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Fatigability is the hallmark of myasthenia gravis (MG). It is not clear, however, whether there is an analogous increase in jitter during the course of a single fiber electromyography (SFEMG) session. The individual jitter values of all potentials of 76 normal and 44 myasthenic orbicularis oculi muscles were assigned a rank number according to their temporal order in which they were collected and linear regression was performed to determine if the slope of the regression line was significantly different from zero. Control and MG subjects displayed rather flat linear regression lines with non-significant positive or negative slopes. Accordingly, ROC analysis yielded areas under the curve near 0.5. We conclude that there is no systematic jitter increase during the collection of 20 potential pairs in a typical SFEMG session.

Considering genetic influence on brain structure and function, including motor control, we report a case of right-handed monozygotic twins with atypical organization of fine motor movement control that might imply genetic influence. Structural and functional organization of the twins' motor function was assessed using transcranial magnetic stimulation (TMS), fMRI with a motor-task paradigm, and diffusion tensor imaging (DTI) tractography. TMS revealed that both twins presented the same unexpected activation and inhibition of both motor cortices during volitional unilateral fine hand movement. The right ipsilateral corticospinal tract was weaker than the left contralateral one. The motor-task fMRI identified activation in the left primary motor cortex and bilateral secondary motor areas during right-hand (dominant) movement and activation in the bilateral primary motor cortex and secondary motor areas during left-hand movement. Based on DTI tractography, both twins showed a significantly lower streamline count (number of fibers) in the right corticospinal tract compared with a control group, which was not the case for the left corticospinal tract. Neither twin reported any difficulty in conducting fine motor movements during their activities of daily living. The combination of TMS and advanced neuroimaging techniques identified an atypical motor control organization that might be influenced by genetic factors. This combination emphasizes that activation of the unilateral uncrossed pyramidal tract represents an alternative scheme to a "failure" of building a standard pattern but may not necessarily lead to disability.

Muscle fascicle length and muscle fibre conduction velocity (MFCV) are thought to be important parameters for power performance. It might be expected that faster muscle fibre conduction velocities would compensate for longer fascicle lengths to increase the speed of action potential propagation along the elongated fibres. However, the relationship between muscle fascicle length and MFCV remains unknown. The aim of the present study was to explore the relationship between average vastus lateralis MFCV and average fascicle length. In 17 moderately trained, healthy, male, physical education students (age, 23.4 ± 3.1 years; body height, 178 ± 5.5 cm; body mass, 82.7 ± 6.9 kg; body mass index, 24.6 ± 1.5 kg·m−2) resting MFCV was measured with intramuscular microelectrodes while muscle architecture was evaluated with ultrasonography. Fascicle length was highly correlated with total MFCV (r = 0.923, p = 0.000), maximum MFCV (r = 0.949, p = 0.000), and MFCV of the fastest (r = 0.709, p = 0.001), but not of the slowest fibres (r = 0.131, p = 0.616). No significant correlations were also found between vastus lateralis thickness or fascicle angle with any of MFCV parameters (r = 0.145–0.430; R2 < 0.130; p > 0.05). These data indicate that average MFCV is associated with average fascicle length in vastus lateralis muscle in different individuals. It seems that participants with longer fascicle lengths have also higher MFCVs.

The comorbidity of myasthenia gravis (MG), with other autoimmune disorders like systemic lupus erythematosus (SLE), is relatively frequent but the co-occurrence with chronic inflammatory demyelinating polyneuropathy (CIDP) along with various autoimmune manifestations in the absence of thymoma is of extreme rarity. Our aim is to report a case of a woman who presented the concomitant appearance of MG, axonal sensory-motor polyneuropathy and hepatitis that may indicate an underlying pathogenetic link among the different autoimmune disorders.We present a case of a 54-year-old woman, with a generalized MG and a chronic sensory-motor polyneuropathy, hypothyroidism, anaemia, hepatitis, livedo reticularis and facial flush, of assumed autoimmune background, like SLE, although with persistent negative ANA antibodies, from the beginning and through the whole following years. The Human Leukocyte Antigen (HLA)-DRB1 genotyping showed a profile of alleles (To raise awareness among clinicians about the exceptional combination of autoimmune manifestations driven by a specific HLA background.

Considering genetic influence on brain structure and function, including motor control, we report a case of right-handed monozygotic twins with atypical organization of fine motor movement control that might imply genetic influence. Structural and functional organization of the twins' motor function was assessed using transcranial magnetic stimulation (TMS), fMRI with a motor-task paradigm, and diffusion tensor imaging (DTI) tractography. TMS revealed that both twins presented the same unexpected activation and inhibition of both motor cortices during volitional unilateral fine hand movement. The right ipsilateral corticospinal tract was weaker than the left contralateral one. The motor-task fMRI identified activation in the left primary motor cortex and bilateral secondary motor areas during right-hand (dominant) movement and activation in the bilateral primary motor cortex and secondary motor areas during left-hand movement. Based on DTI tractography, both twins showed a significantly lower streamline count (number of fibers) in the right corticospinal tract compared with a control group, which was not the case for the left corticospinal tract. Neither twin reported any difficulty in conducting fine motor movements during their activities of daily living. The combination of TMS and advanced neuroimaging techniques identified an atypical motor control organization that might be influenced by genetic factors. This combination emphasizes that activation of the unilateral uncrossed pyramidal tract represents an alternative scheme to a "failure" of building a standard pattern but may not necessarily lead to disability.

Characterization of the least number of muscle fibers analyzed for a quick and reliable, evaluation of intramuscular fiber conduction velocity (MFCV) is of importance for sport scientists. Aim of the present study was to evaluate the reliability of vastus lateralis’ intramuscular MFCV measuring either 25 or 50 different muscle fibers per participant, as well as to compare intramuscular MFCV measured in 25 (C25), 50 (C50) or 140 (C140) muscle fibers. Resting vastus lateralis’ MFCV was measured in twenty-one young healthy males (age 22.1 ± 2.4 yrs) using intramuscular microelectrodes in different days. Test-retest reliability of MFCV's parameters were calculated for C25 and C50 while MFCV was compared among C25, C50 and C140. Significant differences of MFCV parameters were observed between C25 condition and those of C50 and C140. The differences in MFCV values between conditions C50 and C140 were non-significant. A close correlation was found for MFCV between C50 and C140 (r = 0.884 - 0.988, p = 0.000). All reliability measures of MFCV measured with 50 fibers were high (e.g. ICC = 0.813 - 0.980, p = 0.000), in contrast to C25 (e.g. ICC = 0.023 - 0.580 p > 0.05). In conclusion, an average of 50 different fibers per subject is sufficient to provide a quick and reliable intramuscular evaluation of vastus lateralis MFCV. This article is protected by copyright. All rights reserved.

PURPOSE The aim of our study was the comparison of active denervation (fibrillation and/or positive sharp wave potentials) in thoracic paraspinal muscles with rectus abdominis in patients with definite amyotrophic lateral sclerosis. METHODS Ninety-five consecutive patients with clinically definite amyotrophic lateral sclerosis according to the revised El Escorial criteria were studied prospectively over a 5-year period. Concentric needle electromyogram was performed in thoracic paraspinal muscles, in the rectus abdominis at the T9 level, and in limb muscles. RESULTS Active denervation was present in thoracic paraspinal muscles in 75 patients (79%) and in rectus abdominis in 62 patients (65.3%) (P = 0.02). No significant difference was found between the two muscles regarding the type of onset (bulbar, upper, and lower limbs), amyotrophic lateral sclerosis functional rating scale values, and creatine phosphokinase levels. CONCLUSIONS Thoracic paraspinal muscles are the first to be tested in patients with amyotrophic lateral sclerosis. Absence of active denervation in T-PSM is rarely associated with active denervation in rectus abdominis.

The aim of this study was to explore the relationship between muscle fiber conduction velocity (MFCV), fiber type composition, and power performance in participants with different training background.Thirty-eight young males with different training background participated: sedentary (n = 10), endurance runners (n = 9), power trained (n = 10), and strength trained (n = 9). They performed maximal countermovement jumps (CMJ) and maximal isometric leg press for the measurement of the rate of force development (RFD). Resting vastus lateralis MFCV was measured with intramuscular microelectrodes on a different occasion, whereas muscle fiber type and cross-sectional area (CSA) of vastus lateralis were evaluated through muscle biopsies 1wk later.MFCV, CMJ power, RFD, and % CSA of type II and type IIx fibers were higher for the power-trained group (P0.001). No difference was found between sedentary participants and endurance runners in these variables, but both of these groups performed worse than strength/power participants. Close correlations were found between MFCV and fiber CSA as well as the % CSA of all fiber types as well as with RFD and CMJ power (r = 0.712-0.943, P0.005). Partial correlations revealed that the % CSA of IIx fibers dictates a large part of the correlation between MFCV and RFD, power performance. Significant models for the prediction of the % CSA of type IIa and type II as well as the CSA of all muscle fibers based upon MFCV, RFD, and CMJ were revealed (P = 0.000).MFCV is closely associated with muscle fiber % CSA. RFD and jumping power are associated with the propagation of the action potentials along the muscle fibers. This link is regulated by the size and the distribution of type II, and especially type IIx muscle fibers.

Background Oxaliplatin is an effective drug used mainly for advanced colorectal cancer. Neurotoxicity is the major side effect of oxaliplatin. The present clinical and neurophysiologic study was conducted to evaluate patients receiving oxaliplatin therapy. Patients and Methods Thirty-one consecutive patients with colorectal cancer who received oxaliplatin therapy were followed up for more than 3 years. The patients underwent clinical and neurophysiologic tests for large and small fiber function at every visit. Results Most of the patients received oxaliplatin-based chemotherapy at the initial dose of 130 mg/m2 for 6 to 8 cycles, normally every 3 weeks. Acute neurotoxicity with cold and mechanical hyperalgesia was reported by the vast majority of patients after each cycle of therapy and was confirmed by the quantitative sensory, filament, and axon reflex test. Chronic sensory cumulative neuropathy developed in most of the patients after the middle of therapy with numbness and was assessed using clinical scales, nerve conduction studies, and the vibration threshold. Our results support the persistence of the sensory nerve deficits for years after cessation of oxaliplatin therapy. Conclusion Our study has confirmed the results of a few previous long-term studies concerning the persistence of chronic large sensory fiber neuropathy and the influence of the cumulative dose of oxaliplatin on the development and severity of the chronic neuropathy. Our findings have improved the knowledge about the acute oxaliplatin-induced neurotoxicity using the C-fiber axon reflex response.

The association between gray matter (GM) density and neurophysiologic changes is still unclear in amyotrophic lateral sclerosis (ALS). We evaluated the relationship between GM density and motor system integrity combining voxel-based morphometry (VBM) and transcranial magnetic stimulation (TMS) in ALS. We included 17 ALS patients and 22 healthy controls (HC) who underwent 3D-T1-weighted imaging. Among the ALS group, we applied left motor cortex single-pulse TMS. We used whole-brain VBM comparing ALS and HC in GM density. We also conducted regression analysis to examine correlations between GM density and the following TMS parameters: motor evoked potential (MEP)/M ratio and central motor conduction time (CMCT). We found significantly decreased GM density in ALS patients in several frontal, temporal, parietal/occipital and cerebellar regions (p

PURPOSE The authors have observed that in healthy people, the Ulnar wrist-to-first dorsal interosseous distal motor latency does not differ significantly compared with median wrist-to-abductor pollicis brevis distal motor latency. The aim of our study was to investigate whether the difference between these two latencies can be used as a screening tool for diagnosing carpal tunnel syndrome and how this technique compares with other established techniques. METHODS The study was set up as a prospective observational study. As gold standard for the clinical diagnosis of carpal tunnel syndrome, the authors used the opinion of two neurologists who independently examined the patients. A third neurologist, also independently, performed the electrophysiological study. RESULTS Eighty-four subjects, 42 patients and 42 age- and sex-matched controls, participated in the study. Among all subjects using a receiver operating characteristic curve analysis, the area under the curve was 0.924 (95% CI, 0.857-0.991; SE, 0.034; P < 0.001). To detect carpal tunnel syndrome, at a cutoff score of equal to or greater than 0.575 milliseconds, our technique showed a sensitivity of 91%, a specificity of 93%, a positive predictive value of 93%, and a negative predictive value of 91%. Compared with other "classical" techniques, our technique showed better area under the receiver operating characteristic curve and better Youden index. CONCLUSIONS The median wrist-to-abductor pollicis brevis motor latency minus ulnar wrist-to-first dorsal interosseous motor latency may be used as a novel rapid screening tool of patients suffering from carpal tunnel syndrome.

Purpose/aim of the study: An increased serum level of creatine kinase (CK) in asymptomatic individuals is a diagnostic challenge, as it may be associated with either physiological conditions, such as exercise or even signal an ominous neuromuscular disease at a presymptomatic stage. The electromyogram (EMG) and the muscle biopsy play a key role in the evaluation of asymptomatic hyperckemia. The objective of this study was to investigate asymptomatic individuals with increased CK levels.We comparatively studied EMG, quantitative EMG and muscle biopsy in asymptomatic clinically normal individuals with repeatedly increased CK levels.Conventional EMG was abnormal in 76% of patients, while quantitative EMG showed abnormal results in 88.9%. Muscle biopsy was diagnostic in 28%, one patient had neurogenic findings, 40% showed non-specific changes and 28% had normal results.EMG and especially quantitative EMG are highly sensitive in detecting subclinical neuromuscular diseases, whereas muscle biopsy may better contribute in the final diagnosis. No strong correlations were found between histological abnormalities and electrophysiological data, but further research is needed.

The aim of this study was the diagnosis of patients with isolated ocular manifestations (ptosis and/or diplopia) referred for electrophysiological evaluation to the electrodiagnostic laboratory of a University Neurological Department. Examination was performed either in inpatient status or in outpatient basis. We analyzed the clinical, electrophysiological and other laboratory data in 79 subjects. Myasthenia gravis (MG) was diagnosed in 38 %, 45.6 % in other diseases (Graves disease, blepharospasm, IIId cranial verve palsy, multiple sclerosis, stroke, etc.), while in 16.5 %, the cause remained unidentified. Symptoms fluctuation was significantly more frequent in the myasthenic patients, compared to patients with other diseases. The presence of both diplopia and ptosis are more likely due to MG rather than other pathology.

The aim of this study was to compare Bangladeshi immigrants with diabetes to native Greeks with diabetes and to distinguish the different risk factors for polyneuropathy (PN) in the two ethnic groups. Subjects were recruited from the outpatient diabetic clinic of a general hospital. A total of 111 Bangladeshi immigrants (97 men and 14 women of mean age 47 years) and 101 native Greeks (82 men and 19 women of mean age 49 years) were included in the study. Sex, mean age, age at diabetes diagnosis, and diabetes duration did not differ between the two groups. PN was diagnosed in 53 (48%) Bangladeshi and in 59 (58%) Greek patients (p = 0.12). Large fiber neuropathy was less prevalent among Bangladeshis (18%) than in Greeks (53%) (p

In EMG interference pattern analysis, the peak value of turns to mean amplitude ratio [peak(T/A)] is an established clinically significant marker, but its calculation requires specific software available only in few EMG apparatuses. On the contrary, the turns to mean amplitude ratio obtained at maximal muscle contraction (T/Amax) is easily calculated but less well standardized. We aimed to quantitatively assess the association between T/Amax and peak(T/A). Data were derived from 642 muscle contractions (Nc) from 270 consecutive patients (Np) who underwent EMG at our laboratory (software Dantec Keypoint, QEMG) from May 2015 to September 2016 and had interference patterns obtained from at least one of the following muscles: triceps-lateral head, brachioradialis, extensor digitorum communis and biceps. Statistics were calculated separately for normal and neurogenic muscles. Peak(T/A) was calculated by the built-in "peak ratio" function. T/Amax was calculated by the built-in Interference Pattern analysis function. The ratio with the highest amplitude was selected as T/Amax. Linear regression models provided high Pearson correlation coeffficientscoefficients (R) between peak(T/A) and T/Amax for all 4 muscles, normal or neurogenic, except a subgroup of biceps in patients aged40y. Specifically, R were: (A) triceps normal 0.79 (Nc = 99), neurogenic 0.83 (Nc = 50) (B) brachioradialis normal 0.81 (Nc = 84), neurogenic 0.78 (Nc = 66) (C) extensor digitorum communis normal 0.72 (Nc = 92), neurogenic 0.73 (Nc = 61) (D) biceps (age 40y) normal 0.77 (Nc = 77), neurogenic 0.67 (Nc = 62). We conclude that T/Amax has a strong linear association with peak(T/A) and, therefore, the former may be further investigated as a potentially useful quantitative diagnostic marker, especially in cases where the latter is not available.

Background: The Boston Carpal Tunnel Questionnaire (BCTQ) is an easy, brief, self-administered questionnaire developed by Levine et al for the assessment of severity of symptoms and functional status of patients with carpal tunnel syndrome. The aim of our study was to develop and validate the Greek version of BCTQ. Methods: We conducted a cross-sectional study of 90 patients with idiopathic carpal tunnel syndrome. The original English version of BCTQ was adapted into Greek using forward and backward translation. Reliability was assessed by internal consistency (Cronbach α and item-total correlation) and reproducibility. Validity was examined by correlating the Boston Questionnaire scores to Canterbury severity scale for electrodiagnostic severity grading. Results: The Greek version showed high reliability (Cronbach α 0.89 for Symptom Severity Scale and 0.93 for Functional Status Scale) and construct validity (Pearson correlation coefficient 0.53 for Symptom Severity Scale and 0.68 for Functional Status Scale). Test-retest were 0.75 for Symptom Severity Scale and 0.79 for Functional Status Scale ( P < .05). Receiver operating characteristic curve analysis showed that the optimal cutoff of Symptom Severity Scale for the discrimination of subjects with low electrodiagnostic severity grading than subjects with high electrodiagnostic severity grading was 1.95 with sensitivity equal to 75.5% and specificity equal to 68.3%. Conclusions: The Greek version of the BCTQ is a valid, reliable screening tool for assessment in daily practice of symptoms and functional status in patients with carpal tunnel syndrome.

The phenotypic heterogeneity in amyotrophic lateral sclerosis (ALS) implies that patients show structural changes within but also beyond the motor cortex and corticospinal tract and furthermore outside the frontal lobes, even if frank dementia is not detected. The aim of the present study was to investigate both gray matter (GM) and white matter (WM) changes in non-demented amyotrophic lateral sclerosis (ALS) patients with or without cognitive impairment (ALS-motor and ALS-plus, respectively). Nineteen ALS-motor, 31 ALS-plus and 25 healthy controls (HC) underwent 3D-T1-weighted and 30-directional diffusion-weighted imaging on a 3 T MRI scanner. Voxel-based morphometry and tract-based spatial-statistics analysis were performed to examine GM volume (GMV) changes and WM differences in fractional anisotropy (FA), axial and radial diffusivity (AD, RD, respectively). Compared to HC, ALS-motor patients showed decreased GMV in frontal and cerebellar areas and increased GMV in right supplementary motor area, while ALS-plus patients showed diffuse GMV reduction in primary motor cortex bilaterally, frontotemporal areas, cerebellum and basal ganglia. ALS-motor patients had increased GMV in left precuneus compared to ALS-plus patients. We also found decreased FA and increased RD in the corticospinal tract bilaterally, the corpus callosum and extra-motor tracts in ALS-motor patients, and decreased FA and increased AD and RD in motor and several WM tracts in ALS-plus patients, compared to HC. Multimodal neuroimaging confirms motor and extra-motor GM and WM abnormalities in non-demented cognitively-impaired ALS patients (ALS-plus) and identifies early extra-motor brain pathology in ALS patients without cognitive impairment (ALS-motor).

Objective. We investigated differences in pain perception between men and women of reproductive age by using Laser-Evoked Potentials (LEPs). Design, Setting, Subjects. Forty-four right-handed healthy volunteers (19 males/25 females), aged 30–40 years were studied. A CO2 laser generated three series of 10 thermal pulses (4.5 W) on the radial aspect of the dorsum of the left hand. A recording montage for late LEPs was used, and the potentials of each series of stimuli were averaged to calculate mean latency and amplitude for each subject. Volunteers scored verbally pain intensity (Numerical rating scale [NRS]; 0–10). Three series of 10 numbers were averaged for calculation of mean NRS score. Methods. LEP peak-to-peak amplitude, latency, and NRS scoring were compared between genders, and correlations between LEP amplitude/latency and NRS scores were assessed. Results. Data from 44 subjects were analyzed. LEP amplitudes differed significantly ( P

Background Spastic paretic hemifacial contracture (SPHC) is characterized by sustained unilateral contraction of the facial muscles associated with mild ipsilateral facial paresis. Rarely described in the context of multiple sclerosis (MS), it has never been reported as presenting symptom of MS. Case reports Two patients developed SPHC within the context of a clinically isolated syndrome suggestive of MS. EMG revealed continuous resting activity of irregularly firing motor unit potentials, associated with impaired recruitment upon voluntary contraction. SPHC remitted fully in both patients. Conclusions SPHC, a rare but distinct clinical and EMG entity, can occasionally be the presenting feature of MS.

& AbstractBackground: The Douleur Neuropathique 4 questionnaire(DN4) was developed by the French Neuropathic Pain Groupand is a simple and objective tool, primarily designed toscreen for neuropathic pain. The aim of our study is tovalidate the DN4 in the Greek language.Methods: The study was set up as a prospective observa-tional study. Two pain specialists independently examinedpatients and diagnosed them with neuropathic, nociceptive,or mixed pain, according to the International Association forthe Study of Pain (IASP) definitions. A third and a fourthphysician administered the DN4 questionnaire to thepatients.Results: Out of the 237 patients who met our inclusioncriteria and had identical diagnoses regarding the type ofpain, 123 were diagnosed with neuropathic, 59 with noci-ceptive, and 55 with mixed pain. Among patients withidentical diagnoses of neuropathic or nociceptive pain, usinga receiver operating characteristic (ROC) curve analysis, thearea under the curve (AUC) was 0.92. A cutoff point of equalor greater than 4 resulted in a sensitivity of 93% and aspecificity of 78%. Among patients suffering from pain withneuropathic element (neuropathic or mixed pain) or painwith no neuropathic element (nociceptive pain), using a ROCcurve analysis, the AUC was 0.89. A cutoff point of equal orgreaterthan4resultedinasensitivityof89%andaspecificityof 78%.Conclusion: The Greek version of DN4 is a valid tool fordiscriminating between neuropathic and nociceptive painconditions in daily practice. &Key Words: pain, neuropathic, DN4, Douleur Neuropathi-que 4, validation, Greek

Aim Determination of the 25(OH) vitamin D levels in Greek-born and in Bangladeshi immigrant patients in Greece with diabetes with and without polyneuropathy. Materials and methods The method for the detection and staging of polyneuropathy proposed by Dyck, 1988 was used. Results A total of 111 Bangladeshi immigrants and 101 Greek diabetic patients took part in the study. Vitamin D levels were significantly lower in Bangladeshi than in Greek diabetic patients, and were significantly lower in Greek patients with small-fiber neuropathy. In Bangladeshi patients, there was no statistically significant difference in the subgroup of patients with polyneuropathy in comparison to those without polyneuropathy. Conclusion The association of vitamin D deficiency only with a small number of Greek patients with exclusively small-fiber neuropathy does not allow us to draw a definite conclusion on the role of vitamin D in the pathogenesis of diabetic neuropathy.

To investigate the underlying mechanisms of polyneuropathy induced by HIV infection or antiretroviral drugs.We tested 100 HIV patients (59 with AIDS). Ninety-three patients received antiretroviral drugs. Forty-four were treated with neurotoxic compounds (ddI, ddC, d4T). Nerve conduction velocities and the sympathetic skin response (SSR) in palms and soles were measured in all patients. In skin biopsies (ankle and thigh), the intraepidermal nerve fiber density (IENFD) and the number of epidermal fibers without contact to the basal membrane (fragments) were quantified using PGP9.5 staining.Severity of the disease (CD4 +count) correlated to conduction velocities of peroneal (p0.01, Spearmans rank correlation), sural (p0.01) and median nerves (p0.05/p0.001, sensory/motor). In contrast, the duration of neurotoxic treatment did not impair conduction velocities (p0.3) but correlated to reduced IENFD in the ankle (r = -0.24, p0.05). Despite their reduced IENFD, patients with long neurotoxic treatment had a high number of fragments irrespective of their CD4 +count.Neurotoxic treatment appears to primarily impair thin fiber conduction, whereas HIV neuropathy is linked to large fiber impairment and reduction of fragments of nerve fibers.These findings emphasize the differential pattern of polyneuropathy in HIV patients caused by the infection or induced by antiretroviral treatment.

Myasthenia gravis (MG) is usually caused by antibodies against the muscle acetylcholine receptor (AChR). Experimental autoimmune MG (EAMG) is the animal model of MG, typically induced by immunization of rodents with AChR isolated from the electric organ of Torpedo californica. We have successfully induced EAMG in Lewis rats by immunization with the extracellular domains (ECDs) of the human AChR subunits (α, β, γ, δ and e) expressed in yeast. Analysis of the antibody titers revealed a robust antigenic response against all the peptides, but a marked difference in their pathogenicity; the α subunit ECD was the most pathogenic, resulting in the highest percentage of affected animals. Measurements of antibody titers, electromyographic tests and quantitation of the muscle AChR content, offered further support to these findings. The EAMG models presented herein, could be used for studying subunit-specific pathogenic mechanisms, and, more importantly, as tools for the evaluation of antigen-specific therapeutic approaches, which rely on the human AChR.

Objective To integrate neurophysiological findings with clinical and imaging data in a consecutive series of multiple sclerosis (MS) patients developing facial numbness during the course of an MS attack. Methods Nine consecutive patients with MS and recent-onset facial numbness were studied clinically, imaged with routine MRI, and assessed neurophysiologically with trigeminal somatosensory evoked potential (TSEP), blink reflex (BR), masseter reflex (MR), facial nerve conduction, facial muscle and masseter EMG studies. Results All patients had unilateral facial hypoesthesia on examination and lesions in the ipsilateral pontine tegmentum on MRI. All patients had abnormal TSEPs upon stimulation of the affected side, excepting one that was tested following remission of numbness. BR was the second most sensitive neurophysiological method with 6/9 examinations exhibiting an abnormal R1 component. The MR was abnormal in 3/6 patients, always on the affected side. Facial conduction and EMG studies were normal in all patients but one. Conclusions Facial numbness was always related to abnormal TSEPs. A concomitant R1 abnormality on BR allowed localization of the responsible pontine lesion, which closely corresponded with MRI findings. We conclude that neurophysiological assessment of MS patients with facial numbness is a sensitive tool, which complements MRI, and can improve lesion localization.

Objectives Mirror movements (MMs) are unintended and unnecessary movements accompanying voluntary activity in homologous muscles on the opposite side of the body, particularly in distal arm muscles. Congenital MMs may be sporadic or familial. Several mechanisms have been proposed to explain persistent congenital MMs. Hypothesis 1 assumes the existence of an ipsilateral corticospinal pathway, and Hypothesis 2 the activation of both motor cortices. We report a new case of congenital mirror movements in a healthy woman. Methods Electromyographic recordings and focal transcranial magnetic stimulation (TMS) were used for neurophysiological evaluation. Results Voluntary contraction of either abductor pollicis brevis (APB) elicited mirror activation of the other APB. Focal TMS of either M1 elicited motor evoked potential (MEP) of normal latency and amplitude in both resting APB. TMS of the left cortex upon maximal contraction of the right APB and mirror contraction of the left APB produced interhemispheric inhibition (IHI) in the former and silent period (SP) in the later. Conclusions The electrophysiological evaluation using transcranial magnetic stimulation provides evidence of the concurrent action of both mechanisms in this patient. Significance The combination of more than one hypothesis could be more appropriate for understanding the underlying mechanism in some MM cases.

Summary Aims of the study To detect amplitude differences between the sensory nerve action potentials (SNAP) obtained by simultaneous recording of the two main branches of the superficial peroneal sensory nerve (SPSN), the medial and intermediate dorsal cutaneous sensory nerves (MDCN, IDCN); to investigate whether these differences, if any, are correlated with gender, age, body mass index (BMI), and height of normal subjects; to discuss their clinical significance. Population and methods Seventy-six healthy volunteers (36 males) were included (mean age: 36.5 years, range 20–80). Simultaneous MCND and IDCN recordings were performed via surface electrodes placed at precise positions on the intermalleolus line. Stimulation was performed 14 cm proximally on two different sites over the anterolateral aspect of the right leg. Results Responses were obtained for both nerve branches in all subjects. Median value and lower normal limit for the amplitude of the greater among both MDCN and IDCN responses was 10.95 μV and 4.9 μV, respectively. Statistically significant differences were found between the two branches in median amplitude and frequency of the greater value. These differences were not correlated with gender, age, BMI, or height. Conclusion We propose simultaneous recording of the two main branches of the superficial peroneal sensory nerve, placing the recording electrodes and stimulation device on precise positions and measuring the amplitude of the best of both responses. This method is an improvement of an already existent one, and may be clinically useful in detecting abnormal responses of the SPSN.

The study assessed HIV-related and anti-retroviral therapy-induced neuropathy in myelinated and unmyelinated nerve fibers. One hundred consecutive HIV patients were examined clinically and standard nerve conduction velocities were measured. In addition, electrically induced sympathetic skin response (SSR) was assessed in the palms and soles. The difference in delay of SSR in palms and soles (DeltaSSR) was calculated as an indirect measure of C-fiber conduction velocity. Thick fiber conduction velocities significantly decreased with age and increasing stage of the disease, whereas no effect of stage was found for DeltaSSR (p=0.6). In contrast, medication of at least one of the most known neurotoxic drugs zalcitabine, stavudine, or didanosine did not result in significantly lower conduction velocities in thick fibers (51.29+/-3.4 m/s vs. 50.86+/-3.5 m/s), but was related to an increased DeltaSSR. DeltaSSR allows an indirect measurement of C-fiber conduction velocity. In HIV this measure of unmyelinated sympathetic fibers was most sensitive to anti-viral treatment whereas conduction velocity of myelinated somatic fibers was more sensitive to disease-related neuropathy. The results suggest that HIV neuropathy preferably affects myelinated and anti-retroviral therapy unmyelinated fibers.

There has been limited information from population studies regarding the overall frequency of the common 1.5-Mb 17p11.2 deletion and even scarcer data regarding the overall frequency of PMP22 micromutations in patients with a clinical suspicion of hereditary neuropathy with liability to pressure palsies (HNPP). We have analysed 100 consecutive Greek patients referred for HNPP genetic testing over a 15-year period to our Neurogenetics Unit in Athens, a reference centre for all regions of Greece. All patients were screened for the 1.5-Mb deletion and a selected subgroup of deletion-negative patients for PMP22 micromutations. Mutation-positive and mutation-negative patients were compared for various clinical parameters. In total, 54 mutation-positive patients were identified. In index cases, the deletion frequency was 47.8%, and the PMP22 micromutation frequency was 2.2%. Within mutation-positive patients, the common deletion represented 95.7% and PMP22 micromutations 4.3% of cases. Two previously reported PMP22 micromutations (c.364_365delCC and c.79-2AG) were detected. HNPP index cases had a 2.8-1 male-to-female ratio, similar to mutation-negative patients. A typical phenotype (recurrent or isolated palsies) was present in 82.4% of symptomatic HNPP cases, significantly higher than mutation-negative patients. Sensitivity of proposed electrophysiological diagnostic criteria for HNPP was calculated at 95.7% and specificity at 80.5%. In conclusion, the common HNPP deletion accounts for ∼50% and PMP22 micromutations for ∼2% of cases in a large consecutive cohort of patients with suspected HNPP. The mutational and phenotypic spectrum of HNPP is similar in the Greek population compared with other populations. Proposed electrophysiological diagnostic criteria perform satisfactorily in everyday clinical practice.

Background and Purpose— A simple score derived in the Oxfordshire Community Stroke Project (ABCD score) was able to identify individuals at high early risk of stroke after a transient ischemic attack (TIA) both in a population-based and a hospital-referred clinic cohort. We aimed to further validate the former score in a cohort of hospitalized TIA patients. Methods— We retrospectively reviewed the emergency room and hospital records of consecutive patients hospitalized in our neurological department with a definite TIA according to the World Health Organization (WHO) criteria during a 5-year period. The 6-point ABCD score (age [140 mm Hg and/or diastolic >90 mm Hg=1]; clinical features [unilateral weakness=2, speech disturbance without weakness=1, other symptom=0]; duration of symptoms [ Results— The 30-day risk of stroke in the present case series (n=226) was 9.7% (95% CI, 5.8% to 13.6%). The ABCD score was highly predictive of 30-day risk of stroke (ABCD=0 to 2: 0%, ABCD=3: 3.5% [95% CI, 0% to 8.2%], ABCD=4: 7.6% [95% CI, 1.2% to 14.0%], ABCD=5: 21.3% [95% CI, 10.4% to 33.0%], ABCD=6: 31.3% [95% CI, 8.6% to 54.0%]; log-rank test=23.09; df=6; P =0.0008; P for linear trend across the ABCD score levels P Conclusions— Our findings validate the predictive value of the ABCD score in identifying hospitalized TIA patients with a high risk of early stroke and provide further evidence for its potential applicability in clinical practice.

Objective To explore the presence and grade of denervation in three mimic muscles in facial nerve palsy. Methods The frontalis, orbicularis oculi (OO), and mentalis muscles were explored in 54 patients with Bell's palsy, after 30 days from the beginning of symptoms. Concentric needle electromyography, including fibrillation detection, was performed in the three muscles on the affected side. Compound muscle action potentials (CMAP) were recorded on both sides and a CMAP ratio was calculated from each muscle. The House–Brackman scale was used to assess the initial clinical evaluation and the follow-up until recovery. Results Fibrillation was recorded in 32 patients on the 30th day after the onset and was present in 100% of mentalis and in 90% of frontalis, but in only 34% of OO, a statistically significant difference. The grade of fibrillation in OO was also statistically lower from those in the other two muscles. The 32 patients were grouped according to the presence (A) or not (B) of fibrillation in OO. In group A, CMAP ratios (affected/normal side) did not differ significantly among the three muscles. However, in group B they were significantly greater in OO and frontalis than in mentalis. In between-group comparison, group B muscles had significantly higher CMAP ratios, lower degree of fibrillation, and better functional recovery. Conclusion In facial palsy, the presence and grade of fibrillation in OO are lower than in frontalis and mentalis muscles and prognosis is more favorable for the non-fibrillating OO group of patients.

In elderly patients, peripheral neuropathies are common and may lead to disability. In order to investigate the relative incidence of different polyneuropathies in the elderly focusing on the contribution of nerve biopsy to their diagnosis, the authors studied 74 patients over 65 years of age with clinical, laboratory, electrophysiological, and sural nerve biopsy findings of different types of polyneuropathy. Vasculitic polyneuropathy seemed to be the most common cause of disabling neuropathy in the elderly, followed by paraneoplasia and diabetes. The possible diagnosis of idiopathic axonal neuropathy in the nine cases with neuropathy of unknown origin is discussed.

The aim of this study was to compare Bangladeshi immigrants with diabetes to native Greeks with diabetes and to distinguish the different risk factors for polyneuropathy (PN) in the two ethnic groups. Subjects were recruited from the outpatient diabetic clinic of a general hospital. A total of 111 Bangladeshi immigrants (97 men and 14 women of mean age 47 years) and 101 native Greeks (82 men and 19 women of mean age 49 years) were included in the study. Sex, mean age, age at diabetes diagnosis, and diabetes duration did not differ between the two groups. PN was diagnosed in 53 (48%) Bangladeshi and in 59 (58%) Greek patients (p = 0.12). Large fiber neuropathy was less prevalent among Bangladeshis (18%) than in Greeks (53%) (p 0.01). Small fiber neuropathy on the contrary were more frequent in Bangladeshis (18% vs. 7%) (p 0.02). Regarding the risk factors for PN, Greek patients were taller, with higher BMI, and smoked more cigarettes (p 0.001). They were also treated with more anti-lipid and antihypertensive agents. The higher percentage of SFN in Bangladeshi was mainly a result of the significantly greater incidence of erectile dysfunction (ED) in their group (68 Bangladeshi vs. 38 Greek men). It is well known that there are many causes of ED aside from SFN which were not evaluated in this study. Thus this conclusion should be taken with caution.

Aims: The investigation of the association between known risk factors and laterality in patients with carpal tunnel syndrome (CTS). Patients and Methods: 130 consecutive subjects with CTS only, or mainly, in the left hand were compared with 130 consecutive subjects with CTS only, or mainly, in the right hand. The following parameters were recorded: age, sex, job, handedness, hand mainly used in daily activities, BMI, diabetes mellitus, thyroid dysfunction, wrist trauma and connective tissue diseases. Results: A left dominant hand was independently associated with 13-fold higher odds for left-hand CTS, while a right dominant hand had 5-fold higher odds for right-hand CTS. Right-hand CTS was more frequent in younger subjects and females. Conclusion: Older age, higher BMI and diabetes mellitus were more prevalent in patients with bilateral CTS. Age and BMI were independently associated with bilateral CTS.

Objective Age and height influence on sural sensory nerve action potential (SNAP) have been studied separately. Our aim was to develop an equation for predicting the lower normal limits as a function of both these factors. Methods One hundred fifty-eight healthy volunteers, 63 male, with mean age 45.8 and mean height 167.3 without symptoms or signs of peripheral neuropathy participated in the study. The sural SNAP was recorded at the level of the ankle joint, just posterior to the lateral malleolus, using surface electrodes. Antidromic supramaximal stimulation was performed 13 cm proximally at the posterior midcalf. Results The mean sural SNAP amplitude was 19.9 ± 6.89 μV. Pearson linear correlation showed a negative correlation of the SNAP amplitude with age ( R = −0.22, p = 0.005) and height ( R = −0.19, p = 0.03). The multiple linear regression model was applied for both parameters of age and height with SNAP amplitude as the dependent parameter, producing the following equation: SNAP amplitude = 62.45 − 0.1447 × Age − 0.2147 × Height. Conclusions Using our normal data, the computed lower limits of the 95% prediction interval for the sural SNAP amplitude of an individual subject, depending on his age and height, were calculated. Significance The individualized normal values provided by our equation are essential for the correct interpretation of sural nerve studies.

Limb hypoplasia is a rare congenital disorder. Is usually encountered in patients with segmental spinal dysplasia (SSD), in progressive facial hemiatrophy (Parry-Romberg syndrome) and in other rare conditions. We performed an extensive electrophysiological study in a 18-year-old female with congenital left lower limb hypoplasia, but with no motor and sensory deficit. Electrophysiological investigation comprised motor and sensory nerve conduction velocities, needle EMG, quantitative sensory studies and SEP with standard techniques. The study showed markedly involved large diameter peripheral sensory nerve fibers and intact motor and small diameter peripheral sensory nerve fibers. Extensive electrophysiological investigation in cases of limb hypoplasia has not been previously performed. In this patient congentital hypoplasia of the muscles also involved the peripheral large diameter sensory nerve fibers.

Monoclonal IgM-related neuropathies constitute a heterogeneous group of disorders, which are generally poorly responsive to treatment. Rituximab, a chimeric monoclonal antibody against the CD20 molecule, has been used with success in patients with neuropathy and monoclonal IgM with anti-MAG or anti-GM1 ganglioside activity. Based on this observation, four patients were treated with IgM-related neuropathy with rituximab. Between January 1999 - December 2000, four patients with IgM-related neuropathy (one with chronic inflammatory demyelinating polyneuropathy (CIDP) and three with sensorimotor demyelinating neuropathy) were treated with rituximab. Rituximab was administered at a standard dose of 375 mg m(-2) iv weekly for a consecutive 4 weeks; 3 months later, four additional weekly courses were administered to patients who did not experience deterioration of their neuropathy symptoms. Neurological evaluation was performed before each rituximab infusion and at 1 week and 2 months after last infusion and every 6 months the following years; including motor (MRC in six muscle groups, 9-hole peg test, 10 m walk, hand grip strength), sensory neuropathy (vibration threshold and sensory subjective score) assessment. Neurophysiological parameters were also assessed (MNCV, SNCV, CMAP, SNAP). Strength improved in three of four patients; including the patient with CIDP. This patient developed a significant worsening of her weakness 3 weeks after the initiation of rituximab. This phenomenon coincided with a serum monoclonal IgM flare and resolved spontaneously 1 week later. Her improvement is ongoing for more than 5 years. Considering neurophysiological parameters, two patients showed a slight improved regarding conduction velocities and CMAP (10%) and the patient with IgM flare had a transient worsening of conduction velocities followed by improvement. In conclusion, rituximab is a safe and well-tolerated treatment which may be effective in some patients with IgM-related neuropathy.

Rickettsia conorii is endemic in the Mediterranean region. Infections are mostly benign and neurological involvement is unusual. We describe a case of a man who presented with acute facial nerve palsy followed by flaccid tetraparesis due to an electrophysiologically established polyneuritis with distal conduction failure. Elevated IgM antibody titres for R. conorii were documented by indirect immunofluorescent antibody test. After doxycycline therapy, the patient presented a rapid clinical improvement. Repeated electrophysiological examinations revealed significantly restored compound muscles, and sensory action potentials, corresponding to the clinical course after treatment and ex juvantibus, indicate the causative relation between R. conorii infection and the described clinical syndrome.

Background Peripheral nerve injury and brachial plexopathy are known, though rare complications of coronary artery surgery. The ulnar nerve is most frequently affected, whereas radial nerve lesions are much less common accounting for only 3% of such intraoperative injuries. Case presentations Two 52- and 50-year-old men underwent coronary artery surgery. On the first postoperative day they both complained of wrist drop on the left. Neurological examination revealed a paresis of the wrist and finger extensor muscles (0/5), and the brachioradialis (4/5) with hypoaesthesia on the radial aspect of the dorsum of the left hand. Both biceps and triceps reflexes were normoactive, whereas the brachioradialis reflex was diminished on the left. Muscles innervated from the median and ulnar nerve, as well as all muscles above the elbow were unaffected. Electrophysiological studies were performed 3 weeks later, when muscle power of the affected muscles had already begun to improve. Nerve conduction studies and needle electromyography revealed a partial conduction block of the radial nerve along the spiral groove, motor axonal loss distal to the site of the lesion and moderate impairment in recruitment with fibrillation potentials in radial innervated muscles below the elbow and normal findings in triceps and deltoid. Electrophysiology data pointed towards a radial nerve injury in the spiral groove. We assume external compression as the causative factor. The only apparatus attached to the patients' left upper arm was the sternal retractor, used for dissection of the internal mammary artery. Both patients were overweight and lying on the operating table for a considerable time might have caused the compression of their left upper arm on the self retractor's supporting column which was fixed to the table rail 5 cm above the left elbow joint, in the site where the radial nerve is directly apposed to the humerus. Conclusion Although very uncommon, external compression due to the use of a self retractor during coronary artery surgery can affect – especially in obese subjects – the radial nerve within the spiral groove leading to paresis and should therefore be included in the list of possible mechanisms of radial nerve injury.