gms | German Medical Science

Objective: Benign intracranial hypertension (BIH) is a rare disorder, presenting with elevated intracranial pressure (ICP), headache and a gradual worsening of vision. Predominantly, BIH occurs in overweight women of childbearing age leading to the hypothesis that female sex hormones are involved in the pathophysiology of the condition. Standard management of BIH includes spinal fluid taps, weight loss and medical therapy with acetazolamide. In patients refractory to conservative therapy, ventriculo-peritoneal (VP) shunting is usually performed. The aim of the case report is to raise awareness for a rare cause of BIH refractory to standard conservative therapy that did not require VP-shunting but a different tailored treatment option.

Methods: Case report.

Results: A 21-year-old patient with a male phenotype became symptomatic with holocephalic headache, blurred vision and papilledema. Five lumbar punctures revealed elevated opening pressures between 40 and 50 cm H2O. After exclusion of venous sinus stenosis on MRI, BIH was diagnosed, and the patient was referred to our department for VP shunting. Since BIH is an unusual condition in a young male, the patient was questioned about hormone intake and then forwarded the information to be a biological female in the process of female-to-male (FTM) gender reassignment. Two years prior to symptom onset, he started testosterone injections every three weeks with additional usage of testosterone skin cream. Under therapy, he gained a total of 35 kg. At presentation, testosterone levels were the upper reference range for men and estradiol was only partially suppressed. Since it was assumed that fluctuating testosterone levels and an insufficient suppression of estrogens caused BIH in this case, treatment with a gonadotropin releasing hormone analogue (leuprorelinacetate) was initiated to suppress ovarian function. Testosterone medication was changed to a daily gel to ensure constant testosterone levels. Under this regimen and weight loss, ICP decreased to 25 cm H2O and papilledema improved.

Conclusion: It is likely that incomplete suppression of female sex hormones in addition to the surplus of endocrinologically active adipose tissue has propagated the development of BIH in this patient. Peripheral enzymatic conversion of androgens to estrogens might have constituted a contributing factor. Tailored therapy, by means of which VP-shunting could be avoided, only became possible because of thorough history taking and interdisciplinary knowledge sharing.