Citations 16

Response to: An International ASXL3 Natural History Study: Deep Phenotypic Analyses Including Detailed Reports of a Milder Phenotype, Novel Associations,

American Journal of Medical Genetics Part A | September 29, 2025 | Volume: 200 | Issue: 2 | Pages: 559-561

10.1002/ajmg.a.64272 | PMID 41017488

Tongue Myokymia in IgLON5-IgG Autoimmunity

JAMA Neurology | April 14, 2025 | Volume: 82 | Issue: 6 | Pages: 626

10.1001/jamaneurol.2025.0523 | PMID 40227725

This case report discusses a man who tested positive for IgLON5-IgG antibody after intermittent episodes of paresthetic feeling involving the throat and jaw, associated with subtle dysarthria and dysphagia, and upper-extremity paresthesia with piloerection of the forearms.
Anti-SRP Necrotizing Myopathy in Association with Neuromyelitis Optic Spectrum Disorder (P11-8.004)

Neurology | April 7, 2025 | Volume: 104 | Issue: 7_Supplement_1 | Pages: No page available

10.1212/wnl.0000000000212508

Immune Checkpoint Inhibitor-associated Kelch-like Protein-11 IgG Rhombencephalitis (P3-14.002)

Neurology | April 9, 2024 | Volume: 102 | Issue: 7_supplement_1 | Pages: No page available

10.1212/wnl.0000000000205023

The Risk of Epilepsy in Systemic Autoimmune Diseases: A Population-based Study in Olmsted County, Minnesota (P5-14.001)

Neurology | April 9, 2024 | Volume: 102 | Issue: 7_supplement_1 | Pages: No page available

10.1212/wnl.0000000000206254

Clinical Characterization of Neurofascin-155 IgG4 and Contactin-1 IgG Dual Positive Neuropathies (P4-14.003)

Neurology | April 9, 2024 | Volume: 102 | Issue: 7_supplement_1 | Pages: No page available

10.1212/wnl.0000000000205112

Neuroinvasive West Nile Virus Infection in Immunosuppressed and Immunocompetent Adults

JAMA Network Open | March 28, 2024 | Volume: 7 | Issue: 3 | Pages: e244294

10.1001/jamanetworkopen.2024.4294 | PMID 38546642

Importance West Nile virus (WNV) is the leading cause of human arboviral disease in the US, peaking during summer. The incidence of WNV, including its neuroinvasive form (NWNV), is increasing, largely due to the expanding distribution of its vector, the Culex mosquito, and climatic changes causing heavy monsoon rains. However, the distinct characteristics and outcomes of NWNV in individuals who are immunosuppressed (IS) and individuals who are not IS remain underexplored. Objective To describe and compare clinical and radiographic features, treatment responses, and outcomes of NWNV infection in individuals who are IS and those who are not IS. Design, Setting, and Participants This retrospective cohort study used data from the Mayo Clinic Hospital system collected from July 2006 to December 2021. Participants were adult patients (age ≥18 years) with established diagnosis of NWNV infection. Data were analyzed from May 12, 2020, to July 20, 2023. Exposure Immunosuppresion. Main Outcomes and Measures Outcomes of interest were clinical and radiographic features and 90-day mortality among patients with and without IS. Results Of 115 participants with NWNV infection (mean [SD] age, 64 [16] years; 75 [66%] male) enrolled, 72 (63%) were not IS and 43 (37%) were IS. Neurologic manifestations were meningoencephalitis (98 patients [85%]), encephalitis (10 patients [9%]), and myeloradiculitis (7 patients [6%]). Patients without IS, compared with those with IS, more frequently reported headache (45 patients [63%] vs 18 patients [42%]) and myalgias (32 patients [44%] vs 9 patients [21%]). In contrast, patients with IS, compared with those without, had higher rates of altered mental status (33 patients [77%] vs 41 patients [57%]) and myoclonus (8 patients [19%] vs 8 patients [4%]). Magnetic resonance imaging revealed more frequent thalamic T2 fluid-attenuated inversion recovery hyperintensities in individuals with IS than those without (4 patients [11%] vs 0 patients). Individuals with IS had more severe disease requiring higher rates of intensive care unit admission (26 patients [61%] vs 24 patients [33%]) and mechanical ventilation (24 patients [56%] vs 22 patients [31%]). The 90-day all-cause mortality rate was higher in the patients with IS compared with patients without IS (12 patients [28%] vs 5 patients [7%]), and this difference in mortality persisted after adjusting for Glasgow Coma Scale score (adjusted hazard ratio, 2.22; 95% CI, 1.07-4.27; P = .03). Individuals with IS were more likely to receive intravenous immunoglobulin than individuals without IS (12 individuals [17%] vs 24 individuals [56%]), but its use was not associated with survival (hazard ratio, 1.24; 95% CI, 0.50-3.09; P = .64). Conclusions and Relevance In this cohort study of individuals with NWNV infection, individuals with IS had a higher risk of disease complications and poor outcomes than individuals without IS, highlighting the need for innovative and effective therapies to improve outcomes in this high-risk population.
Immune Checkpoint Inhibitor-Associated Kelch-Like Protein-11 IgG Brainstem Encephalitis

Neurology Neuroimmunology & Neuroinflammation | March 14, 2024 | Volume: 11 | Issue: 3 | Pages: No page available

10.1212/nxi.0000000000200218 | PMID 38484218

Traumatic brain injury and erectile dysfunction preceding clinically diagnosed alpha-synucleinopathies: a case – control study in Olmsted County, MN (1991–2010). (P2.063)

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.p2.063

Infections leading to hospitalization or sepsis preceding clinically diagnosed α-synucleinopathies: a case-control study in Olmsted County, MN (1991–2010). (P3.8-049)

Neurology | December 7, 2023 | Volume: 92 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.92.15_supplement.p3.8-049