The neurophysiological lesson from the Italian CIDP database

Introduction Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database. Methods We calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP. Results The ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively. Conclusion Our findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP

Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

OBJECTIVE: to identify the clinical and diagnostic investigations that may help supporting a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the EFNS/PNS electrodiagnostic criteria. METHODS: we retrospectively reviewed the data from patients with a clinical diagnosis of CIDP included in a national database. RESULTS: we included 535 patients with a diagnosis of CIDP. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in 3, while two had CISP). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients raising to 54 (80.6%) if we also included a history of a relapsing course as a possible supportive criteria. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% versus 85.9%) CONCLUSIONS: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

OBJECTIVES: A few variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been described, but their frequency and evolution to typical CIDP remain unclear. To determine the frequency and characteristics of the CIDP variants, their possible evolution to typical CIDP, and treatment response. METHODS: We applied a set of diagnostic criteria to 460 patients included in a database of Italian patients with CIDP. Clinical characteristics and treatment response were reviewed for each patient. The Kaplan-Meier curve was used to estimate the progression rate from atypical to typical CIDP. RESULTS: At the time of inclusion, 376 (82%) patients had a diagnosis of typical CIDP while 84 (18%) had atypical CIDP, including 34 (7%) with distal acquired demyelinating symmetric neuropathy (DADS), 17 (4%) with purely motor, 17 (4%) with Lewis-Sumner syndrome (LSS) and 16 (3.5%) with purely sensory CIDP. Based on retrospective review of the symptoms and signs present at onset and for at least 1 year, 180 (39%) patients had an initial diagnosis compatible with atypical CIDP that in 96 (53%) patients evolved to typical CIDP. Mean disease duration was longer in patients evolving to typical CIDP than in those not evolving (p=0.0016). Patients with DADS and LSS had a less frequent response to immunoglobulin than those with typical CIDP, while patients with purely motor and sensory CIDP had a similar treatment response. CONCLUSIONS: The proportion of patients with atypical CIDP varies during the disease course. DADS and LSS have a less frequent response to intravenous immunoglobulin compared with typical CIDP, raising the possibility of a different underlying pathogenetic mechanism

Tarsal tunnel syndrome: still more opinions than evidence. Status of the art.

Tarsal tunnel syndrome is an entrapment neuropathy of the posterior tibial nerve or its terminal branches within its fibro-osseous tunnel beneath the flexor retinaculum on the medial side of the ankle. The condition is frequently underdiagnosed leading to controversies regarding its epidemiology and to an intense debate in the literature. With the advent of nerve imaging techniques, the diagnostic confirmation and the etiological identification have become more accurate. However, management of this entrapment neuropathy remains challenging because of many intervention strategies but limited robust evidence. Uncertainties still exist about the best conservative treatment, timing of surgical intervention, and best surgical approach. In the attempt to clarify these aspects and to provide the reader some understanding of the status of the art, we have reviewed the published literature on this controversial condition

Ulnar neuropathy at wrist: entrapment at a very "congested" site

Ulnar tunnel syndrome indicates ulnar neuropathy at different sites within the wrist. Several classifications of ulnar tunnel syndrome are present in literature, based upon typical nerve anatomy. However, anatomical variations are not uncommon and can complicate assessment. The etiology is also complex, due to the numerous potential causes of entrapment. Clinical examination, neurophysiological testing, and imaging are all used to support the diagnosis. At present, many therapeutic approaches are available, ranging from observation to surgical management. Although ulnar neuropathy at the wrist has undergone extensive prior study, unresolved questions on diagnosis and treatment remain. In the current paper, we review relevant literature and present the current knowledge on ulnar tunnel syndrome

Post-traumatic neuroma due to closed nerve injury. Is recovery after peripheral nerve trauma related to ultrasonographic neuroma size?

OBJETVICE: traumatic neuroma is a pathological condition of peripheral nervous system consisting of localized proliferation of injured nerve elements. The symptoms depend on the type of involved nerve (motor and/or sensitive) and on the site and the extension of the lesion. Ultrasound is the best tool to depict the morphology of nerve, especially in traumatic conditions. We present a study aimed to assess the correlation between the degree of nerve function and the ultrasound morphology of neuromas. PATIENTS AND METHODS: we retrospectively evaluated 18 patients with neuromas (not transected) occurred after a closed nerve trauma evaluated with clinical and ultrasound assessment. The clinical evaluation was related to the % of increase of cross sectional area as detected by nerve ultrasound respect to normal nerve. RESULTS: we observed that dimensions of neuromas are not related to function until neuroma have cross sectional area 5 times enlarged respect to normal nerve, in this case recovery never occurs. CONCLUSION: our study failed to clear detect a relation between cross sectional area enlargement of neuroma and nerve function, but showed a cut off beyond which prognosis is negative. This result provide some useful information for prognosis, nevertheless we believe that future perspective studies are needed to better understand the timing of developing neuromas and its evolutio

Thoracic outlet syndrome: wide literature for few cases. Status of the art

Despite its low prevalence and incidence, considerable debate exists in the literature on thoracic outlet syndrome (TOS). From literature analysis on nerve entrapments, we realized that TOS is the second most commonly published entrapment syndrome in the literature (after carpal tunnel syndrome) and that it is even more reported than ulnar neuropathy at elbow, which, instead, is very frequent. Despite the large amount of articles, there is still controversy regarding its classification, clinical picture, diagnostic objective findings, diagnostic modalities, therapeutical strategies and outcomes. While some experts believe that TOS is underrated, overlooked and very frequent, others even doubt its existence as a nosological entity. In the attempt to shed more light on this condition, we performed a systematic review of the literature and report evidence and opinions around this controversial subject. Only articles focused on neurogenic TOS were considered. Understanding the status of the art and the underlying reasons of doubts and weaknesses could help clinical practice and set the stage for future research