Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . 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. 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.