Unusual case of intraosseous primary intracranial malignant melanoma.

Primary intracranial malignant melanoma (PIMM) represents 0.07% of central nervous system tumours; clinical behaviour and prognosis are not well documented. Preoperative diagnosis of PIMM is complex and it could be easily misdiagnosed, especially with malignant meningioma.We are reporting a case of a man with a history of rapidly arising motor slowing associated with urinary incontinence, presenting with mild convergent strabismus caused by paralysis in abduction in the right eye. A brain CT showed a lesion compatible with malignant spheno-orbital meningioma, and the patient underwent gross total resection. Intraoperatively, the blackish lesion infiltrated and eroded the bone; it was placed externally on the dura mater with a mild reaction and without attachment. Histological examination confirmed PIMM.Intraosseous localisation of PIMM has been observed in the basic bone structure of the oral cavity. We report the first intraosseous spheno-orbital PIMM case and present an embryological theory about how this unusual tumour can develop.

Myoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?

BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) is a rare and catastrophic clinical syndrome occurring in previously healthy patients. Aetiology is still unknown and outcome usually poor. We describe a case of myoclonic prolonged super refractory status epilepticus (P-SRSE) in FIRES in a patient admitted to the paediatric intensive care unit of Padova, Italy. CASE REPORT: A previously healthy 14-year-old girl with onset of myoclonic status epilepticus after a mild febrile illness was admitted to our hospital with a diagnosis of FIRES. Extensive diagnostic work-up was inconclusive. Status epilepticus and electroclinical seizures recurred every time weaning from anaesthetic agents was attempted. Eventually, a vagal nerve stimulator (VNS) was implanted and cannabidiol (CBD) administered, 43 days and 70 days after P-SRSE onset, respectively. Two days after CBD introduction, status epilepticus weaned and the girl rapidly regained complete consciousness showing a brilliant and unexpected recovery. At last follow-up, 12 months later, she is 8-months seizure free on multiple antiseizure medications, has only mild neuropsychological impairment with no neurological and intellective deficit. CONCLUSIONS: To our knowledge, this represents a unique case with an extremely favourable evolution with a possible effect of the association of VNS and CBD to traditional antiseizure medications.

Petrous apex's dural arteriovenous fistula (DAVF) mimicking a pineal region tumour: a rare cause of Hakim triad.

A dural arteriovenous fistula (DAVF) is a dural-based shunt between meningeal arteries and meningeal veins, sinuses and/or cortical veins; they have been classified and named according to the location and the flow pattern. Petrous apex DAVFs are located where the petrosal vein penetrates the dura mater into the superior petrosal sinus; there are only few cases reported in the literature, they can show an aggressive behaviour (subarachnoid haemorrhage, severe brainstem oedema) with a high mortality rate. The described case is, to the best of our knowledge, the first case of a DAVF presenting with symptoms mimicking idiopathic normal pressure hydrocephalus. After worsening of gait impairment, memory loss and urinary incontinence an urgent CT of the brain showed hydrocephalus and a hyperdense mass in the pineal region mimicking a pineal tumour; an emergent digital subtraction angiogram showed a left petrous apex Borden type III DAVF. A transvenous embolisation was performed obtaining a complete obliteration.

Transnasal Endoscopic Approach for Osteoid Osteoma of the Odontoid Process in a Child: Technical Note and Systematic Review of the Literature.

Osteoid osteoma (OO) is a primary benign tumor that accounts for up to 3% of all bone tumors. The cervical spine is less affected by OOs, and very few cases of C2 OOs have been reported in the literature, both in adults and children. Surgery may be required in case of functional torticollis, stiffness, and reduced range of motion (ROM) due to cervical OOs refractory to medical therapy. Several posterior and anterior surgical techniques have been described to remove C2 OOs. In particular, anterior approaches to the cervical spine represent the most used surgical route for treating C2 OOs. We describe the first case of OO of the odontoid process removed through a transnasal endoscopic approach with the aid of neuronavigation in a 6-year-old child. No intraoperative complications occurred, and the post-operative course was uneventful. The patient had immediate relief of neck pain and remained pain-free throughout the follow-up period, with complete functional recovery of the neck range of motion (ROM). In this case, based on the favorable anatomy, the transnasal endoscopic approach represented a valuable strategy for the complete removal of an anterior C2 OO without the need for further vertebral fixation since the preservation of ligaments and paravertebral soft tissue.

Preoperative Repetitive Navigated TMS and Functional White Matter Tractography in a Bilingual Patient with a Brain Tumor in Wernike Area.

Awake surgery and intraoperative neuromonitoring represent the gold standard for surgery of lesion located in language-eloquent areas of the dominant hemisphere, enabling the maximal safe resection while preserving language function. Nevertheless, this functional mapping is invasive; it can be executed only during surgery and in selected patients. Moreover, the number of neuro-oncological bilingual patients is constantly growing, and performing awake surgery in this group of patients can be difficult. In this scenario, the application of accurate, repeatable and non-invasive preoperative mapping procedures is needed, in order to define the anatomical distribution of both languages. Repetitive navigated transcranial magnetic stimulation (rnTMS) associated with functional subcortical fiber tracking (nTMS-based DTI-FT) represents a promising and comprehensive mapping tool to display language pathway and function reorganization in neurosurgical patients. Herein we report a case of a bilingual patient affected by brain tumor in the left temporal lobe, who underwent rnTMS mapping for both languages (Romanian and Italian), disclosing the true eloquence of the anterior part of the lesion in both tests. After surgery, language abilities were intact at follow-up in both languages. This case represents a preliminary application of nTMS-based DTI-FT in neurosurgery for brain tumor in eloquent areas in a bilingual patient.