Carmen Erra

Intra- and internerve cross-sectional area variability: new ultrasound measures.

Introduction: Nerve involvement in immune‐related neuropathies is non‐homogeneous, and therefore characterization of ultrasound (US) abnormalities is difficult. We developed two measures to quantify US abnormalities in immune‐related neuropathies. Methods: Intranerve cross‐sectional area (CSA) variability for each nerve was calculated as: maximal CSA/minimal CSA. Internerve CSA variability for each patient was calculated as: maximal intranerve CSA variability/minimal intranerve CSA variability. Six patients underwent US evaluation of the median, ulnar, and fibular nerves, and the abnormalities were scored with our newly developed measures. Results: The new measures were applicable to all nerves and patients. The highest degree of intra‐ and internerve CSA variability was observed in multifocal motor neuropathy, consistent with the asymmetric characteristics of this neuropathy. Conclusions: The application of intra‐ and internerve CSA variability measures allows us to quantify the heterogeneity of nerves and nerve segments and identify different US patterns in diverse immune‐related neuropathies. Muscle Nerve, 2012

Carpal tunnel syndrome: clinical features, diagnosis, and management

Carpal tunnel syndrome is the most common peripheral nerve entrapment syndrome worldwide. The clinical symptoms and physical examination findings in patients with this syndrome are recognised widely and various treatments exist, including non-surgical and surgical options. Despite these advantages, there is a paucity of evidence about the best approaches for assessment of carpal tunnel syndrome and to guide treatment decisions. More objective methods for assessment, including electrodiagnostic testing and nerve imaging, provide additional information about the extent of axonal involvement and structural change, but their exact benefit to patients is unknown. Although the best means of integrating clinical, functional, and anatomical information for selecting treatment choices has not yet been identified, patients can be diagnosed quickly and respond well to treatment. The high prevalence of carpal tunnel syndrome, its effects on quality of life, and the cost that disease burden generates to health systems make it important to identify the research priorities that will be resolved in clinical trials.

Nerve ultrasound follow-up in a child with Guillain-Barré syndrome

Introduction: Nerve ultrasound has been used increasingly in neurophysiology laboratories, but data on Guillain‐Barré syndrome (GBS) are still limited, and no follow‐up studies are available. Case report: An 8‐year‐old boy was admitted with severe demyelinating GBS. Serial neurophysiological evaluations were performed initially and in follow‐up. Ultrasound studies showed diffuse and heterogeneous nerve swelling and focal enlargement of single fascicles inside the nerve. Together with clinical and electrophysiological improvement, progressive normalization of ultrasound changes was seen. Conclusions: Ultrasound demonstrated structural nerve abnormalities in GBS. These changes normalized as the patient improved clinically and electrophysiologically. Further studies are needed to elucidate the diagnostic and prognostic value of ultrasound in GBS. Muscle Nerve 46: 270–275, 2012

Sonographic diagnosis of anatomical variations associated with carpal tunnel syndrome

Bifid median nerve is an anatomic variation that occurs in about 18% of patients with symptoms suggestive of carpal tunnel syndrome and in about 15% of symptom‐free subjects. Reversed palmaris longus is a rare anatomic muscular variation. The simultaneous presence of a bifid median nerve and a reversed palmaris longus has been very rarely described, usually during surgical exploration or in cadavers. We present two cases where ultrasound showed the presence of both abnormalities, allowing a correct diagnosis and influencing the treatment plan.

Neuromyelitis optica spectrum disorder as a paraneoplastic manifestation of lung adenocarcinoma expressing aquaporin-4

Background: The observations of neuromyelitis optica spectrum disorders (NMOSD) occurring in the setting of cancer suggest that aquaporin-4 (AQP4) autoimmunity may in some cases be paraneoplastic. Results: We describe a 72-year-old patient who developed a longitudinally extensive transverse myelitis associated with AQP4 autoantibodies in the setting of a lung adenocarcinoma recurrence. AQP4 expression was demonstrated in tumor cells. IgG in patient’s cerebrospinal fluid bound to tumor cells co-localizing with AQP4 immunoreactivity. Conclusions and relevance: This case expands the spectrum of paraneoplastic AQP4 autoimmunity highlighting the importance of considering an oncological screening in patients with late-onset NMOSD.

High ultrasound variability in chronic immune-mediated neuropathies. Review of the literature and personal observations

Chronic immune-mediated neuropathies show high clinical variability. Diagnosis is based on clinical and neurophysiological studies, but recently ultrasound (US) of peripheral nerves has been shown to provide useful morphological information. US has already been shown to crucially influence diagnosis and clinical care in entrapment neuropathies, in traumatic nerve lesions and in tumors. The role of US in the evaluation of polyneuropathies is still not clearly defined, but increasing attention has recently been focused on the immune-mediated neuropathies and specific US measures (namely the intra- and inter-nerve cross-sectional area variability) have been developed. The aim of the current paper is to make a review of the available nerve US studies and provide data from personal observations in the most common chronic immune-mediated neuropathies.

Ultrasound diagnosis of bony nerve entrapment: case series and literature review.

Introduction: Nerve entrapment due to osseous callus formation is a rare complication after bone fracture. Electrodiagnostic studies and routine radiographic imaging often fail to demonstrate the pathology. The diagnosis is difficult and is often made incidentally upon surgical exploration. Nerve ultrasonography has not been used routinely to assess such lesions. Methods: We report 5 cases of nerve entrapment in osseous callus after fractures that occurred in 2011 and 2012. The diagnosis was made by ultrasound (US). We then performed a review of the relevant literature. Conclusions: US is becoming an invaluable tool for diagnosing peripheral nerve entrapments. The current cases suggest that nerve US should be strongly considered as an adjunctive diagnostic tool for nerve palsies developing after trauma. Muscle Nerve 48: 445–450, 2013

Secondary posterior interosseous nerve lesions associated with humeral fractures

Radial nerve lesions associated with humeral shaft fractures are the most common traumatic nerve lesions observed with long bone fractures. Secondary indirect posterior interosseous nerve (PIN) lesions can be associated with traumatic radial nerve palsy. The aim of this study was to identify cases of traumatic double‐site radial nerve involvement through ultrasound (US).

Pain and the Alpha‐Sleep Anomaly: A Mechanism of Sleep Disruption in Facioscapulohumeral Muscular Dystrophy

OBJECTIVE To measure the presence of the alpha-sleep anomaly in facioscapulohumeral muscular dystrophy (FSHD) and to evaluate the association between the sleep electroencephalogram (EEG) pattern and the presence of musculoskeletal pain. DESIGN Cross-sectional study. SETTING Sleep laboratory. SUBJECTS Fifty-five consecutive adult FSHD patients, 26 women and 29 men, age 49.6 ± 15.1 years (range 18-76). INTERVENTIONS Questionnaires and polysomnography. OUTCOME MEASURES Patients were asked to indicate if in the 3 months before the sleep study they presented persisting or recurring musculoskeletal pain. Patients who reported pain were asked to fill in the Italian version of the Brief Pain Inventory and the McGill Pain questionnaire, and a 101-point visual analog scale (VAS) for pain intensity. Polysomnographic recordings were performed. EEG was analyzed by means of Fast Fourier Transform. Four power spectra bands (δ 0-4 Hz, θ 4-8 Hz, α 8-14 Hz, β 14-32 Hz) were computed. Sleep macrostructure parameters and alpha/delta EEG power ratio during non rapid eye movement (NREM) sleep were compared between patients with and without pain. RESULTS Forty-two patients in our sample reported chronic pain. VAS mean score was 55.2 ± 23.8 (range 10-100), pain rating index score was 13.8 ± 10.2, and present pain intensity was 2.5 ± 0.8. The statistical analysis documented an increased occurrence of the alpha and beta rhythms during NREM sleep in FSHD patients with pain. Significant correlations were observed between the alpha/delta power ratio during NREM sleep and pain measures. CONCLUSIONS Chronic musculoskeletal pain is frequent in FSHD patients, and it represents a major mechanism of sleep disruption.

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

OBJECTIVE 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 evolution.