Overall Neuropathy Limitation Scale Research Articles

Residual disability 10 years after falling ill in Guillain–Barré syndrome: A prospective follow-up study

ObjectiveTo describe residual disability 10years after onset of Guillain–Barré syndrome (GBS) and longitudinal changes from 2weeks after onset until 10years afterwards. The Erasmus GBS Outcome score (EGOS) was applied for predicting prognosis at 2 and 10years. MethodsTwenty-nine patients, mean age at onset 49years, were followed prospectively from 2weeks to 10years after GBS onset. Measures included; GBS disability score, EGOS, Barthel Index, Frenchay Activity Index, Sickness Impact Profile (SIP), Overall Neuropathy Limitations Scale (ONLS), Walk-12, and Fatigue Severity Scale. ResultsAt 10years, the facial paralysis found in 5 participants at 2years was still present, 11 participants (38%) experienced paresthesia, 6 (21%) had limitations in their arms, and 15 (52%) had limitations in walking. Decreased health-related quality of life on comparison to the general population was seen in the physical dimension of SIP at 10years. The median EGOS at 2weeks was 4.5, which correlated highly only with the Barthel Index at 2years and the ONLS arm scale at 10years. ConclusionThe residual disabilities at 1–2years comprised mainly of reduced walking ability, and are still persistent 10years after GBS onset. For some individuals, facial paralysis caused major disability. The EGOS only partly predicted residual disability at 2 and 10years after onset.

Pain assessment in Charcot-Marie-Tooth (CMT) disease

ObjectiveThe objective of our study was to describe and evaluate the prevalence of chronic pain in persons with Charcot-Marie-Tooth (CMT) disease during a multidisciplinary consultation at the Center of Reference for Neuromuscular Diseases. MethodologyThis prospective study was conducted between 2008 and 2010, it was a partnership between a Center of Reference for Neuromuscular Diseases (Centre de référence des maladies neuromusculaires [CRMD]) and a Department for the Assessment and Treatment of Pain (Département d’évaluation et de traitement de la douleur [DETD]). The evaluation consisted in a complete assessment of each patient during the first multidisciplinary consultation, with a previously established diagnosis validated by genetic testing, by various specialists: neurologist, PM&R physician, pain management specialist and physiotherapist. The evaluation tools used were Visual Analogical Scale (VAS), Hospital Anxiety and Depression Scale (HAD), DN4 scale, Neuropathic Pain Symptom Inventory (NPSI) (if DN4≥4), Pain Questionnaire of Saint Antoine (QDSA) (if DN4<4), body representation to define the painful areas, Overall Neuropathy Limitations Scale (ONLS), Medical Research Council scale (MRC), Short Questionnaire on Pain (QCD), VAS during transfers, self-care, getting dressed and physiotherapy sessions and quantified use of analgesics. ResultsA total of 50 patients were included (28 women, 22 men); two patients (one man and one woman) were discarded from the study because of missing pain assessment data. Mean age was 47years (R: 14–85), in average the symptoms had been present for the past 20years (R: 0.3–68), most patients had little impairment, the mean MRC was 53 (R: 36–60), with CMT1A being predominant (CMT1A: 76.9%, CMTX: 13.5%, CMT2: 5.8%, CMT4: 3.8%). It is noted that 65.4% of patients reported some pain with a mean duration of pain at 140months (R: 5–660). The mean VAS was 5.5 (R: 1–10), greater than 4 in 79.4% of cases, requiring the use of analgesics in 38.4% of cases (step 1: 60%, step 2: 40% on the WHO pain relief ladder). The predominant location of the pain was distal, peripheral and symmetric (64.7%); furthermore the feet were affected in 80% of cases. DN4 was positive in 40.6% of painful patients. In 62.5% of the cases, the pain did not have an underlying mechanical origin. The emotional impact remained quite modest (HAD: A=8; D=5). Patients with CMT1A seemed less affected by pain (P=0.03). ConclusionThis original study describes the pain in patients with CMT disease during a primary multidisciplinary consultation. We see that in 66% of cases, patients do report some pain, this pain is usually moderate, preferentially located in the extremities and is symmetric. In 62.5% of cases, the pain has a neuromuscular origin with a positive DN4 in 50% of cases in this etiology. In our study, patients with CMT1A report less pain than patients with other CMT types. This disease being quite rare (rare disease), the number of patients did not allow us to bring up statistically significant results. The pain must be thoroughly screened for because of its frequency in persons with CMT.

Outcome measures for Charcot‐Marie‐Tooth disease: clinical and neurofunctional assessment in children

Charcot-Marie-Tooth (CMT) disease is the most common inherited neuromuscular disorder, presenting with symptoms often occurring since childhood, and showing a progressive course. At present, there are no valid and reliable measures for evaluation of impairment and disability in the pediatric population. The aim of this study was to determine the usefulness of outcome measures, commonly used in adult patients, in CMT children. We report the results of a comprehensive evaluation of 21 children affected with CMT type 1A, including clinical examinations, measure of hand and foot muscle strength with a hand-held dynamometer, and the following scales: CMT Neuropathy Score or its clinical component CMT Examination Score, Overall Neuropathy Limitations Scale (ONLS), Walk-12 questionnaire, and nine-hole peg test (9-HPT). Hand grip, three-point pinch, and foot dorsiflexion strength were significantly lower than age/sex equivalent in almost all cases. 9-HPT was significantly abnormal in 62% of patients and CMT Examination Score was <10 points in all cases. ONLS showed presence of minor disability in the upper limbs in 57% and mild abnormalities of gait in 71% of patients. Overall, these scales demonstrated limited potential to measure disability and severity of the disease confirming that it is necessary to identify specific scales for children with CMT.

Amélioration d’un cas de neuropathie motrice multifocale avec blocs de conduction sévère, dépendant des immunoglobulines intraveineuses, après adjonction de rituximab au long cours

Introduction Some patients suffering from multifocal motor neuropathy with conduction blocks (MMNCB) are still disabled after treatment with intravenous immunoglobulin (IVIg). Case report We report the benefits of a combination of rituximab (RTX) and IVIg in the case of a 72-year-old man with MMNCB. Discussion Despite an IVIg treatment, the patient had severe motor weakness of the four limbs which limited daily living activity. Azathioprine, mycophenolate mofetyl and cyclophosphamid did not improve the patient's status. Adjunction of rituximab to IVIg therapy increased muscle strength measured on MRC sum score and reduced disability evaluated on ONLS (Overall Neuropathy Limitation Scale) score in the long term (37 months). In spite of the improvement of his neurological status, the patient remained dependent on IVIg. Conclusion RTX could be proposed as a long-term complementary treatment for some severe cases of IVIg-dependent NMMBC. These results must be confirmed in a randomized controlled study.

Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome

AbstractPOEMS syndrome is a rare clonal plasma cell disorder without standard treatment. Based on the efficacy and low toxicity of a combination of melphalan and dexamethasone (MDex) for light chain amyloidosis, we conducted a prospective study of MDex treatment for patients with newly diagnosed POEMS syndrome. Thirty-one patients (19 men) were enrolled and the median age at the time of diagnosis was 44 years (range, 32-68 years). All patients received 12 cycles of MDex treatment. Twenty-five patients (80.6%) achieved hematologic response including 12 (38.7%) complete remission and 13 (41.9%) partial remission. Of all 31 patients, the neurologic response rate was 100%, assessed by overall neuropathy limitation scale (ONLS). The initial neurologic response was observed in 24 patients (77.4%) at 3 months after treatment and the median time to maximal neurologic response was 12 months (range, 3-15 months). Moreover, MDex substantially improved the level of serum vascular endothelial growth factor and relieved organomegaly, extravascular volume overload, and pulmonary hypertension. Only 6 patients (19.3%) suffered from grade 3 adverse events during treatment. All patients are alive and free of neurologic relapse after the median follow-up time of 21 months. Therefore, MDex is an effective and well-tolerated treatment option for patients with newly diagnosed POEMS syndrome.

Do patients having a decrease in SNAP amplitude during the course of MMN present with a different condition?

A decrease in sensory nerve action potentials (SNAP) amplitude has been recently reported in some patients during the course of multifocal motor neuropathy with conduction blocks (MMNCB). It is not known if those patients have different clinical expression and disability when compared with typical MMNCB. Clinical, biological and electrophysiological assessments were performed in 15 patients fitting the diagnosis criteria of MMNCB, including normal SNAP amplitude at initial examination. Patients presenting with nerve entrapment or associated disease causative of sensory neuropathy were excluded. Median time of follow-up was 3 years (1-17 years). At the last examination, four patients had at least one SNAP amplitude below 50% of normal value. None had clinically objective sensory loss. Clinical and electrophysiological data obtained at the last examination were compared between patients with normal SNAP amplitude and patients with decreased SNAP amplitude. No difference between both population in term of age, sex, disease duration, anti-GM1 antibody titers, CSF data and number of conduction blocks was noted. In contrast, patients with decreased SNAP amplitude had worse overall neuropathy limitation scale (ONLS) scores (7 vs. 2; p = 0.02), a higher number of affected nerves (12.5 vs. 4; p = 0.018), a higher number of affected limb regions (6 vs. 2; p = 0.019) and lower median CMAP amplitude (2 mV vs. 6.5 mV; p = 0.04). They were all dependent on higher doses of IVIg (1.4 g/(kg 4 weeks vs. 0.6; p = 0.018). A reduction in SNAP amplitude during the course of MMNCB is associated with a more severe disease and a more prominent axonal loss. This result needs to be confirmed in a larger cohort.

Reliability of clinical outcome measures in Charcot-Marie-Tooth disease

We assessed inter- and intra-rater reliability of outcome measures in Charcot-Marie-Tooth disease (CMT) patients. In 40 CMT patients, we assessed reliability of Overall Neuropathy Limitations Scale (ONLS), 10-m timed walk (T10MW), 9-hole-peg test (9-HPT), maximal voluntary isometric contraction (MVIC) of arm (elbow flexion, hand-grip, and three-point pinch) and leg (knee extension, foot dorsiflexion/plantar flexion). Reliability was substantial for ONLS, excellent for T10MW and 9-HPT. For MVIC, inter and intra-rater reliability was excellent for hand contractions; for leg contractions, intra-rater agreement was moderate to substantial, whereas inter-rater agreement was poor. An ad hoc device was produced to immobilize the foot and MVIC reliability was re-assessed in 26 CMT patients, resulting in excellent inter-rater and intra-rater reliability for foot dorsiflexion, and clear inter-rater improvement for foot plantar flexion. All outcome measures appear adequate for CMT assessment. Use of an immobilization device improves foot MVIC reliability, preventing biased findings in patients with greater strength.