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Case Reports
. 2015 Oct 29:15:64.
doi: 10.1186/s12902-015-0062-x.

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

Kanchana Ngaosuwan  1 , Therdkiat Trongwongsa  2 , Shanop Shuangshoti  3
Affiliations
Case Reports

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

Kanchana Ngaosuwan et al. BMC Endocr Disord. .

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.

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Figures

Fig. 1
Fig. 1
Magnetic resonance imaging of the brain. a-b T2W and T1W with Gadolinium contrast images on September 17, 2013. MRI scan showed hypersignal intensity and gyral contrast enhancement at the right frontal lobe lesion. c-d On February 17, 2014, after steroid initiation, MRI scan showed complete resolution of the right frontal lobe lesion
Fig. 2
Fig. 2
Magnetic resonance imaging of the pituitary. a-b T2W and T1W with Gadolinium contrast images on September 17, 2013. MRI scan showed sellar mass abutted to optic chiasm. c-d On September 2, 2014, the patient developed new temporal hemianopia. There was increase contrast enhancement in T1W and increase intensity in T2W at optic chiasm but slightly decreased in size of sellar mass. e-f On January 13, 2015, after treatment of high dose steroid, the sellar mass and intensity in T2W at optic chiasm were markedly decreased
Fig. 3
Fig. 3
Pituitary histopathology. a Pituitary tissue was infiltrated by lymphocytes and plasma cells. b Fibrous tissue and plasma cells were partly found. c Immunohistochemical study, there was up to 80 per high power field of IgG4 positive plasma cells
See this image and copyright information in PMC

References

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