The authors declare that they have no competing interests.
KN cared the patient, prepared the manuscript, and arranged for the radiological imaging of the patient. TT participated in histopathological study. SS interpreted histopathological study and arranged for the pathological imaging of the patient. All authors read and approved the final manuscript.
Kanchana Ngaosuwan, MD.
Staff at Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, Srinakharinwirot University, Ongkarak Nakhon Nayok, Thailand.
Therdkiat Trongwongsa, MD.
Staff at Department of Pathology, Faculty of Medicine, Srinakharinwirot University, Ongkarak Nakhon Nayok, Thailand.
Shanop Shuangshoti, MD.
Professor at Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
This 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.
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.
This 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.
Bando H, Iguchi G, Fukuoka H, Taniguchi M, Kawano S, Saitoh M, et al. A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis. Pituitary. 2015.
Osawa S, Ogawa Y, Watanabe M, Tominaga T. Hypophysitis presenting with atypical rapid deterioration: with special reference to immunoglobulin G4-related disease-case report. Neurol Med Chir (Tokyo). 2009;49(12):622–5. CrossRef
Sosa GA, Bell S, Christiansen SB, Pietrani M, Glerean M, Loto M. et al. Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women. Endocrinol Diabetes Metab Case Rep 2014; doi: 10.1530/EDM-14-0062.
Nakasone Y, Oguchi K, Sato Y, Okubo Y, Yamauchi K, Aizawa T. Rapid conversion of autoimmune hypophysitis to an empty Sella with immediate lowering of the serum IgG4 level. Case report. Neuro Endocrinol Lett. 2015;36:112–4. PubMed
Li LF, Tse PY, Tsang FC, Lo RC, Lui WM, Leung GK. IgG4-related hypertrophic pachymeningitis at the falx cerebrii with brain parenchymal invasion: a case report. World Neurosurg. 2015. doi: 10.1016/j.wneu.2015.03.035.
- Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis
- BioMed Central
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