Endocrine Abstracts

Introduction: Morgagni–Stewart–Morel syndrome is defined as the absolute presence of hyperostosis frontalis interna, associated with metabolic, endocrine, and neuropsychiatric disorders. There are very few cases reported in the literature and some experts do not even recognize it but the exact etiology of the syndrome remains unclear; some theories relate to estrogen dysfunction, obesity and leptin dysfunction, and genetic alterations.

Case report: We present the case of a 33 year old, Caucasian woman, referred to us for frontal cranial hyperostosis associated with intense, invalidating headaches with nausea, vomiting, tremor and palpitations. She was seen by the neurology department that confirmed the frontal cranial hyperostosis with CT and MRI imaging. She was already in treatment with Tramadol 150 mg 4 times a day, Carbamazepine 200 mg twice a day, Metamizole 400 mg+Caffeine 60 mg+Drotaverine 40 mg three times a day, Sumatriptan one administration when needed. From the patient’s history we note a lost pregnancy due to oligohydramnios at 30 weeks, allergic bronchitis, left micropolycystic ovary and secondary amenorrhea for 2 years at the moment of her presentation in our clinic. The clinical exam showed an altered general state due to constant multidrug resistant headache, photophobia, hyperemic face, grade II obesity (BMI=39 kg/m²), acanthosis nigricans in the nucal area, accelerated intestinal movements, normal blood pressure and pulse with no other modifications. She had hypercolesterolemia, HbA1c=6.1% and an insufficient status of vitamin D3=17.2 ng/ml, with no other modified parameters on lab work. The bone resorbtion and formation markers were in the normal range. As far the treatment is concerned, we had to choose between neurosurgery of the frontal bone to reduce the frontal hyperostosis or to find a medical approach, to stop the bone formation process. We decided to administer one vial of zolendronic acid 4 mg/5 ml, with benefit in pain reduction and mild flu-like syndrome 24 h after administration. Six months later, the patient comes for a check-up and we note a BMI=32.08 kg/m², a significant decrease in the headaches intensity with a significant reduced use of pain medication, no more tremors, normal menstrual cycles and a CT that showed stationary frontal hyperostosis.

Discussion: Morgagni–Stewart–Morel syndrome is a rare entity, mostly diagnosed on the clinical setting, with speculative theories about its pathogenesis. Its management is usually symptomatic, but in this particular case we needed a more specific approach as the use of the pain medication was very high and the quality of life was severely affected.