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Table of Contents    
CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 5  |  Page : 1214-1216

Hypophysitis: A Rare Cause of Impaired Consciousness in a Middle-Aged Woman


1 Department of Neurology, Jubilee Mission Medical College Hospital and Research Institute, Thrissur, Kerala, India
2 Department of Radiology, Jubilee Mission Medical College Hospital and Research Institute, Thrissur, Kerala, India

Date of Web Publication27-Oct-2020

Correspondence Address:
Dr. Neena Baby
Department of Neurology, Renai Medicity Multi Super Speciality Hospital, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.299159

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 » Abstract 


Background: Hypophysitis occurs predominantly in women, usually during pregnancy or postpartum period. Hypophysitis involving the anterior pituitary often presents with adrenal insufficiency.
Design/Methods: We describe a 43-year-old woman who presented with recurrent episodes of alteration of sensorium due to hyponatremia. She had a 3-month history of amenorrhea and on evaluation had evidence of pan-hypopituitarism. The magnetic resonance images of the brain showed mild pituitary enlargement and thickened pituitary stalk with homogenous contrast enhancement indicating hypophysitis. She was treated with oral steroids and thyroxine supplements. She remained asymptomatic at 15-month follow-up on low-dose oral steroids and thyroxine supplements.
Results: Hypopituitarism due to hypophysitis resulted in recurrent episodes of hyponatremia in our patient.
Conclusion: Our case highlights the importance of a systematic search for the etiology for hyponatremic encephalopathy.


Keywords: Hyponatremia, hypophysitis, hypopituitarism
Key Message: Clinical presentation of hypophysitis as recurrent encephalopathy is rare. Pan-hypopituitarism due to hypophysitis may be considered in the differential diagnosis of recurrent hyponatremic encephalopathy.


How to cite this article:
Baby N, Varghese P, Kunnathuparambil SG, Kuriakose AM. Hypophysitis: A Rare Cause of Impaired Consciousness in a Middle-Aged Woman. Neurol India 2020;68:1214-6

How to cite this URL:
Baby N, Varghese P, Kunnathuparambil SG, Kuriakose AM. Hypophysitis: A Rare Cause of Impaired Consciousness in a Middle-Aged Woman. Neurol India [serial online] 2020 [cited 2020 Nov 24];68:1214-6. Available from: https://www.neurologyindia.com/text.asp?2020/68/5/1214/299159




Hypophysitis occurs predominantly in women, usually during pregnancy or postpartum period.[1] Hypophysitis involving the anterior pituitary often presents with adrenal insufficiency. We report a middle-aged woman with hypophysitis who presented with recurrent episodes of impaired consciousness due to hyponatremia.

Patient description

A 43-year-old woman initially presented with headache and vomiting for 1-day followed by impaired consciousness. There was no history of seizures, head trauma, or fever. At admission, she had E3V4M5 on Glasgow Coma Scale. On examination, she had no focal neurological deficits or any signs of meningeal irritation. However, her metabolic workup showed hyponatremia (serum sodium level = 115 mEq/L), normal thyroid stimulating hormone levels (TSH), (1.1 uIU/mL; reference range: 0.27–4.2) and fasting serum cortisol levels (7.9 ug/dl; reference range: 6.2–19.4). The computerized tomogram (CT) of brain, cerebrospinal fluid study, X-ray chest, and ultrasound abdomen showed normal findings. She had clinical improvement on correction of sodium level.

Almost a month later, she developed a similar event and was readmitted. There was no associated fever or any history of drug intake. Her serum sodium level was 112 mEq/L. In addition, a history of amenorrhea of 3-month duration was reported. Her laboratory workup during readmission showed evidence of pan-hypopituitarism [TSH = 0.19 uIU/L, free T4 = 0.39 ng/dl, fasting serum cortisol = 2.57 μg/dl, Follicle stimulating hormone (FSH) = 3.36 mIU/mL, Luteinizing hormone (LH) = 1.38 IU/L, and prolactin = 6.10 ng/mL]. Her serum Angiotensin-converting enzyme (ACE) level was 12.4 U/L (reference range: 8.0–65.0). The magnetic resonance images (MRI) of the brain showed mild pituitary enlargement and thickened pituitary stalk with homogenous contrast enhancement indicating hypophysitis of probable autoimmune origin [Figure 1]. However, her CT images of chest and abdomen with contrast were normal. Autoimmune workup including Antinuclear antibody (ANA) profile was negative. In addition, the serum IgG4 assay was also within normal limits (0.79 g/L). She was treated with oral steroids (prednisolone 20 mg twice daily, tapered over 1 month, and maintained on 5 mg daily) and thyroxine supplements (50 μg/day). In view of the clinical response to treatment, she refused for a biopsy. She remained asymptomatic at 15-month follow-up on low-dose oral steroids and thyroxine supplements.
Figure 1: Axial pre and post gadolinium T1 weighted magnetic resonance images (a and b) showing enlarged pituitary gland with homogenous enhancement (white arrow).(c) T2 weighted sagittal image showing enlarged pituitary gland and stalk (black arrow). (d) depicting the post contrast T1 weighted image which shows thickened and intensely enhancing pituitary stalk (white arrow) suggestive of hypophysitis

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 » Discussion Top


Hypophysitis occurs predominantly in women, usually during pregnancy or postpartum period.[1] Isolated inflammation of the pituitary leads to primary hypophysitis, while secondary hypophysitis often results from lymphocytic autoimmune neurohypophysitis, sarcoidosis, histiocytosis, tuberculosis, metastasis, and lymphoma.[2] Additional causes of hypophysitis include IgG4-related hypophysitis and anti-PIT-1 antibody syndrome associated hypophysitis.[3] Furthermore, recent literature also reports autoimmune hypophysitis (AH) as a complication of therapy with ipilimumab, an immune checkpoint inhibitor used in the treatment of metastatic melanoma.[4]

Hypophysitis involving the anterior pituitary often presents with adrenal insufficiency. However, inflammation more commonly affects the posterior lobe of the pituitary (neurohypophysitis) and pituitary stalk (infundibulum).[2] Hyponatremia in our patient was due to hypoaldosteronism secondary to hypoadrenalism from hypopituitarism suggesting anterior pituitary involvement. Involvement of posterior lobe of the pituitary and pituitary stalk may lead to severe dehydration and diabetes insipidus in contrast to pituitary adenoma which results in the syndrome of inappropriate antidiuretic hormone secretion (SIADH).[5] Symptoms of partial or pan-hypopituitarism occur in approximately 80% of cases and multiple deficiencies are found in approximately 75% of cases.[2]

AH mimics nonsecreting pituitary adenomas. However, confirmatory diagnosis of AH requires histological examination which was not done in our patient. Approximately, 40% of patients with AH are misdiagnosed as having pituitary macroadenoma and undergo unnecessary surgery.[1] Although no single radiological sign is diagnostically accurate, MRI is the best noninvasive diagnostic tool to differentiate AH from nonsecreting adenomas.[1] Radiological findings such as homogenous enhancement of the pituitary, diffuse symmetric gland enlargement, thickened nontapering pituitary stalk, normal sellar size, dural thickening, parasellar T2-weighted hypointensity, parasellar mucosal thickening, and an absence of a posterior pituitary bright spot may however support the diagnosis of AH.[4] All these features were favoring the diagnosis of AH in this patient. In contrast, pituitary macroadenomas are frequently asymmetric, show heterogenous enhancement, and rarely involve the stalk or erode the sellar floor.

The management strategy in lymphocytic/granulomatous hypophysitis is debatable. Steroids are considered as the first line of management in lymphocytic hypophysitis. For steroid nonresponsive patients, other chemotherapeutic agents like methotrexate, azathioprine, and cyclosporine A have been tried with a positive response.[6] Surgical resection should be considered in the presence of optic nerve and/or chiasmal compression, and in the presence of lesions that are not responsive to medical management.[6]


 » Conclusion Top


Hypophysitis presenting as recurrent hyponatremia is rare. Proper history and evaluation give an etiological clue for diagnosis. Pan-hypopituitarism due to hypophysitis may be considered in the differential diagnosis of recurrent hyponatremic encephalopathy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Gutenberg A, Larsen J, Lupi I, Rohde V, Caturegli P. A radiologic score to distinguish autoimmune hypophysitis from non-secreting pituitary adenoma preoperatively. AJNR Am J Neuroradiol 2009;30:1766-72.  Back to cited text no. 1
    
2.
Johnston PC, Chew LS, Hamrahian AH, Kennedy L. Lymphocytic infundibulo-neurohypophysitis: A clinical overview. Endocrine 2015;50:531-6.  Back to cited text no. 2
    
3.
Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: An emerging clinical entity. Endocr J 2009;56:1033-41.  Back to cited text no. 3
    
4.
Faje A. Hypophysitis: Evaluation and management. Clin Diabetes Endocrinol 2016;2:15.  Back to cited text no. 4
    
5.
Kanda M, Omori Y, Shinoda S, Yamauchi T, Tamemoto H, Kawakami M, et al. SIADH closely associated with non-functioning pituitary adenoma. Endocr J 2004;51:435-8.  Back to cited text no. 5
    
6.
Panigrahi M, Kumari M, Vooturi S. Corticosteroids in the management of lymphocytic hypophysitis: Case series. Neurol India 2018;66:400-4.  Back to cited text no. 6
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