Enlarged high frequency oscillations of the median nerve somatosensory evoked potential and survival in amyotrophic lateral sclerosis

ObjectiveA large N20 and P25 of the median nerve somatosensory evoked potential (SEP) predicts short survival in amyotrophic lateral sclerosis (ALS). We investigated whether high frequency oscillations (HFOs) over N20 are enlarged and associated with survival in ALS.MethodsA total of 145 patients with ALS and 57 healthy subjects were studied. We recorded the median nerve SEP and measured the onset-to-peak amplitude of N20 (N20o-p), and peak-to-peak amplitude between N20 and P25 (N20p-P25p). We obtained early and late HFO potentials by filtering SEP between 500 and 1 kHz, and measured the peak-to-peak amplitude. We followed up patients until endpoints (death or tracheostomy) and analyzed the relationship between SEP or HFO amplitudes and survival using a Cox analysis.ResultsPatients showed larger N20o-p, N20p-P25p, and early and late HFO amplitudes than the control values. N20p-P25p was associated with survival periods (p = 0.0004), while early and late HFO amplitudes showed no significant association with survival (p = 0.4307, and p = 0.6858, respectively).ConclusionsThe HFO amplitude in ALS is increased, but does not predict survival.SignificanceThe enlarged HFOs in ALS might be a compensatory phenomenon to the hyperexcitability of the sensory cortex pyramidal neurons.     

Predictors of survival in 171 patients with systemic sclerosis (scleroderma)

Summary Predictors of survival were determined in 171 patients with systemic sclerosis by univariate analysis, and the Cox proportional hazards model using both cross sectional data at entry into the follow-up and time-dependent follow-up data. Clinical and laboratory data were evaluated from 1982 to the end of 1993. The presence of diffuse scleroderma, kidney and cardiac involvements were unfavourable prognostic signs in both the univariate analysis, and the Cox proportional hazards models. The Cox model, using the variables detected at study entry, indicated that pericarditis, and anaemia were bad prognostic signs. Analysis with time dependent data has not been reported in systemic sclerosis. The appearance of pigmentation disturbances, anaemia, and respiratory failure during the follow-up also caused a poor prognosis of the disease by the Cox model. In the stepwise selection models, diffuse scleroderma, internal organ manifestations including renal, and cardiac involvements were predominantly selected as the most unfavourable factors for survival. As to the extent of skin involvement and internal organ manifestations, the general behaviour of the disease seems to be similar throughout the world. The early appearance of pericarditis and pigmentation disturbances at study entry are bad prognostic signs.This work was supported by the Hungarian Ministry of Health and Social Welfare and by the National Foundation for Scientific Research.     

Antinuclear antibodies in scleroderma,mixed connective tissue disease and “primary” Raynaud's phenomenon

Summary The diversity of antibodies in patients with scleroderma, mixed connective tissue disease or primary Raynaud's phenomenon could be used as a laboratory aid in the clinical diagnosis. In serum samples of 75 patients we screened for antinuclear antibodies (HEp 2 cells), anti DNA, soluble nucleoprotein and extractable nuclear antigens (Sm, rRNP, Ul-nRNP, SSA/Ro, SSB/La and Scl-70). Distinctive antinuclear antibodies pattern was identified in each group of patients. This immunologic profile is valuable for clinical diagnosis and the preferential association of certain autoantibodies with some diseases and not with others, suggest an antigen-driven stimulus for its production.     

Prolonged dynamic clinico-immunological observation of 85 patients with definite multiple sclerosis: First steps towards monitoring process activity

Prolonged clinico-immunological observation of 85 patients with definite multiple sclerosis (MS) was performed in order to elucidate the connections between the clinical and immune state. A battery of immunological investigations was performed, including estimation of T-cell subpopulations in blood and cerebrospinal fluid (CSF); proliferative responses of circulating lymphocytes to mitogens, recombinant interleukin-2 (rIL2) and myelin basic protein levels in different culture conditions; levels of immunoglobulin (Ig) in sera and CSF, and of Ig production in vitro; indices of IL2 synthesis and IL2 sensitivity; production of prostaglandin E2 and tumour necrosis factor (TNF) alpha by monocytes and levels of -endorphin in sera and supernatants phytohaemagglutinin of (PHA)-activated cells. Clinical observation was performed periodically using Kurtzke scales and was supplemented by repeated recording of evoked potentials and magnetic resonance imaging. Initial investigations showed specific differences between patients with MS and the control groups (donors and patients with other neurological disorders of the same age). Correlative and regressive analyses showed no association between immunological and clinical parameters at the initial investigation, although immunological indexes were inter-related, and indicated specific alterations in immuno-regulation in MS. Retrospective analysis revealed associations between the clinical status of patients with MS and their previous immune status. Evidence of cell activation — including a decreased percentage of circulating cells with differential antigens, lower cell mitogen-induced proliferative responses in vitro, with restoration following the addition of autoserum, greater IL2 sensitivity, and increased TNF-alpha production by macrophages — often predicted the clinical manifestation of deterioration. It is proposed that the immunopathological process in MS has a number of stages with characteristic features, and that progression from one stage to another can be subclinical. No single immunological index can be used to determine stage. Only systemic alterations reflect the real situation, whilst every patient has some abnormalities. A system of clinico-immunological monitoring could severe as the basis for a new approach to the dynamic treatment of MS.     

脊髓MRI对多发性硬化的诊断和鉴别诊断价值

目的探讨脊髓MRI对多发性硬化(MS)的诊断和鉴别诊断价值。资料与方法80例早期MS患者作为研究对象,作脑部和脊髓MRI检查,分析脊髓和脑部病灶的特征,脊髓病变的发病率与MS诊断的相关性。结果(1)80例脊髓检查中,脊髓异常者65例(81.25%),其中颈髓18例(27.69%),胸髓13例(20.0%),腰骶髓5例(7.69%),颈髓和胸髓同时受累29例(44.62%)。(2)脊髓局灶性病灶37例(46.25%),弥漫性11例(13.75%),局灶并弥漫性病灶17例(21.25%)。(3)不联合脊髓病灶53例可诊断为MS,敏感性为66.25%,若1个脊髓病灶替代1个脑部病灶,68例可诊断为MS,敏感性为85%,两者差异具有统计学意义(P<0.05)。结论脊髓MRI可显示MS在空间的播散性,能提高MS诊断的敏感性也有助于对MS的鉴别诊断。     

脊髓型颈椎病与肌萎缩性侧束硬化的电生理对比研究

目的：探讨电生理检查对脊髓型颈椎病（CSM）与肌萎缩性侧束硬化（ALS）的签别诊断价值。方法：对124例其中52例脊髓型颈椎病和72例肌萎缩性侧束硬化症进行上、下肢肌、胸锁乳突肌肌电图检查,及C5～C8皮节体感诱发电位（DSEP）检查。结果：肌电图（EMG）检查脊髓型颈椎病上、下肢肌、胸锁乳突肌肌电图异常率分别为54％、8％、8％;肌萎缩性侧束硬化异常率分别为97％、89％、69％。DSEP检查脊髓型颈椎病异常率为92％;肌萎缩性侧束硬化异常率为l％,差异具有非常显著性意义（P〈0、001）。结论：选择上、下肢肌、胸锁乳突肌肌电图检查及C5-C8皮节体感诱发电位检查应作为两种病的常规电生理检查项目,可提高两种病的鉴别诊断率。     

Magnetic resonance imaging and 1H-magnetic resonance spectroscopy in amyotrophic lateral sclerosis

We aimed to increase confidence in the combined use of MRI and proton MR spectroscopy (¹H-MRS) in diagnosis of amyotrophic lateral sclerosis (ALS). We investigated 12 patients with ALS, seven definite and five probable, taking into account clinical measures of motor neuron function. On T2-weighted images we found high signal in the corticospinal tract in six and low signal in the primary motor cortex in seven of the 12 patients. Atrophy of the precentral gyrus was apparent in all the patients apart from one with probable ALS. Absolute quantification of cerebral metabolites using ¹H-MRS demonstrated a significantly lower mean concentration of N-acetylaspartate (NAA) in the precentral gyrus of patients with probable and definite ALS (8.5 ± 0.62) than in control subjects (10.4 ± 0.71; P < 0.001). NAA concentration in primary motor cortex correlated with Norris scale scores (r = 0.30; P < 0.0001) but not with the ALS Functional Rating Scale score or disease duration. Significantly lower levels of NAA were detected in patients with low signal in the motor cortex than in those without (P < 0.01). Mean choline (Cho) and creatine (Cr) values did not differ between patients with ALS and controls. Received: 20 July 2000 Accepted: 1 September 2000     

Measuring the validation of assessing the non-dominant-hand function by ALSFRS-r in Chinese ALS patients

ALSFRS-r is a widely accepted rating scale for measuring the global function of Amyotrophic Lateral Sclerosis (ALS) patients, but we found some limitations of ALSFRS-r in assessing the function of non-dominant hand in Chinese ALS patients. We reviewed 95 ALS patients who expressed upper-limb symptoms at first visit and analyzed the ALSFRS-r score and subscale. In both upper limb involved patients, the ALSFRS-r had no difference between dominant-hand and non-dominant-hand onset groups (39.15 ± 5.55 vs 38.0 ± 5.91, p = 0.477). But in only one upper limb involved patients, the ALSFRS-r score in non-dominant-hand onset patients was higher than dominant-hand onset patients (43.94 ± 3.44 vs 40.87 ± 4.42, p < 0.05), especially in item of handwriting, cutting food and handing utensils (3.56 ± 0.89 vs 2.2 ± 1.27 p = 0.001, 3.44 ± 1.03 vs 1.8 ± 1.21 p = 0.000). When the item of cutting food and handing utensils was replaced by using food bowl and chopsticks to assess the function of non-dominant-hand, the modified ALSFRS-r score was significantly lower than original ALSFRS-r (43.94 ± 3.44 vs 42.88 ± 3.07 p = 0.001), the progression rate was slower (0.81 ± 0.63 vs 0.64 ± 0.63, p = 0.001). So, for Chinese ALS patients, using food bowl and chopsticks should replace the item of cutting food and handling utensils to assess the non-dominant-hand function, especially in non-dominant-hand onset patients.