Case Reports in Neurological Medicine The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Rheumatoid Meningitis Occurring during Etanercept Treatment Mon, 13 Feb 2017 10:04:23 +0000 We report a 65-year-old man who had repetitive seizures 6 months after receiving etanercept, methotrexate, and prednisolone for rheumatoid arthritis. Mononuclear cells were mildly increased in the cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) showed high intensity along sulci of the frontal and parietal lobes. Brain biopsy revealed lymphocyte and plasma cell infiltration in the meninges, confirming the diagnosis of rheumatoid meningitis. After steroid pulse therapy, seizures resolved and clinical findings improved. When etanercept was replaced by tocilizumab, rheumatoid meningitis did not recur. Although TNF-α inhibitors can control joint symptoms of rheumatoid arthritis, they may induce rheumatoid meningitis. Koji Tsuzaki, Takashi Nakamura, Hiroyuki Okumura, Naoko Tachibana, and Toshiaki Hamano Copyright © 2017 Koji Tsuzaki et al. All rights reserved. Improvement and Neuroplasticity after Combined Rehabilitation to Forced Grasping Mon, 06 Feb 2017 11:15:40 +0000 The grasp reflex is a distressing symptom but the need to treat or suppress it has rarely been discussed in the literature. We report the case of a 17-year-old man who had suffered cerebral infarction of the right putamen and temporal lobe 10 years previously. Forced grasping of the hemiparetic left upper limb was improved after a unique combined treatment. Botulinum toxin type A (BTX-A) was first injected into the left biceps, wrist flexor muscles, and finger flexor muscles. Forced grasping was reduced along with spasticity of the upper limb. In addition, repetitive facilitative exercise and object-related training were performed under low-amplitude continuous neuromuscular electrical stimulation. Since this 2-week treatment improved upper limb function, we compared brain activities, as measured by near-infrared spectroscopy during finger pinching, before and after the combined treatment. Brain activities in the ipsilesional sensorimotor cortex (SMC) and medial frontal cortex (MFC) during pinching under electrical stimulation after treatment were greater than those before. The results suggest that training under electrical stimulation after BTX-A treatment may modulate the activities of the ipsilesional SMC and MFC and lead to functional improvement of the affected upper limb with forced grasping. Michiko Arima, Atsuko Ogata, Kazumi Kawahira, and Megumi Shimodozono Copyright © 2017 Michiko Arima et al. All rights reserved. Is Intravenous Heparin a Contraindication for TPA in Ischemic Stroke? Sun, 05 Feb 2017 10:36:37 +0000 There are approximately 2 million cardiac catheterizations that occur every year in the United States and with an aging population this number continues to rise. Adverse events due to this procedure occur at low rates and include stroke, arrhythmia, and myocardial infarctions. Due to the high volume of procedures there are a growing number of adverse events. Stroke after cardiac catheterization (SCC) has an incidence between 0.27 and 0.5% and is one of the most debilitating complications leading to high rates of mortality and morbidity. Given the relatively uncommon clinical setting of stroke after cardiac catheterization, treatment protocols regarding the use of IV or IA thrombolysis have not been adequately developed. Herein, we describe a case of a 39-year-old male who developed a stroke following a cardiac catheterization where IV thrombolysis was utilized although the patient was on heparin prior to cardiac catheterization. Zain Kulairi, Nisha Deol, Renee Tolly, Rohan Manocha, and Maliha Naseer Copyright © 2017 Zain Kulairi et al. All rights reserved. A Case of “Refractory” Neuropsychiatric Lupus Responsive to Anticoagulation Mon, 30 Jan 2017 08:32:22 +0000 Neuropsychiatric disorder is a severe complication in 14% to 75% of systemic lupus erythematosus (SLE) patients, which can result in significant morbidity. A 15-year-old female SLE patient with coexistence of dural sinus thrombosis and intracerebral hemorrhage resistant to two pulses of high dose of glucocorticoid was treated with anticoagulation of the low-molecular-weight [LMW] heparin subcutaneously followed by warfarin. The patient demonstrated a remarkable clinical response. Rui Wu and Sun Hu Copyright © 2017 Rui Wu and Sun Hu. All rights reserved. A Pediatric Tumor Found Frequently in the Adult Population: A Case of Anaplastic Astroblastoma in an Elderly Patient and Review of the Literature Mon, 23 Jan 2017 07:51:31 +0000 Astroblastomas are rare, potentially curable primary brain tumors which can be difficult to diagnose. We present the case of astroblastoma in a 73-year-old male, an atypical age for this tumor, more classically found in pediatric and young adult populations. Through our case and review of the literature, we note that this tumor is frequently reported in adult populations and the presentation of this tumor in the elderly is well described. This tumor is an important consideration in the differential diagnosis when managing both pediatric and adult patients of any age who present with the imaging findings characteristic of this rare tumor. Christopher Payne, Ali Batouli, Kristen Stabingas, Dunbar Alcindor, Khaled Abdel Aziz, Cunfeng Pu, Elizabeth Tyler-Kabara, Robert Williams, and Alexander Yu Copyright © 2017 Christopher Payne et al. All rights reserved. Hemorrhagic Colloid Cyst Presenting with Acute Hydrocephaly Sun, 22 Jan 2017 07:43:00 +0000 Colloid cysts are benign slow-growing cystic lesions located on the roof of the third ventricle that usually present with symptoms related to gradual rise of intracranial pressure. They mostly remain asymptomatic and sometimes grow progressively and cause diverse symptoms associated with increased intracranial pressure such as headache, diplopia, and sixth cranial nerve palsy. Here we report a 47-year-old female who presented to the emergency department with acute severe headache and nausea/vomiting. On MRI examination acute hydrocephaly due to hemorrhagic colloid cyst was detected. Acute hemorrhage in colloid cysts is extremely rare and may present with symptoms of acute increase in the intracranial pressure. Intracystic hemorrhage is very rarely reported as a complication of colloid cyst presenting with paroxysmal symptoms of acute hydrocephaly. Reza Akhavan, Behrouz Zandi, Masoud Pezeshki-Rad, Donya Farrokh, and Bita Abbasi Copyright © 2017 Reza Akhavan et al. All rights reserved. Seizure following the Use of the COX-2 Inhibitor Etoricoxib Sun, 22 Jan 2017 00:00:00 +0000 We describe a case of epileptic seizures occurring after the use of a COX-2 inhibitor. A 61-year-old man was admitted to our department because of a generalized tonic-clonic seizure. EEG showed generalized slowdown of the activity. Neuroimaging and blood samples studies did not evidence alterations, but a careful pharmacological history revealed that the patient had taken the COX-2 inhibitor etoricoxib to treat lumbago few days before the onset of clinical symptoms. No seizures were reported after etoricoxib discontinuation and an EEG resulted to be normal two months after this. Conclusion. Knowing the pharmacological history of a patient is important for understanding the clinical presentation and selecting appropriate treatment. This is, to the best of our knowledge, the first reported case of generalized seizures associated with the use of COX-2 inhibitors. Valentina Arnao, Marianna Riolo, Brigida Fierro, and Paolo Aridon Copyright © 2017 Valentina Arnao et al. All rights reserved. Nonconvulsive Status Epilepticus Resembling Clinical Absence with Atypical EEG Pattern Thu, 19 Jan 2017 00:00:00 +0000 Objective. We are reporting two cases: a patient with steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and another patient with secondary progressive multiple sclerosis (SPMS), both presenting with altered mental status (AMS) and later diagnosed with nonconvulsive atypical absence status epilepticus (AS), with atypical EEG changes. Methods. A report of two cases. Results. A patient with history of SREAT and the other with SPMS had multiple admissions due to AMS. For both, EEG revealed the presence of a high voltage generalized sharply contoured theta activity. A diagnosis of NCSE with clinical features of AS was made based on both clinical and EEG features. There was significant clinical and electrographic improvement with administration of levetiracetam for both patients in addition to sodium valproate and Solumedrol for the SREAT patient. Both patients continued to be seizure free on follow-up few months later. Conclusions. This is a report of two cases of atypical AS, with atypical EEG, in patients with different neurological conditions. Prompt clinical and EEG recovery occurred following appropriate medical treatment. We think that this condition might be underreported and could significantly benefit from prompt treatment when appropriately diagnosed. Channaiah Srikanth Mysore, Najib Murr, Rana Zabad, and John Bertoni Copyright © 2017 Channaiah Srikanth Mysore et al. All rights reserved. Variability in Clinical Presentation of Neonatal Stroke: Report of Four Cases Tue, 17 Jan 2017 00:00:00 +0000 Neonatal stroke can be a cause of long term neurodevelopmental disability, seizures, and impaired cognitive function. We present four cases of neonatal stroke, associated with different risk factors and clinical presentations. Two of these newborns were born to mothers with no prenatal care. Sonia Joseph, Dimitrios Angelis, Robert Bennett, Bhargavi Kola, and Amanda Hughes Copyright © 2017 Sonia Joseph et al. All rights reserved. A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome Sun, 15 Jan 2017 08:59:15 +0000 A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer’s test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia. João Peres, Simão Cruz, Rita Oliveira, Luís Santos, and Ana Valverde Copyright © 2017 João Peres et al. All rights reserved. Evolution of Cerebral Atrophy in a Patient with Super Refractory Status Epilepticus Treated with Barbiturate Coma Sun, 15 Jan 2017 08:22:52 +0000 Introduction. Status epilepticus is associated with neuronal breakdown. Radiological sequelae of status epilepticus include diffusion weighted abnormalities and T2/FLAIR cortical hyperintensities corresponding to the epileptogenic cortex. However, progressive generalized cerebral atrophy from status epilepticus is underrecognized and may be related to neuronal death. We present here a case of diffuse cerebral atrophy that developed during the course of super refractory status epilepticus management despite prolonged barbiturate coma. Methods. Case report and review of the literature. Case. A 19-year-old male with a prior history of epilepsy presented with focal clonic seizures. His seizures were refractory to multiple anticonvulsants and eventually required pentobarbital coma for 62 days and midazolam coma for 33 days. Serial brain magnetic resonance imaging (MRI) showed development of cerebral atrophy at 31 days after admission to our facility and progression of the atrophy at 136 days after admission. Conclusion. This case highlights the development and progression of generalized cerebral atrophy in super refractory status epilepticus. The cerebral atrophy was noticeable at 31 days after admission at our facility which emphasizes the urgency of definitive treatment in patients who present with super refractory status epilepticus. Further research into direct effects of therapeutic coma is warranted. Christopher R. Newey, Pravin George, Premkumar Nattanmai, Christine Ahrens, Stephen Hantus, and Aarti Sarwal Copyright © 2017 Christopher R. Newey et al. All rights reserved. Hyperechogenicity of the Substantia Nigra in Parkinson’s Disease: Insights from Two Brothers with Markedly Different Disease Durations Wed, 11 Jan 2017 00:00:00 +0000 We present clinical features and substantia nigra morphology for two brothers with Parkinson’s disease (PD) aged 60 and 59 years. The brothers were diagnosed at 41 and 50 years of age, respectively. Both patients exhibited an abnormally large area of substantia nigra echogenicity bilaterally when viewed with transcranial ultrasound. The abnormality was similar in both brothers despite one having a much longer disease duration than the other. These findings further highlight that transcranial ultrasound is not associated with severity of clinical symptoms, but it might assist in the diagnosis of PD provided that it is combined with other variables known to precede PD. Julie M. Hall, Matthew J. Georgiades, Deborah A. Hammond, Xiaoting Feng, Ahmed A. Moustafa, Simon J. G. Lewis, and Gabrielle Todd Copyright © 2017 Julie M. Hall et al. All rights reserved. Chronic Subdural Hematoma Associated with Thrombocytopenia in a Patient with Human Immunodeficiency Virus Infection in Cameroon Tue, 10 Jan 2017 08:02:58 +0000 Hematological abnormalities including thrombocytopenia are common in patients living with HIV infection. Patients with HIV infection related thrombocytopenia present generally with only minor bleeding problems. But cases of subdural hematoma are very rare. A 61-year-old female with a history of HIV infection of 9 years’ duration presented with a 3-month history of generalized headache associated with visual blurring and anterograde amnesia. There was no history of trauma or fever. She was treated empirically for cerebral toxoplasmosis for 6 weeks without any improvement of the symptoms. One week prior to admission, she developed weakness of the left side of the body. Clinical examination revealed left-sided hemiparesis. Computed tomography scan of the brain showed a 25 mm chronic right frontoparietotemporal subdural hematoma compressing the lateral ventricle with midline shift. There was no appreciable cerebral atrophy. A complete blood count showed leucopenia and thrombocytopenia at 92,000 cells/mm3. Her CD4-positive cell count was 48 cells/mm3 despite receiving combination antiretroviral therapy for 9 years. A complete blood count analysis suggestive of thrombocytopenia should raise suspicion of possibilities of noninfectious focal brain lesions like subdural hematoma amongst HIV infected patients presenting with nonspecific neurological symptoms. This will enable prompt diagnosis and allow early appropriate intervention. Clovis Nkoke, Engelbert Bain Luchuo, Denis Teuwafeu, Ines Nepetsoun, and Cyrille Nkouonlack Copyright © 2017 Clovis Nkoke et al. All rights reserved. Treatment Challenges of a Primary Vertebral Artery Aneurysm Causing Recurrent Ischemic Strokes Tue, 10 Jan 2017 00:00:00 +0000 Background. Extracranial vertebral artery aneurysms are a rare cause of embolic stroke; surgical and endovascular therapy options are debated and long-term complication may occur. Case Report. A 53-year-old man affected by neurofibromatosis type 1 (NF1) came to our attention for recurrent vertebrobasilar embolic strokes, caused by a primary giant, partially thrombosed, fusiform aneurysm of the left extracranial vertebral artery. The aneurysm was treated by endovascular approach through deposition of Guglielmi Detachable Coils in the proximal segment of the left vertebral artery. Six years later the patient presented stroke recurrence. Cerebral angiography and Color Doppler Ultrasound well characterized the unique hemodynamic condition developed over the years responsible for the new embolic event: the aneurysm had been revascularized from its distal portion by reverse blood flow coming from the patent vertebrobasilar axis. A biphasic Doppler signal in the left vertebral artery revealed a peculiar behavior of the blood flow, alternately directed to the aneurysm and backwards to the basilar artery. Surgical ligation of the distal left vertebral artery and excision of the aneurysm were thus performed. Conclusion. This is the first described case of NF1-associated extracranial vertebral artery aneurysm presenting with recurrent embolic stroke. Complete exclusion of the aneurysm from the blood circulation is advisable to achieve full resolution of the embolic source. Davide Strambo, Luca Peruzzotti-Jametti, Aurora Semerano, Giovanna Fanelli, Franco Simionato, Roberto Chiesa, Enrico Rinaldi, Vittorio Martinelli, Giancarlo Comi, Marco Bacigaluppi, and Maria Sessa Copyright © 2017 Davide Strambo et al. All rights reserved. Chronic Cluster Headache with an Atypical Presentation and Treatment Response Thu, 29 Dec 2016 09:25:01 +0000 The management of cluster headache (CH) may be challenging. We report a 50-year-old male with recurrent attacks of dull and severe unilateral periorbital pain, lasting 30–45 minutes, twice a day, exclusively during sleep, and accompanied by ipsilateral rhinorrhea and lacrimation. The pain switched sides within every attack. CH treatment was initiated but the patient maintained recurrence rates compatible with chronic CH, even after increasing verapamil to 460 mg/day. Afterwards we decided to add lithium (800 mg/day). With this treatment the severity and recurrence of CH substantially decreased, despite the patient’s autonomous decision to take lithium only during the acute phase of the cluster. The exclusively alternating location and the excellent response to short cycles of lithium represent two unique features of CH. Telma Santos and Hugo Morais Copyright © 2016 Telma Santos and Hugo Morais. All rights reserved. Relative Hypodense Vertebral Artery Sign on Computerized Tomography in Atherosclerotic Near Occlusion Thu, 29 Dec 2016 09:14:21 +0000 A 52-year-old white male presented with an acute onset of slurred speech along with hypoesthesia in the entire left arm. The acute computed tomography (CT) showed relative hypodensity in the intracranial segment of left vertebral artery (VA) that was not present in historical images, pointing to the possible lack of flow. The site of occlusion was confirmed by magnetic resonance imaging (MRI) that showed susceptibility effect in the affected artery. By means of historical native CT comparison the site of VA thrombosis was correctly predicted. Local atherosclerotic thrombosis of the VA could be relatively hypodense on native CT and still have positive susceptibility weighted imaging (SWI) sign. Muhammad Faraz Raghib and Slaven Pikija Copyright © 2016 Muhammad Faraz Raghib and Slaven Pikija. All rights reserved. A Successful Treatment of Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization for Hydrocephalus in Walker-Warburg Syndrome Tue, 27 Dec 2016 14:42:53 +0000 Walker-Warburg syndrome (WWS) is a rare autosomal recessive congenital muscular dystrophy with brain malformations and ocular abnormalities that falls under the wider phenotypic spectrum of the dystroglycanopathies. Mutations in a number of genes including POMT1, POMT2, POMGNT1, POMGNT2, FKTN, FKRP, LARGE, and ISPD are known to cause alpha dystroglycan-related muscular dystrophy. Mutations in these genes result in a broad phenotypic spectrum ranging from the severe WWS to a mild congenital muscular dystrophy with no brain involvement. WWS is fatal to most patients early in life with mean survival of 9 months. The most common brain finding is cobblestone lissencephaly with the vast majority of patients (97%) also having ventricular dilation with or without hydrocephalus. Surgical treatment has not been frequently detailed. This report describes our successful treatment of a patient with WWS and hydrocephalus with Endoscopic Third Ventriculostomy (ETV) with choroid plexus cauterization (CPC). Fourteen months following treatment, a follow-up MRI CSF flow study demonstrated robust CSF flow through floor of third ventricle from interpeduncular cistern to lateral ventricle. Tomoko Tanaka, Catharine J. Harris, Sarah S. Barnett, and N. Scott Litofsky Copyright © 2016 Tomoko Tanaka et al. All rights reserved. Fast-Growing Meningioma in a Woman Undergoing Fertility Treatments Mon, 26 Dec 2016 08:40:13 +0000 Meningiomas have long been known to be associated with sexual hormones. We discuss here the case of a woman with a huge meningioma that rapidly grew over the course of a couple years while the patient was simultaneously taking fertility treatments. There is substantial evidence suggesting that fertility treatments can fuel the growth of meningiomas. The potential risks should be considered in women with a previous or family history of meningiomas who plan to undergo fertility treatment. These patients need to be evaluated and a screening imaging of brain MRI (Magnetic Resonant Imaging) should be offered in the middle or toward the end of such a treatment to control and prevent complications of these meningiomas. Adam Patterson and Abdurrahim Elashaal Copyright © 2016 Adam Patterson and Abdurrahim Elashaal. All rights reserved. Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome Thu, 22 Dec 2016 13:10:47 +0000 Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS), in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness) at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA). Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease. Shahvaiz Magsi, Adeel Khoja, Mansoor Ali Merchant Rameez, Ariba Khan, and Noman Ishaque Copyright © 2016 Shahvaiz Magsi et al. All rights reserved. Hirayama Disease: A Rare Disease with Unusual Features Wed, 21 Dec 2016 09:31:41 +0000 Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord atrophy with asymmetric flattening. We report a case of Hirayama disease in a 25-year-old Indian man presenting with gradually progressive asymmetrical weakness and wasting of both hands and forearms along with unusual features of autonomic dysfunction and upper motor neuron lesion. S. Anuradha and Vanlalmalsawmdawngliana Fanai Copyright © 2016 S. Anuradha and Vanlalmalsawmdawngliana Fanai. All rights reserved. Bamboo Leaf Sign as a Sensitive Magnetic Resonance Imaging Finding in Spinal Subependymoma: Case Report and Literature Review Thu, 15 Dec 2016 07:36:18 +0000 Background and Importance. Subependymoma occurs very rarely in the spinal cord. We report another case of spinal subependymoma along with a review of the literature and discussion of a radiological finding that is useful for preoperative diagnosis of this tumor. Clinical Presentation. A 51-year-old man presented with a 2-year history of progressive muscle weakness in the right lower extremity. Sagittal magnetic resonance imaging (MRI) showed spinal cord expansion at the Th7–12 vertebral level. Surgical resection was performed and the tumor was found to involve predominantly subpial growth. Histological diagnosis was subependymoma, classified as Grade I according to criteria of World Health Organization. We made an important discovery of what seems to be a characteristic appearance for spinal subependymoma on sagittal MRI. Swelling of the spinal cord is extremely steep, providing unusually large fusiform dilatation resembling a bamboo leaf. We have termed this characteristic MRI appearance as the “bamboo leaf sign.” This characteristic was apparent in 76.2% of cases of spinal subependymoma for which MRI findings were reported. Conclusion. The bamboo leaf sign on spinal MRI is useful for differentiating between subependymoma and other intramedullary tumors. Neurosurgeons encountering the bamboo leaf sign on spinal MRI should consider the possibility of subependymoma. Hiroyuki Toi, Yukari Ogawa, Keita Kinoshita, Satoshi Hirai, Hiroki Takai, Keijiro Hara, Nobuhisa Matsushita, Shunji Matsubara, and Masaaki Uno Copyright © 2016 Hiroyuki Toi et al. All rights reserved. Small-Cell Lung Cancer with Positive Anti-NMDAR and Anti-AMPAR Antibodies Paraneoplastic Limbic Encephalitis Tue, 13 Dec 2016 13:59:08 +0000 We report the case of a 66-year-old woman, with paraneoplastic limbic encephalitis, treated 6 months earlier for bladder neoplasia. The patient presented to the emergency room with rapidly increasing symptoms, noninfectious cerebral spinal fluid associated with positive anti-NMDAR (as well as in serum) and positive AMPAR antibodies in the serum. Four months later, the patient was diagnosed with a small-cell lung cancer for which chemotherapy and radiotherapy was commenced. Simultaneously, endoscopic surgical treatment was undertaken for an in situ relapse of the bladder neoplasm. After the completion of 3 cycles of chemotherapy her neurological status temporarily worsened. The cerebral MRI did not show signs of encephalitis such as increased T2/FLAIR signal intensity in the mesial temporal lobes and limbic systems. No specific treatment was prescribed. Limbic encephalitis can be associated with malignant tumors such as lung carcinoma. Several cases reported in the literature have shown cognitive improvement after tumoral therapy. Regarding our experience, significant progress was achieved through immuno-modulatory treatment. A transitory deterioration of the cognitive process was perceived during the chemotherapy sessions. Sabina Boangher, Pascal Mespouille, Corina-Mihaela Filip, and Sophie Goffette Copyright © 2016 Sabina Boangher et al. All rights reserved. Reversible Vitamin B12 Deficiency Presenting with Acute Dementia, Paraparesis, and Normal Hemoglobin Tue, 13 Dec 2016 08:06:36 +0000 Vitamin B12 is essential for neurological function and its deficiency is associated with many neuropsychiatric disorders. We report the case of a previously healthy 53-year-old male patient presenting with delirium and multiple neurological findings. Complete blood analysis indicated megaloblastic anemia. All infectious causes were excluded owing to negative cultures (blood and urine). Tests for human immunodeficiency virus, syphilis, and toxoplasma were also negative. Metabolic workup showed severe vitamin B12 deficiency, decreased reticulocyte count, and increased direct bilirubin and lactate dehydrogenase. Intramuscular injection of cobalamin was started, and the patient showed significant improvement. Hani Almoallim, Fahtima S. Mehdawi, Mohammed M. Cheikh, Fahmi Al-dhaheri, and Abdullah Mahir Aqeel Copyright © 2016 Hani Almoallim et al. All rights reserved. Acute Stroke due to Electrocution: Uncommon or Unrecognized? Mon, 12 Dec 2016 14:12:04 +0000 The growing dependence on electricity in our daily lives has increased the incidence of electrocution injuries. Although several neurological injuries have been described previously, acute stroke due to electrocution is rare. Our patient, a previously healthy man, was electrocuted after he grabbed a “live” high-voltage wire. Although he was hemodynamically stable, he remained confused with language defects. MRI of the brain showed acute stroke in the bilateral anterior cerebral artery territory and watershed regions of the left middle cerebral artery territory. MR angiogram incidentally showed A1 segment aplasia of the right anterior cerebral artery. Electrocution is known to cause vasospasm leading to end-organ damage similar to that seen in stroke. In our patient, vasospasm of the left anterior circulation likely led to watershed infarcts in the left parietal lobe and bilateral frontal lobes. Due to aplasia of the A1 segment on the right side, perfusion to both frontal lobes was solely from the left anterior cerebral artery. Laxmi Kokatnur and Mohan Rudrappa Copyright © 2016 Laxmi Kokatnur and Mohan Rudrappa. All rights reserved. Secondary Myelitis in Dermal Sinus Causing Paraplegia in a Child with Previously Normal Neurological Function Tue, 06 Dec 2016 09:36:32 +0000 Neural tube defects result from failure of neural tube fusion during early embryogenesis, the fourth week after conception. The spectrum of severity is not uniform across the various forms of this congenital anomaly as certain presentations are not compatible with extrauterine life (anencephaly) while, on the other hand, other defects may remain undiagnosed as they are entirely asymptomatic (occult spina bifida). We report a child with previously normal neurological development, a devastating clinical course following superinfection of a subtle spina bifida defect which resulted in a flaccid paralysis below the level of the lesion and permanent neurological deficits following resolution of the acute infection and a back closure surgery. Sakina Rashid, Grace Kinabo, Marissa Kellogg, William P. Howlett, and Marieke C. J. Dekker Copyright © 2016 Sakina Rashid et al. All rights reserved. Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome Thu, 01 Dec 2016 14:30:42 +0000 Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies). For each patient, we carried out a pathological analysis (nerve and muscle) and an electrophysiological study (and follow-up). To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders. Stéphane Mathis, Laurent Magy, Philippe Corcia, Karima Ghorab, Laurence Richard, Jonathan Ciron, Mathilde Duchesne, and Jean-Michel Vallat Copyright © 2016 Stéphane Mathis et al. All rights reserved. Motor Speech Apraxia in a 70-Year-Old Man with Left Dorsolateral Frontal Arachnoid Cyst: A [18F]FDG PET-CT Study Thu, 24 Nov 2016 09:04:27 +0000 Motor speech apraxia is a speech disorder of impaired syllable sequencing which, when seen with advancing age, is suggestive of a neurodegenerative process affecting cortical structures in the left frontal lobe. Arachnoid cysts can be associated with neurologic symptoms due to compression of underlying brain structures though indications for surgical intervention are unclear. We present the case of a 70-year-old man who presented with a two-year history of speech changes along with decreased initiation and talkativeness, shorter utterances, and dysnomia. [18F]Fluorodeoxyglucose (FDG) Positron Emission and Computed Tomography (PET-CT) and magnetic resonance imaging (MRI) showed very focal left frontal cortical hypometabolism immediately adjacent to an arachnoid cyst but no specific evidence of a neurodegenerative process. Nicolaas I. Bohnen, Jacob Haugen, Karen Kluin, and Vikas Kotagal Copyright © 2016 Nicolaas I. Bohnen et al. All rights reserved. Progressive Multifocal Leukoencephalopathy in a Multiple Sclerosis Patient Diagnosed after Switching from Natalizumab to Fingolimod Tue, 22 Nov 2016 14:36:38 +0000 Background. Natalizumab- (NTZ-) associated progressive multifocal leukoencephalopathy (PML) is a severe and often disabling infectious central nervous system disease that can become evident in multiple sclerosis (MS) patients after NTZ discontinuation. Recently, novel diagnostic biomarkers for the assessment of PML risk in NTZ treated MS patients such as the anti-JC virus antibody index have been reported, and the clinical relevance of milky-way lesions detectable by MRI has been discussed. Case Presentation and Conclusion. We report a MS patient in whom PML was highly suspected solely based on MRI findings after switching from NTZ to fingolimod despite repeatedly negative (ultrasensitive) polymerase chain reaction (PCR) testing for JC virus DNA in cerebrospinal fluid. The PML diagnosis was histopathologically confirmed by brain biopsy. The occurrence of an immune reconstitution inflammatory syndrome (IRIS) during fingolimod therapy, elevated measures of JCV antibody indices, and the relevance of milky-way-like lesions detectable by (7 T) MRI are discussed. Tim Sinnecker, Jalal Othman, Marc Kühl, Imke Metz, Thoralf Niendorf, Annett Kunkel, Friedemann Paul, Jens Wuerfel, and Juergen Faiss Copyright © 2016 Tim Sinnecker et al. All rights reserved. Proximal Limb Weakness in a Patient with Celiac Disease: Copper Deficiency, Gluten Sensitivity, or Both as the Underlying Cause? Tue, 22 Nov 2016 08:49:21 +0000 Celiac disease has been associated with several neurologic disorders which may result from micronutrient deficiencies, coexisting autoimmune conditions, or gluten sensitivity. Copper deficiency can produce multiple neurologic manifestations. Myeloneuropathy is the most common neurologic syndrome and it is often irreversible, despite copper replacement. We report the case of a 55-year-old man who presented with progressive proximal limb weakness and weight loss in the setting of untreated celiac disease without gastrointestinal symptoms. He had anemia, neutropenia, and severe hypocupremia. The pattern of weakness raised the suspicion that there was an underlying myopathy, although this was not confirmed by electrodiagnostic studies. Weakness and hematologic abnormalities resolved completely within 1 month of total parenteral nutrition with copper supplementation and a gluten-free diet. Myopathy can rarely occur in patients with celiac disease, but the mechanism is unclear. Pure proximal limb weakness has not been previously reported in copper deficiency. We propose that this may represent a novel manifestation of hypocupremia and recommend considering copper deficiency and gluten sensitivity in patients presenting with proximal limb weakness. J. David Avila and David Lacomis Copyright © 2016 J. David Avila and David Lacomis. All rights reserved. A 66-Year-Old Woman with a Progressive, Longitudinally Extensive, Tract Specific, Myelopathy Mon, 21 Nov 2016 11:29:35 +0000 A 66-year-old woman presented with progressive lancinating pain and sensory deficits attributable to a myelopathy of unclear etiology. Spinal cord magnetic resonance imaging showed a longitudinally extensive T2-hyperintense lesion of the dorsal columns. Comprehensive serum, urine, and cerebrospinal fluid analyses failed to identify an etiology. Empiric intravenous methylprednisolone and intravenous immunoglobulin were of no benefit and serial screens for an occult malignancy were negative. She developed dysesthesias and allodynia affecting her entire body and lost the use of her arms and legs due to severe sensory ataxia that was steadily progressive from onset. She opted against additional aggressive medical management of her condition and passed away on hospice eleven months after symptom onset. Autopsy revealed findings most consistent with polyphasic spinal cord ischemia affecting the dorsal and lateral white matter tracts and, to a lesser extent, adjacent gray matter. The underlying etiology for the progressive vasculopathy remains unknown. Spinal cord ischemia affecting the posterior spinal cord is rare and to our knowledge this case represents the only instance of a progressive spinal cord tractopathy attributable to chronic spinal cord ischemia. Elizabeth O’Keefe, Katherine E. Schwetye, John Nazarian, Richard Perrin, Robert E. Schmidt, and Robert Bucelli Copyright © 2016 Elizabeth O’Keefe et al. All rights reserved.