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OBJECTIVE: Motor conduction velocity may yield false-negative results in mild ulnar nerve entrapment at elbow (UNE). There is evidence that the clinical heterogeneity of UNE may be due to the different involvement of fascicles. We hypothesized that, if fibres to FDI are more damaged than fibres to ADM, a relative slowing of motor conduction velocity (CV) at the segment across the elbow recording from FDI (FDI-CV) versus CV at the same segment recording from ADM (ADM-CV) would occur. METHODS: We calculated the ratio between FDI-CV and ADM-CV (IN-RATIO) in 60 consecutive UNE patients, 40 norms, and 16 patients with lower cervical radiculopathy. The UNE sample consisted of (1) patients with neurophysiological evidence (UNE NF+), (2) patients without neurophysiological evidence (UNE NF-). We evaluated the possible usefulness of the IN-RATIO to increase sensitivity in diagnosing UNE. RESULTS: The IN-RATIO was lower in the UNE NF- than in norms (p<0.001) and cervicobrachialgia sample (p=0.02). We found that if the IN-RATIO is

OBJECTIVE: Motor conduction velocity may yield false-negative results in mild ulnar nerve entrapment at elbow (UNE). There is evidence that the clinical heterogeneity of UNE may be due to the different involvement of fascicles. We hypothesized that, if fibres to FDI are more damaged than fibres to ADM, a relative slowing of motor conduction velocity (CV) at the segment across the elbow recording from FDI (FDI-CV) versus CV at the same segment recording from ADM (ADM-CV) would occur. METHODS: We calculated the ratio between FDI-CV and ADM-CV (IN-RATIO) in 60 consecutive UNE patients, 40 norms, and 16 patients with lower cervical radiculopathy. The UNE sample consisted of (1) patients with neurophysiological evidence (UNE NF+), (2) patients without neurophysiological evidence (UNE NF-). We evaluated the possible usefulness of the IN-RATIO to increase sensitivity in diagnosing UNE. RESULTS: The IN-RATIO was lower in the UNE NF- than in norms (p<0.001) and cervicobrachialgia sample (p=0.02). We found that if the IN-RATIO is

Background: There is only one small single-center study on the reliability of the diagnosis of focal dystonia. The aim of this study was to assess the inter-rater reliability of dystonia diagnosis among neurologists with different professional experience. Methods: Twenty-nine adults (18 with dystonia, 9 with other movement disorders, and 2 healthy controls) were videotaped while undergoing neurological examination and during the process of collecting information on the history of their condition. Each case was diagnosed by 35 blind raters (12 general neurologists, 21 neurology residents, and 2 experts in movement disorders) from different hospitals. Sensitivity and specificity were calculated confronting raters with the gold standard (the caring physician). Inter-rater agreement was measured by the Kappa statistic. Results: Specificity and sensitivity were 95.2 and 66.7%, 76.3 and 75.2%, 84.6 and 71.6% for experts, general neurologists, and residents, respectively. Kappa values on dystonia diagnosis ranged from 0.30 to 0.46. The agreement was moderate for experts and residents (0.40-0.60) and fair for general neurologists (0.20-0.40). Kappas were the highest among experts for cranial and laryngeal dystonia (0.61-1), but not for cervical dystonia (0.37). Conclusions: The diagnosis of dystonia is difficult and only partially mirrors a physician's background. © 2014 S. Karger AG, Basel.

Comment on Ulnar neuropathy at the elbow in diabetes. [Am J Phys Med Rehabil. 2009]

Background: Pain is a common symptom of peripheral neuropathies that may severely affect patients’ Quality of Life. Pain questionnaires, based on verbal descriptors, are a useful way to investigate it. Methods: We performed a multicentre study through validated measures to characterize pain in a sample of consecutive patients affected by immune-mediated neuropathies. Results: Ninety-three patients were enrolled in 16 Italian centres. Based on the numeric rating scale, almost half of the patients complained of moderate pain and one-third of the patients severe pain. Overall, up to 50% of our patients with immune-mediated neuropathies complained of neuropathic pain. The most common neuropathic symptoms were paraesthesia/dysesthesia and superficial spontaneous pain. Surprisingly, also patients with neuropathies commonly thought to be painless (such as multifocal motor neuropathy) reported discomfort and painful symptoms. Conclusions: Pain questionnaires should be considered in the clinical evaluation of immune-mediated neuropathies, also when evaluating therapy efficacy, because they may provide clinicians with useful information on painful symptoms and patients’ quality of life.

Previous studies have reported that patients with Parkinson's disease (PD) show, in the "off medication" state, a reduced activation of tibialis anterior (TA) in the late swing-early stance phase of the gait cycle. In PD patients the pathophysiological picture may cause differences among the stride cycles. Our aims were to evaluate how frequently TA activity is reduced in the late swing-early stance phase and if there is a relationship between the TA pattern and the clinical picture. Thirty PD patients were studied 2 h after Levodopa administration ("on-med") and 12 h after Levodopa wash-out ("off-med"). They were evaluated by the Unified Parkinson's Disease Rating Scale (UPDRS III) and surface electromyography of TA and gastrocnemius medialis (GM). The root mean square (RMS) of the TA activity in late swing-early stance phase (RMS-A) was normalized as a percent of the RMS of the TA activity in late stance-early swing (RMS-B). RMS-A was reduced in 30% of patients in the "off-med" condition. Within these patients, the percentage of stride cycles with reduced RMS-A, ranged between 28% and 83%. After Levodopa intake, no stride cycle showed reduced RMS-A. Patients with reduced RMS-A had a lower UPDRS III total score in the "on-med" rather than in the "off-med" condition (p=0.02). Our data confirm and extend previous observations indicating that, in "off-med" the function of TA is impaired in those patients clinically more responsive to Levodopa. TA activation is reduced in a relatively high percent of gait cycles in the "off-med" state. Since the variability of TA activation disappears after Levodopa administration, this phenomenon could be the expression of an abnormal dopaminergic drive.

Facioscapulohumeral muscular dystrophy (FSHD) is the third most frequent inherited myopathy. We previously demonstrated that mesoangioblasts can be efficiently isolated from FSHD muscles, although their differentiation ability into skeletal muscle was variably impaired. This correlates with overall disease severity and degree of histopathologic abnormalities, since mesoangioblasts from morphologically normal muscles did not show any myogenic differentiation block. The aim of our present study was to verify whether mesoangioblasts from differentially affected FSHD muscles reproduce in vivo the same differentiation ability shown in vitro by studying their capability to form new muscle fibers during muscle regeneration of experimentally damaged muscles. We show that a diverse ability of FSHD mesoangioblasts to engraft and differentiate into skeletal muscle of SCID mice is strictly related to the characteristics of the muscle of origin, closely replicating in vivo what was previously observed in vitro. Moreover, we demonstrate that mesoangioblasts obtained from severely affected muscles scarcely integrate into muscle fibers, remaining mainly localized in the connective tissue. This suggests a defective migration in response to chemoattractants released by damaged fibers, as indicated by cell migration assays in response to HMGB1 and very low levels of RAGE expression, along with a decreased ability to fuse or to appropriately trigger the myogenic program. Our study indicates that FSHD mesoangioblasts from unaffected muscles can be used as selective treatment to halt muscle degeneration in severely affected muscles, and suggests that pharmacological and molecular interventions aimed to ameliorate homing and engraftment of transplanted autologous mesoangioblasts may open the way to cell therapy for FSHD patients, without requiring immunosuppression or genetic correction in vitro.