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Copyright © 2020 Asian Journal of Neurosurgery.Moyamoya disease (MMD) is an incompletely understood malady that affects many age groups, primarily in a bimodal age distribution. We present a patient with the association of type 1 diabetes mellitus (type 1 DM) and MMD followed by a review of the existing literature. We found five papers that describe this association, in the form of one case report, one case series, and three retrospective reviews. Despite a poor understanding of the underlying pathophysiology, a clear association between autoimmune conditions and MMD appears to exist. Clinicians who manage such patients ought to be vigilant and have a high index of suspicion when young patients with type 1 DM present with new onset of neurological symptoms. Copyright © 2020 Asian Journal of Neurosurgery.Ependymoma is a rare tumor central nervous system that arises from the ependymal lining of the ventricles or the central canal of the spinal cord. They are of neuroectodermal in origin and constitute about 30%-86% of tumors arising in the spinal cord. The occurrence of these tumors in the cervicomedullary region is very rare. Sudden symptomatic neurologic presentations due to hemorrhage in cervicomedullary ependymoma is very rare and so far have never been reported. Mostly presenting as neurologic deficits involving limbs, these tumors pose a technical challenge in their removal. We present a patient who presented with sudden-onset dysesthesia of the upper and lower limbs. On imaging, he was found to have a cystic medullary tumor extending to the cervical region with hemorrhage. We discuss the epidemiology, surgical challenges, and outcome along with review of literature of these rare tumors located in this precarious location. Copyright © 2020 Asian Journal of Neurosurgery.Spontaneous cerebrospinal fluid (CSF) rhinorrhea is a rare occurrence. We present a case of spontaneous CSF rhinorrhea in a 57-year-old patient secondary to a sphenoid osseous defect involving the foramen rotundum and maxillary nerve with an associated arachnoid cyst and meningocele compressing the maxillary nerve. The location of the defect made correction amenable to an open skull-based approach. To the best of our knowledge, this is the first reported case of a spontaneous meningocele herniating into the sphenoid osseous defect through the medial aspect of the foramen rotundum. Apcin Early detection of these defects, open or endoscopic approaches and definitive treatment by closing the defect can result in excellent outcomes. Copyright © 2020 Asian Journal of Neurosurgery.Juxtafacet cysts and ligamentum flavum hematoma have the potential to cause acute root or spinal cord compression despite their low incidences. Their simultaneous presence with acute nerve compression has not been reported. Herein, we present a case who reported with low back and leg pain to the emergency department. Copyright © 2020 Asian Journal of Neurosurgery.Here, we report a case of idiopathic epidural lipomatosis presented with a clinical picture of lumbar canal stenosis with neurogenic claudication which resolved completely only by weight loss. A 53-year-old obese male with a body mass index of 36 without significant past medical history presented to the outpatient clinic with neurogenic claudication and bilateral sciatic radiculopathy. Initially, magnetic resonance imaging (MRI) showed epidural lipomatosis at the level of L5 vertebral body and L5-S1 intervertebral disc. A conservative treatment was decided with dietary regime program. After 6 months of follow-up, his bilateral sciatic radiculopathy disappeared, and updated MRI showed complete disappearance of epidural lipomatosis. Based on the Grand Round case and relevant literature, we present a case of an unusual epidural lipomatosis with mixed clinical picture of degenerative lumbar disease. This case report set out the importance of Borré classification for differentiating the mixed clinical complaint of degenerative discopathy and epidural lipomatosis. Copyright © 2020 Asian Journal of Neurosurgery.Anterior cranial fossa (ACF) dural arteriovenous fistulas (DAVFs) are mainly fed by the ethmoidal arteries and sometimes have pial arterial feeders. DAVFs with pial arterial supply in ACF are extremely rare because most of the reported cases of DAVFs with pial arterial supply are located at the transverse sigmoid sinus and tentorium. A 68-year-old male presented with dizziness. Angiography showed cortical venous reflex (CVR) through an ACF DAVF fed by both bilateral ethmoidal arteries and by the right orbitofrontal artery as a pial feeder. The ethmoidal feeders were disconnected by craniotomy. The pial arterial feeder from the anterior cerebral artery was not found during surgery, and disconnection of the draining vein was not performed. CVR showed a significant reduction after the surgery. After 2 years of follow-up, angiography revealed an increased shunt flow from the pial feeder. Endovascular treatment using n-butyl-2-cyanoacrylate was performed, resulting in the complete occlusion of the fistula. DAVFs with pial supply are reported to carry a high risk of perioperative complications because of the restriction of the venous outflow and retrograde thrombosis of the pial artery. Endovascular pial feeder occlusion after surgical dural arterial feeder disconnection might achieve a safe and effective outcome. With close follow-up, the recurrence of increased shunt flow may be an appropriate timing for additional treatment. This rare condition may offer a new insight into the mechanisms of pial feeder development. Copyright © 2020 Asian Journal of Neurosurgery.The dermal sinus tract of the spine is associated with other occult spinal dysraphisms, such as the split cord malformation (diastematomyelia) in a 40% of the cases and embryologically is not clearly defined if the dermal sinus and split cord malformation have origin in gastrulation or late primary neurulation, but the most accepted theory of the dermal sinus tract consists in early incomplete disjunction, which explains the relation with other spinal dysraphisms. Here, we present two cases, with a dermal sinus tract of the spine associated with Type I and Type II split cord malformation. Copyright © 2020 Asian Journal of Neurosurgery.