Pseudotumor Cerebri in a Patient with Graves Disease, Is There an Association?

By:Gibson, S., Abou-Samra, A.|Date: July 31, 2013|Source: Endocr Rev, Vol. 33. 2012: SUN-443

Backround: Idiopathic Intracranial Hypertension (IIH), commonly referred as pseudotumor cerebri, is characterized by a clinical picture dominated by signs and symptoms of intracranial hypertension with normal composition of cerebrospinal fluid (CSF) and neuro-imaging that shows no other cause of IHH. Several endocrinopathies have been linked to IHH, including Addison’s disease and hypoparathyroidism.We present a case of a 25 year old African-American woman who presented in May 2011 complaining of severe headaches. Her headaches began in February 2010 after a C-section. Past medical history revealed an emergency-room (ER) visit in January 2011 with intractable vomiting and visual disturbances. She had papilledema and a lumbar puncture (LP) with an opening pressure of 48 mm/Hg indicating high intracranial pressure (ICP) and normal CSF composition. Head computed tomography (CT) showed no intracranial process. The patient was not taking oral contraceptive pills or any other medication. Acetazolamide was started and a follow-up visit was arranged. In June 2011, the patient visited the ER again with similar complaints; LP offered little symptomatic relief. Persistent sinus tachycardia was noted. Thyroid function tests (TFTs) revealed TSH <0.008 uIU/ml, free T4 of 3.2 ng/dL and total T3 of 355 ng/dL. Patient had thyromegaly, mild periorbital edema, no exophthalmos or lid lag. Methimazole 20 mg daily was started. The thyroid uptake was 51% at 24 hrs and the scan was homogenous; compatible with Graves’ disease. A few weeks post discharge, her headaches improved; however, the patient stopped Methimazole in August 2011 due to GI side effects. In September 2011 she presented with acute right eye blindness secondary to worsening ICP causing optic nerve damage. She was thyrotoxic and a head CT showed increased size of ventricles. A ventriculo-peritoneal (VP) shunt was placed to alleviate the IC pressure. The patient also underwent ablation with 14.9 mCi of 131I-Na in December 2011. Symptoms cleared as her TFTs approached a euthyroid state. She remained legally blind in her right eye.Conclusion: IIH is a rare entity in which endocrine pathologies have been previously described as possible etiologies. Graves’ Disease may be associated with IIH, with few cases previously reported in the literature. This seems to be the first case reported of IIH causing blindness associated with Graves’ disease.