Calcifying pseudoneoplasm of the spine

Calcifying pseudoneoplasms have been rarely reported to involve the spine. Diagnostically, MRI characteristics have not been well-described. We present the case of a 59-year-old woman with a C8 radiculopathy and history significant for metastatic breast cancer. MRI showed a C7-T1 extradural mass with an isointense signal to the spinal cord on T1- and T2-weighted sequences. Mild peripheral enhancement was also noted. The patient underwent a laminectomy to accomplish resection of the underlying lesion. The mass was firm, tan-colored, and adherent to the adjacent dura. A gross total resection was achieved and, postoperatively, the patient had resolution of her radiculopathy. Pathologic analysis confirmed a calcifying pseudoneoplasm. Calcifying pseudoneoplasms can cause exclusively axial pain or may induce radicular or myelopathic symptoms. The pathogenesis is unclear, although the lesions are usually benign. MRI findings can vary, but typically the lesions are extradural, well-demarcated, and mildly enhance peripherally. Surgical resection, either subtotal or total, is highly successful.     

Paediatric intramedullary spinal cord cavernous malformations: Case report and review of the literature

Cavernous malformations are vascular lesions which can occur throughout the entire neuraxis. This term is synonymous to cavernous angioma, cavernous haemangioma, and cavernoma. They comprise of closely packed, capillary-like vascular channels, without intervening neural tissue. MR imaging is currently the study of choice for the diagnosis of cavernous malformations as they are considered angiographically occult lesions. Intramedullary location represents only 3–5% of all central nervous system cavernous malformations, with the majority of them being supratentorial. Only 10% of the intramedullary cavernous malformations present in the paediatric population. As the natural history of these lesions is not well known, the debate on the optimal management of them is still ongoing. Here we describe the case of a 14-year-old male with progression of symptoms over an 18 months period, which necessitated microsurgical removal of a T9 intramedullary cavernous malformation.     

The role of interleukin-1 in seizures and epilepsy: A critical review

Interleukin-1 (IL-1) has a multitude of functions in the central nervous system. Some of them involve mechanisms that are related to epileptogenesis. The role of IL-1 in seizures and epilepsy has been investigated in both patients and animal models. This review aims to synthesize, based on the currently available literature, the consensus role of IL-1 in epilepsy.Three lines of evidence suggest a role for IL-1: brain tissue from epilepsy patients and brain tissue from animal models shows increased IL-1 expression after seizures, and IL-1 has proconvulsive properties when applied exogeneously. However, opposing results have been published as well. More research is needed to fully establish the role of IL-1 in seizure generation and epilepsy, and to explore possible new treatment strategies that are based on interference with intracellular signaling cascades that are initiated when IL-1 binds to its receptor.     

Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature

A 37-year old woman, who had presented 5 years earlier with suspected simple partial seizures, returned with seizures increasing in frequency and intensity, confirmed by video/electroencephalography (VEEG) monitoring with left frontotemporal onset. A low-grade tumor was suspected, given a magnetic resonance imaging (MRI) study demonstrating enlargement of the left amygdala, anterior hippocampus, and adjacent mesial temporal neocortex, with modest gadolinium enhancement, and a positron emission tomography (PET) scan showing increased metabolism within that region. Surgical resection of the left mesial temporal lobe was performed and pathology revealed pathogen-free granulomas. She was given a diagnosis of sarcoidosis (following chest computed tomography that showed hilar adenopathy). She was treated with oral steroids for neurosarcoidosis with no further epileptic seizures in 19 months of follow-up. The second case was a young man, with known pulmonary sarcoidosis, who developed simple partial seizures and, later, complex partial seizures, with MRI revealing a left insular mass. Stereotactic biopsy again demonstrated pathogen-free granulomas. He has also done well in 4 years of follow-up. Review of the literature suggests that seizures associated with sarcoidosis do not invariably imply a poor prognosis. Certain features-multifocal parenchymal involvement, hydrocephalus, and chronic meningitis-were associated with poor outcome. In contrast, cases with isolated mass lesions often fared well.     

Calcifying pseudoneoplasm of the neuraxis in direct continuity with a low‐grade glioma: A case report and review of the literature

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are presumed to be a non‐neoplastic reactive pathology, based on the frequent finding of granulomatous inflammation. To our knowledge, there are few reports of CAPNON in association with a neoplasm. Here, we report the case of a 62‐year‐old man presenting with headache, which was caused by CAPNON in the left cingulate gyrus. CT scan revealed a calcified mass exhibiting gradual growth and increasing peritumoral edema. MRI showed an intra‐axial hypointense mass on T1‐ and T2‐weighted images. Development of a peri‐lesional hyperintense lesion on T2‐weighted images suggested local edema or tumoral invasion. Gadolinium‐enhanced T1‐weighted images revealed mild peripheral enhancement of the calcified nodule. L‐methyl‐¹¹C methionine‐positron emission tomography revealed the uptake of tracer in the calcified nodule. The calcified mass and its enveloping brain tissue were removed using a parietal craniotomy. The calcified tissue was surrounded by spindle‐shaped cells positive for GFAP and nestin. The MIB‐1 labeling index of spindle cells was around 10% (i.e. a hot spot). Fourteen months after surgery, gadolinium‐enhanced MRI evidenced growth of a tiny residual lesion. Therefore, this report illustrates a potential case of CAPNON arising from low‐grade glial neoplasm.     

Photic- and pattern-induced seizures: a review for the Epilepsy Foundation of America Working Group

PURPOSE: This report summarizes background material presented to a consensus conference on visually provoked seizures, convened by the Epilepsy Foundation of America. METHODS: A comprehensive review of literature was performed. RESULTS: Photosensitivity, an abnormal EEG response to light or pattern stimulation, occurs in approximately 0.3-3% of the population. The estimated prevalence of seizures from light stimuli is approximately 1 per 10,000, or 1 per 4,000 individuals age 5-24 years. People with epilepsy have a 2-14% chance of having seizures precipitated by light or pattern. In the Pokemon cartoon incident in Japan, 685 children visited a hospital in reaction to red-blue flashes on broadcast television (TV). Only 24% who had a seizure during the cartoon had previously experienced a seizure. Photic or pattern stimulation can provoke seizures in predisposed individuals, but such stimulation is not known to increase the chance of subsequent epilepsy. Intensities of 0.2-1.5 million candlepower are in the range to trigger seizures. Frequencies of 15-25 Hz are most provocative, but the range is 1-65 Hz. Light-dark borders can induce pattern-sensitive seizures, and red color also is a factor. Seizures can be provoked by certain TV shows, movie screen images, video games, natural stimuli (e.g, sun on water), public displays, and many other sources. CONCLUSIONS: Recommendations on reducing risk of seizures have been developed by agencies in the United Kingdom, Japan, and the International Telecommunications Union, affiliated with the United Nations. The Epilepsy Foundation of America has developed a consensus of medical experts and scientists on this subject, reported in an accompanying work.     

Seizures and EEG findings in an adult patient with DiGeorge syndrome: A case report and review of the literature

This is the first case report to describe the EEG findings in a patient with DiGeorge syndrome who survived into adulthood. The patient developed generalized tonic–clonic seizures when she was 9 years old and these were associated with hypocalcemia. Despite treatment with calcium, seizures persisted and the patient required antiepileptic medications. She was eventually controlled with oxcarbazepine. An MRI of the head was normal. An EEG showed independent spike and wave discharges emanating from the left temporal and right frontal region. The presence of focal findings on EEG, the lack of complete response to calcium therapy, and the need for antiepileptic drug therapy indicate that some of these patients may be inherently predisposed to developing epilepsy.     

Intramedullary spinal cord cavernous angioma in children: case report and literature review

Introduction Intramedullary cavernous angiomas (ImCA) of the spinal cord are very uncommon malformations especially in the pediatric population where only ten cases have yet been reported within the available literature. Case report In this paper, the authors report the case of a 12-year-old girl presenting with a T11 level hematomyelia because of a cavernous angioma. Microsurgical excision was performed with good clinical outcome and no magnetic resonance imaging evidence of residual cavernoma 30 months later. Discussion Despite the small number of cases reported in children, this one contributes to the literature identifying special features of presentation and management of ImCA for pediatric patients. Because a higher risk of recurrent bleeding has been demonstrated for ImCA, with dramatic clinical consequences, microsurgical removal remains the only definitive treatment.     

A possible role of dystrophin in neuronal excitability: A review of the current literature

Duchenne muscular dystrophy (DMD) is a recessive hereditary form of muscular dystrophy caused by a mutation in the dystrophin gene on the X chromosome. Clinical observations show that in addition to progressive muscular degeneration, DMD is more often accompanied by neurocognitive symptoms and learning disabilities, especially in automatisation of reading, attention processes, and expressive language skills. Additionally, three studies reported a higher prevalence of epilepsy in DMD, suggesting that the absence of dystrophin might be related to increased CNS excitability. In this article, we aim to review current clinical and experimental evidence for a potential role of brain dystrophin in seizure generation.     

Voxel-based morphometry of temporal lobe epilepsy: an introduction and review of the literature

We review the applications and results of voxel-based morphometry (VBM) studies that have reported brain changes associated with temporal lobe epilepsy (TLE). A PubMed search yielded 18 applications of VBM to study brain abnormalities in patients with TLE up to May 2007. Across studies, 26 brain regions were found to be significantly reduced in volume relative to healthy controls. There was a strong asymmetrical distribution of temporal lobe abnormalities preferentially observed ipsilateral to the seizure focus, particularly of the hippocampus (82.35% of all studies), parahippocampal gyrus (47.06%), and entorhinal (23.52%) cortex. The contralateral hippocampus was reported as abnormal in 17.65% of studies. There was a much more bilateral distribution of extratemporal lobe atrophy, preferentially affecting the thalamus (ipsilateral = 61.11%, contralateral = 50%) and parietal lobe (ipsilateral = 47.06%, contralateral = 52.94%). VBM generally reveals a distribution of brain abnormalities in patients with TLE consistent with the region-of-interest neuroimaging and postmortem literature. It is unlikely that VBM has any clinical utility given the lack of robustness for individual comparisons. However, VBM may help elucidate some unresolved important research questions such as how recurrent temporal lobe seizures affect hippocampal and extrahippocampal morphology using serial imaging acquisitions.     

Glioblastoma presenting as spontaneous intracranial haemorrhage: Case report and review of the literature

Glioblastoma (GB) classically presents with symptoms of raised intracranial pressure and gradual progressive neurological deficits. An acute presentation, with intracerebral haemorrhage (ICH) and rapid clinical deterioration, occurs infrequently. Contemporary imaging modalities do not reliably reflect underlying mass lesions in parenchymal brain haemorrhage at first presentation. We report a delayed diagnosis of GB in a 21-year-old patient presenting with spontaneous ICH and a negative initial neurovascular workup. A comprehensive literature review was performed to investigate the incidence of malignant aetiology for spontaneous ICH in young adults, and to underscore the importance of early utilisation of diagnostic magnetic resonance imaging (MRI) in such cases.     

Epilepsy,seizures, physical exercise,and sports: A report from the ILAE Task Force on Sports and Epilepsy

People with epilepsy (PWEs) are often advised against participating in sports and exercise, mostly because of fear, overprotection, and ignorance about the specific benefits and risks associated with such activities. Available evidence suggests that physical exercise and active participation in sports may favorably affect seizure control, in addition to producing broader health and psychosocial benefits. This consensus paper prepared by the International League Against Epilepsy (ILAE) Task Force on Sports and Epilepsy offers general guidance concerning participation of PWEs in sport activities, and provides suggestions on the issuance of medical fitness certificates related to involvement in different sports. Sports are divided into three categories based on potential risk of injury or death should a seizure occur: group 1, sports with no significant additional risk; group 2, sports with moderate risk to PWEs, but no risk to bystanders; and group 3, sports with major risk. Factors to be considered when advising whether a PWE can participate in specific activities include the type of sport, the probability of a seizure occurring, the type and severity of the seizures, seizure precipitating factors, the usual timing of seizure occurrence, and the person's attitude in accepting some level of risk. The Task Force on Sports and Epilepsy considers this document as a work in progress to be updated as additional data become available.     

全面性癫癎伴热性惊厥附加症的临床和脑电图特征分析

目的探讨全面性癫伴热性惊厥附加症(GEFS )的临床和脑电图(EEG)特点。方法收集4个GEFS 的家系资料,通过详细的调查建立完善的家系谱,并对受累者的临床资料、EEG进行分析总结。结果4个家系共有60名成员,其中受累者20例,表现为FS者5例,FS 者7例,FS 与失神发作2例,FS 与肌阵挛发作1例,FS 与失神和肌阵挛发作1例,此例患者发作间期EEG呈现局灶性癫放电和全面性癫放电共存的现象,1例表现为FS 和部分性发作,其发作间歇期EEG呈现中央中颞棘波灶,个体诊断符合良性罗兰多区癫。另外,受累者有肯定的临床发作,但是由于不能收集到可靠的发作表现资料,无法进行发作分类者3例。受累者神经系统检查以及头颅CT或磁共振成像(MRI)检查均未见异常。结论GEFS 的正确诊断需要注重个体,立足于整个家系进行,其临床发作谱还包括部分性发作,脑电图也有局灶的癫样放电。良性罗兰多区癫也许是GEFS 的一个新表现型。     

表现有立毛运动的复杂部分性癫痫(附3例报告及文献复习)

目的了解立毛运动性发作的病理生理、病因及临床特点。方法结合3例立毛运动性发作病例及文献复习(已报过21例)，探讨立毛运动性发作的特点。结果立毛运动性发作的最重要临床表现是反复的立毛运动，可能与右颞叶额叶(眶面皮层，运动前区)下丘脑和边缘系统学结构有关，脑电图(EEG)表现为非特异性异常，但是大多数病例有发作期发放或癫痫样波，视频监测脑电图(Video—EEG)对于评价其分型及发作期、发作间期变化有帮助。结论立毛运动性发作极罕见。但是注意结合病史，体检和EEG检查还是可以发现的。     

Neonatal hypocalcemic seizures: case report and literature review

Seizures during the neonatal period have a broad differential diagnosis, many with a specific treatment and prognosis. In the case reported, a combination of dietary and endocrinologic abnormalities resulted in hypocalcemic seizures, which continued despite aggressive correction of serum ionized calcium levels. Serial electroencephalograms (EEG) performed during the hospitalization were markedly abnormal, and treatment with anticonvulsant drugs was considered given the persistence of seizures despite normalization of serum calcium levels. After 4 days of intravenous calcium administration, the seizure activity resolved, and the patient returned to his normal baseline level of functioning. This case highlights the clinical course of neonatal hypocalcemic seizures, EEG findings in several cases, and possible mechanisms for both hypocalcemic precipitation of seizures and anticonvulsant ineffectiveness.     

大脑半球切开术一例报道并文献复习

目的通过我们的1例患者探讨大脑半球切开术的操作技术并附有简单的文献复习。方法 1例12岁女孩,诊断为药物难治性癫痫。临床表现为简单部分性发作,发作频繁。影像学显示左侧大脑半球弥漫性软化灶。经过充分的术前准备,在导航系统引导下行改良的经岛周大脑半球切开术,手术过程顺利。结果术后患者恢复良好,右侧肢体运动功能无明显下降,言语功能正常。术后随访6个月未再有癫痫发作。结论结合文献复习,大脑半球切开术是功能性大脑半球切除术的彻底改良,手术操作可行,具有和大脑半球切除术相同的疗效,远期并发症明显减少。