Multifocal motor neuropathy: correlation of nerve ultrasound,electrophysiological, and clinical findings

We present nerve ultrasound findings in multifocal motor neuropathy (MMN) and examine their correlation with electrophysiology and functional disability. Eighty healthy controls and 12 MMN patients underwent clinical, sonographic, and electrophysiological evaluation a mean of 3.5 years (standard deviation [SD] ± 2.1) after disease onset. Nerve ultrasound revealed significantly higher cross‐sectional area (CSA) values of the median (forearm, p < 0.001), ulnar (p < 0.001), and tibial nerve (ankle, p < 0.001) when compared with controls. Electroneurography documented signs of significantly lower values of the motor conduction velocity and compound muscle action potentials (cMAPs) in the upper arm nerves (median, ulnar, radial, p < 0.001). A significant correlation between sonographic and electrophysiological findings in the MMN group was found only between cMAP and CSA of the median nerve at the upper arm (r = 0.851, p < 0.001). Neither nerve sonography nor electrophysiology correlated with functional disability. MMN seems to show inhomogeneous CSA enlargement in various peripheral nerves, with weak correlation to electrophysiological findings. Neither nerve sonography nor electrophysiology correlated with functional disability. Multicentre, prospective studies are required to prove the applicability and diagnostic values of these findings.     

The long‐term functional status in patients with Guillain‐Barré syndrome

Background and purpose: The purpose of this study was to analyse the long‐term impact of Guillain‐Barré syndrome (GBS) on quality of life, and the relationship between clinical variables at disease onset and symptoms at follow‐up to general health status. Methods: Forty‐two GBS patients were examined at median 6 years after disease onset and were compared with 50 healthy controls. The fatigue severity scale (FSS), visual analogue scale (VAS) for pain, disability rating index (DRI) and medical outcome study 36‐item short‐form health status scale (SF‐36) were applied. Variables at onset and symptoms at follow‐up were correlated with outcome measurements in GBS. Results: VAS [2.9 (SD 3.3) vs. 1.5 (SD 1.9); P = 0.01] and DRI [2.5 (SD 2.1) vs. 1.0 (SD 1.5); P < 0.001] were significantly higher in patients with GBS, compared with healthy controls. Decreased physical functioning and general health were found on SF‐36. Differences between GBS patients with shorter (<6 years) and longer (≥6 years) follow‐up after onset were not found. Conclusions: Relatively independent from various variables at onset, patients with GBS seem to have a reduced quality of life and functioning, and the distress seems to have become persistent after the first few years with improvement following the acute disease.     

Ultrasonography of the peripheral nervous system in the early stage of Guillain‐Barré syndrome

Ultrasonography can be used to visualize peripheral nerve abnormalities in immune‐mediated neuropathies. The objective of this study was to prove the role of ultrasonography (US) in acute phase of Guillain‐Barré syndrome (GBS). Systematic ultrasonic measurements of several peripheral nerves including the vagal nerve as well as the sixth cervical nerve root were performed in 18 patients with GBS at days 1–3 after symptom onset and compared to 21 healthy controls. Nerve conduction studies (NCS) of corresponding nerves were undertaken. Consequently, significant differences between the groups were found in compound muscle action potential amplitudes, F‐wave latency, and persistency. Ultrasonic cross‐sectional areas (CSAs) showed significant enlargement in all nerves except of the ulnar nerve (upper arm) and the sural nerve compared to healthy controls, most prominent in proximal and middle median nerve (p < 0.01). The vagal nerve also showed enlargement compared to controls (p < 0.05), which was most pronounced in patients with autonomic dysfunction compared to patients without (p < 0.05). C6 root diameter showed a significant correlation to the amount of cerebrospinal fluid (CSF)‐protein (Pearson correlation, p < 0.05). US shows nerve enlargement in several peripheral nerves including vagal nerve and C6 root in acute phase of GBS and could be an additional diagnostic tool for example, in GBS of atypical onset and autonomic dysfunction.     

Normal values of median nerve cross‐sectional area obtained by ultrasound along its course in the arm with electrophysiological correlations,in 100 Asian subjects

Introduction: The objective of this study is to obtain normative cross‐sectional area (CSA) values for median nerve by ultrasound at predetermined sites and correlate them with electrophysiological variables in healthy Asian subjects. Methods: The median nerve was examined ultrasonographically in 100 healthy volunteers, mean age 39 years (range, 18–75 years). CSA of the median nerve was measured at wrist, mid‐forearm, mid‐arm, and axilla. All subjects underwent simultaneous standardized nerve conduction studies. Results: The mean median nerve CSAs ± SD at the distal wrist crease was 7.2 ± 1 mm²; mid‐forearm 4.8 ± 0.9 mm²; mid‐arm 6.1 ± 1 mm²; axilla 5.9 ± 0.9 mm². The CSA at the wrist was the largest compared with other levels (P < 0.001), and it increased with advancing age (P < 0.002). Conclusions: These normative data show that median nerve CSA is not uniform along its length. There are differences between gender, and values increase with advancing age. Muscle Nerve 49 : 284–286, 2014     

Is there a need for long‐term follow‐up in chronic idiopathic polyneuropathy?

Objective – To evaluate the long‐term functional status and well‐being in patients with chronic idiopathic polyneuropathy (CIP) in comparison to Guillain–Barré syndrome (GBS) and healthy controls. Materials and methods – Forty‐two CIP and 42 GBS‐patients were examined at median 5 and 6 years after disease onset and were compared with 50 healthy controls. The Fatigue Severity Scale (FSS), Visual Analogue Scale for pain (VAS), Disability Rating Index (DRI) and Medical Outcome Study 36‐item short‐form health status scale (SF‐36) were used. Variables at onset and symptoms at follow‐up were correlated with outcome measurements in GBS. Results – Patients with CIP and GBS had more pain and disability than healthy controls. Additionally, CIP‐patients were more fatigued than healthy controls. Patients with CIP were more fatigued [FSS 4.9 (SD 1.6) vs 3.8 (SD 1.8); P < 0.01] and disabled [DRI 4.1 (SD 2.3) vs 2.5 (SD 2.1); P = 0.05] than those with GBS. Physical functioning on the SF‐36 was more impaired in CIP than GBS, compared with healthy controls. Conclusions – Patients with CIP and GBS seem to develop persistent impairment on long‐term functional status and well‐being, more clearly in CIP, reflecting the importance of long‐term follow‐up in further disease management.     

Innovative quantitative testing of hand function in Charcot‐Marie‐Tooth neuropathy

To describe a new test to quantitatively evaluate hand function in patients affected by Charcot‐Marie‐Tooth neuropathy (CMT). The sensor‐engineered glove test (SEGT) was applied to CMT patients (N: 26) and compared with a cohort of healthy controls (HC, N: 26). CMT patients were further divided into subjects with clinically normal (group 1) or impaired hand (group 2) function. The SEGT parameters evaluated were touch duration, inter‐tapping interval, and movement rate parameters of two different sequences: finger tapping (FT) and index‐medium‐ring‐little (IMRL) performed at self‐paced mode (SPM) and maximum velocity (MV). Hand function and strength were assessed by the 9‐hole peg test (9HPT) and dynamometry. Disability of patients was measured by the CMT neuropathy score. CMT patients had significantly worst performances at SEGT than controls regarding the rate of execution of both FT (at MV) and IMRL sequences (at SPM and MV). The rate parameter at MV in IMRL sequence showed a significant trend of decreasing in its average between HC (n: 26, rate = 3.08 ± 0.52 Hz), group 1 (n: 9, rate = 2.64 ± 0.66 Hz) and group 2 (n: 17, rate = 2.19 ± 0.45 Hz) (p for trend <0.001). No correlations were found with either 9HPT, dynamometry, electrophysiology, and the CMT neuropathy score. The SEGT test is sensitive to show hand dysfunction in CMT patients, with and without clinically impaired hands.     

Diabetes mellitus may affect short‐term outcome of Guillain‐Barré syndrome

We sought to determine influence of diabetes mellitus on Guillain‐Barré syndrome (GBS) course and short‐term prognosis. Among the 257 GBS patients included in this retrospective study, diabetes mellitus was present in 17%. The degree of disability at admission and on discharge was assessed according to the GBS Disability Scale (mild disability = 0–3, severe disability = 4–6). Even after correction for age, diabetes mellitus was significantly associated with more severe disability at nadir (odds ratio, OR = 3.4, p < 0.05) and on discharge (OR = 2.0, p < 0.05). Linear regression analysis with multiple factors included showed that age and presence of diabetes were significant predictors of severe disability at nadir (adjusted R² = 0.21, p < 0.05), and on discharge (adjusted R² = 0.19, p < 0.05). The presence of diabetes mellitus affects short‐term prognosis of GBS, independent of age.     

Immunoglobulin IgG Fc‐receptor polymorphisms and HLA class II molecules in Guillain–Barré syndrome

Sinha S, Prasad KN, Jain D, Nyati KK, Pradhan S, Agrawal S. Immunoglobulin IgG Fc‐receptor polymorphisms and HLA class II molecules in Guillain–Barré syndrome.
Acta Neurol Scand: 2010: 122: 21–26.
© 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. Objective – To analyze host genetic factors immunoglobulin G Fc receptors (FcγRs) and human leukocyte antigen (HLA) class II in GBS patients. Methods – FcγRIIA, IIIA and IIIB polymorphisms were studied in 80 each GBS patients and healthy controls by allele specific PCR. HLA class II DRβ1 and DQβ1 typing was performed at the two‐digit level by PCR in randomly selected 54 GBS patients and 202 controls. Results – FcγRIIA‐H/H (56% vs 9%; P < 0.0001) and FcγRIIIA‐V/V (40% vs 13%; P < 0.0001) genotypes, H131 allele frequencies (0.73 vs 0.26, P < 0.0001) and HLA DQβ1*060x (OR, 1.96; 95% CI, 1.26–3.04; P < 0.01) were significantly increased in GBS than controls. DRβ1*0701 alone (OR, 10; 95% CI, 45.90–2.25; P < 0.001) and together with FcγRIIA‐H/H (OR, 11.03; 95% CI, 2.63–46.20; P < 0.001) was significantly associated with GBS patients having microbiological evidence of recent infection. Conclusions – The study indicates that homozygous FcγRIIA and FcγRIIIA genotypes and FcγRIIA H131 allele are associated with GBS. HLA class II molecule DRβ1*0701 is identified as novel genetic risk factor for development of GBS in patients with preceding infection.     

Guillain‐Barré syndrome in the elderly

The aim of the study was to analyze specific features of Guillain‐Barré syndrome (GBS) in old people. The study included 403 GBS patients (62% young [<60 years], 35% young‐old [60–80 years], and 3% old‐old [>80 years]). Diagnosis of GBS was made according to the National Institute of Neurological Disorders and Stroke (NINDS criteria). Severe disability (GBS disability score of >3) at nadir was more common in old compared with young patients (p = 0.0001) as was mortality (9% vs. 2%, respectively). Acute motor and sensory axonal neuropathy and hyponatremia were more common in old compared with young patients (12% vs. 6% and 27% vs. 18%, respectively, p = 0.04). A positive history for malignancy was more than three times more common in old than young patients (11% vs. 3%, respectively, p = 0.01). Disability on nadir was similar in young‐old and old‐old subjects with disability on discharge being more severe in old‐old (p = 0.04) suggesting slower recovery in this subgroup. Bulbar symptoms were more common in old‐old compared with young‐old (50% vs. 19%, respectively, p = 0.01). Comorbidities were present in virtually all old‐old patients compared with 66% of young‐old patients (p = 0.04). In conclusion, Elderly patients, and especially old‐old patients, with GBS have more severe disease with slower recovery than do younger patients.     

Validation of the Serbian version of inflammatory Rasch‐built overall disability scale in patients with chronic inflammatory demyelinating polyradiculoneuropathy

Inflammatory Rasch‐built overall disability scale (I‐RODS) seems to be a valid activity measure for use in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our aim was to translate and validate the I‐RODS for use in CIDP patients from Serbia. Study comprised 83 patients diagnosed with CIDP. I‐RODS was translated and cross‐culturally validated using the standard guidelines. Following scales were also administered: Medical Research Council (MRC) sum score, Inflammatory Neuropathy Cause and Treatment (INCAT) sensory and disability scores, Krupp's Fatigue Severity Scale, Beck Depression Inventory, Visual Analogue Scale for pain, and health survey‐36 (SF‐36) as a quality of life measure. According to the I‐RODS, significant proportion of our patients reported that “running” (51%), “dancing” (41%), and “standing for hours” (40%) were impossible tasks to perform, while “teeth brushing” (94%), “eating” (88%), and “reading a newspaper/book” (82%) were noted as the easiest items. Patients with more muscle weakness (lower MRC sum score) and more severe INCAT sensory score had lower I‐RODS score (P < .01). Also, patients with fatigue, depression and pain had lower I‐RODS scores (P < .01). I‐RODS score correlated with the INCAT disability score (P < 0.01) was 78 ± 19 compared to 51 ± 30 in patients with INCAT >1 (P < .01). I‐RODS score correlated with the total SF‐36 score (rho = +0.73, P < .01), as well as with all SF‐36 domain scores. Serbian version of the I‐RODS seems to be a valid activity measure for use in CIDP patients. I‐RODS was able to assess different levels of disability, it was in association with impairment measures, INCAT disability scale and quality of life. Further studies are needed to assess its responsiveness.     

Determinants of health‐related quality of life in anti‐MAG neuropathy: a cross‐sectional multicentre European study

Our objective was to assess determinants of quality of life (QoL) in anti‐myelin associated glycoprotein antibody (MAG) neuropathy. The SF‐36 questionnaire was assessed in 55 patients, from Marseille, Angers (France) and Birmingham (UK). Routine clinical evaluations included Medical Research Council (MRC) sum score, inflammatory neuropathy cause and treatment (INCAT) sensory score, inflammatory Rasch‐built overall disability score (I‐RODS), ataxia score, Jamar grip dynamometry, timed 10‐m walk, neuropathic pain symptom inventory (NPSI) score, and fatigue severity score (FSS). Physical component summary (PCS) and mental component summary (MCS) of the SF36 questionnaire were significantly lower than in reported normal subjects of both countries (p < 0.001). All SF‐36 domains correlated with I‐RODS, except MCS for which significance was, however, approached (p = 0.056). PCS correlated with MRC sum score, ataxia score, timed 10‐m walk, tremor, Jamar grip dynamometry, NPSI pain score, FSS and level of social support. MCS correlated exclusively with FSS and level of social support. In multivariate regression, PCS was associated independently with I‐RODS (p < 0.001) and NPSI pain score (p = 0.011), whereas MCS was associated independently with FSS (p = 0.022). QoL is accurately predicted in anti‐MAG neuropathy by the I‐RODS and FSS, lending support to their use in clinical and research settings. Effective measures to improve QoL should include tremor and neuropathic pain treatment, fatigue management, and improved social support.     

The utility of Guillain‐Barré syndrome prognostic models in Malaysian patients

Guillain‐Barré syndrome (GBS) is an acute immune‐mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P < .001; EGOS: r = .484, P < .001). However, there were no significant correlations between mEGOS or EGOS and outcome in patients with MFS (mEGOS on admission: r = .152, P = .523; mEGOS at day 7 of admission: r = .008, P = .973; EGOS: r = .110; P = .644). The score of EGRIS for GBS patients with mechanical ventilation was significantly higher than those patients without mechanical ventilation (4 ± 2 vs 3 ± 1; P < .001). We conclude that mEGOS and EGOS are clinically useful and relevant to the Malaysian GBS population but not in patients with classic MFS. EGRIS could be used to predict the need for mechanical ventilation in our local GBS patients.     

Clinical and serological prognostic factors in childhood Guillain‐Barré syndrome: A prospective cohort study in Bangladesh

Guillain‐Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. The objective of this study was to investigate the preceding infections, clinical, serological and electrophysiological characteristics and outcome of childhood GBS in Bangladesh. We included 174 patients with GBS aged <18 years from a prospective cohort in Bangladesh between 2010 and 2018. We performed multivariate logistic regression to determine the risk factors for poor outcome. Among 174 children with GBS, 74% (n = 129) were male. Around half of the patients (49%, n = 86) had severe muscle weakness, 65% (n = 113) were bedbound (GBS disability score 4) and 17% (n = 29) patients required mechanical ventilation at admission. Campylobacter jejuni serology and anti‐GM1 IgG antibody were positive in 66% and 21% of the patients respectively. One hundred and forty‐three (82%) patients did not receive standard treatment and half of them recovered fully or with minor deficits at 6‐month. Twenty patients (11%) died throughout the study period. At 3‐month of onset of weakness, complete recovery or recovery with minor deficit was significantly higher in demyelinating GBS patients compared to axonal GBS patients (86% vs 51%, P = .001). Cranial nerve palsy (OR = 4.00, 95%CI = 1.55‐10.30, P = .004) and severe muscle weakness (OR = 0.16, 95%CI = 0.06‐0.45, P = .001) were the important risk factors of poor outcome in children with GBS. Further large‐scale studies are required for better understanding of factors associated with mortality and morbidity in childhood GBS.     

[11C]d-threo-methylphenidate PET in patients with Parkinson’s disease and essential tremor

Summary. Twenty Parkinson’s disease (PD) patients, 6 patients with essential tremor and 10 healthy controls were studied with the dopamine transporter ligand [¹¹C]d-threo-methylphenidate ([¹¹C]dMP) and positron emission tomography (PET) to assess dopamine terminal loss in relation to disease duration and motor disability. Dopamine transporter availability was expressed as [¹¹C]dMP binding potential (BP_dMP) in percentage of the mean of healthy controls. In PD patients (age at onset 57.7 ± 8.9 yrs; disease duration 5.2 ± 3.3 yrs; UPDRS motor score 24.2 ± 9.8; Hoehn & Yahr 2.1 ± 0.8; mean ± SD) BP_dMP was reduced to 30% (range: 11–55%) in the putamen and 52% (range: 14–96%) in the caudate nucleus. BP_dMP in the putamen closely correlated with the UPDRS motor score (r = −0.79, p < 0.001), and disease duration (r = −0.76, p < 0.001) but not with age at onset. The correlation with the UPDRS score depended on akinesia and rigidity, while the tremor scores were related neither to putamen nor caudate BP_dMP. Interestingly, when plotted over disease duration, PD patients with severe asymmetry of symptoms showed significantly lower BP_dMP in the contralateral putamen (exponential fit: 34% at onset) than the other PD patients (41% at onset), indicating a different symptomatic threshold of these subgroups and an even closer correlation with the hypothetical “true” disease duration. The exponential fit across all patients indicated a mean symptomatic threshold of 37% contra- and 62% ipsilateral, corresponding with an observed mean BP_dMP of 51% (average contra- and ipsilateral) in those patients with disease duration less than one year. No differences in BP_dMP were observed between patients with essential tremor and healthy controls. [¹¹C]dMP appears to be a useful and sensitive marker of dopaminergic dysfunction in PD and can be used to assess and monitor disease severity. Both authors contributed equally     

Demyelination induced by serum form patients with Guillain-Barré syndrome

Sera from 16 patients with acute Guillain-Barré syndrome (GBS) and 14 healthy control subjects were injected into rat sciatic nerve and assessed for demyelinating activity by electrophysiological and histological techniques. Only fresh GBS serum, and not GBS serum stored at?20°C or ?70°C, blocked conduction to a significantly greater extent than did fresh control serum. Conduction block developed gradually, starting within 24 hours of injection and reaching a maximum between days 3 and 6. Recovery of conduction commenced thereafter, and conduction returned to normal by day 33. Quantitative histological studies on day 6 showed that fresh GBS serum produced significantly more widespread demyelination than did stored GBS serum (p < 0.01). Stored GBS serum showed residual demyelinating activity When compared with fresh control serum (p < 0.01). Fresh serum obtained from 4 patients after recovery from GBS did not produce conduction block, despite it having done so during the acute phase of the disease.     

Investigation of esophageal sensation and biomechanical properties in functional chest pain

Background There is limited and conflicting data regarding the role of esophageal hypersensitivity in the pathogenesis of functional chest pain (FCP). We examined esophageal sensori‐motor properties, mechanics, and symptoms in subjects with FCP. Methods Esophageal balloon distension test was performed using impedance planimetry in 189 (m/f = 57/132) consecutive subjects with non‐cardiac, non‐reflux chest pain, and 36 (m/f = 16/20) healthy controls. The biomechanical and sensory properties of subjects with and without esophageal hypersensitivity were compared with controls. The frequency, intensity, and duration of chest pain were assessed. Key Results One hundred and forty‐three (75%) subjects had esophageal hypersensitivity and 46 (25%) had normal sensitivity. Typical chest pain was reproduced in 105/143 (74%) subjects. Subjects with hypersensitivity demonstrated larger cross‐sectional area (P < 0.001), decreased esophageal wall strain (P < 0.001) and distensibility (P < 0.001), and lower thresholds for perception (P < 0.01), discomfort (P < 0.01), and pain (P < 0.01) compared to those without hypersensitivity or healthy controls. Chest pain scores (mean ± SD) for frequency, intensity and duration were 2.5 ± 0.3, 2.2 ± 0.2, and 2.2 ± 0.2, respectively, and were similar between the two patient groups. Conclusions & Inferences Seventy‐five per cent of subjects with FCP demonstrate esophageal hypersensitivity. Visceral hyperalgesia and sensori‐motor dysfunction of the esophagus play a key role in the pathogenesis of chest pain.     

One‐year follow‐up study of neuropathic pain in chronic inflammatory demyelinating polyradiculoneuropathy

We sought to gather information about frequency and features of neuropathic pain (NeP) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and to investigate course of NeP during 1‐year follow‐up. Study included 105 patients diagnosed with CIDP. Patients with diabetes (N = 26) were excluded. NeP was diagnosed by the official guidelines and painDETECT questionnaire (PD‐Q). Medical Research Council Sum Score (MRC‐SS), INCAT disability and sensory scores, and Beck Depression Inventory were also measured. PD‐Q showed presence of NeP in 16 (20%) of 79 CIDP patients and their mean pain was moderate (5.1 ± 3.0 of 10). Diagnostic delay in CIDP patients with NeP was prolonged compared to CIDP patients without NeP (21 ± 28 vs 9 ± 12 months, P < .05). Slowly progressive course of the disease was more frequent in patients with NeP (81% vs 52%, P < .05). Patients with NeP had worse INCAT sensory score (P < .01), INCAT disability score (P < .05), MRC‐SS, as well as worse disease outcome at time of testing (P < .05). Depression was more common in patients with NeP (69% vs 17%, P < .01). During 1‐year follow‐up, majority of our CIDP patients had good control of NeP with gabapentinoids or amitriptyline. NeP was common in our cohort of non‐diabetic CIDP patients. It was associated with worse functional disability, worse sensory deficit, and depression. Special attention should be paid to CIDP patients with NeP because they request additional symptomatic therapy that appeared efficacious in our cohort.     

Health‐related quality of life in early Parkinson's disease: The impact of nonmotor symptoms

Nonmotor symptoms (NMS) are common in patients with established Parkinson's disease (PD) and have a major impact upon quality of life. We investigated the significance of NMS in relation to health‐related quality of life (HRQoL) in patients with newly diagnosed PD. Patients and healthy controls were recruited as part of the Incidence of Cognitive Impairment in Cohorts with Longitudinal Evaluation in Parkinson's Disease Study. Prevalence of NMS was determined with the Non‐Motor Symptom Questionnaire. HRQoL was recorded with the 39‐item Parkinson's Disease Quality of Life Questionnaire (PDQ‐39). Further assessments included measures of motor disability, depression, sleep, and cognition. One hundred and fifty‐eight patients with newly diagnosed PD and 99 controls participated in this cross‐sectional study. Patients reported greater numbers of NMS than controls (mean 8.3 ± 4.3 versus 2.8 ± 2.5 symptoms; P < 0.001). Patients reported lowest HRQoL in the domains assessing bodily discomfort, mobility, and activities of daily living. Motor and nonmotor symptoms impacted negatively upon HRQoL scores. Patients with the postural instability and gait difficulty motor subtype reported worse HRQoL, compared with those with tremor‐dominant disease. Depression (P < 0.001), incomplete bowel emptying (P < 0.001), anxiety (P < 0.001), impaired concentration (P < 0.001), memory complaints (P < 0.001), and insomnia (P = 0.001) had the greatest negative impact upon HRQoL. NMS are common in patients with early PD and represent a significant cause of poorer health‐related quality of life. Cognitive, neuropsychiatric, and sleep disturbances are particularly associated with reduced well‐being. Screening and management of these symptoms should be prioritized at the time of diagnosis. © 2013 International Parkinson and Movement Disorder Society     

Measurement of motor root conduction time at the early stage of Guillain–Barre syndrome

Background and purpose: As they are mainly performed at distal nerve parts, routine electrophysiological examinations can fail to detect the abnormalities at the early stage of Guillain–Barre syndrome (GBS) because of predominant involvement of proximal nerve segments. Measurements focused on proximal conduction can provide additional findings. We investigated the diagnostic significance of motor root conduction time (MRCT) at the early stage of GBS. Methods: Study was performed in 30 healthy volunteers and within the first week of 12 patients with GBS. MRCT was calculated as the latency differences between motor nerve conduction time (MNCT) obtained by cervical magnetic stimulation and total peripheral motor conduction time obtained by electrical stimulation of ulnar nerve. Also MNCT/MRCT ratio was calculated in each subject. Results: There were statistically significant differences between groups for MRCT (P < 0.0001) and MNCT/MRCT ratio (P < 0.0001). Although the F‐wave latency at ulnar nerve was abnormal only in 33%, MRCT was significantly prolonged in 83% of patients. In three patients, prolongation of MRCT was the only abnormality at the first electrodiagnostic examination. Conclusions: Motor root conduction time as a non‐invasive method can provide additional and confirmative information at the early stage of GBS in which routine nerve conduction studies may fail to detect the focal demyelination.     

Macrophage migration inhibitory factor (MIF) seems crucially involved in Guillain-Barré syndrome and experimental allergic neuritis

Macrophage migration inhibitory factor (MIF) is a proinflammatory type 1 cytokine that plays a pathogenic role in several inflammatory and autoimmune diseases. The role of this cytokine in peripheral nerve inflammatory disease has not been evaluated. Therefore, to evaluate the role of macrophage migration inhibitory factor (MIF) in Guillain-Barré syndrome (GBS) and experimental allergic neuritis (EAN), we determined MIF circulating levels in a series of patients with GBS and healthy subjects with ELISA and evaluated the effect of two specific inhibitors of MIF, a neutralizing monoclonal antibody or a chemical inhibitor ISO1 on the course of murine EAN. The data show increased MIF plasma levels in GBS patients as compared to healthy controls (p<0.0001) and a progressive increase of MIF circulating concentration with patient's disability (p<0.0001). Both anti-MIF mAb and ISO1 favorably influenced the course of EAN. Treated mice had a lower cumulative severity score (p=0.001) and reduced disease duration than the control mice (p<0.03). MIF may promote immune reaction in GBS. Therapeutic effects of both anti-MIF mAb and ISO1 in EAN suggest that MIF could be a promising therapeutic target in inflammatory demyelinating peripheral nerve disorders.