Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Advanced Genetic Testing Comes to the Pain Clinic to Make a Diagnosis of Paroxysmal Extreme Pain Disorder Thu, 21 Jul 2016 12:14:22 +0000 Objective. To describe the use of an advanced genetic testing technique, whole exome sequencing, to diagnose a patient and their family with a SCN9A channelopathy. Setting. Academic tertiary care center. Design. Case report. Case Report. A 61-year-old female with a history of acute facial pain, chronic pain, fibromyalgia, and constipation was found to have a gain of function SCN9A mutation by whole exome sequencing. This mutation resulted in an SCN9A channelopathy that is most consistent with a diagnosis of paroxysmal extreme pain disorder. In addition to the patient being diagnosed, four siblings have a clinical diagnosis of SCN9A channelopathy as they have consistent symptoms and a sister with a known mutation. For treatment, gabapentin was ineffective and carbamazepine was not tolerated. Nontraditional therapies improved symptoms and constipation resolved with pelvic floor retraining with biofeedback. Conclusion. Patients with a personal and family history of chronic pain may benefit from a referral to Medical Genetics. Pelvic floor retraining with biofeedback should be considered for patients with a SCN9A channelopathy and constipation. Ashley Cannon, Svetlana Kurklinsky, Kimberly J. Guthrie, and Douglas L. Riegert-Johnson Copyright © 2016 Ashley Cannon et al. All rights reserved. Diagnostic Challenges of Cryptococcus neoformans in an Immunocompetent Individual Masquerading as Chronic Hydrocephalus Wed, 20 Jul 2016 15:05:28 +0000 Cryptococcus neoformans can cause disseminated meningoencephalitis and evade immunosurveillance with expression of a major virulence factor, the polysaccharide capsule. Direct diagnostic assays often rely on the presence of the cryptococcal glucuronoxylomannan capsular antigen (CrAg) or visualization of the capsule. Strain specific phenotypic traits and environmental conditions influence differences in expression that can thereby compromise detection and timely diagnosis. Immunocompetent hosts may manifest clinical signs and symptoms indolently, often expanding the differential and delaying appropriate treatment and diagnosis. We describe a 63-year-old man who presented with a progressive four-year history of ambulatory dysfunction, headache, and communicating hydrocephalus. Serial lumbar punctures (LPs) revealed elevated protein (153–300 mg/dL), hypoglycorrhachia (19–47 mg/dL), lymphocytic pleocytosis (89–95% lymphocyte, WBC 67–303 mg/dL, and RBC 34–108 mg/dL), and normal opening pressure (13–16 cm H2O). Two different cerebrospinal fluid (CSF) CrAg assays were negative. A large volume CSF fungal culture grew unencapsulated C. neoformans. He was initiated on induction therapy with amphotericin B plus flucytosine and consolidation/maintenance therapy with flucytosine, but he died following discharge due to complications. Elevated levels of CSF Th1 cytokines and decreased IL6 may have affected the virulence and detection of the pathogen. Kedar R. Mahajan, Amity L. Roberts, Mark T. Curtis, Danielle Fortuna, Robin Dharia, and Lori Sheehan Copyright © 2016 Kedar R. Mahajan et al. All rights reserved. Horner’s Syndrome due to a Spontaneous Internal Carotid Artery Dissection after Deep Sea Scuba Diving Wed, 20 Jul 2016 13:39:17 +0000 Internal carotid artery dissection (ICAD) is a rare entity that either results from traumatic injury or can be spontaneously preceded or not by a minor trauma such as sporting activities. It represents a major cause of stroke in young patients. The diagnosis should be suspected with the combination of Horner’s syndrome, headache or neck pain, and retinal or cerebral ischaemia. The confirmation is frequently made with a magnetic resonance angiography (MRA). Although anticoagulation with heparin followed by vitamin-K-antagonists is the most common treatment, there is no difference in efficacy of antiplatelet and anticoagulant drugs at preventing stroke and death in patients with symptomatic carotid dissection. We describe a patient with ICAD following deep sea scuba diving, who presented with Horner’s syndrome and neck pain and was successfully treated with anticoagulants. Jose Enrique Alonso Formento, Jose Luis Fernández Reyes, Blanca Mar Envid Lázaro, Teresa Fernández Letamendi, Ryth Yeste Martín, and Francisco José Jódar Morente Copyright © 2016 Jose Enrique Alonso Formento et al. All rights reserved. Carotid Space Mass Proximal to Vagus Nerve Causing Asystole and Syncope Tue, 19 Jul 2016 14:31:34 +0000 Manipulation of vagal nerve rootlets, whether surgical or through mass effect of a neoplasm, can result in asystole and hypotension, accompanied by ST depression and right bundle branch block. There are few case reports of a neoplasm causing these effects, and this case describes a patient with such a mass presenting with syncopal episodes. A 43-year-old man with a past medical history of HIV, bipolar disorder, and epilepsy was admitted to the neurology service for a video electroencephalogram (vEEG) to characterize syncopal episodes that were felt to be epileptic in origin. During the study, he experienced symptoms of his typical aura, which correlated with a transient symptomatic high degree AV block on telemetry, and an absence of epileptic findings on vEEG. Magnetic Resonance Imaging (MRI) of the brain showed a mass in the left posterior carotid space at the skull base. The patient underwent permanent dual chamber MRI-compatible pacemaker placement for his heart block. His syncopal episodes resolved, but presyncopal symptoms persisted. We discuss the presentation and treatment of vagal neoplasms. Julie Leviter and Daniel H. Wiznia Copyright © 2016 Julie Leviter and Daniel H. Wiznia. All rights reserved. Acute Disseminated Encephalomyelitis following Vaccination against Hepatitis B in a Child: A Case Report and Literature Review Tue, 12 Jul 2016 14:08:57 +0000 Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system, which has been associated with several vaccines such as rabies, diphtheria-tetanus-polio, smallpox, measles, mumps, rubella, Japanese B encephalitis, pertussis, influenza, and the Hog vaccine. Here, we presented a case of 12-year-old child who suffered from ADEM three weeks after hepatitis B vaccination. He was admitted to our hospital with symptoms of weakness of limbs, high fever, and alteration of consciousness. Some abnormalities were also found in CSF. Treatment with high-dose corticosteroids and intravenous immunoglobulin had significant effect, with marked improvement of the clinical symptoms and the results of CSF. The findings of MRI also detected some abnormal lesions located in both brain and spinal cord. The clinical features, the findings of CSF and MRI, and therapeutic effect may contribute to such diagnosis of ADEM. Jun-liang Yuan, Shuang-kun Wang, Xiao-juan Guo, and Wen-li Hu Copyright © 2016 Jun-liang Yuan et al. All rights reserved. Acute Paraplegia due to Thoracic Hematomyelia Tue, 12 Jul 2016 09:11:28 +0000 Spontaneous intraspinal intramedullary hemorrhage is a rare entity with the acute onset of neurologic symptoms. The etiology of idiopathic spontaneous hematomyelia (ISH) is unknown, and there are few published case reports. Hematomyelia is mostly associated with trauma, but the other nontraumatic etiologies are vascular malformations, tumors, bleeding disorders, syphilis, syrinx, and myelitis. MRI is a good choice for early diagnosis. Hematomyelia usually causes acute spinal cord syndrome due to the compression and destruction of the spinal cord. A high-dose steroid treatment and surgical decompression and evacuation of hematoma are the urgent solution methods. We present idiopathic spontaneous hematomyelia of a previously healthy 80-year-old male with a sudden onset of back pain and paraplegia. Aykut Akpınar, Bahattin Celik, Ihsan Canbek, and Ergun Karavelioğlu Copyright © 2016 Aykut Akpınar et al. All rights reserved. Paradoxical Herniation following Decompressive Craniectomy in the Subacute Setting Thu, 30 Jun 2016 10:27:35 +0000 Decompressive craniectomy is reserved for extreme cases of intracranial hypertension. An uncommon complication known as paradoxical herniation has been documented within weeks to months following surgery. Here we present a unique case within days of surgery. Since standard medical treatment for intracranial hypertension will exacerbate paradoxical herniation, any abrupt neurological changes following decompressive craniectomy should be carefully investigated. Immediate treatment for paradoxical herniation is placement of the patient in the supine position with adequate hydration. Cranioplasty is the ultimate treatment option. Alex P. Michael and Jose Espinosa Copyright © 2016 Alex P. Michael and Jose Espinosa. All rights reserved. Management of a Low-Energy Penetrating Brain Injury Caused by a Nail Sun, 26 Jun 2016 13:28:12 +0000 Low-energy penetrating nail injury to the brain is an extremely rare neurosurgical emergency. The most common cause of nail gun injury is work related accidents; other causes result from accidental firing of a nail gun, suicide attempts by firing nail guns into the brain, and bomb blasts containing pieces of nails. Neurosurgical treatment performed by craniotomy still seems to be the safest one; there are reports of complications such as subdural hematoma and intraparenchymal hemorrhages following the blind removal of foreign bodies leading to suggestions that all penetrating foreign bodies should be removed under direct vision. We report a rarely described neurosurgical approach for removal of a penetrating nail from the brain and skull without evidence of associated hematoma and other brain lesions. V. R. Ferraz, G. B. Aguiar, J. L. Vitorino-Araujo, G. L. Badke, and J. C. E. Veiga Copyright © 2016 V. R. Ferraz et al. All rights reserved. Reversible Akinetic Mutism after Aneurysmal Subarachnoid Haemorrhage in the Territory of the Anterior Cerebral Artery without Permanent Ischaemic Damage to Anterior Cingulate Gyri Thu, 23 Jun 2016 11:47:02 +0000 We report on two cases of transient akinetic mutism after massive subarachnoid haemorrhage due to the rupture of an intracranial aneurysm of the anterior cerebral artery (ACA). In the two cases, vasospasm could not be demonstrated by imaging studies throughout the clinical course. Both patients shared common radiological features: a hydrocephalus due to haemorrhagic contamination of the ventricular system and a mass effect of a subpial hematoma on the borders of the corpus callosum. Patients were also investigated using auditory event-related evoked potentials at acute stage. In contrast to previous observations of akinetic mutism, P300 wave could not be recorded. Both patients had good recovery and we hypothesized that this unexpectedly favourable outcome was due to the absence of permanent structural damage to the ACA territory, with only transient dysfunction due to a reversible mass effect on cingulate gyri. François-Xavier Sibille, Philippe Hantson, Thierry Duprez, Vincent van Pesch, and Simone Giglioli Copyright © 2016 François-Xavier Sibille et al. All rights reserved. Posterior Reversible Encephalopathy Syndrome with Bilateral Independent Epileptic Foci Precipitated By Guillain-Barrè Syndrome Wed, 15 Jun 2016 11:17:47 +0000 We report the case of a 56-year-old woman who developed status epilepticus (SE) related to independent occipital foci as clinical manifestation of posterior reversible encephalopathy syndrome (PRES) in the background of Guillain-Barrè syndrome (GBS). SE resulted from a series of focal seizures clinically characterized by left- and rightward deviations of the head and consequent oculoclonic movements. Electroencephalography recorded independent seizure activity in both occipital regions with alternate involvement of the two cerebral hemispheres. The epileptic foci corresponded topographically to parenchymal abnormalities of PRES in the occipital lobes. The manifestation of bilateral, independent occipital seizures with alternate deviations of the head and oculoclonic movements, previously not reported in patients with PRES, highlights the acute epileptogenicity of the cerebral lesions in this syndrome. Despite the variable clinical expression of seizures due to occipital damage in PRES, the development of independent seizure activity in both occipital lobes might represent a distinctive epileptic phenomenon of this encephalopathy. Rosario Rossi, Maria Valeria Saddi, Alessandro Mela, and Anna Ticca Copyright © 2016 Rosario Rossi et al. All rights reserved. Lesion in Scalp and Skull as the First Manifestation of Hepatocellular Carcinoma Tue, 14 Jun 2016 11:54:57 +0000 Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver and the fifth most common cancer in the world. The lungs, bone, and lymph nodes are frequent sites of metastasis of HCC. The purpose of the present study is show that metastases, although rare, must be among the differential diagnosis of skin lesions and that a diagnostic research based on these findings can be conducted. The authors report a rare case of metastatic hepatocellular injury to the scalp and skull treated by a radical surgical approach. Excision of the lesion in the scalp was performed “en bloc.” The tumor was supplied by the frontal branch of the superficial temporal artery. There are few case reports of metastatic HCC to scalp and skull; treatment of these lesions should be individualized in order to control symptoms, improve quality of life, and promote an increase in survival. V. R. Ferraz, J. L. Vitorino-Araújo, L. Sementilli, J. F. Neto, and J. C. E. Veiga Copyright © 2016 V. R. Ferraz et al. All rights reserved. GNE Myopathy in Turkish Sisters with a Novel Homozygous Mutation Thu, 19 May 2016 13:22:19 +0000 Background. Hereditary inclusion body myopathy is caused by biallelic defects in the GNE gene located on chromosome 9p13. It generally affects adults older than 20 years of age. Methods and Results. In this study, we present two Turkish sisters with progressive myopathy and describe a novel mutation in the GNE gene. Both sisters had slightly higher levels of creatine kinase (CK) and muscle weakness. The older sister presented at 38 years of age with an inability to climb steps, weakness, and a steppage gait. Her younger sister was 36 years old and had similar symptoms. The first symptoms of the disorder were seen when the sisters were 30 and 34 years old, respectively. The muscle biopsy showed primary myopathic features and presence of rimmed vacuoles. DNA analysis demonstrated the presence of previously unknown homozygous mutations [c.2152 G>A (p.A718T)] in the GNE genes. Conclusion. Based on our literature survey, we believe that ours is the first confirmed case of primary GNE myopathy with a novel missense mutation in Turkey. These patients illustrate that the muscle biopsy is still an important method for the differential diagnosis of vacuolar myopathies in that the detection of inclusions is required for the definitive diagnosis. Gulden Diniz, Yaprak Secil, Serdar Ceylaner, Figen Tokucoglu, Sabiha Türe, Mehmet Celebisoy, Tülay Kurt İncesu, and Galip Akhan Copyright © 2016 Gulden Diniz et al. All rights reserved. Nonvisualization of the Internal Carotid Artery on Computed Tomography Angiography: Discussion of Two Cases with Review of Literature Thu, 19 May 2016 10:58:09 +0000 Nonvisualization of the internal carotid artery (ICA) on cross-sectional imaging studies can be due to congenital (dysgenesis of the ICA) or acquired (complete occlusion of ICA) causes. We report two cases, one with absent carotid canal on bone window setting of computed tomography (CT) suggestive of congenital cause and the other with normal carotid canal, suggesting acquired cause. Development of aortic arches with six pathways of collateral circulation in brain is also discussed. Sonal Saran, Rengarajan Rajagopal, Pushpinder S. Khera, and Neeraj Mehta Copyright © 2016 Sonal Saran et al. All rights reserved. Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis Tue, 17 May 2016 12:56:17 +0000 Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete external ophthalmoplegia without ptosis, cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of diplopia at four-week follow-up. Conclusions. This patient’s recurrence of diplopia was treated with a five-week course of oral corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness. Scott A. McLeod, Wallace Wee, Francois D. Jacob, Isabelle Chapados, and Francois V. Bolduc Copyright © 2016 Scott A. McLeod et al. All rights reserved. Morvan Syndrome Secondary to Thymic Carcinoma in a Patient with Systemic Lupus Erythematosus Tue, 10 May 2016 16:32:11 +0000 Morvan syndrome (MoS) is a rare paraneoplastic autoimmune disorder characterized by peripheral nerve hyperexcitability, autonomic dysfunction, and sleep disorders. Systemic lupus erythmatosus (SLE) cooccurs in 6–10% of patients with thymoma. It may occur before, concurrently with, or after thymoma diagnosis. This paper reports the first case of cooccurrence of SLE, thymic carcinoma, and MoS. The cooccurrence of SLE, thymoma, and MoS delineates the generalized autoimmunity process. Symptoms of both MoS and SLE abated upon tumor resection. Gabrielle Macaron, Elie El Rassy, and Salam Koussa Copyright © 2016 Gabrielle Macaron et al. All rights reserved. Primary Intracranial Synovial Sarcoma Mon, 09 May 2016 11:33:58 +0000 Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. Mohit Patel, Luyuan Li, Ha Son Nguyen, Ninh Doan, Grant Sinson, and Wade Mueller Copyright © 2016 Mohit Patel et al. All rights reserved. High-Grade Glioma of the Ventrolateral Medulla in an Adult: Case Presentation and Discussion of Surgical Considerations Sun, 08 May 2016 12:24:23 +0000 Background. High-grade gliomas of the brainstem are rare in adults and are particularly rare in the anterolateral medulla. We describe an illustrative case and discuss the diagnostic and treatment issues associated with a tumor in this location, including differential diagnosis, anatomical considerations for options for surgical management, multimodality treatment, and prognosis. Case Description. A 69-year-old woman presented with a 3-week history of progressive right lower extremity weakness. She underwent an open biopsy via a far lateral approach with partial condylectomy, which revealed a glioblastoma. Concurrent temozolomide and radiation were completed; however, she elected to stop her chemotherapy after 5.5 weeks of treatment. She succumbed to her disease 11 months after diagnosis. Conclusions. Biopsy can be performed relatively safely to provide definitive diagnosis to guide treatment, but long-term prognosis is poor. Angela Spurgeon, Viet Le, Sanjay Konakondla, Douglas C. Miller, Tamera Hopkins, and N. Scott Litofsky Copyright © 2016 Angela Spurgeon et al. All rights reserved. Imaging Evidence for Cerebral Hyperperfusion Syndrome after Intravenous Tissue Plasminogen Activator for Acute Ischemic Stroke Tue, 03 May 2016 09:22:01 +0000 Background. Cerebral hyperperfusion syndrome (CHS), a rare complication after cerebral revascularization, is a well-described phenomenon after carotid endarterectomy or carotid artery stenting. However, the imaging evidence of CHS after intravenous tissue plasminogen activator (iv tPA) for acute ischemic stroke (AIS) has not been reported. Case Report. Four patients were determined to have manifestations of CHS with clinical deterioration after treatment with iv tPA, including one patient who developed seizure, one patient who had a deviation of the eyes toward lesion with worsened mental status, and two patients who developed worsened hemiparesis. In all four patients, postthrombolysis head CT examinations were negative for hemorrhage; CT angiogram showed patent cervical and intracranial arterial vasculature; CT perfusion imaging revealed hyperperfusion with increased relative cerebral blood flow and relative cerebral blood volume and decreased mean transit time along with decreased time to peak in the clinically related artery territory. Vascular dilation was also noted in three of these four cases. Conclusions. CHS should be considered in patients with clinical deterioration after iv tPA and imaging negative for hemorrhage. Cerebral angiogram and perfusion studies can be useful in diagnosing CHS thereby helping with further management. Yi Zhang, Abhay Kumar, John B. Tezel, and Yihua Zhou Copyright © 2016 Yi Zhang et al. All rights reserved. Miller-Fisher Syndrome: Are Anti-GAD Antibodies Implicated in Its Pathophysiology? Sat, 30 Apr 2016 13:37:51 +0000 Miller-Fisher syndrome (MFS) is considered as a variant of the Guillain-Barre syndrome (GBS) and its characteristic clinical features are ophthalmoplegia, ataxia, and areflexia. Typically, it is associated with anti-GQ1b antibodies; however, a significant percentage (>10%) of these patients are seronegative. Here, we report a 67-year-old female patient who presented with the typical clinical features of MFS. Workup revealed antibodies against glutamic acid decarboxylase (GAD) in relatively high titers while GQ1b antibodies were negative. Neurological improvement was observed after intravenous gamma globulin and follow-up examinations showed a continuous clinical amelioration with simultaneous decline of anti-GAD levels which finally returned to normal values. This case indicates that anti-GAD antibodies may be associated with a broader clinical spectrum and future studies in GQ1b-seronegative patients could determine ultimately their clinical and pathogenetic significance in this syndrome. Ioannis E. Dagklis, Sotirios Papagiannopoulos, Varvara Theodoridou, Dimitrios Kazis, Ourania Argyropoulou, and Sevasti Bostantjopoulou Copyright © 2016 Ioannis E. Dagklis et al. All rights reserved. Neurological Complications of Middle East Respiratory Syndrome Coronavirus: A Report of Two Cases and Review of the Literature Thu, 28 Apr 2016 06:20:12 +0000 Middle East Respiratory Syndrome Coronavirus (MERS-CoV) was first discovered in September 2012 in Saudi Arabia. Since then, it caused more than 1600 laboratory-confirmed cases and more than 580 deaths among them. The clinical course of the disease ranges from asymptomatic infection to severe lower respiratory tract illness with multiorgan involvement and death. The disease can cause pulmonary, renal, hematological, and gastrointestinal complications. In this paper, we report neurological complications of MERS-CoV in two adult patients, and we hypothesize the pathophysiology. The first patient had an intracerebral hemorrhage as a result of thrombocytopenia, disseminated intravascular coagulation, and platelet dysfunction. The second case was a case of critical illness polyneuropathy complicating a long ICU stay. In these cases, the neurological complications were secondary to systemic complications and long ICU stay. Autopsy studies are needed to further understand the pathological mechanism. Hussein Algahtani, Ahmad Subahi, and Bader Shirah Copyright © 2016 Hussein Algahtani et al. All rights reserved. A Rare Case of Spontaneous Pneumocephalus Associated with Nontraumatic Cerebrospinal Fluid Leak Wed, 27 Apr 2016 14:25:00 +0000 Introduction. Spontaneous nontraumatic pneumocephalus (PNC) and cerebrospinal fluid (CSF) leaks are both very uncommon conditions. We report a rare case of spontaneous pneumocephalus associated with CSF leak secondary to right sphenoid sinus bony defect without history of trauma. Case Description. 51-year-old Hispanic female with past medical history of hypertension and idiopathic intracranial hypertension (Pseudotumor Cerebri) presented to the emergency room complaining of headache and clear discharge from the right nostril. Physical examination was significant for right frontal sinus tenderness and clear discharge from right nostril. Computed Tomography (CT) scan of the brain showed moderate amount of extra-axial air within the right cerebral hemisphere indicative of pneumocephalus. CT scan of facial bones showed bony defect along the right sphenoid sinus with abnormal CSF collection. The patient was started on intravenous antibiotics for meningitis prophylaxis and subsequently underwent transsphenoidal repair of cerebrospinal fluid leak with abdominal fat graft. CSF rhinorrhea stopped completely after the surgery with near complete resolution of pneumocephalus before discharge. Conclusions. Early identification of pneumocephalus and surgical intervention can help decrease the morbidity and avoid possible complications. Idiopathic intracranial hypertension, although rare, can lead to CSF leak and pneumocepahlus. Murad Baba, Omer Tarar, and Amer Syed Copyright © 2016 Murad Baba et al. All rights reserved. An Unusual Case of Asystole Occurring during Deep Brain Stimulation Surgery Wed, 27 Apr 2016 06:37:47 +0000 Background. Symptomatic bradycardia and hypotension in neurosurgery can produce severe consequences if not managed appropriately. The literature is scarce regarding its occurrence during deep brain stimulation (DBS) surgery. Case Presentation. A 67-year-old female presented for left DBS lead placement for essential tremors. During lead implantation, heart rate and blood pressure dropped rapidly; the patient became unresponsive and asystolic. Chest compressions were initiated and epinephrine was given. Within 30 seconds, the patient became hemodynamically stable and conscious. A head CT demonstrated no acute findings. After deliberation, a decision was made to complete the procedure. Assuming the etiology of the episode was the Bezold-Jarisch reflex (BJR), appropriate accommodations were made. The procedure was completed uneventfully. Conclusion. The episode was consistent with a manifestation of the BJR. The patient had a history of neurocardiogenic syncope and a relatively low-volume state, factors prone to the BJR. Overall, lead implantation can still occur safely if preventive measures are employed. Ha Son Nguyen, Harvey Woehlck, and Peter Pahapill Copyright © 2016 Ha Son Nguyen et al. All rights reserved. Endoport-Assisted Microsurgical Treatment of a Ruptured Periventricular Aneurysm Tue, 19 Apr 2016 10:56:02 +0000 Background and Importance. Ruptured periventricular aneurysms in patients with moyamoya disease represent challenging pathologies. The most common methods of treatment include endovascular embolization and microsurgical clipping. However, rare cases arise in which the location and anatomy of the aneurysm make these treatment modalities particularly challenging. Clinical Presentation. We report a case of a 34-year-old female with moyamoya disease who presented with intraventricular hemorrhage. CT angiography and digital subtraction angiography revealed an aneurysm located in the wall of the atrium of the right lateral ventricle. Distal endovascular access was not possible, and embolization risked the sacrifice of arteries supplying critical brain parenchyma. Using the BrainPath endoport system, the aneurysm was able to be accessed. Since the fusiform architecture of the aneurysm prevented clip placement, the aneurysm was ligated with electrocautery. Conclusion. We demonstrate the feasibility of endoport-assisted approach for minimally invasive access and treatment of uncommon, distally located aneurysms. Ching-Jen Chen, James Caruso, Robert M. Starke, Dale Ding, Thomas Buell, R. Webster Crowley, and Kenneth C. Liu Copyright © 2016 Ching-Jen Chen et al. All rights reserved. Profound Autonomic Instability Complicated by Multiple Episodes of Cardiac Asystole and Refractory Bradycardia in a Patient with Anti-NMDA Encephalitis Sun, 17 Apr 2016 10:22:40 +0000 Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is autoimmune encephalitis primarily affecting young adults and children. First described about a decade ago, it frequently manifests as a syndrome that includes progressive behavioral changes, psychosis, central hypoventilation, seizures, and autonomic instability. Although cardiac arrhythmias often accompany anti-NMDARE, the need for long-term electrophysiological support is rare. We describe the case of NMDARE whose ICU course was complicated by progressively worsening episodes of tachyarrhythmia-bradyarrhythmia and episodes of asystole from which she was successfully resuscitated. Her life-threatening episodes of autonomic instability were successfully controlled only after the placement of a permanent pacemaker during her ICU stay. She made a clinical recovery and was discharged to a skilled nursing facility after a protracted hospital course. Stephanie R. Mehr, Roy C. Neeley, Melissa Wiley, and Avinash B. Kumar Copyright © 2016 Stephanie R. Mehr et al. All rights reserved. S3 Dorsal Root Ganglion/Nerve Root Stimulation for Refractory Postsurgical Perineal Pain: Technical Aspects of Anchorless Sacral Transforaminal Lead Placement Wed, 30 Mar 2016 13:24:41 +0000 Chronic perineal pain limits patients in physical and sexual activities, leading to social and psychological distress. In most cases, this pain develops after surgery in the urogenital area or as a consequence of trauma. Neuromodulation is one of the options in chronic postsurgical perineal pain treatment. We present a case of refractory perineal pain after right sided surgical resection of a Bartholin’s cyst which was treated with third sacral nerve root/dorsal root ganglion stimulation using the transforaminal approach. We describe a new anchorless lead placement technique using a unique curved lead delivery sheath. We postulate that this new posterior foraminal technique of lead placement is simple, safe, and reversible and may lower the occurrence of lead related complications. X. Zuidema, J. Breel, and F. Wille Copyright © 2016 X. Zuidema et al. All rights reserved. Unusual Spread of Renal Cell Carcinoma to the Clivus with Cranial Nerve Deficit Tue, 15 Mar 2016 09:53:58 +0000 Renal cell carcinoma (RCC) has unusual presentation affecting elderly males with a smoking history. The incidence of RCC varies while the incidence of spread of RCC to the clivus is rare. The typicality of RCC presentation includes hematuria, flank pain, and a palpable flank mass; however, RCC can also present with clival metastasis. The unique path of the abducens nerve in the clivus makes it susceptible to damage in metastasis. We report a case of a 54-year-old African American female that was evaluated for back pain, weakness, numbness, and tingling of bilateral lower extremities and subsequently disconjugate gaze and diplopia. Brain MRI confirmed metastasis to the clivus. She was started on radiotherapy and was planned for chemotherapy and transfer to a nursing home. When a patient presents with sudden unusual cranial nerve pathology, the possibility of metastatic RCC should be sought. Jerome Okudo and Nwabundo Anusim Copyright © 2016 Jerome Okudo and Nwabundo Anusim. All rights reserved. Spinal Cord Infarction in a Patient with Hereditary Spherocytosis: A Case Report and Discussion Mon, 14 Mar 2016 13:19:37 +0000 The etiology of spinal cord infarcts (SCIs), besides being related to aortic perioperative events, in large subset of SCIs, remains cryptogenic. We present a first case of SCI in a patient with hereditary spherocytosis and discuss the potential pathophysiologic considerations for vascular compromise. A 43-year-old woman with a history of hereditary spherocytosis, post splenectomy status, presented with chest, back, and shoulder pain with subsequent myelopathic picture; SCI extending from C4-T2 was confirmed by MRI. Despite aggressive treatment her stroke progressed leading to her demise. Her autopsy confirmed the SCI and revealed some incidental findings, but the cause of SCI remained unidentified. Exclusion of the known etiologies of SCI by extensive negative workup including autopsy evaluation suggested that SCI in our case was related to her history of hereditary spherocytosis. Both venous and arterial adverse vascular events, at a higher rate, have been associated in patients with hereditary spherocytosis who had their spleens removed compared to nonsplenectomized patients. Postsplenectomy increases in the platelet, red blood cell count, leukocyte count, and cholesterol concentrations are postulated to contribute to increased thrombotic risk. Additional prothrombotic factors include continuous platelet activation and adhesion as well as abnormalities of the red blood cell membrane. Waqar Waheed, Anjali L. Varigonda, Chris E. Holmes, Christopher Trevino, Neil M. Borden, and W. Pendlebury Copyright © 2016 Waqar Waheed et al. All rights reserved. A Rare Case Report on Suboccipital Region Benign Giant Osteoma Wed, 09 Mar 2016 13:37:51 +0000 Herein we report a rare case of a giant suboccipital osteoma in a 55-year-old woman presenting primarily due to cosmetic issue. We discuss the management algorithm taken in the patient, highlighting excision of a potentially curable bony tumor only after ruling out its extension to the ear cavity, mastoid ear cells, transverse sinus, and the intracranial compartment. Sunil Munakomi and Binod Bhattarai Copyright © 2016 Sunil Munakomi and Binod Bhattarai. All rights reserved. Septic Encephalopathy Characterized by Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion and Early Nonconvulsive Status Epilepticus Wed, 09 Mar 2016 13:23:05 +0000 Infection, whether viral or bacterial, can result in various forms of brain dysfunction (encephalopathy). Septic encephalopathy (SE) is caused by an excessive immune reaction to infection, with clinical features including disturbed consciousness and seizures. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is usually accompanied by viral infection in children and is characterized by biphasic seizures and impaired consciousness. The initial neurologic symptom of AESD is typically a febrile seizure that frequently lasts longer than 30 minutes. However, the possible forms this seizure takes are unclear. For example, it is unknown if nonconvulsive status epilepticus (NCSE) could be an early seizure symptomatic of AESD. In addition, thus far no cases of combined SE and AESD have been reported. Here, we describe the first reported case of SE with AESD that notably demonstrated NCSE as an early seizure. Hiroshi Yamaguchi, Tsukasa Tanaka, Azusa Maruyama, and Hiroaki Nagase Copyright © 2016 Hiroshi Yamaguchi et al. All rights reserved. Cerebral Venous Thrombosis with Migraine-Like Headache and the Trigeminovascular System Wed, 17 Feb 2016 14:38:17 +0000 Cerebral venous thrombosis- (CVT-) associated headache is considered a secondary headache, commonly presenting as intracranial hypertension headache in association with seizures and/or neurological signs. However, it can occasionally mimic migraine. We report a patient presenting with a migraine-like, CVT-related headache refractory to several medications but intravenous dihydroergotamine (DHE). The response to DHE, which is considered to be an antimigraine medication, in addition to the neurovascular nature of migraine, points out to a probable similarity between CVT-headache and migraine. Based on experimental studies, we discuss this similarity and hypothesize a trigeminovascular role in the genesis of CVT-associated headache. Fábio A. Nascimento, Marília Grando Sória, Vanessa Rizelio, and Pedro A. Kowacs Copyright © 2016 Fábio A. Nascimento et al. All rights reserved.