{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T08:00:08Z","timestamp":1773820808987,"version":"3.50.1"},"reference-count":33,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T00:00:00Z","timestamp":1773792000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>\n                    Neuronal intranuclear inclusion disease (NIID) can initially present with gastrointestinal symptoms as the sole or primary manifestation for decades before neurological signs emerge. We report the case of a 61-year-old woman with a 22-year history of drug-refractory, recurrent nausea and vomiting. Previous extensive gastrointestinal evaluations were unremarkable, and conventional therapies proved ineffective. The diagnosis of NIID was established on the basis of the following key findings: diffusion-weighted imaging revealed a characteristic \u201ccrown-like\u201d hyperintensity at the corticomedullary junction; genetic analysis revealed a GGC repeat expansion in the\n                    <jats:italic>NOTCH2NLC<\/jats:italic>\n                    gene, with no abnormalities detected in\n                    <jats:italic>FMR1<\/jats:italic>\n                    ; and skin biopsy demonstrated p62-positive intranuclear inclusions within sweat gland cells. The patient\u2019s symptoms improved markedly with corticosteroid therapy but recurred upon repeated withdrawal. Subsequent initiation of a maintenance corticosteroid regimen resulted in sustained clinical stability, with only occasional mild symptom fluctuations over a six-month follow-up period. This case highlights that vomiting can be a prominent and early clinical feature of NIID. The condition should be considered in the differential diagnosis of patients with unexplained, refractory nausea and vomiting, for whom long-term corticosteroid therapy may represent a viable treatment strategy.\n                  <\/jats:p>","DOI":"10.3389\/fimmu.2026.1782547","type":"journal-article","created":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T06:58:17Z","timestamp":1773817097000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Neuronal intranuclear inclusion disease with initial manifestation of intractable nausea and vomiting responsive to corticosteroids: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Long","family":"Luo","sequence":"first","affiliation":[{"name":"Department of Neurology, The Central Hospital of Xiangtan (The Affiliated Hospital of Hunan University)","place":["Xiangtan, Hunan, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ling","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Central Hospital of Xiangtan (The Affiliated Hospital of Hunan University)","place":["Xiangtan, Hunan, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yong","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Central Hospital of Xiangtan (The Affiliated Hospital of Hunan University)","place":["Xiangtan, Hunan, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Yuan","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Central Hospital of Xiangtan (The Affiliated Hospital of Hunan University)","place":["Xiangtan, Hunan, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lei","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Central 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