Case Reports in Neurological Medicine最新文献

{"title":"Incidentally Identified Pulmonary AVM: An Unusual Cause for Stroke in a Young Patient.","authors":"Vinod Kalapurackal Mathai,&nbsp;Dale Sebire,&nbsp;Scarlett Bowen","doi":"10.1155/2020/1203945","DOIUrl":"https://doi.org/10.1155/2020/1203945","url":null,"abstract":"<p><p>Pulmonary arteriovenous malformation is an uncommon though important potential source for embolic right-to-left extracardiac shunt as a cause for both silent and clinically evident stroke. We present this case to highlight this pathology as a cause for stroke, the importance of treating this malformation, even if incidentally identified, and finally the role of echocardiography in screening for extracardiac shunt and indicating those patients that may benefit from further investigations looking for extracardiac shunt.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"1203945"},"PeriodicalIF":0.9,"publicationDate":"2020-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/1203945","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38319010","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease.","authors":"Birte Eikeland","doi":"10.1155/2020/3579419","DOIUrl":"https://doi.org/10.1155/2020/3579419","url":null,"abstract":"<p><p>Research in the last few years has indicated that most voltage-gated potassium channel- (VGKC-) complex antibodies without leucine-rich glioma-inactivated protein 1 or contactin-associated protein-like 2 antibody specificity lack pathogenic potential and are not clear markers for autoimmune inflammation. Here we report on a patient with double-negative VGKC who developed severe peripheral nerve hyperexcitability, central nervous system symptoms with agitation and insomnia, dysautonomia, and systemic symptoms with weight loss, itch, and skin lesions. The disease started acutely one month after an episode of enteroviral pericarditis and responded well to immunotherapy. The patient is presumed to have developed a postinfectious immunotherapy-responsive autoimmune disease. In the setting of anti-VGKC positivity, it seems likely that anti-VGKC contributed to the pathogenesis of the patient's symptoms of nerve hyperexcitability and that the disease was caused by an acquired autoimmune effect on the neuronal kinetics of VGKC. It is still unknown whether or not there are unidentified extracellular molecular targets within the VGKC-complex, i.e., a novel surface antigen and a pathogenic antibody that can cause affected individuals to develop a peripheral nerve hyperexcitability syndrome. This case highlights the fact that less well-characterized autoimmune central and peripheral nervous system syndromes may have infectious triggers.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"3579419"},"PeriodicalIF":0.9,"publicationDate":"2020-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/3579419","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38279479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Postpartum-Onset Moyamoya Disease: A Rare Cause of Stroke in Unexpected.","authors":"Muhammet Ozer,&nbsp;Khadija Merchant,&nbsp;Zulfiya Manning,&nbsp;Suleyman Yasin Goksu,&nbsp;Kirti Juneja,&nbsp;Vernard S Fennell","doi":"10.1155/2020/7689450","DOIUrl":"https://doi.org/10.1155/2020/7689450","url":null,"abstract":"<p><p>Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease that is characterized by progressive bilateral stenosis of the terminal portion of the internal carotid artery and its main branches. Cerebrovascular events are the primary presenting symptoms and are related both to stenosis and occlusion of the ICAs and their main branches. Detection of bilateral stenosis by cerebral angiography is considered the gold standard, but computed tomography angiography (CTA) is also an acceptable method of diagnosis. In the current literature, there are no precise data on the incidence of moyamoya disease in Europe and the United States. Also, the pathogenesis of MMD remains obscure, and genetic factors and inflammation are the two most representative mechanisms. Here, we report the case of MMD in a 29-year-old African American female who presented with an ischemic stroke for the second time that manifested after pregnancy. This case is important to increase awareness of the probability of this rare disease in Western countries as well as to call attention to pregnancy's accelerating effects of MMD. Careful, long-term neurologic and radiologic follow-up is essential in adult patients with MMD to prevent additional stroke events and improve outcomes.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"7689450"},"PeriodicalIF":0.9,"publicationDate":"2020-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/7689450","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38220053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cervical Spine Ischemic Stroke Complicated by Spastic Quadriparesis and Ogilvie Syndrome: A Case Report and Literature Review.","authors":"Salman Assad,&nbsp;Justin Nolte,&nbsp;Dharampreet Singh,&nbsp;Samrina Hanif,&nbsp;Paul Ferguson","doi":"10.1155/2020/7197230","DOIUrl":"https://doi.org/10.1155/2020/7197230","url":null,"abstract":"Infarction or ischemia of the spinal cord is a rare entity and is often misdiagnosed as inflammatory myelopathy in acute settings. Atherosclerotic disease can affect spinal arteries, leading to cord ischemia with clinical presentation mixed with myelopathy. We present a case of a 66-year-old male who came to the hospital with unsteady gait and numbness of all extremities without associated pain for the past 48 hours. The neurological examination on admission directed the diagnosis towards myelopathy of the cervical spine. However, the initial magnetic resonance imaging (MRI) of the cervical spine demonstrated gliosis and restricted diffusion of the cord with multilevel neuroforaminal stenosis but without central canal stenosis or cord compression. The MRI brain, cerebrospinal fluid analysis, and rheumatologic evaluation were unremarkable. Four days into the clinical course, the patient developed weakness and spasticity of all extremities prompting further evaluation. Computed tomography angiography (CTA) scan of the head and neck revealed right vertebral artery occlusion and intracranial atherosclerotic disease. He was started on aspirin and clopidogrel for secondary risk reduction. The hospital course was further complicated by Ogilvie syndrome (OS), and the patient underwent uncomplicated cecostomy.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"7197230"},"PeriodicalIF":0.9,"publicationDate":"2020-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/7197230","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38220052","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transient, Recurrent Central Nervous System Clinical Manifestations of X-Linked Charcot-Marie-Tooth Disease Presenting with Very Long Latency Periods between Episodes: Is Prolonged Sun Exposure a Provoking Factor?","authors":"Andria Tziakouri,&nbsp;Konstantinos Natsiopoulos,&nbsp;Kleopas A Kleopa,&nbsp;Costas Michaelides","doi":"10.1155/2020/9753139","DOIUrl":"https://doi.org/10.1155/2020/9753139","url":null,"abstract":"<p><p>Charcot-Marie-Tooth disease is one of the most common inherited neurological disorders affecting the peripheral nervous system. The common clinical manifestations of the disease are distal muscle weakness and atrophy, often associated with a characteristic steppage gait and foot deformities. Transient acute and recurrent or chronic central nervous system manifestations, predominantly, dysarthria, dysphagia, motor weakness, and ataxia, have been recognized as a feature of the X-linked type 1 of CMT (CMTX1). The CNS symptoms occur typically in young age and often precede the clinical manifestation of the polyneuropathy. Several predisposing factors such as exercise, fever, and returning from areas of high altitude have been described as triggers of the CNS symptoms; however, in many cases, a substantial cause remains undetermined. In this report, we describe a patient with three attacks of transient CNS deficits at the ages of 11, 21, and 38 years, respectively, which were also accompanied by transient white matter abnormalities on MRI. Two of the attacks occurred after prolonged exposure to sunlight. In our knowledge, this is the first documented case with such long latency periods between CNS attacks as well as the only report describing intense sun exposure as a possible provoking factor.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"9753139"},"PeriodicalIF":0.9,"publicationDate":"2020-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9753139","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38169385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurosarcoidosis Presentation as Adipsic Diabetes Insipidus Secondary to a Pituitary Stalk Lesion and Association with Anti-NMDA Receptor Antibodies.","authors":"Jose Gabriel Solis, Arturo Olascoaga Lugo, Marco Antonio Rodríguez Florido, Bayron Alexander Sandoval Bonilla, Jose Malagón Rangel","doi":"10.1155/2020/7956350","DOIUrl":"10.1155/2020/7956350","url":null,"abstract":"<p><p>Sarcoidosis is a multisystemic inflammatory disease of unknown cause. It is characterized by the presence of noncaseating granuloma on a biopsy specimen. Clinical presentation varies across case report series with myriad of symptoms ranging from fever, respiratory symptoms, and skin lesions, or atypical symptoms like heart block or neurological symptoms. Hence, we report the case of a 22-year-old woman with encephalitis, a pituitary mass, and adipsic diabetes insipidus. The diagnostic approach did not end on the biopsy of the lesion, which reported noncaseating granulomas; on the contrary, it was the beginning of a path to exclude other causes of the central nervous system granulomas that ended with the diagnosis of the isolated central nervous system sarcoidosis. Also, we report the first proven association between anti-NMDA receptor antibodies and sarcoidosis.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"7956350"},"PeriodicalIF":0.9,"publicationDate":"2020-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7334767/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38157003","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rasmussen's Encephalitis: A Report of a Tunisian Pediatric Case and Literature Review.","authors":"Hedia Klaa,&nbsp;Thouraya Ben Younes,&nbsp;Hanene Benrhouma,&nbsp;Sonia Nagi,&nbsp;Aida Rouissi,&nbsp;Ichraf Kraoua,&nbsp;Ilhem Ben Youssef-Turki","doi":"10.1155/2020/6810237","DOIUrl":"https://doi.org/10.1155/2020/6810237","url":null,"abstract":"<p><p>Rasmussen's encephalitis (RE) is a rare progressive inflammatory disease of the central nervous system. It is characterized by unilateral hemispheric atrophy, pharmacoresistant focal seizures, and progressive neurological deficit. The exact etiopathogenesis still remains unknown. Brain imaging plays an important role in diagnosis and follow-up. Fluctuation of lesions in brain imaging was reported in few cases. Herein, we report an additional pediatric case of Rasmussen encephalitis with fluctuating changes in brain MRI.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"6810237"},"PeriodicalIF":0.9,"publicationDate":"2020-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/6810237","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38157002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rituximab for Autoimmune Encephalitis with Epilepsy.","authors":"Mohankumar Kurukumbi,&nbsp;Rahul H Dave,&nbsp;Jose Castillo,&nbsp;Tulsi Shah,&nbsp;Joanne Lau","doi":"10.1155/2020/5843089","DOIUrl":"https://doi.org/10.1155/2020/5843089","url":null,"abstract":"<p><p>Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the <i>α</i>-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and <i>N</i>-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"5843089"},"PeriodicalIF":0.9,"publicationDate":"2020-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/5843089","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38144447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Successful Use of Infliximab in a Relapsing Case of Susac's Syndrome.","authors":"Suran L Fernando,&nbsp;Therese Boyle,&nbsp;Annika Smith,&nbsp;John D E Parratt","doi":"10.1155/2020/9317232","DOIUrl":"https://doi.org/10.1155/2020/9317232","url":null,"abstract":"<p><p>Susac's syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may prevent disease sequelae such as permanent cognitive, visual, and hearing loss. We present such a case of Susac's syndrome that was also refractory to conventionally prescribed combination of immunosuppressive treatments including high-dose potent corticosteroids, intravenous cyclophosphamide, methotrexate, plasma exchange, rituximab, and mycophenolate. His disease was stabilized with infliximab in combination with a tapering course of low-dose prednisone. After 2 years of remission with TNF treatment, consideration is being given to ceasing therapy. He has the sequelae of bilateral sensorineural hearing loss but no visual impairment or cognitive deficits on follow-up with neuropsychometric testing. This is the first case report to our knowledge of the successful use of infliximab for a patient with Susac's syndrome that was necessary following treatment with cyclophosphamide and then rituximab.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"9317232"},"PeriodicalIF":0.9,"publicationDate":"2020-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9317232","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38068698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-NMDAR-Positive Small-Cell Lung Cancer Paraneoplastic Limbic Encephalitis: A Case Report and Literature Review.","authors":"Raman Sohal,&nbsp;Steven H Adams,&nbsp;Vishal Phogat,&nbsp;Abha Harish,&nbsp;Carlos Ynigo D Lopez,&nbsp;Michael P A Williams,&nbsp;Kamal K Khurana,&nbsp;Basel Abuzuaiter,&nbsp;Nia Jagroop,&nbsp;Bhavya Narapureddy","doi":"10.1155/2020/5269352","DOIUrl":"https://doi.org/10.1155/2020/5269352","url":null,"abstract":"<p><strong>Introduction: </strong>Paraneoplastic limbic encephalitis (PLE) is a rare disease that presents as rapid onset dementia characterized by short-term memory loss (STM), anxiety, and behavioral changes. Anti-NMDAR antibodies are unfrequently reported in PLE associated with small-cell lung cancer (SCLC). Given that PLE can precede the diagnosis of cancer, it is very important that once infectious, metabolic, nutritional, or structural disorders associated with short-term memory loss are ruled out that vigorous effort must be made to rule out underlying malignancy.</p><p><strong>Case: </strong>We report a rare case of PLE as the presenting symptom of SCLC. A 72-year-old male with history of COPD was brought to the ED by his wife after he was found to have short-term memory loss, including forgetfulness of his wedding anniversary the day before, and anxiety. Neurological exam showed impaired short-term recall on MOCA. CT head showed no evidence of infarct. Lumbar puncture was performed which showed lymphocytic pleocytosis, a nonspecific inflammatory change. CSF panel was negative for HSV, <i>Neisseria</i>, <i>Hemophilus</i>, <i>E. coli</i>, and HIV. Initial EEG was unremarkable, though a repeat EEG showed mild slowing of the posterior dominant rhythm consistent with mild encephalopathy. MRI showed equivocal increased FLAIR on T2-weighted images in the bilateral temporal lobes, left greater than right. CTA thorax showed bulky mediastinal and right hilar LAD. FNA of the R4 lymph node revealed SCLC. The NM bone scan showed no osteoblastic lesions. While the serum autoantibody panel was positive for anti-NMDAR, the CSF autoantibody panel returned entirely negative. Chemotherapy with etoposide and cisplatin was started on Day 4 of admission. The patient's neurological symptoms showed improvement following chemotherapy.</p><p><strong>Conclusion: </strong>This case highlights the importance of recognizing short-term memory loss as a feature of PLE.</p>","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"2020 ","pages":"5269352"},"PeriodicalIF":0.9,"publicationDate":"2020-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/5269352","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38068697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}