Abstract：
:A healthy 43-year-old physician developed gradually progressive and fluctuating fatigable muscle weakness involving ocular, limb, bulbar and respiratory muscles, with episodic acute respiratory failure, eventually necessitating intermittent non-invasive respiratory support (NIV). A mild short episode occurred 15 years earlier with complete resolution. Electromyography (EMG) studies and acetylcholine receptor (AchR) antibodies were repeatedly non-diagnostic. The diagnosis of myasthenia gravis (MG) was finally confirmed by direct measurement of diaphragmatic strength using magnetic nerve stimulation providing clear cut evidence of significant fatigable weakness and the demonstration of muscle-specific kinase (MuSK) serum antibodies using a novel cell-based assay. The cluster of several atypical features and lack of response to commonly used treatment modalities prompted a search for a unifying mechanism and better understanding of the underlying pathophysiology. Review of the literature suggested a possible impairment of excitation-contraction coupling with malfunction of a signaling protein downstream to the AchR, without an accompanying impairment of electrical transmission. This postulated mechanism, resulting in a disturbance of calcium signaling, explained the unusual features in this patient's illness and led to treatment with salbutamol and ephedrine and to significant symptomatic improvement not achieved by any other treatment.
journal_name
Clin Neurol Neurosurgjournal_title
Clinical neurology and neurosurgeryauthors
Haran M,Schattner A,Mate A,Starobin D,Haran G,Shtalrid Mdoi
10.1016/j.clineuro.2012.06.038subject
Has Abstractpub_date
2013-05-01 00:00:00pages
562-6issue
5eissn
0303-8467issn
1872-6968pii
S0303-8467(12)00363-0journal_volume
115pub_type
杂志文章abstract：OBJECTIVE:To determine the frequency of symptomatic and asymptomatic herniated discs and root compression in patients with lumbosacral radicular syndrome (LRS) and to correlate clinical localization with MRI findings. METHODS:Fifty-seven patients with unilateral LRS were included in the study. Using the visual analogu...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2005.10.003
更新日期：2006-09-01 00:00:00
abstract：:Paintball is an "extreme sport" that has been steadily growing in popularity since the early 1980s. Although this activity is considered recreational, there are a number of inherent dangers associated. Most notably, the number of head and neck injuries due to paintball participation has been increasing in recent years...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2008.08.006
更新日期：2009-01-01 00:00:00
abstract：PURPOSE:To analyze sagittal balance of the cervical spine after three operative methods for three consecutive levels. METHODS:A retrospective case selection and observational study was performed from December 2012 to December 2015: 20 patients underwent anterior cervical discectomy and fusion, 22 patients underwent hy...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2018.03.003
更新日期：2018-05-01 00:00:00
abstract：:Ipilimumab is an immunomodulating drug for use in treatment of unresectable or metastatic melanoma with autoimmune lymphocytic hypophysitis as a reported complication. We describe three recent cases of ipilimumab associated autoimmune hypophysitis (IAH) at our institution, and provide a selected literature review show...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,评审
doi：10.1016/j.clineuro.2014.06.011
更新日期：2014-10-01 00:00:00
abstract：:The results of the combination of CW-Doppler ultrasonography and OPG-Gee are described in 68 patients. The Doppler examination had a sensitivity of 92% and a specificity of 89%, the percentages for the OPG-Gee were 69 en 79. The combination of the Doppler examination with the OPG-Gee improves our positive predictive v...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/0303-8467(88)90026-1
更新日期：1988-01-01 00:00:00
abstract：:Moyamoya is the disease which involves the terminal portions of the internal carotid or origins of the middle or anterior cerebral arteries. The posterior communicating arteries are also involved, but not the vertebrals or the basilar artery. The disease occurs more commonly in females than males and it has two age pe...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/s0303-8467(97)00073-5
更新日期：1997-10-01 00:00:00
abstract：:Juxta-facet cysts are relatively uncommon intraspinal lesions, causing radiculopathy, neurogenic claudication or myelopathy. To the best of our knowledge, only 4 cases of bilateral synovial or ganglioncysts were described. We report the 5th case of bilateral ganglioncysts of the lumbar spine. Generally, juxta-facet cy...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/0303-8467(92)90120-r
更新日期：1992-01-01 00:00:00
abstract：OBJECTIVE:Schwannomas of the sciatic nerve, which is the largest nerve of the human body, are very rare accounting for ≤ 1% of all schwannomas. They often may raise confusion with other more common causes of sciatica, such as lumbar degenerative and inflammatory diseases or spinal tumors, which may often lead to a late...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2020.105889
更新日期：2020-08-01 00:00:00
abstract：:There are only sparse data on viral CNS infections in patients with malignant glioma. We report a case of fatal herpes encephalitis in a patient with glioblastoma in partial remission and provide a short review of the literature. ...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,评审
doi：10.1016/j.clineuro.2003.12.002
更新日期：2004-09-01 00:00:00
abstract：OBJECTIVES:The present study aimed to re-evaluate the incidence of early dysphonia after anterior cervical spine surgery (ACSS) and to determine the related risk factors. CLINICAL MATERIALS AND METHODS:Patients underwent ACSS between January 2011 and December 2013 at two sites were identified retrospectively from hosp...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2016.07.030
更新日期：2016-10-01 00:00:00
abstract：:Bilateral Symmetrical Calcifications (B.S.C.) in cerebro represent calcium deposits usually found in the basal ganglia and/or dentate nucleus. They can be the result of diverse disorders, but can also present themselves without any underlying disease. Most often they are asymptomatic, but if the calcifications are ext...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/s0303-8467(88)80036-2
更新日期：1988-01-01 00:00:00
abstract：OBJECTIVE:Early brain injury after aneurysmal subarachnoid hemorrhage (aSAH) comprises a pronounced neuroinflammatory reaction. Nevertheless, its relevance for functional outcome and its role as outcome predictor remains uncertain. We evaluated the relationship of various early inflammatory parameters regarding functio...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2015.08.030
更新日期：2015-11-01 00:00:00
abstract：:We report the first successfully treated case of Cladophialophora bantiana cerebral abscess with the relatively new antifungal agent voriconazole. Infection with this organism is often fatal. A 64-year-old man presented to our institution with progressive neurologic symptoms due to a brain abscess. A stereotactic brai...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2004.11.020
更新日期：2005-10-01 00:00:00
abstract：:Thirty-six parkinsonian patients were selected by age of onset of over 70 and a minimum of five years duration of illness. The mean age of onset was 73.5 years and 30 patients were still alive after a mean of 7.2 years. We found that late onset Parkinson's disease has a relatively benign course with more "axial sympto...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/0303-8467(92)90049-9
更新日期：1992-01-01 00:00:00
abstract：STUDY DESIGN:Blinded, placebo-controlled, prospective clinical trial. PURPOSE:To examine the effects of botulinum toxin type A (BTX-A) injections into plantar flexor muscles in stroke patients with equinovarus gait. SUBJECTS:15 post-stroke and 10 matched neurologically intact subjects. METHODS:Modified Ashworth Scal...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.clineuro.2012.03.014
更新日期：2012-06-01 00:00:00
abstract：OBJECTIVES:To assess the feasibility of cement-augmented fenestrated pedicle screw for patients with spondylolisthesis with osteoporotic spine. PATIENTS AND METHODS:From January 2014 to March 2018, a retrospective study of 88 patients with spondylolisthesis and osteoporosis treated with minimally invasive transforamin...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2019.105377
更新日期：2019-08-01 00:00:00
abstract：OBJECTIVE:To analyse the prevalence and incidence of dementia in a population of community-dwelling elderly (aged 75-80), living in socio-economically differing districts of Antwerp (Belgium), taking into account possible gender and educational differences. METHODS:A longitudinal cohort study (N=825) with a 3-year fol...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2011.07.030
更新日期：2011-11-01 00:00:00
abstract：BACKGROUND:Surgery for medically resistant epilepsy is safe and effective. However, when noninvasive techniques are insufficient, then consideration is given to invasive electrocorticography (EcoG). OBJECTIVE:The aim of the study was to analyze results and complications of subdural electrodes placement in the treatmen...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2012.10.007
更新日期：2013-07-01 00:00:00
abstract：:A case of Von Recklinghausen neurofibromatosis (VRNF) with progression during two pregnancies and obstetrical complications is described to illustrate the effect of pregnancy on VRNF and vice versa. The interaction between VRNF and pregnancy is discussed. The obstetrical complications and progression of VRNF lesions d...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/s0303-8467(87)80052-5
更新日期：1987-01-01 00:00:00
abstract：BACKGROUND:Aneurysmal subarachnoid hemorrhage (aSAH) is an often devastating form of stroke. Aside from the initial hemorrhage, cardiac complications can occur resulting in neurogenic stress cardiomyopathy (NCM), leading to impaired cardiac function. We investigated whether aSAH patients with NCM had poorer long term f...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2012.09.006
更新日期：2013-07-01 00:00:00
abstract：:A brain tumor in a 4-year-old child is described. The neoplasm was partly cystic and showed an a-typical multi-differentiated aspect. Microscopically the neoplasm had a clear-cut 'malignant' morphology. This tumor represents possibly a partly maturated primitive neuroectodermal brain tumor. The term PNET is briefly di...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/s0303-8467(86)80011-7
更新日期：1986-01-01 00:00:00
abstract：BACKGROUND:Until recently, post-radiotherapy brain abscess was considered rare, but it has become an increasingly important aetiology. Discussions of the relationship between bacterial brain abscess and radiotherapy (RT) are rare in the literature. Our clinical study was conducted to analyse the role of RT in the patho...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2015.05.025
更新日期：2015-09-01 00:00:00
abstract：:Tanycytic ependymoma is an uncommon but well-recognized variant of ependymoma. Here we report a case of tanycytic ependymoma occurring at the region of filum terminale in a 44-year male who presented with low backache, bilateral lower limb weakness and urinary incontinence. MR imaging in this patient showed a lesion t...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2011.09.017
更新日期：2012-02-01 00:00:00
abstract：BACKGROUND:While intraoperative neurophysiological monitoring (IOM) for intramedullary tumors has become a standard in neurosurgical practice, IOM for intradural extramedullary tumors (IDEMs) is still under debate. The aim of this study is to evaluate the role of IOM during surgery for IDEMs. METHODS:From March 2008 t...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2015.01.007
更新日期：2015-03-01 00:00:00
abstract：:We investigated the expression of c-myc and c-sis/PDGF mRNA and protein products in 20 cases of meningiomas of various grades: 10 benign, 5 atypical and 5 anaplastic meningiomas. All cases of atypical and anaplastic meningiomas were positive for c-myc protein and mRNA by immunohistochemistry and in situ hybridisation,...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/s0303-8467(00)00119-0
更新日期：2001-04-01 00:00:00
abstract：OBJECTIVES:Surgical excision of pineal parenchymal tumors(PPT), though desirable for good long term outcome, may not always be possible. Role of adjuvant radiotherapy (RT) in residual PPT's is not well delineated. The purpose of this single institutional retrospective study is to assess the role of radiotherapy in resi...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2018.01.027
更新日期：2018-03-01 00:00:00
abstract：:An increasing number of patients referred for neuroimaging studies unrelated to multiple sclerosis (MS) are found to have incidental lesions suggestive of MS in their nervous systems but many such patients do not develop clinical symptoms and signs and remain as "subclinical MS" (SCMS). MRI and cerebrospinal fluid (CS...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,评审
doi：10.1016/j.clineuro.2005.11.010
更新日期：2006-03-01 00:00:00
abstract：:We report a case of thalamic deep brain stimulation (DBS) for treatment of posttraumatic tremor. An 18-year-old right-handed man developed a disabling and medically refractory action tremor in the right upper extremity 9 months after sustaining diffuse axonal injury in a motor vehicle collision. DBS of the left ventra...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章
doi：10.1016/j.clineuro.2003.12.004
更新日期：2004-09-01 00:00:00
abstract：:Colloid cyst of the third ventricle is a known primary brain tumor. The familial occurrence of this cyst and associated conditions has been rarely discussed in the literature. To our knowledge only 10 affected families have been previously reported in the literature. We report the 11th family with this potentially lif...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,评审
doi：10.1016/j.clineuro.2007.04.017
更新日期：2007-09-01 00:00:00
abstract：:Migraine in pediatric is a common neurological disease, and its prevalence is varying widely. The medication for the acute attack of pediatric migraine is various. we take advantage of network meta-analysis to address the efficacy and rank of these medications. Database including Pubmed and Cochrane Library were queri...
journal_title：Clinical neurology and neurosurgery
pub_type： 杂志文章,评审
doi：10.1016/j.clineuro.2020.105853
更新日期：2020-08-01 00:00:00
