Search

Background: Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. In this study, we analyzed presurgery hydrodynamic markers to evaluate if their changes could be associated with clinical and radiological improvement after posterior fossa decompression and duraplasty (PFDD). As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. Methods: In total, in this study, we enrolled 36 consecutive adults with Syr and CM1 who were followed by a multidisciplinary team. All the patients were prospectively evaluated with clinical scales and neuroimaging, including CSF flow, the fourth ventricle area, and the Vaquero Index by using a phase-contrast MRI before (T0) and after surgical treatment (T1-Tlast, with a range of 12–108 months). The CSF flow at the craniocervical junction (CCJ), the fourth ventricle area, and the Vaquero Index changes were statistically analyzed and compared to the clinical and quality of life improvement after surgery. The good outcome prediction ability of presurgical radiological variables was tested. Results: Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. The fourth ventricle area significantly reduced after surgery (T0-Tlast, p = 0.0093), but no significant associations with clinical improvement were found. The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50–0.87 and LH+ = 2.1, IC 95% 1.16–3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). Conclusions: Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. Measurements of the fourth ventricle area could be useful additional information for evaluating surgical long-term follow-up; further experience on larger cohorts is required to better define the prognostic yield of this radiological parameter.

Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. Aim of the study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults. A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement

Purpose. Brainstem syndromes have frequently been reported in Chiari syndrome and in syringobulbia; previous studies have shown that determining the central motor conduction time (CMCT) along the circuit of the phrenic nerve makes the assessment of the voluntary control of the respiratory pathway possible. In our study, we evaluated the transcranial magnetic stimulation (TMS) of the phrenic nerve in patients affected by Chiari syndrome and/or syringomyelia (Syr) with the aim of identifying subclinical neurophysiological alterations. Methods. One hundred patients (75 females; average age: 51 ± 13.08 SD; range: 18–76) affected by Chiari syndrome and/or Syr without dyspnea were selected. The magnetic stimulation of the second motor neuron correlating with the phrenic nerve was performed using cervical magnetic stimulation (C5-MEP); the cortical MEP after magnetic stimulation (Cz-MEP) was recorded by magnetic stimulation of the motor cortex (areas corresponding to the diaphragm). The CMCT was calculated. The differences between the patients and controls were calculated (Student’s t test). Results. In 13% of the patients, the Cz-MEP were absent bilaterally, and the CMCT was not evaluable. In all these cases, bulbar/cervical Syr was present at MRI; in 10 of them, this was associated with CM1. A bilateral response was obtained in all the other patients (87%), and the CMCTs were normal. All the patients with alterations/absence of Cz-MEP presented bulbar/cervical Syr at MRI. The C5-MEP latency was prolonged or absent in 48%; of these, 84% presented bulbar/cervical Syr associated with CM1 at MRI. The C5-MEP latency values were significantly higher in the group of patients. Conclusions. Neurophysiological alterations of the diaphragmatic pathway were recorded in a group of Chiari syndrome and, particularly, in bulbar/cervical Syr. Future studies with larger cohorts of patients are needed to further assess the specific role of the TMS of the phrenic nerve in CM/Syr patients.

In anticipation of the “Chiari and Syringomyelia Consensus Conference” held in Milan in 2019, we performed a systematic literature review on the management of Chiari malformation type 1 (CM1) and syringomyelia (Syr) in children. We aimed to summarize the available evidence and identify areas where consensus has not been reached and further research is needed. In accordance with PRISMA guidelines, we formulated seven questions in Patients-Interventions-Comparators-Outcomes (PICO) format. Six PICOs concerned CM1 children with/without additional structural anomalies (Syr, craniosynostosis, hydrocephalus, tethered cord, and cranio-vertebral junction anomalies), and one PICO Syr without CM1. We searched Medline, Embase, Cochrane, and NICE databases from January 1, 1999, to May 29, 2019. Cohort studies, controlled and randomized clinical trials (CCTs, RCTs), and systematic reviews were included, all pertinent only to patients ≤ 18 years of age. For CM1, 3787 records were found, 460 full texts were assessed and 49 studies (46 cohort studies, one RCT, and two systematic reviews) were finally included. For Syr, 376 records were found, 59 full texts were assessed, and five studies (one RCT and four cohort studies) were included. Data on each PICO were synthetized narratively due to heterogeneity in the inclusion criteria, outcome measures, and length of follow-up of the included studies. Despite decades of experience on CM1 and Syr management in children, the available evidence remains limited. Specifically, there is an urgent need for collaborative initiatives focusing on the adoption of shared inclusion criteria and outcome measures, as well as rigorous prospective designs, particularly RCTs.

In Chiari 1 Malformation (CM1) the most frequent symptom is exertional headache, but other headache types have been reported, such as migraine. This cross-sectional study is aimed to examine the prevalence of migraine in a group of CM1 headache patients and to compare clinical-demographic characteristics between migraine and non-migraine patients. 427 adults were enrolled at the multidisciplinary Chiari Center in Torino. 230 headache patients were classified, based on radiological criteria. Frequencies (absolute/percentage values) were calculated in the whole sample for: migraine presence (with and without aura), gender, age, radiologic phenotypes and headache clinical characteristics. The association between CM1 diagnosis and headache characteristics (independent variables) and migraine diagnosis (dependent variable) was estimated by logistic regression models. Seventy-eight patients (67 females) out of 230 presenting headache had a migraine (34%), 44/78 (56%) with aura; in 58/78 (74%) migraine was comorbid with secondary headache attributable to CM1. Migraine prevalence in patients with isolated CM1 (52/120, 43.3%) was higher (p = 0.0016) than in all the other patients (26/110, 23.6%). Although migraine was prevalent in females (86%; 6:1 female:male ratio), age classes and gender were not risk factors for migraine at multivariate analysis, while migraine was associated with isolated CM1 phenotype (OR = 2.6). This study shows a high prevalence of migraine in CM1 patients and a significant association between migraine and isolated CM1. In patients with radiological evidence of CM1, particularly in the absence of neurological signs, a careful headache clinical characterization, according to the International Headache Society criteria, may be advised.

We performed a prospective multicenter case-control study to explore the association between ulnar neuropathy at elbow (UNE) and body and elbow anthropometric measures, demographic and lifestyle factors, and comorbidities. Cases and controls were consecutively enrolled among subjects admitted to four electromyography labs. UNE diagnosis was made on clinical and neurographic findings. The control group included all other subjects without signs/symptoms of ulnar neuropathy and with normal ulnar nerve neurography. Anthropometric measurements included weight, height, waist, hip circumferences, and external measures of elbow using a caliper. The participants filled in a self-administered questionnaire on personal characteristics, lifestyle factors, and medical history. We estimated odds ratios (ORs) and 95% confidence intervals (CIs) by fitting unconditional logistic regression models adjusted by center and education level. We enrolled 220 cases (males 61.8%; mean age 51.7 years) and 460 controls (47.4% males; mean age 47.8 years). At multivariable analysis, UNE was associated to male gender (OR = 2.4, 95%CI = 1.6-3.7), smoking habits (>25 pack-years (OR = 2.3, 95%CI = 1.3-4.1), body mass index (OR = 1.05, 95%CI 1.01-1.10), polyneuropathies (OR = 4.1, 95%CI 1.5-11.5), and leaning with flexed elbow on a table/desk (OR = 1.5, 95%CI 1.0-2.2). Cubital groove width (CGW) turned out to be negatively associated with UNE (OR = 0.80, 95%CI = 0.74-0.85). Our study suggests that some personal factors especially anthropometric measures of the elbow may play a role in UNE pathogenesis as the measures of wrist in CTS. We demonstrated that for each millimeter of smaller CGW the risk of idiopathic UNE increases of 25%.

To improve patient care and help clinical research, the Neuropathic Pain Special Interest Group of the Italian Neurological Society appointed a task force to elaborate a consensus statement on pharmacoresistant neuropathic pain. The task force included 19 experts in neuropathic pain. These experts participated in a Delphi survey consisting of three consecutive rounds of questions and a face-to-face meeting, designed to achieve a consensus definition of pharmacoresistant neuropathic pain. In the three rounds of questions, the participants identified and described the main distinguishing features of pharmacoresistance. In the face-to-face meeting the participants discussed the clinical features determining pharmacoresistance. They finally agreed that neuropathic pain is pharmacoresistant when "the patient does not reach the 50% reduction of pain or an improvement of at least 2 points in the Patient Global Impression of Change, having used all drug classes indicated as first, second, or third line in the most recent and widely agreed international guidelines, for at least 1 month after titration to the highest tolerable dose." Our consensus statement might be useful for identifying eligible patients for invasive treatments, and selecting patients in pharmacological trials, thus improving patient care and helping clinical research.

Chiari malformation is a group of congenital malformations involving the brainstem, cerebellum, and upper spinal cord, frequently identified in both young adults and in children. Chiari I malformation (CM1), classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord, is the most common clinical type. A syringomyelia can be associated at the time of the diagnosis or appear secondarily and manifest with medullary symptoms. The aim of this paper is to update the knowledge on clinical manifestations specifically related to Chiari I malformation with or without syringomyelia in the pediatric population. Current literature with focus on relevant clinical pediatric issues is reviewed and discussed, comparing with those related to adults; we include the results of a 10-year single-center experience on 600 CM1 patients. Herniation of the cerebellar tonsils may lead to significant clinical symptoms, including neck and cervical pain, short-lasting occipital “cough” headache, dizziness, and gait impairment; in children younger than 3 years, oropharyngeal symptoms are prevalent (sleep apnea, feeding problems) whereas in those older than 3 years, a higher incidence of cough headache and scoliosis is reported. CM1 clinical features, both in children and in adults, have in common the presence of anatomical deformities of the brainstem and cerebellum. Clinical myelopathy (sensory/autonomic disorders, motor weakness) can result from direct compression of the cervical spinal cord by the herniated cerebellar tonsils or can be due to the presence of a syrinx, reported in association with Chiari I between 35 and 75% of pediatric patients. Similarly, in our series (440 females, 160 males, 98% > 18 years), syringomyelia associated with Chiari I was ranging from 40 to 60% (respectively in asymptomatic and symptomatic groups); headache was reported in 65%. Sensory disturbances (48%), cranial nerve deficits (45%), motor weakness (32%), and autonomic disorders (35%) were the most frequent neurological signs in our cohort. In Chiari I malformation, cervical pain and occipital cough headache are the most characteristic presenting symptoms, both in old children and in adults; however, headache is often multifactorial, and CM1 patients can report a wide variety of non-specific symptoms and signs. Clinical diagnostic CM1 criteria, shared at the national and international level, are recommended with the aim to avoid consequent controversies on diagnosis and on surgical decision making.

Objective Prevalence and clinical characteristics of neuropathic pain due to traumatic brachial plexus injury. Design Observational epidemiological study. Setting Hospital-based multicenter study. Subjects One hundred seven prospectively enrolled patients with brachial plexus injury. Methods All the patients underwent clinical examination and neurophysiological testing for a definitive diagnosis of the brachial plexus lesion. The DN4 questionnaire was used to identify neuropathic pain, and the Neuropathic Pain Symptom Inventory (NPSI) to evaluate the different symptoms of neuropathic pain. The SF36 questionnaire and the Beck Depression Inventory (BDI) were used to assess quality of life and mood disturbances in patients with neuropathic pain. Results Of the 107 enrolled patients, 74 had pain (69%); neuropathic pain, as assessed by means of the DN4, was identified in 60 (56%) of these patients. According to the NPSI, the most frequent and severe pain type was the spontaneous burning pain. Clinical and neurophysiological findings showed that pain is unrelated to age but is associated with the severity of peripheral nerve damage. The SF36 questionnaire and BDI showed that neuropathic pain impairs quality of life and causes depression. Conclusions Our study provides information on the prevalence, characteristics, and variables associated with neuropathic pain due to traumatic brachial plexus injuries that might provide a basis for improving the clinical management of this condition.

Introduction: Pain complicates hypertrophic post-burn pathologic scars (PPS) Methods: To investigate the possible neuropathic origin of pain, 13 patients with painful PPS involving at least 1 hand underwent clinical examination, including the Douleur Neuropathique en 4 questions (DN4) questionnaire; median, ulnar, and radial nerve conduction studies (NCS); cold- (CDT) and heat-induced pain threshold evaluation by quantitative sensory testing; and cutaneous silent period (CSP) testing of the abductor pollicis brevis. Controls included 9 patients with non-painful PPS, 52 healthy subjects, and 28 patients with carpal tunnel syndrome (CTS). Results: All patients with painful PPS had possible neuropathic pain (DN4 score ≥4). NCS signs of CTS were similarly present in PPS subjects with or without pain. Hands with painful PPS had lower CDT and CSP duration, more frequent cold- and heat-pain hypesthesia, and more thermal allodynia than controls. Conclusions: In PPS, possible neuropathic pain is associated with psychophysical and neurophysiological abnormalities suggestive of small-fiber damage. Muscle Nerve 45: 883–890, 2012

Cocito D, Maule S, Paolasso I, Castelli L, Ciaramitaro P, Poglio F, Ottobrelli A, Grimaldi S. High prevalence of neuropathies in patients with end-stage liver disease. Acta Neurol Scand: 2010: 122: 36–40. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objectives – Peripheral neuropathy has been reported in association with end-stage liver disease, but there is only a limited number of reports on the incidence and features of these neuropathies. Materials and methods – In this study, 83 patients awaiting liver transplantation were evaluated for the presence of peripheral and autonomic neuropathy. Results – Sixty-five percent of the patients had evidence of neuropathy, in agreement with peripheral NCS or cardiovascular autonomic function test. The neuropathy was more frequent in patients with advanced hepatic failure, evaluated with the MELD score. The most frequent abnormalities in nerve conduction studies were sensory-motor neuropathies and sensory neuropathies, with a length-dependent pattern. Conclusion – Peripheral neuropathy and autonomic neuropathy are common in patients with end-stage liver disease with different etiology and correlate with the severity of the liver disease.

The aim of this study was to verify the extent to which the presence of pain affects the quality of life (QoL) of neuropathic patients. The patients were selected in our Department of Peripheral Nervous System Diseases. We enrolled 120 consecutive patients with chronic polyneuropathy who had not received continuous pain therapy during the two months preceding study entry, and administered them the Total Neuropathy Score (TNS), the official Italian version of the SF-36 and the Italian Pain Questionnaire (QUID). Our main finding was that the QoL is affected not only by the presence of neuropathy, but also by the presence and intensity of pain: the physical aspect of the QoL correlated only weakly with the TNS, but pain was closely related to a worsening in this parameter; moreover, the mental domains of the SF-36 were only correlated with pain. Pain per se worsens the QoL of neuropathic patients, regardless of disease severity.

Alterations of the phrenic nerve (PN) and pulmonary function tests (PFTs) have been described in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). This study was aimed at assessing the relationship between PN and respiratory function in CIDP patients without clinical signs of respiratory failure. Bilateral PN and right median nerve conduction studies were carried out along with blood gas analysis and PFTs: maximal inspiratory pressure; maximal expiratory pressure; forced vital capacity. The amplitude of the compound muscle action potential of the PN was seen to be altered in 19/24 (79%) patients and latency in 22 (92%). Eighteen patients (75%) showed at least one abnormal PFTs or CO2 partial pressure value. Electrophysiological alterations of the PN were observed in a high percentage of the CIDP patients studied. No statistically significant correlation was observed between PN and PFTs alterations.

We evaluated the benefits of teleconsulting for patients hospitalised with minor head injuries in centres without neurosurgery. In the Piedmont region, 1462 consultation requests were received at specialist centres in 2009, relating to 519 patients with a minor head injury diagnosis (ICD 850–854). These were compared with the details of 1895 patients admitted with the same diagnosis during 2009, but for whom no consultations were requested. The mortality risk in the two groups was estimated using logistic regression, after adjusting for the principal confounding factors (sex, age, seriousness of the patient's injury at diagnosis, referral centre). The estimated risk of death for patients for whom no consultation was requested was an odds ratio of 1.32 (95% CI 1.08 to 1.74) compared to those who received a teleconsultation. However, after adjusting for the confounding factors, the risk was not significant (odds ratio = 1.25, 95% CI 0.83 to 1.91). A stratified analysis identified a significant effect for elderly people, aged over 70 years, in whom the odds ratio was 1.14 (95% CI 1.04 to 1.82). The results confirm the benefits of telemedicine, in particular for elderly patients, when teleconsultation is requested in the case of minor head injury.

The distinction between chronic demyelinating polyneuropathies associated with IgM paraproteinemia and anti-myelin-associated glycoprotein (MAG) antibodies (MAG-PN) and chronic inflammatory demyelinating polyneuropathies (CIDPs) relies on the anti-MAG antibodies assay. The aim of the study was to identify clinical and electrophysiological features suggesting a diagnosis of MAG-PN. Fourteen patients with MAG-PN and 35 with CIDP were included, and a discriminant analysis was performed to identify the clinical and electrophysiological features suggestive of MAG-PN. Pure sensory clinical phenotype, low median and ulnar terminal latency index, and absence of M responses in the lower limbs were significantly associated with the diagnosis of MAG-PN, and indicate a moderate to large increase in probability of this diagnosis in patients with chronic dysimmune demyelinating polyneuropathies.

Objective – Thalidomide is a promising therapy for multiple myeloma. Sensory neuropathy is a side effect of thalidomide and resulted to be partially reversible in 50% of cases, suggesting a sensory ganglionopathy. Spinal cord magnetic resonance imaging (MRI) was found to be useful in the diagnosis of sensory ganglionopathies and we use it to determine if thalidomide neuropathy has features of a ganglionopathy. Material and methods – Six patients with multiple myeloma developed thalidomide-induced polyneuropathy. Nerve conduction studies, somatosensory-evoked potentials (SEPs) and cervical and dorsal spinal cord MRI were obtained in all. Results – All patients had a sensory neuropathy, with clinical or electrophysiological abnormalities involving all four limbs. Spinal cord MRI showed high signal intensity in the posterior columns in only one patient, with abnormal central conduction time at SEPs. Conclusion – Our results suggest that thalidomide can induce either an axonal length-dependent neuropathy or, less frequently, a ganglionopathy.

OBJECTIVE: Interferon is regarded as a promising treatment option for patients with chronic inflammatory demyelinating polyneuropathy. However, sporadic cases of axonal PNP and one case of CIDP after treatment with Interferon (INF) alfa-2a were reported. The objective of this study was to test the onset of PNP in patients affected by relapsing-remitting MS after treatment with INF beta-1b. METHODS: from the OPTIMS trial we have selected 23 patients (9 males, 14 females; mean age 30.5; range 22–50) with diagnosis of relapsing-remitting SM in treatment with INF beta-1b (8–12.000.000 UI). In these patients bilateral motor nerve conduction study (NCS) of peroneal, tibial, median, ulnar nerves and antidromic sensory NCS of sural, median and ulnar nerves were performed according standard techniques. All patients (23/23) were submitted to neurophysiological tests before the treatment (T0), 16/23 (7 drop out) after six months (T1) and after nine months of therapy (T2). RESULTS: at T0 no patient showed electrophysiological signs indicating PNP; after six and nine months of therapy (T1 and T2) no patient submitted to NCS showed alterations in the PNS either. CONCLUSIONS: all tested patients showed absence of electrophysiological signs of PNP as side effects after one year of treatment with high dose of INF beta-1b.

BACKGROUND: Chronic demyelinating polyneuropathy associated with both IgM paraproteinemia and anti-MAG antibody (MAG-PN) is regarded as a clinical entity distinct from chronic inflammatory demyelinating PN (CIDP) even in association with paraproteinemia. MAG-PN satisfy current electrophysiological criteria for CIDP and 18 to 37 % of patients fulfilling these criteria have high titers of IgM anti-MAG antibody (Ab). Anti-MAG Ab assay is rather expensive and time consuming, thus resulting sometimes in delaying the appropriate treatment in patients without these Ab. On the contrary, patients with MAG-PN may be treated inappropriately before anti-MAG Ab detection. OBJECTIVE: Determining the predictive value of clinical and electrophysiological features of patients with chronic dysimmune demyelinating PN for the presence of anti-MAG Ab. MATERIALS AND METHODS: 49 patients fulfilling the diagnostic criteria for CIDP established by Rotta et al. (2001) were prospectively included. All of them were submitted to anti-MAG Ab assay and research of paraproteinemia. The following features at the initial evaluation were considered for the analysis: presence of distal or proximal weakness at limbs, asymmetry of motor deficit, presence of sensory ataxia, presence of vibration or pinprick sense deficit, presence of conduction blocks, low terminal latency index (TLI) in at least two nerves at upper limbs, absence of M-response (CMAP) at lower limbs. A discriminative analysis method was used to determine which features were significantly associated with the presence of anti-MAG Ab, then sensitivity, specificity and likelihood ratio of these features were calculated. RESULTS: Low TLI, absence of CMAP at lower limbs, absence of proximal and distal weakness at lower limbs, were the features significantly associated with the presence of anti-;MAG Ab. The combination of low TLI in two nerves and absence of CMAP at lower limbs or low TLI and absence of proximal and distal weakness at lower limbs had the greatest sensitivity, specificity and likelihood ratio, resulting in a very high increase in probability that a patient had MAG-PN. DISCUSSION: Low TLI value in at least two upper limb nerves, absence of CMAP and of proximal weakness at lower limbs are the most informative features indicating the presence of anti-MAG Ab in patients with chronic dysimmune demyelinating PN.

Introduction. This study evaluates the efficacy of palmitoylethanolamide ultramicronized (PEA-um) as an add-on treatment in patients with diabetic or traumatic neuropathic pain (NP). Methods. 30 patients with chronic NP were assessed with Visual Analogue Scale (VAS), NP Symptom Inventory (NPSI), and Health Questionnaire Five Dimensions (EQ-5D), both at baseline and after 10 and 40 days of treatment with 1200 mg/die of PEA-um. All other therapies were maintained stable during the follow-up period. Results. VAS mean score significantly improved within the first 10 days, ranging from 8.20 ± 1.53 to 6.40 ± 1.83 (P<0.002), with a further decrease to 5.80 ± 2.04 (P<0.001) after 40 days of PEA-um administration. Moreover, NPSI total score improved from 5.2 ± 1.5 to 3.8 ± 2.1 (P: 0.025) and EQ-5D ranged from −0.30 ± 0.65 to 0.5 ± 0.34 (P<0.001) between T0 and T2. Conclusions. This study reports the prospective short-term efficacy data of oral PEA-um in patients with diabetic or traumatic NP. A significant improvement was observed both in VAS and NPSI scores and in quality of life scales after 40 days of treatment, although some limitations should be considered, including the short followup and the open-label study design.

Cortico-diaphragmatic pathway was investigated with transcranial magnetic stimulation (TMS) in amyotrophic lateral sclerosis without clinical signs of respiratory impairment revealing subclinical diaphragmatic impairment. Diffusion Tensor Imaging with fiber tracking (DTI-FT) through fractional anisotropy (FA) measurement was tested for the assessment of spinal sensory tract lesions in cervical syringomyelia (Syr). The aims of this work was to investigate: (1) central motor conduction time (CMCT) in Syr and Chiari Malformation (CM) to point out subclinical alterations of phrenic pathway; (2) relationship between CMCT and structural damage in Syr/CM through FA. 100 patients (75F, 18–76 years, 51 ± 13.08 years) were selected. Latency/amplitude of cortical/spinal motor-evoked potentials (Cx/Sp-MEP) and CMCT along phrenic nerve were measured and compared with healthy subjects. FA measurement was carried out in 13 patients and compared with neurophysiological data. CMCT was significantly altered ( p p The reduced values of Sp-MEP may be related to the fibers loss along the cortical-spinal way and to primitive axonal damage of spinal motoneurons linked to diaphragmatic muscle. CMCT and FA alterations might have a prognostic relevance in the clinical monitoring of these patients.

The purpose of this study was to evaluate, in newly diagnosed type 2 diabetes mellitus (DM): (1) the prevalence and staging of peripheral neuropathy, as well as its possible relationship with metabolic profile; (2) the clinical value of both the Diabetic Neuropathy Index (DNI) and the Diabetic Neuropathy Score (DNS), and their reciprocal concordance, as a screening method for neuropathy. Thirty-nine newly diagnosed DM subjects underwent: neurological examination, nerve conduction studies (NCS), quantitative sensory system and cardiovascular autonomic function assessments. Peripheral neuropathy was observed in 72% of the subjects (its staging was similar to that of patients with longer disease history), while another 10% of them showed a borderline neuropathy. The Deep Breathing test was abnormal in 28% of the patients; postural hypotension was found in 6%. The same proportion (82%) of subjects who scored positively on the DNI showed altered NCS, while the quantitative sensory system assessments had a low sensitivity in order to detect the neuropathy. No correlation was found between metabolic indexes and DNI/DNS parameters. The high prevalence of peripheral and autonomic function alterations suggests that each newly diagnosed diabetic subject should be screened for neuropathy by the DNI, to reduce the negative prognostic influence of this complication.

The aim of this study was to investigate phrenic central motor conduction time (CMCT) in patients affected by Syringomyelia (Syr) and Chiari Malformation (CM) to point out subclinical phrenic pathway alterations. Forty-two patients (12 M, 30 F, age 18–71 years, mean 49.43 ± 13.56) without clinical signs of respiratory impairment were included and divided into subgroups according to different clinical phenotypes (cervical Syr, cervical-dorsal Syr, Syr with CM). TMS was bilaterally performed, recording by surface electrodes from hemidiaphragm; latency/amplitude of cortical and spinal motor-evoked potentials (Cx-MEP/Sp-MEP) and CMCT were measured in all patients. No adverse local or systemic reactions were reported. Neurophysiological data were analyzed and compared with 16 healthy subjects (T-test). CMCT was significantly prolonged ( p p

Pain is a subjective condition that cannot be objectively measured; for this reason, self patient-perspective is crucial. Recently, several screening tools to discriminate between nociceptive and neuropathic pain have been developed. We aimed at assessing the consistence and discrepancy of two widely used screening tools, The Douleur Neuropathique 4 (DN4) and the 6-item questionnaire (ID-Pain), by comparing their ability in discriminating neuropathic from nociceptive pain. DN4 and ID-Pain were administered to 392 Italian patients attending 16 outpatient services for peripheral nerve diseases. Based on medical history, clinical findings and diagnostic tools, patients were divided into two groups (neuropathic and nociceptive). Globally, ID-Pain identified neuropathic pain in 60 % of patients (38 % probable, 22 % likely). Interestingly also DN4 diagnosed neuropathic pain in 60 % of cases. A discrepancy was observed in 16 % of cases. DN4 and ID-Pain resulted to be highly interrelated in the identification of neuropathic pain. Sensitivity of DN4 was 82 % and specificity was 81 %, while ID-Pain (considering both probable and likely groups) showed sensitivity 78 % and specificity 74 %. Reliable screening tools for neuropathic pain are well related between them; hence, they are available for researchers and clinicians who may choose the most appropriate for their activity. Since the gold standard for the diagnosis and treatment of neuropathic pain cannot do without a neurological evaluation, perhaps DN4, that includes physician objective measures, may help reducing the percentage of dubious cases. Conversely, when needing a more agile tool (not needing a physician) ID-Pain may be adopted.

The objectives of this study were (1) epidemiological analysis of traumatic peripheral nerve injuries; (2) assessment of neuropathic pain and quality of life in patients affected by traumatic neuropathies. All consecutive patients with a diagnosis of traumatic neuropathies from four Italian centres were enrolled. Electromyography confirmed clinical level and site diagnosis of peripheral nerve injury. All patients were evaluated by disability scales, pain screening tools, and quality of life tests. 158 consecutive patients for a total of 211 traumatic neuropathies were analysed. The brachial plexus was a frequent site of traumatic injury (36%) and the radial, ulnar, and peroneal were the most commonly involved nerves with 15% of iatrogenic injuries. Seventy-two percent of the traumatic neuropathies were painful. Pain was present in 66% and neuropathic pain in 50% of all patients. Patients had worse quality of life scores than did the healthy Italian population. Moreover, there was a strong correlation between the quality of life and the severity of the pain, particularly neuropathic pain (Short Form-36 [SF-36] p < 0.005; Beck Depression Inventory [BDI] p < 0.0001). Traumatic neuropathies were more frequent in young males after road accidents, mainly in the upper limbs. Severe neuropathic pain and not only disability contributed to worsening the quality of life in patients with traumatic neuropathies.

The aim of this study was to validate the Italian version of the Neuropathic Pain Symptom Inventory (NPSI) in patients with neuropathic pain due to peripheral nerve diseases, and also to evaluate the validity of a new NPSI score: a frequency weighted NPSI score (NPSI-FW). First, the original version of the NPSI was translated into Italian. Then the validity and reliability of the Italian NPSI (I-NPSI) were tested in 392 Italian patients consecutively referred to 16 Italian outpatient services for peripheral nerve diseases, by correlating the I-NPSI scores with other pain scales. The repeatability and responsiveness were assessed. A significant correlation between the I-NPSI scores and all the other pain measures was seen. Reproducibility and responsiveness were good. Our study shows the validity of the I-NPSI and demonstrates its reliability for assessing neuropathic pain in patients with peripheral nerve diseases. The I-NPSI scores represent reliable measurements to assess neuropathic symptoms and effectiveness of treatment on them.

Prospective, observational study. Regional Trauma Center, Torino, Italy. Complex spinal surgery carries a significant risk of neurological damage. The aim of this study is to determine the reliability and applicability of multimodality motor-evoked potentials (MEPs) and somatosensory-evoked potentials (SEPs) monitoring during spine and spinal cord surgery in our institute. Recordings of MEPs to multipulse transcranial electrical stimulation (TES) and cortical SEPs were made on 52 patients during spine and spinal cord surgery under propofol/fentanyl anaesthesia, without neuromuscular blockade. Combined MEPs and SEPs monitoring was successful in 38/52 patients (73.1%), whereas only MEPs from at least one of the target muscles were obtained in 12 patients (23.1%); both MEPs and SEPs were absent in two (3.8%). Significant intraoperative-evoked potential changes occurred in one or both modalities in five (10%) patients. Transitory changes were noted in two patients, whereas three had persistent changes, associated with new deficits or a worsening of the pre-existing neurological disabilities. When no postoperative changes in MEP or MEP/SEP modalities occurred, it was predictive of the absence of new motor deficits in all cases. Intraoperative combined SEP and MEP monitoring is a safe, reliable and sensitive method to detect and reduce intraoperative injury to the spinal cord. Therefore, the authors suggest that a combination of SEP/MEP techniques could be used routinely during complex spine and/or spinal cord surgery.

This study was aimed at assessing the electrophysiological signs of peripheral neuropathy in diabetes mellitus (DM) type II patients at diagnosis. Nerve conduction studies (NCS) of median, ulnar, peroneal, tibial and sural nerves were performed in 39 newly diagnosed DM subjects and compared to those of 40 healthy controls. Metabolic indices were also investigated. Electrophysiological alterations were found in 32 (82%) of the DM patients, and more than half of them (62.2%) showed multiple (two to five) abnormal parameters. Because most of the subjects (84.4%) had from two to five nerves involved, these alterations were widespread in the seven nerves evaluated. Forty-two percent of the patients had NCS alterations suggestive of distal median mononeuropathy, implying that metabolic factors in DM make the median nerve more susceptible to focal entrapment. A reduced sensory nerve action potential (SNAP) amplitude was observed in the median nerve in 70% of the patients, in the ulnar in 69% and in the sural nerve only in 22%. In the presence of a decrease in the SNAP amplitude of the ulnar or median nerve, the SNAP amplitude of the sural nerve was normal in 82 or 80% of the subjects, respectively. This finding may be in keeping with a distal involvement of the sensory fibres, as explored by routine median or ulnar NCS. No correlation was found between metabolic indices and NCS parameters. In conclusion, a high percentage of newly diagnosed DM patients show signs of neuropathy, and upper limb nerve sensory NCS seem to be more sensitive in detecting it than lower limb NCS.

The aim of this study was to evaluate the impact of electrophysiological (EDX) tests in the clinical management and diagnosis of patients, and the appropriateness of the referral diagnosis. A study was carried out in three electrodiagnostic services in the Torino area, over a 12-month period. In our study 3,900 individuals (2,340 females, 1,560 males) were evaluated. Patients underwent EDX examinations including nerve conduction study, electromyography and repetitive stimulation test. Most patients had been sent for EDX tests by specialists. Specialists suspected mainly polyneuropathy, whilst general practitioners suspected mainly carpal tunnel syndrome. Seventy-two percent of the requests were correctly formulated, 55% by general practitioners and 77% by specialists. There was a concordance between the results of the EDX tests and diagnostic hypothesis 40% of the time. This study confirms the usefulness and diagnostic impact of EDX examinations and evidences the amount of time and resources wasted as a result of incorrect or incomplete requests.

The clinical and neurophysiologic data from 65 patients taking thalidomide were reviewed. Thalidomide sensory neurotoxicity was found to be cumulative dose dependent but occurs only when the total dose is relatively high (>20 g). The risk of developing sensory neuropathy is around 10% below this threshold but increases with higher doses.

This study was aimed at defining normative values of latency and amplitude of the compound muscle action potential (CMAP) from the orbicularis oculi muscle, obtained by transcranial magnetic stimulation. We evaluated the dependence of latency and amplitude on the age of the subjects, in order to calculate cut-off values for each age decade. A total of 120 healthy normal subjects, aged 15-78 years, were studied. CMAP from the orbicularis oculi muscle, pars inferior, was excited by means of transcranial magnetic stimulation of the homolateral scalp. A suitable mathematical model was developed to evaluate the mean variation of the latency and amplitude of CMAP for each age decade in the studied population. Mean values were, respectively, 4.62+/-0.40 ms for latency and 4.17+/-0.72 mV for amplitude. We found an increase in latency of about 0.12 ms for every ten years, that became 0.15 ms if referring only to subjects over 20 years, and a decrease in amplitude of 0.18 mV each ten years. Normality interval values for the latency and amplitude were calculated for each age decade. Our data, obtained in a representative population for range of age of evaluated subjects, provide normality values and variability coefficients useful for future confronting works.

Persistent neurologic sequelae may be the consequences of brain aneurysms surgery. Aim of this study is to test the role of intraoperative neurophysiology in the prevention of postoperative deficits. Combined monitoring of combined m-MEPs and SEPs was attempted in 164 consecutive surgeries on 158 patients undergoing surgery for the treatment of 173 aneurisms. In 90 surgeries (54.9%) patients have suffered for SAH; 74 (54.1%) surgeries were performed for unrupted aneurysms. In 12 cases (7.3%), all of them affected by SAH, intraoperative neurophysiology documented a severe damage to motor or sensory/motor pathways: the overall monitorability was 92.7% (152 surgeries). Intraoperative modifications of neurophysiologic parameters were observed in 28 (18.4%) of the remaining 124 cases, transient in 17 (11.2%) and persistent in 11 (7.2%). Combined modifications of SEPs and m-MEPs (a pattern of cortical ischemia) were observed in 10 cases (reversible in 4, persistent in 6), isolated modifications of m-MEPs in 16 cases (11 transient, 5 persistent). In 2 cases a transient reduction in amplitude of SEPs was seen. All but one of cases with persistent modifications were followed by postoperative deficits. Intraoperative neurophysiology may detect an impending injury particularly on motor pathways and provide relevant information about sensory/motor outcome.

Background: Alterations of the phrenic nerve (FN), as well as of the pulmonary function tests (PFTs), have been described in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Objective: Our study was aimed at assessing the relationship between FN conduction study and respiratory function in 24 CIDP patients without clinical signs of respiratory failure. Material and Methods: Bilateral FN and right median nerve conduction studies were accomplished, along with emogasanalysis (EGA) and PFTs: maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), forced vital capacity (FVC) and partial CO2 pressure. Results: The amplitude of the compound muscle action potential (CMAP) of the FN was altered in 19 (79%) of our 24 patients, the latency in 22 (92%). Eighteen patients (75%) showed at least one PFT or the pCO2 abnormal. FN alterations showed a low sensitivity and specificity with respect to PFTs or pCO2. Discussion: Our results demonstrated electrophysiological alterations of the FN in a high percentage of CIDP patients. No significant correlation was observed between FN and PFTs alterations.

Despite long-lasting use, several features of thalidomide neurotoxicity have yet to be resolved. A more detailed knowledge of thalidomide-induced sensory neurotoxicity is desirable, particularly in view of the future use of thalidomide derivatives which are currently under investigation. Our study demonstrates that thalidomide sensory neurotoxicity is cumulative dose-dependent, but this effect is evident only when the dose is relatively high (in our study >20 g). In fact, the risk of developing sensory neuropathy is low (around 10%) below this threshold, while it steadily increases with increasing cumulative doses. In our series, a discrepancy between clinical and neurophysiological results was frequent when a low cumulative dose of thalidomide was administered. Regarding the need for thalidomide withdrawal at the onset of any evidence of peripheral neuropathy, we followed up the course of seven patients who were clinically and neurophysiologically normal at baseline, but had treatment-induced neurotoxicity and a very severe change in their SAPs. In all these cases the thalidomide dose was reduced and no clinically-relevant worsening was observed despite a further marked reduction in their SAPs. This finding suggests that thalidomide withdrawal is not mandatory even in the presence of marked abnormalities in sensory neurophysiological parameters, provided that an accurate clinical follow-up is guaranteed and the dose is adjusted.

Descriptive study will be carried out on patients examined in the neurophysiology laboratory of Neurological Clinic of Turin University and in the neurophysiology laboratory of C.T.O. Hospital of Turin in the period February-April 2004. Our purpose is to evaluate the following issues: 1) physicians requiring electrodiagnostic (EDX) test (general and/or specialist practitioner), 2) diagnostic hypothesis or the symptomatology reported from the patients that have induced the doctor to demand the test and 3) the agreement or disagreement with results of EDX test. Moreover, the outcome of EDX study will be compared with eventual previous EDX tests. Anagraphic data and the profession of the patient, eventual presence of concomitant pathologies or therapeutic use of neurotoxic drugs, possible responsibilities of peripheral nervous system diseases, will be also investigated. Our goal is to evaluate the appropriateness of the EDX test relative to requiring physicians (general or specialist practitioner). We would also improve efficiency of the service, optimizing the economic and human resources, and reducing the number of inappropriate EDX tests.

We carried out an electrophysiological retrospective study in 55 patients with chronic demyelinating acquired and hereditary neuropathies. Alterations of motor nerve conduction velocities (MNCV), distal motor latencies (DML), conduction blocks (CB) and compound muscle action potential (CMAP) were compared, considering the whole number of nerves for each disease. MNCV, DML, CB and CMAP were considered suggestive of demyelination when meeting the American Academy of Neurology (AAN) criteria. Abnormally slow MNCV was found respectively in the 46% of all the CMTX female nerves studied, in the 56.5% of CMTX males, 84% of CMT1A, 74% CIDP and 70% of MAG-PNP. Prolonged DML was observed in the 25% of the CMTX female nerves studied, in the 49.5% of CMTX males, 81% of CMT1A, 63% of CIDP and 71% of MAG-PNP. Moreover, CB were quite often evidenced in CIDP and MAG-PNP nerves (respectively in 48% and 29%) and rarely in hereditary neuropathies. Finally, we observed CMAP reduction in the 45% of all the CMTX female nerves studied, in the 50% of CMTX males, 63% of CMT1A, 49% of CIDP and 60% of MAG-PNP. A well-characterized pattern generally allows an electrophysiological distinction between CMT1A, CMTX males, MAG-PNP on one side and CIDP and CMTX females on the other side. A clear electrodiagnostic distinction result is often hard between CMTX males and MAG-PNP and between CIDP and CMTX females.

Aims: To investigate the features which are most predictive of IVIg response in patients with CIDP. Methods: We included 38 consecutive CIDP diagnosed according to Rotta et al. (2000). Anti-MAG antibodies (Ab) assay and serum immunofixation were performed in all. IVIg (0.4 g/Kg/day) were the first treatment in all patients. Response to IVIg was defined as increase by at least 1 grade of the Rankin scale score at 1 month. The following features were considered: 1) proximal as well as distal weakness, 2) pure clinical sensory pattern, 3) disease duration lower than 12 months, 4) conduction block in at least 1 motor nerve, 5) mean lower/upper limbs motor nerve CMAP amplitude lower than 50% of lower limit of normal, 6)M monoclonal gammopathy, and 7) anti-MAG Ab. Likelihood ratio (LR) of the features significantly associated with treatment response was evaluated in order to determine the increase or decrease of response to IVIg probability. Results: Twenty-six patients were classified as responders and 12 as non-responders. Age (60.6 ± 9.7 vs. 63.6 ± 9.4 years), disease duration (24 vs. 42 months) and sex ratio (18 vs. 9 male) were not different among the groups. Presence of conduction block in at least one motor nerve was associated significantly with treatment response (responders 50% vs. non-responders 8.3%, p: 0.02) and presence of anti-MAG Ab was associated with treatment failure (non-responders 50% vs. responders 7.6%, p: 0.007). The remaining features were not significantly associated with treatment response. LR of presence on conduction block was 6, whereas LR of anti-MAG Ab was 0.15. Considering 70% as mean percentage of responders, the finding of conduction blocks increase the probability of response to IVIg to 89%, whereas the finding of anti-MAG antibody decreases this probability to 25%. Conclusion: Presence of conduction blocks and absence of anti-MAG antibodies seem to be good predictors of response to IVIg in patients with CIDP.

Background: In the past few years thalidomide became one of the most interesting chemotherapeutic drugs in the treatment of multiple myeloma. However a predominantly sensory neuropathy is a well-known side effect of thalidomide in a substantial amount of patients. Peripheral nerve damage resulted to be at lest partially reversible only in 50% of cases suggesting a possible toxic effect on dorsal rot ganglion neurons. Spinal cord MRI was indicated as a useful tool in the diagnosis of sensory ganglionopathy. We described MRI findings in 6 patients with thalidomide neuropathy and multiple myeloma. Patients: Six patients (4 men, 2 women, mean age 61.2 ± 8.6 years) were included: two had non-secretory, 3 osteolytic and 1 osteosclerotic myeloma. Thalidomide was administered orally at a dose of 200 mg/day in 4 patients, and at a dose of 100 mg/day in 2. All patients where asymptomatic for peripheral neuropathy before treatment. Five of them were previously treated with vincristine and 4 with cisplatin, both at low doses. Positive sensory symptoms developed 6 to 19 months after treatment beginning: all patients had paresthesia; 2 had pain in stocking and glove distribution. One patient developed sensory ataxia. On nerve conduction studies, all patients had decreased or absent sensory action potential both at upper and lower limbs. Methods: Cervical spine routine MRI was performed in all patients with a 1 T system, with sagittal T2 FSE spin echo (TR/TE excitations 3909 / 112), axial gradient echo (630/22/30)—weighted images. Results: Cervical spine MRI was normal in 5 patients. In 1 patient, however, MRI disclosed a small T2-weighted high signal intensity in the posterior columns. Discussion: Our results suggest that thalidomide could have a different toxic effect on the peripheral nervous system than on dorsal root ganglion neurons and axons.

OBJECTIVE: The finding of predominantly demyelinating polyneuropathies has been considered unusual in diabetic patients, however recent studies reported polyneuropathies satisfying electrophysiological criteria for diagnosis of CIDP (Gorson et al., 2000; Rotta et al., 2000) can be detected in diabetic patients. It is uncertain if this type of PN shows similar response to immunomodulating treatment as compared to other CIDP. In this study we evaluated response to IV IG in 10 diabetic patients with predominantly demyelinating polyneuropathy. PATIENTS AND METHODS: We included all diabetic patients with CIDP evaluated in our department from July 1999 to November 2000. 13 patients were found to be affected with predominantly demyelinating polyneuropathy; 3 of them refused IV IG. Sensory and motor disturbances according to Van Dijk et al. (1999) and nerve conduction studies (NCS) according to standard techniques were performed before IV IG treatment (T0) and 1 month (T1) and 6 months (T2) after IV IG. All patients underwent CSF examination and anti-MAG, anti-sulphatide and anti-GM1 antibody assay. RESULTS: Increased CSF protein levels were found in 6 patients. No patients were positive for anti-MAG, anti-sulphatide or anti-GM1 antibody assay. We did not detect significant clinical improvement after IV IG treatment, however there was improvement of motor deficit in 2 patients and of sensory score in 3. Six patients, 3 with increased CSF proteins level, had improvement on NCS, however there was not overall significant improvement of NCS abnormalities at T1. Patients with normal CSF protein levels had greater, although not significant, improvement on NCS than patients with increased protein levels. All patients with increased CSF protein levels deteriorated clinically or on NCS before T2. All patients with normal CSF protein levels remained stable on both items at T2. DISCUSSION: Our results confirmed low response of diabetic patients with demyelinating polyneuropathy to immunomodulating treatment, outlined by Gorson et al. (2000). In our study patients with normal CSF protein levels seem to have a better response to IV IG treatment probably reflecting a different pathogenesis as compared to patients with increased CSF protein levels. On the other hand, the high rate of relapse after IV IG treatment in patients with increased CSF protein levels seems to confirm the association of diabetes mellitus and CIDP in some patients.