Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Valproate Treatment in an ALS Patient Carrying a c.194G>A Spastin Mutation and SMN2 Homozygous Deletion Thu, 17 Jul 2014 07:40:47 +0000 Here we report the case of an ALS patient found to carry both a novel heterozygous change (c.194G>A) within the spastin gene and a homozygous deletion of the SMN2 gene. The patient was started on valproic acid (VPA, 600 mg/die per os) considering the capacity of this drug of increasing survival motor neuron through an epigenetic mechanism. Patient clinical course and molecular effects of VPA on skin fibroblasts obtained from the proband are described. This c.194G>A spastin mutation might expand the previously known borders of type 4 spastic paraplegia (SPG4) and we suggest the intriguing possibility that the absence of SMN2 might have acted as a contributory risk factor for starting lower motor neuron damage. Exploring the relationship genocopy-phenocopy in selected ALS patients might represent an interesting strategy for understanding its clinical variability. Lucio Tremolizzo, Gessica Sala, Elisa Conti, Virginia Rodriguez-Menendez, Antonella Fogli, Angela Michelucci, Paolo Simi, Silvana Penco, Christian Lunetta, Massimo Corbo, and Carlo Ferrarese Copyright © 2014 Lucio Tremolizzo et al. All rights reserved. A Beautician’s Dystonia: Long-Lasting Effect of Botulinum Toxin Wed, 16 Jul 2014 12:09:45 +0000 Treatment options for dystonia are not curative but symptomatic; the treatment of choice for focal dystonias is repeated botulinum toxin injections. Here, we present the case of a 46-year-old beautician with focal dystonia in her left hand that affected her ability to work. Pharmacological treatment with clonazepam and gabapentin failed to resolve her symptoms and was discontinued due to side effects (sleepiness, gastrointestinal disorders). Intramuscular injection of botulinum toxin (incobotulinumtoxinA, Xeomin) into the extensor digitorum communis (35 U), flexor carpi radialis (35 U), and flexor digitorum superficialis (30 U) muscles resulted in complete resolution of symptoms at clinical assessments at 1, 3, 6, and 10 months after the injections, confirmed by the results of surface electromyography 10 months after treatment. The patient was able to work again 1 month after treatment. No reinjection has been necessary at the last evaluation (12 months after treatment). In conclusion, botulinum toxin is an effective treatment for focal dystonia that can have long-lasting effects and can improve patients’ ability to work and quality of life. Siria Di Martino, Stefania Dalise, Giuseppe Lamola, Martina Venturi, Bruno Rossi, and Carmelo Chisari Copyright © 2014 Siria Di Martino et al. All rights reserved. Cardiac Failure as an Unusual Presentation in a Patient with History of Amyotrophic Lateral Sclerosis Tue, 15 Jul 2014 14:18:45 +0000 Amyotrophic lateral sclerosis (ALS) is the most well-known form of motor neuron diseases in which both upper and lower motor neurons are involved in this disease. We presented an unusual case of ALS whom had presented with chief complaint of dyspnea. Cardiac failure was diagnosed at the final stage of the ALS disease. The pathogenetic mechanism leading to an elevated occurrence of cardiomyopathy in ALS is not comprehensible. Dilated cardiomyopathy has been explained in some previous studies. Based on the collected data, it was hypothesized that cardiomyopathy is underdiagnosed in the ALS population, probably because symptoms are masqueraded as a result of the patients’ disability. It was suggested that in all motor neuron diseases a serial cardiological evaluation should be executed, including annual echocardiography. Mohammad Hasan Namazi, Isa Khaheshi, Habib Haybar, and Shooka Esmaeeli Copyright © 2014 Mohammad Hasan Namazi et al. All rights reserved. Listerial Rhombencephalitis in an Immunocompetent Woman Sun, 13 Jul 2014 11:48:33 +0000 Listeriosis usually affects immunocompromised patients including elderly people and pregnant women, but it may also affect otherwise healthy individuals. In our report, we present a case of a rare and very severe form of listeriosis-rhombencephalitis in a 61-year-old female with no history of immunosuppression, who, because of history, clinical picture, and laboratory results as well as negative cultures, was at first diagnosed with viral encephalitis. This paper underlines that Listeria monocytogenes infection should be taken into consideration in case of lymphocytic encephalitis even in immunocompetent patients. Typical MRI picture may be crucial in establishing a proper diagnosis as the lab results may be misleading. Piotr Czupryna, Agata Zajkowska, Adam Garkowski, Sławomir Pancewicz, Katarzyna Guziejko, Anna Moniuszko, and Joanna Zajkowska Copyright © 2014 Piotr Czupryna et al. All rights reserved. Transient Global Amnesia Associated with an Acute Infarction at the Cingulate Gyrus Sun, 13 Jul 2014 10:24:26 +0000 Background. Transient global amnesia (TGA) is a syndrome of sudden, unexplained isolated short-term memory loss. In the majority of TGA cases, no causes can be identified and neuroimaging, CSF studies and EEG are usually normal. We present a patient with TGA associated with a small acute infarct at the cingulate gyrus. Case Report. The patient, a 62 year-old man, developed two episodes of TGA. He had hypertension and hypercholesterolemia. He was found to have an acute ischemic stroke of small size (15 mm of maximal diameter) at the right cerebral cingulate gyrus diagnosed on brain magnetic resonance imaging. No lesions involving other limbic system structures such as thalamus, fornix, corpus callosum, or hippocampal structures were seen. The remainder of the examination was normal. Conclusion. Unilateral ischemic lesions of limbic system structures may result in TGA. We must bear in mind that TGA can be an associated clinical disorder of cingulate gyrus infarct. Alejandro Gallardo-Tur, Jorge Romero-Godoy, Carlos de la Cruz Cosme, and Adriá Arboix Copyright © 2014 Alejandro Gallardo-Tur et al. All rights reserved. Diagnosis of Exclusion: A Case Report of Probable Glatiramer Acetate-Induced Eosinophilic Myocarditis Thu, 03 Jul 2014 09:14:49 +0000 Importance. Medication-induced eosinophilia is an acknowledged, often self-limiting occurrence. Glatiramer acetate, a biologic injection used in the management of relapsing-remitting multiple sclerosis, is widely regarded as a safe and effective medication and lists eosinophilia as an infrequent side effect in its package insert. Contrary to reports of transient, benign drug-induced eosinophilia, we describe a case of probable glatiramer acetate-induced eosinophilia that ultimately culminated in respiratory distress, shock, and eosinophilic myocarditis. Observations. A 59-year-old female was admitted to the hospital after routine outpatient labs revealed leukocytosis (43,000 cells/mm3) with pronounced hypereosinophilia (63%). This patient had been using glatiramer acetate without complication for over 10 years prior to admission. Leukocytosis and hypereosinophilia persisted as a myriad of diagnostic evaluations returned negative, ultimately leading to respiratory depression, shock, and myocarditis. Glatiramer acetate was held for the first time on day 6 of the hospital stay with subsequent resolution of leukocytosis, hypereosinophilia, respiratory distress, and shock. Conclusions and Relevance. Glatiramer acetate was probably the cause of this observed hypereosinophilia and the resulting complications. Reports of glatiramer-induced eosinophilia are rare, and few case reports regarding medication-induced hypereosinophilia describe the severe systemic manifestations seen in this patient. Christopher J. Michaud, Heather M. Bockheim, Muhammad Nabeel, and Timothy E. Daum Copyright © 2014 Christopher J. Michaud et al. All rights reserved. Cerebellar Dysfunction in a Patient with HIV Mon, 30 Jun 2014 11:14:00 +0000 A 50-year-old AIDS patient with a CD4 T-cell count of 114/mm3 was admitted with cerebellar symptoms of left CN XI weakness, wide-based gait with left-sided dysmetria, abnormal heel-knee-shin test, and dysdiadochokinesia. MRI showed region of hyperintensity in the left inferior cerebellar hemisphere involving the cortex and underlying white matter. Serological tests for HSV1, HSV2, and syphilis were negative. Her CSF contained high protein content and a WBC of 71/mm3, predominantly lymphocytes. The CSF was also negative for cryptococcal antigen and VDRL. CSF culture did not grow microbes. CSF PCR assay was negative for HSV1 and HSV2 but was positive for JC virus (1,276 copies). The most likely diagnosis is granule cell neuronopathy (GCN), which can only be definitively confirmed with biopsy and immunohistochemistry. Fernando Gonzalez-Ibarra, Waheed Abdul, Sahar Eivaz-Mohammadi, Christopher Foscue, Srinivas Gongireddy, and Amer Syed Copyright © 2014 Fernando Gonzalez-Ibarra et al. All rights reserved. Late-Onset Glycogen Storage Disease Type II (Pompe’s Disease) with a Novel Mutation: A Malaysian Experience Mon, 30 Jun 2014 10:13:11 +0000 Pompe’s disease (acid maltase deficiency, glycogen storage disease type II) is an autosomal recessive disorder caused by a deficiency of lysosomal acid -1,4-glucosidase, resulting in excessive accumulation of glycogen in the lysosomes and cytoplasm of all tissues, most notably in skeletal muscles. We present a case of adult-onset Pompe’s disease with progressive proximal muscles weakness over 5 years and respiratory failure on admission, requiring prolonged mechanical ventilation. Electromyography showed evidence of myopathic process with small amplitudes, polyphasic motor unit action potentials, and presence of pseudomyotonic discharges. Muscle biopsy showed glycogen-containing vacuoles in the muscle fibers consistent with glycogen storage disease. Genetic analysis revealed two compound heterozygous mutations at c.444C>G (p.Tyr148*) in exon 2 and c.2238G>C (p.Trp746Cys) in exon 16, with the former being a novel mutation. This mutation has not been reported before, to our knowledge. The patient was treated with high protein diet during the admission and subsequently showed good clinical response to enzyme replacement therapy with survival now to the eighth year. Conclusion. In patients with late-onset adult Pompe’s disease, careful evaluation and early identification of the disease and its treatment with high protein diet and enzyme replacement therapy improve muscle function and have beneficial impact on long term survival. Hiew Fu Liong, Siti Aishah Abdul Wahab, Yusnita Yakob, Ngu Lock Hock, Wong Kum Thong, and Shanthi Viswanathan Copyright © 2014 Hiew Fu Liong et al. All rights reserved. Management of Gamma-Butyrolactone Dependence with Assisted Self-Administration of GBL Thu, 26 Jun 2014 12:15:51 +0000 Gamma-hydroxybutyric acid (GHB) and its liquid precursor gamma-butyrolactone (GBL) have become increasingly popular beyond the clubbing culture resulting in daily consumption and dependence in the broader population. This case report illustrates the challenges of managing GHB-withdrawal and a possibly superior future approach of its management by titration and tapering of the addictive agent. Rafael Meyer, Josef Jenewein, and Soenke Boettger Copyright © 2014 Rafael Meyer et al. All rights reserved. Encephalopathy and Neuropathy due to Glue, Paint Thinner, and Gasoline Sniffing in Trinidad and Tobago-MRI Findings Wed, 18 Jun 2014 11:21:23 +0000 A 29-year-old male petrol station pump attendant was admitted with ataxia and clinical evidence of a sensorimotor polyneuropathy which developed over the preceding 3 months. He had cognitive dysfunction, hearing loss, and cerebellar clinical abnormalities that came on slowly over the three years. He had a fifteen-year history of sniffing mostly glue, occasionally paint thinners, and, in the recent two years, gasoline. Magnetic resonance brain imaging showed abnormalities of the cerebral cortex, cerebral white matter, corpus callosum, hippocampus, brainstem and cerebellar atrophy, hypointensities of basal ganglia, red nuclei, and substantia nigra as previously described in toluene sniffing. Abstinence for six months led to partial clinical improvement. Clinicians need to be aware of this preventable entity which has peculiar radiological findings which are being increasingly accepted as typical. Kanterpersad Ramcharan, Amrit Ramesar, Moshanti Ramdath, Joel Teelucksingh, and Maria Gosein Copyright © 2014 Kanterpersad Ramcharan et al. All rights reserved. Acute Aneurismal Bilateral Subdural Haematoma without Subarachnoid Haemorrhage: A Case Report and Review of the Literature Wed, 18 Jun 2014 00:00:00 +0000 Spontaneous pure acute bilateral subdural haematoma (ASDH) without intraparenchymal or subarachnoid haemorrhage caused by a ruptured cerebral aneurysm is extremely rare. It can follow rupture of different aneurysms specially located in anterior incisural space; the most frequently encountered location is the PcoA aneurysms as demonstrated in the present case. We present a case report of a PcoA aneurysm presenting as pure bilateral ASDH. A high level of suspicion for bleeding of arterial origin should be maintained in all cases of acute subdural haematoma without history of trauma. The neurological status on admission dictates the appropriate timing and methodology of the neuroradiological investigations. Ossama Mansour, Tamer Hassen, and Sameh Fathy Copyright © 2014 Ossama Mansour et al. All rights reserved. Recovery of Dysphagia in Lateral Medullary Stroke Tue, 17 Jun 2014 07:17:15 +0000 Lateral medullary stroke is typically associated with increased likelihood of occurrence of dysphagia and exhibits the most severe and persistent form. Worldwide little research exists on dysphagia in brainstem stroke. An estimated 15% of all patients admitted to stroke rehabilitation units experience a brainstem stroke out of which about 47% suffer from dysphagia. In India, a study showed that 22.3% of posterior circulation stroke patients develop dysphagia. Dearth of literature on dysphagia and its outcome in brainstem stroke particularly lateral medullary stroke motivated the author to present an actual case study of a patient who had dysphagia following a lateral medullary infarct. This paper documents the severity and management approach of dysphagia in brainstem stroke, with traditional dysphagia therapy and VitalStim therapy. Despite being diagnosed with a severe form of dysphagia followed by late treatment intervention, the patient had complete recovery of the swallowing function. Hitesh Gupta and Alakananda Banerjee Copyright © 2014 Hitesh Gupta and Alakananda Banerjee. All rights reserved. Paroxysmal Sneezing at the Onset of Syncopes and Transient Ischemic Attack Revealing a Papillary Cardiac Fibroelastoma Tue, 17 Jun 2014 06:27:32 +0000 Sneezing can at times be associated with neurological disorders. The “sneeze center” is localized in the lateral medulla. We report the case of a 50-year-old man who presented three episodes of sneezing, two of them followed by an episode of transient gait instability and dizziness and the third one followed by an episode of transient left hemiparesis due to fibroelastoma of the aortic cardiac valve. To the best of our knowledge, this is the first description of a transient ischemic attack due to cardiac papillary fibroelastoma and revealed by violent episodes of sneezing. Stéphane Mathis, Matthias Lamy, Jonathan Ciron, Anna Iljicsov, Roxana Arjmand, Pierre Agius, and Jean-Philippe Neau Copyright © 2014 Stéphane Mathis et al. All rights reserved. Microcystic Variant of an Intraosseous Meningioma in the Frontal Area: A Case Report Tue, 17 Jun 2014 06:16:33 +0000 Meningiomas located inside the bone of the calvaria belong to the group of rare types of tumours. The microcystic variant is the least common in this area. Due to their similarity to other tumours in this area, the imaging test of those tumours may constitute the source of an improper preoperative diagnosis. According to the records of the Department of Neurosurgery in Bielsko-Biała, 133 patients diagnosed with an intracranial meningioma confirmed by a histopathological test were operated in the last 10 years (2004–2014). In the histopathological test, three patients were diagnosed with the microcystic variant, which constitutes 2.25% of the cases. Only one variant of microcystic meningioma was located inside the bone, which constitutes 0.75% of all the meningiomas operated. Jan Bujok and Marek Bienioszek Copyright © 2014 Jan Bujok and Marek Bienioszek. All rights reserved. Conus Medullaris Syndrome following Radionuclide Cisternography Thu, 12 Jun 2014 06:59:13 +0000 Radionuclide cisternography is generally considered to be a safe procedure without significant neurological complications. However, in this report we present a patient who developed conus medullaris syndrome following radionuclide cisternography. A 46-year-old woman underwent lumbar puncture followed by radionuclide cisternography with the diagnosis of hydrocephalus. After the cisternography, she developed voiding difficulty with perineal sensory loss. Lumbar MRI revealed a high signal intensity lesion on T2-weighted images at the level of conus medullaris. Considering its clinical course and MRI findings, a spinal cord infarction is highly suggested as a cause of the conus medullaris lesion in this patient. Jay Chol Choi Copyright © 2014 Jay Chol Choi. All rights reserved. Cerebral Venous Sinus Thrombosis following Diagnostic Curettage in a Patient with Uterine Fibroid Sun, 01 Jun 2014 13:04:47 +0000 Cerebral venous sinus thrombosis (CVST) is a relatively rare cerebrovascular disease, of which the risk has been documented in patients with numerous conditions. However, CVST has never been previously described in association with the use of a diagnostic curettage in patient with uterine fibroid. Herein, we described a 43-year-old woman who presented with recurrent convulsive seizures and severe and progressive headache 1 day after a diagnostic curettage of the uterus, which was confirmed to be uterine fibroid pathologically later, and her condition subsequently progressed to confusion. Brain magnetic resonance imaging (MRI) revealed an acute extensive thrombosis of the left transverse and sigmoid sinus and the ipsilateral cerebellum infarction. Evaluation for primary thrombophilia revealed that an iron deficiency anemia (IDA) due to the fibroid bleeding induced menorrhagia together with a diagnostic curettage might be the sole hypercoagulable risk factor identified. Treatment with anticoagulation led to full recovery of her symptoms and recanalization of the thrombosis was proven on magnetic resonance venography (MRV) 2 months later. We suggest that CVST should be recognized as a potential complication related to this diagnostic technique, especially in patient with IDA. The early diagnosis and timely treatment would be of significance in improving the prognosis of this potentially lethal condition. Xiao-Qun Zhu and Li Cao Copyright © 2014 Xiao-Qun Zhu and Li Cao. All rights reserved. A Rare Sequela of Acute Disseminated Encephalomyelitis Mon, 26 May 2014 13:14:31 +0000 Acute disseminated encephalomyelitis is a demyelinating disease, typically occurring in children following a febrile infection or a vaccination. Primary and secondary immune responses contribute to inflammation and subsequent demyelination, but the exact pathogenesis is still unknown. Diagnosis of acute disseminated encephalomyelitis is strongly suggested by temporal relationship between an infection or an immunization and the onset of neurological symptoms. Biopsy is definitive. In general, the disease is self-limiting and the prognostic outcome is favorable with anti-inflammatory and immunosuppressive agents. Locked-in syndrome describes patients who are awake and conscious but have no means of producing limb, speech, or facial movements. Locked-in syndrome is a rare complication of acute disseminated encephalomyelitis. We present a case of incomplete locked-in syndrome occurring in a 34-year-old male secondary to acute disseminated encephalomyelitis. Our case is unique, as acute disseminated encephalomyelitis occurred in a 34-year-old which was poorly responsive to immunosuppression resulting in severe disability. Vijay Kodadhala, Saravana Devulapalli, Mohankumar Kurukumbi, and Annapurni Jayam-Trouth Copyright © 2014 Vijay Kodadhala et al. All rights reserved. A Presentation of Cerebritis Secondary to Granulomatosis with Polyangiitis (Wegener) Tue, 13 May 2014 00:00:00 +0000 Neurological manifestations of GPA are common, most frequently as a peripheral neuropathy. Cerebritis as a principal presentation is extremely rare. We report a patient who presented with subacute progression of ataxia, confusion, and vacant episodes. An MRI of her brain showed bilateral signal abnormalities in the cingulate and superior sagittal gyrus while a staging CT revealed a mass in the right upper lobe of the patient’s lung with a satellite nodule. C-ANCA antibodies specific for PR3 at high titres were positive and a diagnosis of GPA was made. The patient was commenced on intravenous methylprednisolone followed by cyclophosphamide and responded well to treatment. GPA is a rare and treatable differential diagnosis for confused patients with acute or subacute neurological features and unusual MRI findings. James Norman, Ira Pande, Timothy Taylor, and Bruno Gran Copyright © 2014 James Norman et al. All rights reserved. Recurrent Gliosarcoma in Pregnancy Mon, 12 May 2014 11:38:57 +0000 Gliosarcoma is a rare tumor of the central nervous system and it constitutes about 1 to 8% of all malignant gliomas. In this report we are presenting a recurrent gliosarcoma case during a pregnancy in a 30-year-old woman. This is the first report presenting gliosarcoma in the pregnancy. İsmail Gülşen, Hakan Ak, Tevfik Yilmaz, Mehmet Deniz Bulut, İsmet Alkış, and İrfan Bayram Copyright © 2014 İsmail Gülşen et al. All rights reserved. Spinocerebellar Ataxia 7: A Report of Unaffected Siblings Who Married into Different SCA 7 Families Sun, 04 May 2014 12:58:13 +0000 Two families with spinocerebellar ataxia type 7 are presented. Although there are affected cousins, it is not the sibling parents that transmitted the mutation. It is assumed that the affected families share a common ancestor. Fariha Zaheer and Dominic Fee Copyright © 2014 Fariha Zaheer and Dominic Fee. All rights reserved. Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus Thu, 24 Apr 2014 16:43:06 +0000 Comorbid hyperammonemic encephalopathy (HE) and status epilepticus (SE) leading to extensive cortical diffusion restriction (CDR) on MRI have not been previously reported. We describe a patient with HE who subsequently developed provoked SE. Sequential MRIs demonstrated a progressive CDR that involved the entire bilateral supratentorial cortex, thalami, and basal ganglia, resulting in death from cerebral edema and brain herniation. Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience. Such findings have also been described in both HE and SE (Milligan et al. (2009), Chatzikonstantinou et al. (2011), U-King-Im et al. (2011), and Bindu et al. (2009)), but not to the extent seen in our patient. Additionally, our patient had distinct radiologic features of both disease processes, suggesting a cumulative effect. The diagnosis of HE and SE in the setting of extensive CDR should not be missed and could lead to improved outcomes for two progressive, malignant, and treatable illnesses that can be easily overlooked. Adam de Havenon, Kris French, and Safdar Ansari Copyright © 2014 Adam de Havenon et al. All rights reserved. Extensive VZV Encephalomyelitis without Rash in an Elderly Man Wed, 23 Apr 2014 11:33:07 +0000 Introduction. Varicella zoster virus (VZV) encephalomyelitis with cranial nerve involvement is rare. Characteristically it is preceded by a rash and primarily presents in the immunocompromised. The spectrum of VZV neurologic disease is extensive and it is not uncommon to present without rash. We report the case of an elderly otherwise immunocompetent patient who presented with diverse manifestations of VZV CNS infection all occurring without rash. Case Report. A 78-year-old man presented with 1 week of progressive paraparesis and sensory loss, malaise, and fevers. MRI of the neuraxis demonstrated numerous enhancing lesions: intramedullary, leptomeningeal, pachymeningeal, and cranial nerves. Cerebrospinal fluid (CSF) showed a white blood cell count of 420/μL with elevated protein (385 mg/dL). CSF VZV qualitative PCR was positive and CSF VZV immunofluorescence assay detected IgM antibody, confirming the diagnosis of VZV encephalomyelitis. Clinical and radiological improvement was observed after intravenous acyclovir treatment. Conclusion. This is a rare report of an immunocompetent patient with extensive VZV encephalomyelitis. We highlight the importance of considering this diagnosis even in the absence of the characteristic rash, and the potential risk of premature discontinuation of antiviral therapy once HSV has been excluded. Prompt recognition and treatment can dramatically reduce morbidity and mortality in patients. Karen Lynch, Prakhar Agarwal, Anu Paranandi, Susan Hadley, and Mithila Vullaganti Copyright © 2014 Karen Lynch et al. All rights reserved. Prolonged Toxic Encephalopathy following Accidental 4-Aminopyridine Overdose Thu, 17 Apr 2014 00:00:00 +0000 Background. 4-Aminopyridine (4-AP) is a drug that is used to improve motor fatigue in patients suffering from multiple sclerosis (MS). Medication error can occur, as commercial preparation may not be available in some countries. Case Presentation. A 58-year-old woman with progressive MS presented with status epilepticus. She was receiving 4-AP for more than 3 years. The symptoms started soon after the ingestion of a single pill that was supposed to contain 10 mg 4-AP, but further investigations revealed that each pill had been inadvertently prepared with an 100 mg 4-AP concentration. The patient was admitted to the intensive care unit (ICU) for appropriate management (orotracheal intubation, sedation, and antiepileptic drugs). The first electroencephalogram (EEG) showed abundant irregular spike-waves on the left central regions. Neurological condition gradually improved from day 7, while the EEG did not reveal any more electrical seizures but was still consistent with toxic encephalopathy. The patient stayed in the ICU until day 13. At discharge from the rehabilitation ward (2.5 months later), the patient had not yet recovered her previous cognitive and functional condition. Conclusion. A single 100 mg 4-AP accidental overdose may cause serious immediate complications, with a slow and incomplete neurological recovery. Maria Ballesta Méndez, Vincent van Pesch, Arnaud Capron, and Philippe Hantson Copyright © 2014 Maria Ballesta Méndez et al. All rights reserved. Open-Ring Enhancement in Pseudotumoral Multiple Sclerosis: Important Radiological Aspect Tue, 15 Apr 2014 12:30:52 +0000 Introduction. Observation of open-ring enhancement in magnetic resonance imaging (MRI) is considered a specificity marker for diagnosing pseudotumoral multiple sclerosis (MS). This finding is of great value in the differential diagnosis of tumefactive lesions. Case Report. We describe a 55-year-old white woman, with previous history of ovarian cancer and recent history of fatigue and bilateral retroorbital pain. Important bilateral visual impairment evolved over one month. Physical examination detected the presence of right homonymous hemianopia. Cranial MRI showed an expanding lesion with open-ring enhancement. Given the range of diagnostic possibilities, a stereotactic biopsy was performed, and histopathological examination was consistent with an active demyelinating disease. The patient was treated with 1 g of methylprednisolone and symptoms improved following a significant reduction in the lesion. Conclusions. We highlight the MRI results suggestive of pseudotumoral MS, especially open-ring enhancement, which is an important radiologic aspect to diagnosis and can assist in avoiding unnecessary biopsies. Frederico Carvalho de Medeiros, Lucas Alverne Freitas de Albuquerque, Jose Eymard Homem Pittella, Renata Brant de Souza, Antonio Pereira Gomes Neto, and Paulo Pereira Christo Copyright © 2014 Frederico Carvalho de Medeiros et al. All rights reserved. Usefulness of Preoperative Surgical Simulation with Three-Dimensional Fusion Images for Resection of Cerebral Cavernous Malformations Near Broca’s Area Mon, 14 Apr 2014 10:28:30 +0000 Treating subcortical brain lesions in or near eloquent areas is challenging not only because lesions must be resected while preserving brain tissue involved in essential functions, but also because lesions often cannot be easily identified from the surface of the brain. Here, we report 2 cases of cerebral cavernous malformations near Broca’s area. In both cases, lesions were surgically removed by utilizing three-dimensional fusion images created using preoperative magnetic resonance imaging and computed tomography data. Excisions were completed without any worsening of speech function, and the use of presurgical simulations was found to be useful in the design and execution of the actual operations. The technique described in this report serves as a useful tool in simulating surgical strategies by using brain gyri and sulci as surgical landmarks. Furthermore, in contrast to other intraoperative techniques, this method can aid in shortening the duration of surgery and can help limit damage to eloquent areas of the brain. Satoshi Takahashi, Yoshio Tanizaki, Kazunori Akaji, Tadashige Kano, Kenji Hiraga, and Ban Mihara Copyright © 2014 Satoshi Takahashi et al. All rights reserved. An Adult Patient with Ocular Myasthenia and Unusually Long Spontaneous Remission Sun, 13 Apr 2014 00:00:00 +0000 A male patient developed ocular myasthenia gravis (MG) at the age of 33. He was anti-acetylcholine receptor antibody (anti-AChR Ab) negative. He received cholinesterase blocker for 5 months and went into a complete clinical remission that lasted untreated for 17 years. He relapsed recently with ocular symptoms only. He is now anti-AChR Ab positive and SFEMG is abnormal in a facial muscle. The patient is controlled with steroids. He had one of the longest spontaneous remissions reported in the natural history of MG, particularly unusual for an adult with the disease. Jasem Al-Hashel, Hanaa M. Rashad, and Rossen T. Rousseff Copyright © 2014 Jasem Al-Hashel et al. All rights reserved. The Middle Fossa Approach for the Removal of a Trochlear Schwannoma Wed, 12 Mar 2014 13:34:06 +0000 Objectives. Schwannomas originating from the trochlear nerve are extremely rare; only 30 cases have been reported in the literature. Many operative approaches have been utilized for lesion resection, but the advantages of the anterior transpetrosal approach are numerous and include excellent exposure, minimal extradural retraction of the temporal lobe, and minimal cerebrospinal fluid leaks. We report the second case of a trochlear schwannoma resected via the anterior transpetrosal approach. Setting. A 64-year-old male presented with 3-month history of diplopia and headaches. On physical examination, he was found to have a right fourth nerve palsy. Brain magnetic resonance imaging revealed a mass within the right ambient cistern compressing the adjacent midbrain. A right-sided anterior transpetrosal approach was used—which confirmed that the trochlear nerve entered the mass—to achieve gross total resection. Pathological examination confirmed diagnosis of schwannoma. The patient was discharged on postoperative day 3. He experienced a persistent fourth nerve palsy postoperatively with an otherwise normal neurological examination. Follow-up imaging confirmed complete removal of the tumor. Conclusion. The anterior transpetrosal approach is an excellent approach for removal of trochlear schwannomas involving the cisternal course of the trochlear nerve. It affords complete visualization of this anatomical region while introducing minimal morbidity. Andrew B. Boucher and L. Madison Michael II Copyright © 2014 Andrew B. Boucher and L. Madison Michael II. All rights reserved. Persistent Cryptococcal Brain Infection despite Prolonged Immunorecovery in an HIV-Positive Patient Wed, 05 Mar 2014 00:00:00 +0000 Background. HIV-positive people starting combined antiretroviral therapy may develop immune reconstitution to latent or treated opportunistic infections. Immune reconstitution to cerebral Cryptococcus is poorly understood and can be fatal. Case Presentation. A 33-year-old Zimbabwean female presented with cryptococcal meningitis and newly diagnosed HIV with a CD4 count of 51 cells/μL (4%). She was treated with amphotericin and flucytosine. Combined antiretroviral therapy was started four weeks later and she showed early improvement. However, over the ensuing 18 months, her clinical course was marked by periodic worsening with symptoms resembling cryptococcal meningitis despite having achieved CD4 counts ≥400 cells/μL. Although initially treated for relapsing cryptococcal immune reconstitution syndrome, a brain biopsy taken 17 months after initial presentation showed budding Cryptococci. Conclusion. This unusually protracted case highlights the difficulties in differentiating relapsing cryptococcal meningitis from immune reconstitution and raises questions concerning the optimum timing of initiation of combined antiretroviral therapy in such patients. Tom Wingfield, Jo Baxter, Amit Herwadkar, Daniel du Plessis, Tom J. Blanchard, F. Javier Vilar, and Anoop Varma Copyright © 2014 Tom Wingfield et al. All rights reserved. Synovial Cyst Mimicking an Intraspinal Sacral Mass Tue, 04 Mar 2014 09:58:25 +0000 A 68-year-old female had a three-week history of severe low back pain radiating down the posterior left buttocks and left leg exacerbated by standing and walking. Lumbar spine MRI revealed cystic mass with similar intensity to cerebrospinal fluid located on dorsolateral left side of the sacral spinal canal inferior to the S1 pedicle. There was compression of left exiting S1 and traversing S2 nerve roots. Neurosurgery consult was requested to evaluate the cystic mass in the sacral spinal canal. After clinical evaluation, an unusually located synovial cyst was thought possible. Cyst contents were heterogeneous, suggestive of small hemorrhage and acute clinical history seemed reasonable. Left S1 and partial left S2 hemilaminectomy was performed and an epidural, partially hemorrhagic cyst was removed. There was no obvious connection to the ipsilateral L5-S1 facet joint. Pathology revealed synovial cyst, and the patient’s leg pain was improved postoperatively. This synovial cyst was unusual as it had no connection with the facet joint intraoperatively and its location in the sacral canal was uncommon. Jason Hoover and Stephen Pirris Copyright © 2014 Jason Hoover and Stephen Pirris. All rights reserved. Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report Tue, 04 Mar 2014 08:38:36 +0000 Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord. Sajish Jacob, Harsh Gupta, Dejan Nikolic, Betul Gundogdu, and Shirley Ong Copyright © 2014 Sajish Jacob et al. All rights reserved.