Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Thermoregulatory Instability in Childhood: Linking the Normal Brain to Hypothalamic Storm Wed, 26 Oct 2016 13:56:29 +0000 Central core temperature is tightly controlled by hypothalamic centers, a feature that makes sudden changes in body temperature very unusual. A dysfunction of these hypothalamic pathways leads to Shapiro’s syndrome, comprising spontaneous hypothermia, hyperhidrosis, and corpus callosum dysgenesis. Although it may affect any age, usually it presents in childhood. Variants to this syndrome with completely normal brain anatomy have been consistently reported, expanding the clinical spectrum of the syndrome. Herein, we report the case of a 4-year-old girl with Shapiro’s syndrome and unaffected corpus callosum. William Alves Martins, Rafael do Amaral Cristovam, Helena Fussiger, Viviane Maria Vedana, and Marta Hemb Copyright © 2016 William Alves Martins et al. All rights reserved. Hemorrhagic Longitudinally Extensive Transverse Myelitis Tue, 25 Oct 2016 08:42:04 +0000 Longitudinally extensive transverse myelitis (LETM) may be associated with viral triggers, including both infections and vaccinations. We present a case of a healthy immunocompetent 33-year-old woman who developed a hemorrhagic LETM 2 weeks after seasonal influenza vaccination. Hemorrhagic LETM has not to our knowledge been reported after influenza vaccination. It may represent a forme fruste variant of acute hemorrhagic leukoencephalitis. Chris Y. Wu, Tanawan Riangwiwat, and Beau K. Nakamoto Copyright © 2016 Chris Y. Wu et al. All rights reserved. Internal Carotid Artery Stenosis Presenting with Limb Shaking TIA Thu, 20 Oct 2016 14:35:47 +0000 Internal carotid artery (ICA) stenosis may lead to a wide range of clinical symptoms. We describe the case of a 66-year-old female who experienced a transient ischemic attack (TIA) with episodes of limb shaking caused by ICA stenosis. After epilepsy had been suspected and ruled out, studies of her left ICA showed extensive blockage as a result of atherosclerosis. Magnetic resonance angiography (MRA) revealed total occlusion of the left ICA and the patient was eventually medically managed due to the strong possibility of surgical complications. We reported this patient’s clinical course to shed light on a rare manifestation of carotid stenosis that may be confused with other diagnoses if not closely scrutinized. Awad Javaid and Mostafa Alfishawy Copyright © 2016 Awad Javaid and Mostafa Alfishawy. All rights reserved. Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease Thu, 20 Oct 2016 14:25:46 +0000 Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. On the contrary, ophthalmic involvement and localisation in the central nervous system are rare. Case Report. An old man was admitted to our hospital for first seizure. Brain imaging studies revealed on the left an extra-axial thickening of the dura mater with enhancement and perilesional oedema, infiltrating the sphenoorbital fissure and an isointense mass with enhancement in the orbital region with dislocation of the optic nerve. Pathological and immunohistochemistry examination of the bioptical specimen was consistent with a diagnosis of RDD. Treatment with levetiracetam and steroids was started obtaining only remission of seizures. Because of the patient refusal of the surgical debulking, therapy with mercaptopurine was started, stopping disease progression. Conclusion. So far, very few cases of extranodal RDD with multiple CNS lesions involving the orbital region have been described. Our case is significant because it is the first case in which the efficacy of mercaptopurine treatment has been documented in an adult patient with isolated ocular and intracranial RDD. Valentina Arnao, Marianna Riolo, Giovanni Savettieri, and Paolo Aridon Copyright © 2016 Valentina Arnao et al. All rights reserved. Stroke from Vasospasm due to Marijuana Use: Can Cannabis Synergistically with Other Medications Trigger Cerebral Vasospasm? Wed, 19 Oct 2016 12:22:08 +0000 We present a case of imaging proven cerebral vasospasm causing ischemic stroke in a young patient chronically on buprenorphine-naloxone for heroin remission who started smoking cannabis on a daily basis. With cannabis legalization spreading across the states in the USA, it is important for physicians not only to be aware of cannabis reported association with cerebral vasospasm in some patients but also to be on the lookout for possible interacting medications that can synergistically affect cerebral vessels causing debilitating strokes. Marium Jamil, Atif Zafar, Syed Adeel Faizi, and Ifrah Zawar Copyright © 2016 Marium Jamil et al. All rights reserved. Management of Stable Angina with Ivabradine as Safe Alternative to Patients with Myasthenia Gravis Sun, 16 Oct 2016 11:41:03 +0000 Management of cardiac symptoms in myasthenia gravis (MG) patients can be challenging. The aim of this report is to describe the safe use of ivabradine for stable angina in MG patients. A 48 y.o. woman, with MG diagnosis, presented stable angina. Therapies choices were reduced considering concomitant disease as well as previous and unsuccessful cardiologic managements. Ivabradine showed unexpected results. The patient presented an improvement of neurological and cardiac symptoms, bringing ivabradine as one more therapeutic option to similar patients. In this report we recommend ivabradine as an effective and safe drug for treatment of stable angina in MG patients. Giuliano Ohde Dalledone, Gustavo Lenci Marques, Renata Dal-Prá Ducci, Arnaldo Laffitte Stier Junior, Cláudia Suemi Kamoi Kay, Lineu Cesar Werneck, Paulo José Lorenzoni, and Rosana Herminia Scola Copyright © 2016 Giuliano Ohde Dalledone et al. All rights reserved. Familial Hemiplegic Migraine with Severe Attacks: A New Report with ATP1A2 Mutation Thu, 13 Oct 2016 07:31:02 +0000 Introduction. Familial hemiplegic migraine (FHM) is a rare disorder characterized by migraine attacks with motor weakness during the aura phase. Mutations in CACNA1A, ATP1A2, SCN1A, and PRRT2 genes have been described. Methods. To describe a mutation in ATP1A2 gene in a FHM case with especially severe and prolonged symptomatology. Results. 22-year-old woman was admitted due to migraine-type headache and sudden onset of right-sided weakness and aphasia; she had similar episodes in her childhood. Her mother was diagnosed with hemiplegic migraine without genetic confirmation. She presented with fever, decreased consciousness, left gaze preference, mixed aphasia, right facial palsy, right hemiplegia, and left crural paresis. Computed tomography (CT) showed no lesion and CT perfusion study evidenced oligohemia in left hemisphere. A normal brain magnetic resonance (MR) was obtained. Impaired consciousness and dysphasia began to improve three days after admission and mild dysphasia and right hemiparesis lasted for 10 days. No recurrences were reported during a follow-up of two years. We identified a variant in heterozygous state in ATP1A2 gene (p.Thr364Met), pathogenic according to different prediction algorithms (SIFT, PolyPhen2, MutationTaster, and Condel). Conclusion. Prolonged and severe attacks with diffuse hypoperfusion in a FHM seemed to be specially related to ATP1A2 mutations, and p.T364M should be considered. E. Martínez, R. Moreno, L. López-Mesonero, I. Vidriales, M. Ruiz, A. L. Guerrero, and J. J. Tellería Copyright © 2016 E. Martínez et al. All rights reserved. CNS Vasculitis Associated with Waldenström Macroglobulinemia Wed, 12 Oct 2016 07:49:29 +0000 Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient’s mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment. Tanawan Riangwiwat, Chris Y. Wu, Alberto S. Santos-Ocampo, Randal J. Liu, Aaron M. McMurtray, and Beau K. Nakamoto Copyright © 2016 Tanawan Riangwiwat et al. All rights reserved. A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset Mon, 10 Oct 2016 13:39:11 +0000 Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease. Various neurodegenerative disorders are manifested with CBS as a clinical phenotype, including corticobasal degeneration (CBD), progressive supranuclear palsy, Alzheimer’s disease, and CJD. In our patient, mutation E200K and M129M polymorphism of the PRNP gene and typical immunohistochemical findings pointed to a diagnosis of CJD. The patient’s mother died of CJD many years ago. Several CBS-CJD cases were described, but the atypical stroke-like onset of CBS-CJD, an extremely rare presentation of CJD, makes our case unique worldwide. Ján Necpál, Martin Stelzer, Silvia Koščová, and Michal Patarák Copyright © 2016 Ján Necpál et al. All rights reserved. Two Cases of Human T-Lymphotropic Virus Type I-Associated Myelopathy/Tropical Spastic Paraparesis Caused by Living-Donor Renal Transplantation Thu, 29 Sep 2016 16:57:14 +0000 In rare instances, recipients of organ transplants from human T-lymphotropic virus type I- (HTLV-I-) positive donors reportedly developed neurologic symptoms due to HTLV-I-associated myelopathy (HAM). We present herein two cases of HAM associated with renal transplantation from HTLV-I seropositive living-donors. The first patient was a 42-year-old woman with chronic renal failure for twelve years and seronegative for HTLV-I. She underwent renal transplantation with her HTLV-I seropositive mother as the donor, and she developed HAM three years after the transplantation. The second patient was a 65-year-old man who had been suffering from diabetic nephropathy. He was seronegative for HTLV-I and underwent renal transplantation one year previously, with his HTLV-I seropositive wife as the donor. He developed HAM eight months after renal transplantation. Both cases showed neurological improvements after the immunomodulating therapies. We tried to shed some light on the understanding of immunological mechanisms of transplantation-associated HAM, focusing on therapeutic strategies based on the immunopathogenesis of the condition. Yasutaka Tajima, Mariko Matsumura, Hiroaki Yaguchi, and Yasunori Mito Copyright © 2016 Yasutaka Tajima et al. All rights reserved. Embolic Stroke due to a Common Carotid Artery Thrombus in a Young Patient with Severe Iron-Deficiency Anemia without Thrombocytosis Mon, 26 Sep 2016 16:43:18 +0000 This case report describes a 41-year-old previously healthy male who presented with stuttering transient ischemic symptoms and radiographic evidence of a left common carotid artery thrombus as well as acute and subacute ischemic infarcts in the left middle cerebral artery territory. An exhaustive stroke work-up did not provide a plausible etiology for his symptoms. His complete blood count and iron studies, however, revealed evidence of severe iron-deficiency anemia without reactive thrombocytosis. His stool guaiac test was positive. He was discharged home on oral antithrombotic agents and aggressive iron replacement therapy with a plan for repeat vascular imaging in 3 months and a colonoscopy. This case report suggests that severe iron-deficiency anemia with or without reactive thrombocytosis should be viewed as a possible hematologic condition associated with thrombotic tendencies and a risk factor for ischemic stroke, especially in young adults. Aggressive iron supplementation and short-term antithrombotic therapy with follow-up vascular imaging are a reasonable treatment for these patients. David Roshal Copyright © 2016 David Roshal. All rights reserved. Diffusion Tensor Imaging Tractography in Pure Neuritic Leprosy: First Experience Report and Review of the Literature Wed, 21 Sep 2016 11:14:56 +0000 Five years after both right ulnar and median nerve decompression for paraesthesias and palsy, a patient, coming from Nigeria but living in Italy, came to our unit claiming to have persistent pain and combined median and ulnar palsy. Under suspicion of leprosy, skin and left sural nerve biopsy were performed. Skin tests were negative, but Schwann cells resulted as positive for acid-fast bacilli (AFB), leading to the diagnosis of Pure Neuritic Leprosy (PNL). The patient was given PB multidrug therapy and recovered from pain in two months. After nine months both High Resolution Ultrasonography (HRUS) and Magnetic Resonance Imaging (MRI) were performed, revealing thickening of the nerves. Since demyelination is common in PNL, the Authors started to use Diffusion Tensor Imaging Tractography (DTIT) to get better morphological and functional data about myelination than does the traditional imaging. DTIT proved successful in showing myelin discontinuity, reorganization, and myelination, and the Authors suggest that it can give more information about the evolution of the disease, as well as further indications for surgery (nerve decompression, nerve transfers, and babysitting for distal effector protection), and should be added to traditional imaging tools in leprosy. Michele R. Colonna, Giuseppe Tallarida, Francesco Stagno d’Alcontres, Salvatore Noto, Aurora Parodi, and Alberto Tagliafico Copyright © 2016 Michele R. Colonna et al. All rights reserved. A Case of West Nile Encephalitis That Develops into a Disease of Deep White Matter on MRI over a Two-Week Span Wed, 14 Sep 2016 13:07:02 +0000 We present a case of serologically proved West Nile encephalitis. This patient had a normal MRI on admission. Given that the patient’s clinical picture had worsened during her admission, a repeat MRI was performed, which demonstrated diffuse disease in the deep white matter of the brain. This is a case of West Nice encephalitis presenting as a disease of deep white matter that developed over a two-week span. Adam Puchalski and Antonio Liu Copyright © 2016 Adam Puchalski and Antonio Liu. All rights reserved. A Case Report of Neurosarcoidosis Presenting as a Lymphoma Mimic Thu, 08 Sep 2016 11:10:28 +0000 Objective. To describe a unique presentation of neurosarcoidosis. Background. Central nervous system involvement is rare in sarcoidosis. Sarcoidosis can be severe and can be mistaken for systemic lymphoma. Case Description. A 55-year-old right-handed white male with past medical history of obstructive sleep apnea, Raynaud’s disease, and Hashimoto’s thyroiditis was noted to have cognitive decline over a duration of few weeks and 20 lb weight loss. His neurologic exam (including cranial nerves) was normal except for five-minute recall. Head CT revealed a lacrimal gland mass, confirmed on brain MRI, which was suspicious for lymphoma on brain PET/MRI. Subsequent whole-body FDG PET/CT scan showed multiple enlarged lymph nodes. Bone marrow biopsy was negative. Serum and CSF ACE levels were within normal limits. Supraclavicular lymph node biopsy before steroids therapy was initiated and revealed multiple noncaseating granulomas, diagnostic of “sarcoidosis.” He was treated with daily prednisone for two months, followed by weekly infliximab. Brain MRI two months after treatment with prednisone showed decrease in size of lacrimal lesion, and brain PET/MRI showed normal brain metabolism pattern after five months. Neurocognitive evaluation three months after diagnosis demonstrated improvements in memory abilities. Discussion. Both clinically and radiographically, neurosarcoidosis can mimic systemic lymphoma. Biopsy in these types of cases is necessary to establish the diagnosis. Gurcharanjeet Kaur, Lauren Cameron, Olga Syritsyna, Patricia Coyle, and Agnes Kowalska Copyright © 2016 Gurcharanjeet Kaur et al. All rights reserved. Dementia Improvement after Plasma Exchange for Familial Hypercholesterolemia Thu, 08 Sep 2016 09:03:38 +0000 Worldwide dementia related memory issues affect a great number of patients and families. In this case, a “senior moment” was noted at age fifty and issues with memory and mind progressed resulting in early retirement from work. The patient described here was given a diagnosis of “Pre-Alzheimer’s disease” and presented for further accurate evaluation, diagnosis, and management. The medical management resulted in an improvement in the patients memory and cognitive ability. Allen J. Orehek Copyright © 2016 Allen J. Orehek. All rights reserved. A Patient with Eight Intracranial Aneurysms: Endovascular Treatment in Two Sessions Wed, 07 Sep 2016 10:32:54 +0000 The frequency of multiple intracranial aneurysms seen in patients with or without subarachnoid hemorrhage is high. The advancement of the endovascular technique and devices has ensured that endovascular treatment of intracranial aneurysms is the first choice in most cases, especially in unruptured ones. Different combinations of treatment modalities and techniques can be used in the management of multiple aneurysms. But in selected patients without subarachnoid hemorrhage, treatment of all aneurysms in one or more sessions with endovascular techniques is less traumatic than that with surgery. In the literature, the maximum number of aneurysms in one patient treated endovascularly and/or surgically is seven. In this case report, we present, with a review of the literature, a patient with eight intracranial aneurysms, all of which were treated in two sessions with various endovascular techniques. A 40-year-old female patient was admitted due to headache. Angiography showed eight aneurysms in the posterior circulation and, bilaterally, in the anterior circulation. All aneurysms were treated endovascularly in two sessions. In the treatment of the aneurysms, different endovascular techniques were used including flow diverters stents, stent-assisted coiling, Y-stent-assisted coiling, and coiling alone. Erol Akgul, Hasan Bilen Onan, Huseyin Tugsan Balli, and Nuri Eralp Cetinalp Copyright © 2016 Erol Akgul et al. All rights reserved. Atypical Features in a Large Turkish Family Affected with Friedreich Ataxia Wed, 07 Sep 2016 10:04:25 +0000 Here, we describe the clinical features of several members of the same family diagnosed with Friedreich ataxia (FRDA) and cerebral lesions, demyelinating neuropathy, and late-age onset without a significant cardiac involvement and presenting with similar symptoms, although genetic testing was negative for the GAA repeat expansion in one patient of the family. The GAA repeat expansion in the frataxin gene was shown in all of the family members except in a young female patient. MRI revealed arachnoid cysts in two patients; MRI was consistent with both cavum septum pellucidum-cavum vergae and nodular signal intensity increase in one patient. EMG showed demyelinating sensorimotor polyneuropathy in another patient. The GAA expansion-negative 11-year-old female patient had mental-motor retardation, epilepsy, and ataxia. None of the patients had significant cardiac symptoms. Description of FRDA families with different ethnic backgrounds may assist in identifying possible phenotypic and genetic features of the disease. Furthermore, the genetic heterogeneity observed in this family draws attention to the difficulty of genetic counseling in an inbred population and to the need for genotyping all affected members before delivering comprehensive genetic counseling. Semiha Kurt, Betul Cevik, Durdane Aksoy, E. Irmak Sahbaz, Aslı Gundogdu Eken, and A. Nazli Basak Copyright © 2016 Semiha Kurt et al. All rights reserved. Marchiafava-Bignami and Alcohol Related Acute Polyneuropathy: The Cooccurrence of Two Rare Entities Wed, 07 Sep 2016 09:22:55 +0000 Objectives. The aim of this article is to represent the first reported case with cooccurrence of two rare alcohol related complications. Case Report. We report a 38-year-old man with chronic alcoholism who presented with both cranial and peripheral nerve palsy. On MRI examination characteristic findings of Marchiafava-Bignami disease were recognized. Discussion. Marchiafava-Bignami disease (MBD) is a rare complication of long-term, heavy alcohol abuse that has characteristic MRI findings. Acute alcohol related polyneuropathy (AARP) is another rare and not-well-understood complication of chronic alcohol abuse. We could not find any previous report of the cooccurrence of these two complications in the literature. Samine Boloursaz, Sirous Nekooei, Farrokh Seilanian Toosi, Hossein Rezaei-Dalouei, Behrooz Davachi, Sahar Kazemi, and Bita Abbasi Copyright © 2016 Samine Boloursaz et al. All rights reserved. Parkinson’s Disease and Cryptogenic Epilepsy Mon, 05 Sep 2016 14:02:12 +0000 Epilepsy is an uncommon comorbidity of Parkinson’s disease (PD) and has been considered not directly associated with PD. We present five patients (3 men and 2 women; ages 49–85) who had concomitant PD and cryptogenic epilepsy. Although rare, epilepsy can coexist with PD and their coexistence may influence the progression of PD. While this may be a chance association, an evolving understanding of the neurophysiological basis of either disease may suggest a mechanistic association. Andre Y. Son, Milton C. Biagioni, Dorian Kaminski, Alec Gurevich, Britt Stone, and Alessandro Di Rocco Copyright © 2016 Andre Y. Son et al. All rights reserved. A Case of Painless Legs and Moving Toes Syndrome in Parkinson’s Disease Responsive to Dopaminergic Therapy Sun, 28 Aug 2016 11:57:54 +0000 Painless Legs and Moving Toes Syndrome (PoLMT) is a rare movement disorder characterized by flexion, extension, abduction, adduction, and torsion of toes without pain. It is considered a variant of Painful Legs and Moving Toes Syndrome (PLMT), which is characterized by similar movements but with pain. Although neuropathy and several central nervous system (CNS) involvements have been reported to be associated with PoLMT, the actual cause and mechanism remain unclear. Here we describe the first case of PoLMT in Parkinson’s Disease (PD), parallel to parkinsonism in severity, who demonstrated a good response to dopaminergic therapy. Sumihiro Kawajiri, Yasunobu Hoshino, Ryota Nakamura, Kazuyuki Noda, Yuji Tomizawa, Nobutaka Hattori, and Yasuyuki Okuma Copyright © 2016 Sumihiro Kawajiri et al. All rights reserved. Internal Occipital Crest Misalignment with Internal Occipital Protuberance: A Case Report of Posterior Cranial Fossa Anatomic Variations Thu, 25 Aug 2016 16:27:57 +0000 During gross anatomy head and neck laboratory session, one dissection group observed an abnormal anatomic variation in the posterior cranial fossa of a 94-year-old male cadaver. The internal occipital crest was not aligned with internal occipital protuberance and groove for superior sagittal sinus. It seemed that the internal occipital protuberance was shifted significantly to the right side. As a result the skull was overly stretched in order to connect with the internal occipital ridge. These internal skull variations of occipital bone landmarks can influence the location of adjacent dural venous sinuses and possibly influence cerebrospinal fluid flow. Similar anatomical anomalies have been attributed to presence of hydrocephalus and abnormalities in cisterna magna. Jae Ha Kim and Maha Ahmad Copyright © 2016 Jae Ha Kim and Maha Ahmad. All rights reserved. FLAIR Hyperintense Vessel Sign of Both MCAs with Severe Heart Failure Thu, 25 Aug 2016 08:09:05 +0000 Introduction. Fluid-attenuated inversion recovery hyperintense vessels (FHVs) can be seen in patients with occlusion or severe stenosis of the cerebral arteries. FHVs are known to reflect stagnant or slow blood flow within the cerebral artery. Case Report. A 75-year-old woman presented with suddenly developed gait disturbance. She had a history of hypertension, heart failure, and dementia. Brain MRI demonstrated FHVs within both middle cerebral arteries (MCAs). However, there was no acute ischemic lesion and severe stenosis or occlusion of the cerebral arteries. In the baseline routine laboratory investigations, the AST, ALT, and B-type natriuretic peptide levels were elevated. Transthoracic echocardiography (TTE) showed mitral valve prolapse with severe regurgitation. Blood pressure control and conservative management for ischemic hepatitis were performed. After 7 days, the transaminase levels were normalized, and the patient was able to walk with normal gait. Conclusions. In this patient, underlying chronic cerebral hypoperfusion and additionally decreased systemic perfusion seemed to provoke ischemic hepatitis and contribute to the development of FHVs. Donghee Kim, Seung-Yul Lee, and Kwon-Duk Seo Copyright © 2016 Donghee Kim et al. All rights reserved. Colon Adenoma Implicating Myasthenia Gravis: A Case Report of a Patient with Postcolectomy Complications Wed, 17 Aug 2016 14:01:51 +0000 We report the case of a 63-year-old patient with myasthenia gravis (MG) due to acetylcholine receptor antibodies (AChR) who underwent colectomy due to colon adenoma and developed myasthenic crisis and anastomosis leakage after surgery. The patient underwent two plasma exchanges, 4 and 6 days preoperatively, and received intravenous prednisolone and immunoglobulin infusion due to the crisis, which included primarily bulbar symptoms. The patient developed on the 10th postoperative day bowel obstruction symptoms and anastomosis leakage which required surgical repair and ileostomy. Bowel obstruction occurred in a patient with AChR related myasthenia after plasma exchange and during immunosuppression although it is more commonly reported in patients with thymoma related myasthenia. Y. Papachatzakis, E. Tseliou, I. Tatouli, I. Dialoupi, F. Michas, E. Papadopoulou, D. Kousouris, S. Kontogiannis, and M. A. Dimopoulos Copyright © 2016 Y. Papachatzakis et al. All rights reserved. Adult Primary Spinal Epidural Extraosseous Ewing’s Sarcoma: A Case Report and Review of the Literature Wed, 17 Aug 2016 07:59:29 +0000 Background. Extraosseous Ewing’s sarcoma in the spinal epidural space is a rare malignancy, especially in adults. Case Presentation. A 40-year-old male presented with back pain and urinary hesitancy. MRI revealed a thoracic extradural mass with no osseous involvement. He underwent surgery for gross total resection of the mass, which was diagnosed as Ewing’s sarcoma. He was subsequently treated with chemoradiotherapy. He remains disease-free 1 year after surgery. Review of the literature indicated only 45 previously reported cases of spinal epidural extraosseous Ewing’s sarcoma in adults. Conclusions. Extraosseous Ewing’s sarcoma in the spinal epidural space is a rare clinical entity that should be included in the differential for spinal epidural masses. Its treatment is multidisciplinary but frequently requires surgical intervention due to compressive neurologic symptoms. Gross total resection appears to correlate with improved outcomes. Mark Bustoros, Cheddhi Thomas, Joshua Frenster, Aram S. Modrek, N. Sumru Bayin, Matija Snuderl, Gerald Rosen, Peter B. Schiff, and Dimitris G. Placantonakis Copyright © 2016 Mark Bustoros et al. All rights reserved. Aphasic Dystextia as Presenting Feature of Ischemic Stroke in a Pediatric Patient Sun, 07 Aug 2016 14:31:23 +0000 Aphasia is an important presenting symptom of acute stroke. With increasing reliance on electronic communication, incoherent texting or “dystextia,” which is a subset of aphasia that is reflected in text messages, can be a useful tool for symptom recognition and analysis. It can be a red flag for the family and therefore can help in early identification of an acute neurological deficit. It is also useful for providers to reliably analyze the deficit as well as establish a timeline of evolution of symptoms. There have been case reports where dystextia has been the presenting feature of stroke or complicated migraine and in one case of meningioma. We present the case of a teenage patient that in our knowledge is the youngest reported case of dystextia, whose aphasia recorded in a text message assisted with stroke localization. This also adds to the literature of dystextia which so far has only seven other cases reported. Arpita Lakhotia, Alok Sachdeva, Supriya Mahajan, and Nancy Bass Copyright © 2016 Arpita Lakhotia et al. All rights reserved. Effect of Spinal Cord Stimulation on Gait in a Patient with Thalamic Pain Sun, 07 Aug 2016 13:34:46 +0000 Thalamic pain is a central neuropathic pain disorder which occurs after stroke. Its severe chronic pain is often intractable to pharmacotherapies and affects the patients’ activities of daily living (ADL) and quality of life (QOL). Recently, spinal cord stimulation (SCS) has been reported to be effective in relieving the pain of thalamic pain; however, the effect of SCS on gait performance in patients is unknown. Therefore, we evaluated the gait performance before and after SCS in a case with thalamic pain. A 73-year-old male with thalamic pain participated in this study. We evaluated the gait of the patient two times: before SCS insertion and after 6 days of SCS. At the second evaluation, we measured the gait in three conditions: stimulation off, comfortable stimulation, and strong stimulation. SCS succeeded in improving the pain from 7 to 2 on an 11-point numerical rating scale. Step frequency and the velocity of gait tended to increase between pre- and poststimulation periods. There were no apparent differences in gait among the three stimulation conditions (off, comfortable, and strong) at the poststimulation period. SCS may be effective on gait in patients with thalamic pain. Arito Yozu, Masahiko Sumitani, Masahiro Shin, Kazuhiko Ishi, Michihiro Osumi, Junji Katsuhira, Ryosuke Chiba, and Nobuhiko Haga Copyright © 2016 Arito Yozu et al. All rights reserved. Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient Wed, 27 Jul 2016 12:21:47 +0000 Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease most common in immunodeficient patients. It occurs due to reactivation of the John Cunningham Virus (JCV) and carries a poor prognosis, with a median life expectancy of 6 months. We report a case of a 66-year-old man with a history of HCV related cirrhosis (HCV) and hepatocellular carcinoma (HCC) who was found to have PML in the setting of a negative viral load in the CSF and a CD4+ >200. He initially presented with two weeks of mild confusion and word-finding difficulty concerning for hepatic encephalopathy. An MRI was notable for extensive T2/FLAIR hyperintensity signal in the left temporal lobe. Brain biopsy was positive for JCV. PML is rare in immunocompetent individuals, especially in the setting of a negative viral load. It is possible, however, that transient states of immunosuppression may have been responsible in this case. Although viral load was reported as negative, virus may still have been detected but was below the quantifiable threshold. It is important for clinicians to note that a negative result does not necessarily exclude the possibility of PML, and care should be taken to review lab values on viral load in closer detail. T. Nanda Copyright © 2016 T. Nanda. All rights reserved. Unusual Late Onset of Parenchymal Neuro-Behçet Disease Tue, 26 Jul 2016 17:39:41 +0000 Neuro-Behçet disease (NBD) is a multisystem inflammatory disorder characterized by oral lesions, genital lesions, uveitis, and neurological deficits. If left untreated, it may lead to worsening neurological function and can be fatal. Here we present a case of a 52-year-old woman who was diagnosed with Behçet disease (BD) as a teenager and had a relatively mild disease course. Decades later after her initial DB diagnosis, she presented to our hospital with a chief complaint of headache. She did not have focal neurological deficits or any active mucosal lesions. Upon further investigation, the patient was found to have multiple inflammatory changes on neuroimaging and abnormal cerebrospinal fluid (CSF), consistent with the diagnosis of NBD. She was treated with intravenous corticosteroid therapy and her symptoms resolved. Although our patient presented with minimal symptoms decades after her initial diagnosis, any neurological complaint warranted a thorough investigation for a proper diagnosis and treatment given the multisystem involvement of BD. Wai Wai Miller, Demetrios Konstas, Chetan Gandhy, and Derrick Robertson Copyright © 2016 Wai Wai Miller et al. 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.