ArticleBMC endocrine disorders2015
Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis.
Article in BMC endocrine disorders, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 21 citations in OpenAlex.
- Proptosis Revealing a Neurological Form of IgG4-Related Disease: A Case Report and Literature Review.Cureus · 2026Article
- IgG4-Related Disease: Current and Future Insights into Pathological Diagnosis.International journal of molecular sciences · 2025Review
- Typing of inflammatory lesions of the pituitary.Pituitary · 2022Article
- IgG4-related hypophysitis.Endocrine · 2021Review
- Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature.Virchows Archiv : an international journal of pathology · 2019Review
- IgG4-related intracranial disease.The neuroradiology journal · 2019Article
- IgG4-related disease in endocrine practice.Archives of medical science : AMS · 2019Article
- Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review.International journal of endocrinology · 2019Review
- Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center.International journal of endocrinology · 2018Article
- IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis.Brain pathology (Zurich, Switzerland) · 2017Article
- IgG4-related Hypophysitis with Subtle Hypopituitarism in an Elderly Diabetic Patient: Is Treatment or Observation Preferable?Internal medicine (Tokyo, Japan) · 2017Article
- Review
- Critical review of IgG4-related hypophysitis.Pituitary · 2017Review
- Hypophysitis: Evaluation and Management.Clinical diabetes and endocrinology · 2016Review
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThis is the first case report of focal seizure as a manifestation of Immunoglobulin G4 (IgG4)-related hypophysitis. IgG4-related hypophysitis is a novel category of hypophysitis. The clinical presentations, imaging studies and initial pathology studies can mimic lymphocytic hypophysitis. Here we report additional clinical clues in differentiating these two conditions. CASE PRESENTATION: A 43-year-old Thai male presented with focal seizure, headache, and anterior pituitary hypofunction. His MRI study showed typical hypophysitis lesion with abnormal cerebral parenchymal signal intensity at right frontal lobe. The pituitary biopsied was obtained and the patient was initially diagnosed with lymphocytic hypophysitis. Following initial low-dose steroid therapy, his seizure and headache resolved but his anterior pituitary hormones remained deficient. However, during steroid tapering, he developed new onset acute visual loss. Upon rigorous pathologic review, his diagnosis of IgG4-related hypophysitis with suspected CNS involvement was established. He was subsequently treated with high-dose steroid and rapidly regained his sight.
conclusionThis case report highlights the important distinguishing features of IgG4-related hypophysitis from lymphocytic hypophysitis. These include the relapsing clinical course of hypophysitis after steroid decrement and concomitant pachymeningitis particularly in middle-aged to elderly Asian male who presented with hypophysitis. With appropriate dosage of steroids, medical treatment is usually sufficient to control the disease and surgical interventions are usually not required.
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