Lymphocytic hypophysitis and headache in pregnancy

Abstract

A 33 year old woman in her second pregnancy developed frontal headaches at 29 weeks of gestation. Her pregnancy had previously been uneventful. Her first pregnancy had ended in a spontaneous miscarriage at eight weeks of gestation. She had no past history of migraine and was otherwise asymptomatic. There was no family history of autoimmune disease. Her blood pressure was normal and there was no proteinuria.In the 33rd week of pregnancy, her headaches became persistent, more severe on awakening and were accompanied by vomiting. We recommended computed tomography or magnetic resonance imaging of the brain but she declined investigation because of the perceived radiation risk. Her pregnancy progressed uneventfully until term, when following the development of an abnormal cardiotocogram, she was delivered by caesarean section under epidural anaesthesia. There was no evidence of placental abruption. The weight of her infant was 2.9 kg. Her blood pressure was again normal and she had no symptoms or signs of pre-eclampsia. Her headaches persisted after delivery and were associated with visual blurring and failure to lactate. Computed tomography of the brain with contrast was performed three weeks postpartum and showed a uniformly enhancing pituitary mass with suprasellar extension (Fig. 1). Formal visual field perimetry was normal. Endocrine assays on her blood three weeks after her delivery revealed: free thyroxine 9 pmol/L (normal range 11 -25 pmol/L); thyroid stimulating hormone 1.6 mu/L (0.35 -5.0 mu/L); prolactin <50 mu/L (100 -420 mu/L); 9:00 am cortisol <30 nmol/L ( > 400 nmol/L); growth hormone 10.7 mu/L (9.5 -45 mu/L); insulin-like growth factor-1 36.6 nmol/L (9.5 -45 nmol/L); follicle stimulating hormone 4.2 u/L; luteinising hormone 1.3 u/L.The reduced concentrations of free thyroxine, prolactin and cortisol suggested partial hypopituitarism related to the pituitary mass. A diagnosis of lymphocytic hypophysitis was made on the basis of the typical peripartum presentation, the pituitary mass and the endocrine investigations suggesting partial hypopituitarism. Treatment was started with hydrocortisone 10 mg at 0800 and 1500 h and thyroxine 150 mcg daily. Her headaches completely resolved over the next three days. Regular menstruation resumed two months after her delivery but she failed to lactate. Four months postpartum, magnetic resonance imaging showed complete resolution of the pituitary mass (Fig. 2). At 12 months, thyroxine was withdrawn and her thyroid function remained normal. At 14 months, following omission of the morning dose of hydrocortisone, cortisol and prolactin levels remained undetectable, indicating residual deficiency of ACTH and prolactin production. There was no serologic evidence of autoimmune disease. Two years after her delivery, physiological steroid replacement therapy is maintained and long term follow up in the endocrine clinic is planned. The woman and her partner have not yet tried for a further pregnancy and are using barrier contraception.

Post‐partum hypoglycemia and hypothermia as first manifestations of lymphocytic adenohypophysitis: A case report

“…During pregnancy, there are many causes of headache, including tension headaches, migraine and pre-eclampsia. 7 Diagnosis of LAH is classically difficult; it can be easily mistaken for a pituitary adenoma; therefore, definitive diagnosis of LAH is pathological following surgery. 2 However, nowadays, non-invasive diagnosis of LAH is feasible by pituitary MRI and endocrinological deficit patterning.…”

The Young Woman With Postpartum “Thunderclap” Headache

“…Finally, lymphocytic hypophysitis, an autoimmune inflammation of the pituitary gland, can present with an acute-onset headache or more commonly as a subacute or chronic headache with visual disturbances and symptoms of hypopituitarism. 17,18 Lymphocytic hypophysitis is rare, but is more common during pregnancy, particularly during the final trimester or postpartum period. Pituitary apoplexy may be misdiagnosed as subarachnoid hemorrhage or meningoencephalitis because of the presence of leukocytes and erythrocytes in the cerebrospinal fluid.…”