A 36-year-old male presented to the Emergency Department with clinical symptoms of blurred vision of progressive onset of two years of evolution. The ophthalmological examination revealed the existence of bilateral papilledema. Using cranial computed tomography and magnetic resonance imaging, the presence of a right occipital pial arteriovenous malformation was certified. Arteriographically, pial arterial contributions dependent on the right middle cerebral artery and the right posterior cerebral artery were identified. Venous drainage was located at the level of the superior sagittal sinus. An associated right transverse sinus stenosis was also identified. The existence of secondary intracranial hypertension was corroborated by monitoring with an intracranial pressure sensor. An interventional procedure was carried out consisting of embolization of the arterial supplies of the lesion using Onyx®. The clinical-radiological findings after the procedure were favorable: the papilledema disappeared and complete exclusion of the malformation was achieved. A new intracranial pressure measurement showed resolution of intracranial hypertension. Subsequent regulated radiological controls showed complete exclusion of the malformation up to 5 years later.

BACKGROUND: We present a rare case of NeuroBehcet\'s-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet\'s. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic.
METHODS: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\'s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.
METHODS: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.
CONCLUSIONS: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.

The management of intracranial malignancies in pregnancy poses unique challenges to the perioperative team. We describe the successful surgical management of a meningioma in a 28-year-old previously healthy patient, in her third trimester of pregnancy, who first presented with a generalised seizure. Without clear guidelines on the management of intracranial malignancies in pregnancy, a multidisciplinary approach was essential in providing a management plan for the patient\'s seizures and on the timing of her surgical intervention. Hormone-mediated tumour growth was a significant factor in opting for urgent surgical intervention and we discuss the current evidence linking hormones to tumour growth in pregnancy.

UNASSIGNED: Endolymphatic sac tumor (ELST) is a rare lesion. It may be sporadically or associated with Von Hippel-Lindau syndrome. Progressive audiovestibular symptoms characterize the typical clinical presentation. Here, we report a unique case of ELST with acute intracranial hypertension (IH) due to tumor compression, successfully treated with an urgent suboccipital decompressive craniectomy (SDC).
UNASSIGNED: A 33-year-old woman previously underwent a biopsy and ventriculoperitoneal shunt. The histopathological finding revealed an ELST. One year later, she developed headache, vomiting, and somnolence due to brainstem compression. An urgent SDC was performed. One month later, preoperative endovascular embolization and partial tumor resection were carried out. After 6 months adjuvant radiotherapy (RT) therapy was administered. She has been under follow-up for 8 years since the last surgical procedure, and the tumor remains stable.
UNASSIGNED: ELST generally has a progressive clinical course. This is a unique case with acute IH due to tumor compression. The tumor\'s high vascularity and the unavailability of endovascular embolization precluded its resection. SDC was an alternative approach. The final treatment included tumor embolization, surgical resection, and RT. No progression was observed for 8 years after the last procedure, and long-term follow-up is warranted.

OBJECTIVE: To examine whether increased systemic arterial blood pressure (ABP) and decreased heart rate (HR) under inhalant anesthesia were suggestive of the Cushing reflex (CR) in dogs with neurological diseases based on magnetic resonance imaging (MRI) findings.
METHODS: Retrospective case-control study.
METHODS: A sample of 137 client-owned dogs admitted to two veterinary teaching hospitals for diagnosis and patient care owing to suspected intracranial disease from 2005 to 2020. All dogs underwent general anesthesia for MRI of the brain.
METHODS: Based on MRI study reports, dogs were divided into three study groups: group A, dogs with suspected brain herniation; group B, dogs with an abnormal MRI finding but without suspicion of herniation; and group C, dogs with structurally unremarkable brains (control). Initial evaluation by an anesthesiologist resulted in 58 cases selected for further review based on suspicion of having intracranial hypertension, as indicated by ABP and HR changes under anesthesia. Anesthetic records were randomized and independently reviewed by three board-certified anesthesiologists blinded to MRI diagnosis who allocated each dog into one of three groups: 1) likely to have CR; 2) unlikely to have CR; or 3) insufficient data for determination.
RESULTS: Of the 58 cases evaluated, nine were considered likely to have CR and 48 were considered unlikely to have CR. In one case, the anesthesiologists were unable to determine whether CR occurred. Of the nine dogs with likely CR, three had an MRI diagnosis of intracranial herniation. The remaining six dogs suspected of CR belonged to the normal brain group.
CONCLUSIONS: The ABP and HR changes associated with CR are not reliably associated with brain herniation in dogs undergoing general anesthesia.

Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, represents a challenging diagnostic entity in neurology, characterized by elevated intracranial pressure of unknown origin. The classical clinical triad of headache, visual disturbances, and papilledema provides a well-established framework for diagnosis; however, the heterogeneity of IIH presentations, combined with the absence of an overt causative factor, continues to perplex clinicians. This case report delves into the complexities of a rare IIH presentation in a 32-year-old male, highlighting the need for a nuanced understanding of this condition beyond its traditional confines.

BACKGROUND: Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review.
METHODS: A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations.
RESULTS: A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment.
CONCLUSIONS: The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.

BACKGROUND: Raised intracranial pressure (ICP) can be due to varied etiology. Differentiating among these various etiologies is crucial in making appropriate therapeutic decisions. A patient with a known past history of the primary or secondary headache of any etiology, when presenting with new onset severe headache, needs to be evaluated with imaging to rule out an alternative diagnosis.
CONCLUSIONS: Here, we describe the case details of a young lady who presented with recurrent raised ICP headaches due to three different etiologies. At her third visit, isolated intracranial hypertension (IH) was the only manifestation of cerebral venous sinus thrombosis (CVST), which could have been missed if a repeat magnetic resonance imaging (MRI) brain and venogram were not done.
CONCLUSIONS: Our case highlights the importance of having a high degree of suspicion for CVST in the clinical setting of raised ICP headache in view of its crucial therapeutic implications.

Idiopathic intracranial hypertension (IIH) is a rare condition characterized by increased intracranial pressure, with an unknown cause. However, the pathophysiology of antibiotic-induced IIH remains unclear. The clinical symptoms include headache, visual disturbances, and vomiting. The diagnosis is confirmed by an elevated intracranial pressure (ICP) with normal CSF study and cerebral imaging. Management includes discontinuing the offending antibiotic and reducing ICP with medications such as acetazolamide or diuretics. Therefore, surgical intervention may be necessary in severe cases. In this article, we report the case of a 19-year-old patient, admitted with symptoms of intracranial hypertension syndrome, occurring three days after receiving antibiotics (gentamicin, penicillin). Physical examination revealed bilateral optic disc edema. Cerebral magnetic resonance imaging (MRI) revealed indirect signs of intracranial hypertension. The CSF pressure measurement was approximately 290 mmHg, while CSF and other laboratory blood tests were normal. The patient received methylprednisolone bolus and topiramate (50 mg/day). A month later, the clinical outcome showed regression of headaches and regression of the papilledema.

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