil. This is the first case reporting Adie's pupil as a postinfectious manifestation of COVID-19. An immune-mediated mechanism is presumed.

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Background and purpose Anti‐IgLON5 disease is a rare disorder characterized by a heterogeneous myriad of symptoms that may include sleep disorders, bulbar dysfunction, gait problems, movement disorders, cognitive impairment, oculomotor abnormalities, and nervous system hyperexcitability. Its physiopathology remains unknown, with a combination of both autoimmune and neurodegenerative findings. Methods We describe clinical, cerebrospinal fluid (CSF), and ioflupane single‐photon emission computed tomography (SPECT) findings of a positive case of anti‐IgLON5 disease mimicking probable progressive supranuclear palsy (PSP). We performed a literature review of previous publications reporting on anti‐IgLON5 disease and ioflupane SPECT. Results We report the case of a 66‐year‐old male who met clinical criteria for probable PSP, in whom ioflupane SPECT showed an alteration of the left presynaptic dopaminergic pathway. However, the presence of atypical neurological symptoms for PSP led to further complementary tests, and IgLON5 antibodies were detected in CSF. According to our literature review, ioflupane SPECT findings have been previously described in only three other patients with anti‐IgLON5 disease, with a reduced uptake in the striatum in two of them. Conclusions Ioflupane SPECT abnormalities, though scarcely described, are not uncommon in anti‐IgLON5 disease. They could be related to nigrostriatal dopaminergic degeneration in the context of the tauopathy component of the disease, but further case descriptions are necessary.

A 74-year-old woman presented with a 5-year history of progressive gait instability with recurrent falls, dysphonia, dysphagia, and sleep problems, which included insomnia, breathing difficulties, and complex vocalizations during sleep (talking, singing, laughing). Examination showed bilateral ptosis, upward gaze palsy, hoarseness, dysprosody, and gait instability (video 1). No parkinsonism, chorea, or dementia was detected.Brain MRI and PET as well as CSF measures were unremarkable. Video-polysomnography revealed a disorganized sleep architecture but not parasomnia. Anti-IgLON5 antibodies were positive in CSF and serum by cell-based assay of HEK cells transfected with IgLON5 and immunohistochemistry on rat brain. Human leukocyte antigen genotyping detected HLA-DQB1*05:01.Immunotherapy with IV methylprednisolone 1 g/d for 5 days and immunoglobulins resulted in improvement of gait and speech over a month (video 1). A second course of immunoglobulins provided clinical stability.Anti-IgLON5 disease represents a paradigm of autoimmune neurodegeneration with core features of a specific sleep disorder, bulbar symptoms, and gait abnormalities. 1 Its recognition is important because some cases respond to immunotherapy. 2 Study FundingNo targeted funding reported.

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