胸锁乳突肌肌电图在鉴别肌萎缩侧索硬化与颈椎病性脊髓病的研究

对106例肌萎缩侧索硬化(ALS)与颈椎病性脊髓病(CSM),及两病鉴别困难者进行胸锁乳突肌、肢体肌及舌肌EMG检查。结果ALS组胸锁乳突肌神经源性损害的异常率高于三肢体肌、舌肌;CSM组胸锁乳突肌无1例异常。表明该肌神经源性损害能明显提高ALS亚临床的阳性率,有助于ALS的早期诊断及ALS与CSM两病的鉴别。     

Amyotrophic lateral sclerosis versus cervical spondylotic myelopathy: a study using transcranial magnetic stimulation with recordings from the trapezius and limb muscles.

OBJECTIVE: We report an electrophysiological method to differentiate amyotrophic lateral sclerosis (ALS) from cervical spondylotic myelopathy (CSM). METHODS: Motor evoked potentials (MEPs) by transcranial magnetic stimulation were investigated in patients with ALS (n=10) and CSM (n=9). In addition to limb MEPs using the triple stimulation technique (TST) at upper limbs, MEPs recorded from trapezius muscles were compared with those obtained from 23 normal subjects. The parameters studied were: central motor conduction time, amplitude ratio and, for the trapezius, the interside asymmetry. RESULTS: Whereas limb MEPs were abnormal in most ALS and CSM patients (17/19), trapezius MEPs were abnormal in all ALS patients, and normal in 8 out of 9 CSM patients. CONCLUSION: Recording of trapezius MEPs is a valuable addition to the limb MEPs study, since it distinguishes ALS from SCM in most patients.     

Responses of masseter muscles to transcranial magnetic stimulation in patients with amyotrophic lateral sclerosis

We recorded motor responses evoked by transcranial magnetic stimulation (TMS) in the masseter muscles of 30 patients with amyotrophic lateral sclerosis (ALS), 10 patients with cervical spondylotic myelopathy (CSM) and 22 age-matched normal controls. Responses to direct activation of the trigeminal motor root (R-MEPs) were normal both in ALS and CSM patients. Responses to activation of cortico-bulbar descending fibers (C-MEPs) were absent or delayed in 19 ALS patients (63.3%). Abnormalities of masseter C-MEPs were more frequent than abnormalities of limb MEPs and could be observed both in patients with (77.8%) and without (41.7%) clinical bulbar signs. Masseter C-MEPs were normal in all CSM patients. Recording masseter responses to TMS can reveal the frequent impairment of cortico-bulbar projections in ALS and can be useful in the differential diagnosis of spinal cord compression disorders mimicking ALS because of combination of upper and lower motor neuron signs.     

斜方肌肌电图在肌萎缩侧索硬化早期诊断中的应用

目的 探讨斜方肌肌电图在检测肌萎缩侧索硬化(ALS)延髓部下运动神经元损害中的价值.方法 对100例ALS患者、80例颈椎病患者和100名健康志愿者进行斜方肌肌电图检测,AKS组和颈椎病组同时进行胸锁乳突肌、第一骨间肌、腹直肌、胫前肌肌电图检测,比较3组肌电图的差异.颈椎病组中43例患者进行手术前后斜方肌肌电图比较.结果 ALS组中,病程≤8个月的患者斜方肌肌电图自发电位的出现率高于病程>8个月者[分别为21/30(70%)和28/70(40%),X~2=7.56,P=0.004];斜方肌肌电图与胸锁乳突肌肌电图异常率比较差异无统计学意义.ALS组[波幅(1086.9±152.6)μV,时限(17.2±6.5)ms,多相波23.6%±3.4%]与对照组[波幅(606.7±82.7)μV,时限(11.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P<0.01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P< .01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±     

上肢DSSEPs对ALS与CSM鉴别诊断的研究

以上肢皮节体感诱发电位(DSSEPs)鉴别肌萎缩侧索硬化(ALS)与脊髓型颈椎病(CSM),结果发现前者阳性率8％,后者阳性率100％,二者存在极显著性差异(P<0.001)。进一步将异常结果与病人颈部MR对照分析,发现两方法对颈部病损定位诊断间存在线性关系。结果提示:此检查有助于ALS与CSM的鉴别。     

肌萎缩侧索硬化与颈椎病鉴别诊断方法的评估

目的 探讨早期肌萎缩侧索硬化（ＡＬＳ）与颈椎病鉴别诊断的临床首选方法。方法 对临床已确诊的５３例ＡＬＳ,１４例脊髓型颈椎病（ＣＳＭ）,２３例神经根型颈椎病（ＣＳＲ）患者的临床症状／体征和肌电图异常特征进行百分比统计及其比值比分析,对依据高比值比的临床症状／体征组合与肌电图所作出的鉴别诊断的可行性作比较。结果 以高比值比的临床症状／体征组合实施多步鉴别诊断的真阳性率９８％,真阴性率８６．５％,单凭肌电图难以作出鉴别诊断。结论 高比值比的临床症状／体征组合是ＡＬＳ与ＣＳＭ和ＣＳＲ鉴别诊断的首选临床方法。     

Illness perceptions,mood and health-related quality of life in patients with amyotrophic lateral sclerosis

ObjectivesTo divide amyotrophic lateral sclerosis (ALS) patients in two clusters according to their illness representation, and to identify, between the two clusters, the differences in functional state, mood, and quality of life.MethodsSeventy-four patients with ALS were recruited at our ALS Centre from different Italian regions, having been for multidisciplinary consultations. The patients' functional impairment was evaluated by the ALS Functional Rating Scale as well as the Bulbar Score and Forced Vital Capacity. Psychological Characteristics and quality of life of ALS patients were evaluated by Profile of Mood State, Illness Perception Questionnaire, and 36-item Short Form Health Survey.ResultsOnly few of the ALS patients studied showed critical mood ratings. On the whole, the perceived quality of life, mood state, and the dimensions relating to their illness representation seem to be correlated to the functional state and respiratory capacity. The clustering of patients according to their illness representations allowed to highlight that ALS patients can be divided into two groups: adaptors and nonadaptors. The patients of the two groups, adaptors and nonadaptors, differed in respiratory capacity as well as in their mood and health-related quality of life.ConclusionsThis study supports the Common Sense Model (CSM) of illness representation when considering ALS patients. Their psychological reactions to illness and quality of life depend not only on the severity of the illness but also on the way the illness is represented. Therefore, CSM could become the theoretical framework for psychological interventions in ALS patients.     

Illness perceptions, mood and health-related quality of life in patients with amyotrophic lateral sclerosis

Objectives

To divide amyotrophic lateral sclerosis (ALS) patients in two clusters according to their illness representation, and to identify, between the two clusters, the differences in functional state, mood, and quality of life.

Methods

Seventy-four patients with ALS were recruited at our ALS Centre from different Italian regions, having been for multidisciplinary consultations. The patients' functional impairment was evaluated by the ALS Functional Rating Scale as well as the Bulbar Score and Forced Vital Capacity. Psychological Characteristics and quality of life of ALS patients were evaluated by Profile of Mood State, Illness Perception Questionnaire, and 36-item Short Form Health Survey.

Results

Only few of the ALS patients studied showed critical mood ratings. On the whole, the perceived quality of life, mood state, and the dimensions relating to their illness representation seem to be correlated to the functional state and respiratory capacity. The clustering of patients according to their illness representations allowed to highlight that ALS patients can be divided into two groups: adaptors and nonadaptors. The patients of the two groups, adaptors and nonadaptors, differed in respiratory capacity as well as in their mood and health-related quality of life.

Conclusions

This study supports the Common Sense Model (CSM) of illness representation when considering ALS patients. Their psychological reactions to illness and quality of life depend not only on the severity of the illness but also on the way the illness is represented. Therefore, CSM could become the theoretical framework for psychological interventions in ALS patients.     

Abnormal cardiovascular responses to carotid sinus massage also occur in vasovagal syncope – implications for diagnosis and treatment

Background and purpose: Carotid sinus massage (CSM) is commonly used to identify carotid sinus hypersensitivity (CSH) as a possible cause for syncope, especially in older patients. However, CSM itself could provoke classical vasovagal syncope (VVS) in pre disposed subjects. Methods: Retrospective analysis of CSM, cardiovascular autonomic function tests (including tilt table testing) and medical history in 388 patients with recurrent syncope to identify and characterize patients in whom an abnormal response to CSM was more likely to reflect VVS than CSH. Results: CSM was abnormal in 79 patients. In 53 patients (77.2 ± 8.7 years), CSH was the likely cause of syncope. VVS was the more likely diagnosis in 26 younger patients (59.7 ± 12.6 years) with longstanding syncope from youth, in whom fear or pain was as a trigger; 7/26 suffered from intense chronic or intermittent neck pain and one exacerbation of syncopal attacks followed a physical and emotional trauma to the neck. In VVS, 4/26 had spontaneous VVS during head‐up tilt, another six after venepuncture (performed in 17/26). In 6/26, the abnormal response to CSM was delayed, occurring 62.8 ± 28.4 s after completion of CSM. The response to CSM was predominantly of the mixed type (20/26) and abnormal on both sides in 14/26. Conclusions: An abnormal response to CSM may not indicate syncope caused by CSH and needs to be considered in the light of the patient’s age, duration of syncopal episodes and detailed history of provocative stimuli. Differentiating CSH from VVS with an abnormal response to CSM has various implications from advice on driving to treatment strategies.     

Quantitative methods for cross-species mapping (CSM)

Cross-species extrapolation will be defined as prediction from one species to another without empirical vetification. Cross-species mapping (CSM) is the same except empirical vetification is performed. CSM may be viewed as validation of methods for extrapolation. Algorithms for CSM may originate from theory, from empirical observations or a combination of the two. Regardless of their origins, CSM algorithms must be explicated and confidence intervals given around their predictions. This paper offers a quantitative method for constructing CSM equations which is useful in evaluation of the CSM and as an aid in the design of new experiments in CSM and extrapolation. The method requires fitting mathematical models for the physiological or behavioral phenomena to be mapped across species. A CSM equation can then be derived from the models in each species and approximate confidence limits may be obtained for predictions from the equation. The method is useful even when the models in the two species differ in form, implying differences in physiology or behavioral principles between species. The method proposed has a number of remaining uncertainties and possible problems.     

Ezio Sciamanna: The Italian contribution to the origin of cortical stimulation mapping in humans

Influenced by Ferrier's 1873 publication documenting his initial experience with cortical stimulation mapping (CSM) across several species, 19th-century experiments applying electric current to exposed human brain soon followed. Bartholow is commonly credited with the first report of CSM in a conscious human in 1874. What is not well established is that prominent Italian neurologist, Ezio Sciamanna, localized sensorimotor function in a human demonstration of CSM shortly thereafter in 1882. Sciamanna was in the vanguard of functional localization of brain function through direct stimulation of human gray matter. Unlike Bartholow, who has been canonized in the annals of CSM, Sciamanna has remained relatively obscure, despite the fact that his case may represent a better example of true subdural CSM than his better known contemporary.     

Effects of brain derived neurotrophic factor Val66Met polymorphism in patients with cervical spondylotic myelopathy

Cervical spondylotic myelopathy (CSM) is the leading cause of spinal cord related disability in the elderly. It results from degenerative narrowing of the spinal canal, which causes spinal cord compression. This leads to gait instability, loss of dexterity, weakness, numbness and urinary dysfunction. There has been indirect data that implicates a genetic component to CSM. Such a finding may contribute to the variety in presentation and outcome in this patient population. The Val66Met polymorphism, a mutation in the brain derived neurotrophic factor (BDNF) gene, has been implicated in a number of brain and psychological conditions, and here we investigate its role in CSM. Ten subjects diagnosed with CSM were enrolled in this prospective study. Baseline clinical evaluation using the modified Japanese Orthopaedic Association (mJOA) scale, Nurick and 36-Item Short Form Health Survey (SF-36) were collected. Each subject underwent objective testing with gait kinematics, as well as hand functioning using the Purdue Peg Board. Blood samples were analyzed for the BDNF Val66Met mutation. The prevalence of the Val66Met mutation in this study was 60% amongst CSM patients compared to 32% in the general population. Individuals with abnormal Met allele had worse baseline mJOA and Nurick scores. Moreover, baseline gait kinematics and hand functioning testing were worse compared to their wild type counterpart. BDNF Val66Met mutation has a higher prevalence in CSM compared to the general population. Those with BDNF mutation have a worse clinical presentation compared to the wild type counterpart. These findings suggest implication of the BDNF mutation in the development and severity of CSM.     

Single-nucleotide polymorphisms in uncoding regions of ALS2 gene of Japanese patients with autosomal-recessive amyotrophic lateral sclerosis

ALS2 is an autosomal recessive form of amyotrophic lateral sclerosis (AR-ALS) with juvenile onset, and has been mostly found in North African and Middle Eastern countries. Deletion mutations in the coding exons of a new gene ALS2, encoding a protein with guanine-nucleotide exchange factor (GEF) domains, have recently been identified in ALS2 patients. These mutations are predicted to cause a loss of protein function, indicating that ALS2 is the causative gene underlying ALS2. To examine whether ALS2 is mutated in Japanese ALS patients sharing some characteristics of ALS2, we analyzed ALS2 gene from three patients with AR-ALS. While no deletion mutation was detected in the coding regions of ALS2 gene, several single-nucleotide polymorphisms (SNPs) that have been found in healthy controls as well as in Tunisian ALS2 patients were found mostly in intronic regions of the gene. These results suggest that deletion mutations in ALS2 gene detected in ALS2 patients seem to be uncommon in Japanese AR-ALS, and that SNPs in uncoding regions might possibly be relevant to predisposition to ALS.     

Neuropathology of parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam: an update

A comparative study was performed to investigate the differences and similarities of the neuropathological findings in the parkinsonism-dementia complex (PDC) and amyotrophic lateral sclerosis (ALS) of Guam, progressive supranuclear palsy and classic ALS. Based on the findings, it is proposed that (a) PDC is a discrete disease entity, (b) NFTs in Chamorro ALS are merely a background feature widely distributed in this population, (c) Chamorro ALS is a disease combined with classic ALS and neurofibrillary degeneration, (d) thus a subtype of “Guam ALS” is not present, and (e) PDC and ALS of Guam are different diseases.

     

Tract pain because of cervical spondylotic myelopathy: A case series

Spinal cord compression may lead to pain that is sometimes directed to areas far below the compression level. In certain cases, it may present as sciatica pain, knee pain or low back pain (LBP). These types of pain are called tract pain or funicular pain. Tract pain because of cervical spondylotic myelopathy (CSM) may lead to delays in the diagnosis and treatment of CSM in some cases, and sometimes unnecessary medical and surgical treatments. This study evaluated the results of four patients who presented to the outpatient clinic with complaints of LBP accompanying CSM findings. This study aims to present the improvement in low back pain as a result of anterior cervical microdiscectomy and cage procedure in four patients who presented with tract pain because of CSM, which is a rare condition.     

Epidurally recorded spinal cord evoked potentials in patients with cervical myelopathy and normal central motor conduction time measured by transcranial magnetic stimulation.

OBJECTIVE: Use epidural recording of evoked spinal cord potentials (ESCPs) to investigate the pathology of cervical spondylotic myelopathy (CSM) in patients with normal central motor conduction time (CMCT) in upper and lower limbs. METHODS: A total of 75 patients with CSM were studied. All patients were examined before surgery for motor evoked potentials (MEPs) following transcranial magnetic stimulation (TMS). They were also evaluated during surgery by epidurally recorded ESCPs following stimulation of the median nerve, brain and spine. RESULTS: Seven patients (9%) showed normal CMCT in upper and lower limbs upon TMS examination. Only the ESCPs following median nerve stimulation (MN-ESCPs) were abnormal in these patients. In 5 of the 7 patients, a marked block in conduction of MN-ESCPs was observed at the C3-4 intervertebral level. The remaining two patients showed attenuation in the amplitude of MN-ESCPs at mid-cervical levels. CONCLUSIONS: We present 7 cases of CSM with negative CMCT findings. From the MN-ESCP results, we surmise that the pathology of CSM with normal CMCT is due predominantly to dysfunction of sensory systems involved in the upper limbs. SIGNIFICANCE: Examination by TMS is useful in the diagnosis of CSM but the possibility of negative CMCT findings upon TMS must be borne in mind. Multi-functional evoked spinal cord responses demonstrate that lesions in the sensory system are the major underlying pathology.     

Detection of fMRI activation using cortical surface mapping

A methodology for fMRI data analysis confined to the cortex, Cortical Surface Mapping (CSM), is presented. CSM retains the flexibility of the General Linear Model based estimation, but the procedures involved are adapted to operate on the cortical surface, while avoiding to resort to explicit flattening. The methodology is tested by means of simulations and application to a real fMRI protocol. The results are compared with those obtained with a standard, volume-oriented approach (SPM), and it is shown that CSM leads to local differences in sensitivity, with generally higher sensitivity for CSM in two of the three subjects studied. The discussion provided is focused on the benefits of the introduction of anatomical information in fMRI data analysis, and the relevance of CSM as a step toward this goal.     

Transplantation of immortalized mesencephalic progenitors (CSM14.1 cells) into the neonatal parkinsonian rat caudate putamen

The present study analyzed whether grafts of the mesencephalic progenitor cell line CSM14.1 into the neonatal rat caudate putamen (CPu) differentiate into neurons and whether this is accompanied by a functional improvement in 6-hydroxydopamine (6-OHDA)-lesioned animals. As in previous studies, a neuronal differentiation of CSM14.1 cells transplanted into the CPu of adult animals could not be observed, so we here used neonatal rats, because graft location and host age seemingly are crucial parameters for neural transplant differentiation and integration. Rats bilaterally lesioned at postnatal day 1 by intraventricular 6-OHDA-injections 2 days later received 100,000 CSM14.1 cells prelabelled with the fluorescent dye PKH26 into the right CPu. Five weeks after grafting, the cylinder test was performed, and the data compared with data from age-matched intact controls and bilaterally lesioned-only animals. Brain slices immunostained for tyrosine hydroxylase (TH) were quantified by optical densitometry. We observed a significant preference of left forelimb use exclusively in transplanted animals. In these rats, TH-containing perikarya were found in the grafted CPu, presumedly leading to the significant increase of TH-immunoreactive fibers in this region. Moreover, confocal laser microscopy revealed a differentiation of transplanted PKH26-labelled CSM14.1 cells into neuronal nuclei antigen or TH-immunoreactive cells. Thus, CSM14.1 cells differentiate into TH-containing neurons, which most probably contribute to the preferred forelimb use, indicating a functional integration of CSM14.1 cells into the host basal ganglia loops during early postnatal development. These findings that are in contrast to observations in adult rats suggest instructive cues for neuronal differentiation and integration given by the neonatal microenvironment.     

Genetic factors of cervical spondylotic myelopathy-a systemic review

BackgroundCervical spondylotic myelopathy (CSM) is a degenerative disorder of the neck. Recent studies have reported the roles of single nucleotide polymorphisms and abnormal gene expression in the etiology and development of CSM. However, a systemic review of these findings is currently unavailable.MethodsA systemic review of genetic factors of CSM was conducted through searching PubMed and EMbase databases. A total of 9 studies were included in this study, which included 8 genes: brain derived neurotrophic factor (BDNF), osteopontin (OPN), bone morphogenic protein (BMP) 4, collagen IX, vitamin D receptor (VDR), apolipoprotein E (ApoE), hypoxia-inducible factor α (HIF-1α), and cyclooxygenase 2 (COX-2).ResultsThe polymorphisms of 6 genes (OPN, BMP-4, collagen IX, VDR, HIF-1α) showed significant association with the susceptibility to or risk of CSM. The polymorphisms of 3 genes (BMP-4, ApoE4, HIF-1α) were significantly associated with the postoperative outcome. The polymorphism of BDNF, VDR, and expression of COX-2 were associated with the severity of disease.ConclusionThis review demonstrates that 8 genes were associated with CSM although there is no repeated study. This review also suggests that large scale and high quality studies are needed to provide more reliable evidence for future evaluation.     

Mirror movements and ipsilateral motor evoked potentials in ALS.

It can be difficult to detect upper motor neuron (UMN) involvement in early amyotrophic lateral sclerosis (ALS). The present study tested the usefulness of mirror movements (MMs), i.e., contralateral coactivation of hand muscles, as a UMN sign in ALS. Thirty-seven patients with possible, probable or definite ALS and 19 patients with suspected ALS without clinical signs of UMN involvement, 21 disease controls and 15 healthy volunteers were included. MMs were studied clinically, electromyographically and by transcranial magnetic stimulation (TMS), looking for ipsilateral motor evoked potentials (IMEPs). MMs were observed clinically in 15 ALS patients (27%) and electromyographically in 28 (50%). IMEPs in the abductor pollicis brevis muscle following TMS were recorded in 34 (61%) of all ALS patients and in nine (47%) out of 19 patients with suspected ALS, but not in healthy controls. Central motor conduction times were prolonged in 15 ALS patients (27%). It is concluded that MMs, especially if studied by EMG and TMS (IMEPs) can essentially improve detection of UMN involvement, especially in early stages of ALS.