Endocrine Abstracts

JOINT1707

Rasmussen’s encephalitis is ultra-rare with precocious puberty reported in approximately 20% of cases but not endocrinopathy. Sodium valproate has been reported as causing impaired gonad, thyroid and adrenal axes function. Rathke’s cleft cysts affect pituitary function to varying degrees. We report a case of a patient with Rasmussen’s encephalitis, long-term valproate treatment and a Rathke’s cleft cyst. A 41 year old female presented with a 1 year history of secondary amenorrhea, scalp hair loss and leg oedema. Menarche occurred at 12 years of age with subsequent regular menses. At 8 months of age she developed a right hemiparesis and epilepsy but no appertaining records were available. She had received treatment with valproate for 27 years. Examination: weight 57.6 kg, mild right hemiparesis, scalp alopecia and moderate leg oedema. Investigations at 09:00h: cortisol 202 nmol/l, ACTH 48.8 ng/l(arose at 07:00h), fT4 10.3 pmol/l, fT3 5.3 pmol/l, TSH 2.02 mmol/l, FSH 3.0 IU/l, LH 1.0 IU/l, oestradiol <100 pmol/l, prolactin 394 mU/land IGF-1 6.3 nmol/L. Renal function, liver function, bone profile and full blood count were normal. Her symptoms, signs and hypogonadotropic hypogonadism were attributed to valproate side-effects. Anticonvulsant change was declined as she had been seizure-free for 23 years, as was HRT. No clinical or endocrine changes were recorded at 1 year. She subsequently presented with general deterioration, anorexia, weight loss (weight 52.6 kg), decreased mobility, worsening leg oedema and hypotension. Investigations at 09:00h: cortisol 93 nmol/l, ACTH 10.6 ng/l, fT4 6.7 pmol/l, TSH 2.98 mU/l, FSH 1.8 IU/l, LH 0.6 IU/l, oestradiol <100 pmol/l, valproate 201 mg/land pancytopenia. MRI was declined. Treatment with hydrocortisone and change in anticonvulsant to lamotrigine resulted in improved clinical status. HRT was declined. At follow up thyroid function and IGF-1 returned to normal but ACTH, cortisol, and gonadotrophins remained low. MRI was accepted and demonstrated the presence of an intrasellar Rathke’s cleft cyst.

Discussion: 1. This patient’s varying endocrine dysfunction is difficult to explain. Valproate could explain the original endocrine abnormalities but not those that have persisted.

2. The Rathke’s cleft cyst could be responsible if it had changed in size on stopping valproate.

3. An episode of hypophysitis would be an alternative explanation.