Publication:
Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis

2
0

Issued Date

2015

Resource Type

Article

File Type

application/pdf

ISSN

14726823

DOI

10.1186/s12902-015-0062-x

Other identifier(s)

2-s2.0-84945955517

Rights Holder(s)

Scopus

Bibliographic Citation

BMC Endocrine Disorders. Vol 15, No.1 (2015)

Suggested Citation

Ngaosuwan K., Trongwongsa T., Shuangshoti S. Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis. BMC Endocrine Disorders. Vol 15, No.1 (2015). doi:10.1186/s12902-015-0062-x Retrieved from: https://hdl.handle.net/20.500.14740/6040

Title

Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis

Author(s)

Ngaosuwan K.
 Trongwongsa T.
 Shuangshoti S.

Abstract

Background: 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. 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. Conclusion: 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. © 2015 Ngaosuwan et al.

Subject(s)

Acetylsalicylic acid
 Adenohypophysis hormone
 Immunoglobulin G4
 Levothyroxine
 Phenytoin
 Prednisolone
 Simvastatin
 Steroid
 Adenohypophysis hypofunction
 Adult
 Article
 Brain biopsy
 Brain infarction
 Case report
 Differential diagnosis
 Drug dose reduction
 Drug megadose
 Focal epilepsy
 Frontal lobe
 Headache
 Hemianopia
 Histopathology
 Human
 Human tissue
 Hypophysitis
 Immunoglobulin blood level
 Immunoglobulin G4 related hypophysitis
 Low drug dose
 Lymphocytic hypophysitis
 Male
 Nuclear magnetic resonance imaging
 Pituitary incidentaloma
 Right hemisphere
 Visual impairment
 Autoimmune hypophysitis
 Complication
 Lymphocytosis
 Pathology
 Pituitary Diseases
 Prognosis
 Seizures
 Adult
 Autoimmune Hypophysitis
 Humans
 Lymphocytosis
 Male
 Pituitary Diseases
 Prognosis
 Seizures
 Steroids

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URI

https://hdl.handle.net/20.500.14740/6040

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Scopus: Year 1983-2021

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