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

Ngaosuwan K, Trongwongsa T, Shuangshoti S - BMC Endocr Disord (2015)

Bottom Line: However, during steroid tapering, he developed new onset acute visual loss.He was subsequently treated with high-dose steroid and rapidly regained his sight.With appropriate dosage of steroids, medical treatment is usually sufficient to control the disease and surgical interventions are usually not required.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Faculty of Medicine, Srinakharinwirot University, Ongkarak, Nakhon Nayok, Thailand. dream.kanchana@gmail.com.

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.

No MeSH data available.


Related in: MedlinePlus

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
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Fig1: 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

Mentions: In September 2013, a 43-year-old Thai man was admitted to the hospital with three consecutive episodes of rhythmic jerky movement of left face and arm, as well as speech arrest. These symptoms spontaneously resolved prior to hospital arrival. He also had a two-year history of malaise, loss of appetite, cold intolerance, 10-kg weight loss, headache, and loss of libido. Upon initial physical examination, there was no residual neurological deficit. His visual acuity and visual fields were normal in both eyes. A brain MRI revealed T1W hypointense and T2W hyperintense lesion at right frontal lobe with gyral enhancement (Fig. 1a, b). There was a homogeneous enhancing sellar-suprasellar mass that was abutted to the optic chiasm with pituitary stalk enlargement (Fig. 2a, b) and loss of posterior bright spot. At this point, a neurologist suspected that he had a subacute cerebral infarction with pituitary incidentaloma. An endocrine assessment at the presentation revealed multiple anterior pituitary hormone deficiencies without diabetes insipidus (Table 1). Due to the unavailability of Growth hormone releasing hormone and glucagon, Growth hormone stimulation test was not performed at that time. Insulin tolerance test was also omitted due to the concern of recurrent seizure. Serum alpha-fetoprotein and beta-human chorionic gonadotropin were within normal limits. Anti-thyroperoxidase and Anti-thyroglobulin were negative. He was initially treated with 15 mg of prednisolone daily, levothyroxine replacement, low dose aspirin, simvastatin and phenytoin. The patient gradually improved and was discharged two weeks later.Fig. 1


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

Ngaosuwan K, Trongwongsa T, Shuangshoti S - BMC Endocr Disord (2015)

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
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4625849&req=5

Fig1: 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
Mentions: In September 2013, a 43-year-old Thai man was admitted to the hospital with three consecutive episodes of rhythmic jerky movement of left face and arm, as well as speech arrest. These symptoms spontaneously resolved prior to hospital arrival. He also had a two-year history of malaise, loss of appetite, cold intolerance, 10-kg weight loss, headache, and loss of libido. Upon initial physical examination, there was no residual neurological deficit. His visual acuity and visual fields were normal in both eyes. A brain MRI revealed T1W hypointense and T2W hyperintense lesion at right frontal lobe with gyral enhancement (Fig. 1a, b). There was a homogeneous enhancing sellar-suprasellar mass that was abutted to the optic chiasm with pituitary stalk enlargement (Fig. 2a, b) and loss of posterior bright spot. At this point, a neurologist suspected that he had a subacute cerebral infarction with pituitary incidentaloma. An endocrine assessment at the presentation revealed multiple anterior pituitary hormone deficiencies without diabetes insipidus (Table 1). Due to the unavailability of Growth hormone releasing hormone and glucagon, Growth hormone stimulation test was not performed at that time. Insulin tolerance test was also omitted due to the concern of recurrent seizure. Serum alpha-fetoprotein and beta-human chorionic gonadotropin were within normal limits. Anti-thyroperoxidase and Anti-thyroglobulin were negative. He was initially treated with 15 mg of prednisolone daily, levothyroxine replacement, low dose aspirin, simvastatin and phenytoin. The patient gradually improved and was discharged two weeks later.Fig. 1

Bottom Line: However, during steroid tapering, he developed new onset acute visual loss.He was subsequently treated with high-dose steroid and rapidly regained his sight.With appropriate dosage of steroids, medical treatment is usually sufficient to control the disease and surgical interventions are usually not required.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Faculty of Medicine, Srinakharinwirot University, Ongkarak, Nakhon Nayok, Thailand. dream.kanchana@gmail.com.

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.

No MeSH data available.


Related in: MedlinePlus