Headache due to intracranial hypertension at the time of first manifestation of multiple sclerosis

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Headache due to intracranial hypertension at the time of first manifestation of multiple sclerosis Angelo Pascarella 1,2 & Antonio De Martino 2 & Lucia Manzo 1,2 & Stefania Barone 2 & Paola Valentino 2 & Francesco Bono 1,2

# Fondazione Società Italiana di Neurologia 2020

Patients with multiple sclerosis (MS) frequently suffer from headache. In fact, recent studies have shown that about 78% of patients with multiple sclerosis suffer from headache. In addition, the occurrence of headache at the time of the first clinical manifestation of MS has recently been reported in a large proportion of patients [1]. Although an increased risk of headache is now recognized among MS patients, the secondary etiology of MS-associated headache has been poorly studied. Indeed, the possibility that intracranial hypertension (IH) is the cause of headache in MS patients has been rarely considered [2]. Here we report data from patients who had headache as the first manifestation of MS, in whom the cause of headache was an IH syndrome secondary to a cerebral venous flow disorder. Patient 1 was a 28-year-old obese (BMI: 35) female with polycystic ovary syndrome, suffering from severe daily headache. She complained a daily, diffuse, severe, tightening pain, worsening with lying position associated with dizziness and transient visual disturbances. Neurological examination was normal. Brain MRI demonstrated empty sella, perioptic subarachnoid space distension, and posterior sclera flattening. Cerebral MR venography (MRV) revealed bilateral transverse sinus stenosis (BTSS), indicating an altered intracranial venous outflow. One-hour lumbar CSF pressure monitoring via spinal puncture needle [3] showed an elevated opening pressure and mean pressure (272 mmH2O and 244 mmH2O, respectively), with maximum peak of 302 mmH2O and presence of abnormal CSF pulsations (B waves). CSF analysis

Angelo Pascarella and Antonio De Martino contributed equally to this work. * Francesco Bono [email protected] 1

Center for Headache and Intracranial Pressure Disorders, A. O. U. “Mater Domini” of Catanzaro, Viale Europa, 88100 Catanzaro, Italy

2

Institute of Neurology, University “Magna Graecia”, Catanzaro, Italy

was normal. On this base, the diagnosis of headache due to IH syndrome was performed and medical treatment with acetazolamide was administered. After 6 months, the patient experienced sudden worsening of headache associated to transient visual loss and tinnitus. Neurological examination revealed left hemiparesis and hypoesthesia. A contrastenhanced MRI of brain and spine displayed white matter lesions fulfilling diagnostic criteria for MS. Patient 2 was a 19-year-old female suffering from headache (right frontotemporal, throbbing, almost daily pain, worsening when she was lying down) and transient diplopia in the last 6 months. Neurological examination showed right nystagmus and brisk tendon reflexes at both lower limbs. Contrastenhanced MRI of the brain disclosed exclusively the presence of empty sella, whereas MRV showed left unila