Neuromuscular Ultrasound Research Articles

Neuromuscular ultrasound of cranial nerves.

Ultrasound of cranial nerves is a novel subdomain of neuromuscular ultrasound (NMUS) which may provide additional value in the assessment of cranial nerves in different neuromuscular disorders. Whilst NMUS of peripheral nerves has been studied, NMUS of cranial nerves is considered in its initial stage of research, thus, there is a need to summarize the research results achieved to date. Detailed scanning protocols, which assist in mastery of the techniques, are briefly mentioned in the few reference textbooks available in the field. This review article focuses on ultrasound scanning techniques of the 4 accessible cranial nerves: optic, facial, vagus and spinal accessory nerves. The relevant literatures and potential future applications are discussed.

The Diagnostic Role of Neuromuscular Ultrasound in Chronic Inflammatory Demyelinating Polyneuropathy

The immune-neuropathies are a heterogenous group of peripheral nerve disorders. Their diagnostic classification is mainly based on the documentation of the distribution pattern of peripheral nerve impairment and the results of nerve conduction studies. Nerve conduction studies remain nowadays fundamental not only for the diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), but also for the followup and measurement of response to immune-treatment. The challenge though of acquiring the best static and dynamic image of the relevant nerve structures, led to the development of high frequency ultrasound technology. Neuromuscular ultrasound has been able to detect thickened or swollen roots, peripheral nerves or plexus, findings that are consistent with ongoing inflammation, especially in cases of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Similar findings have been described also in other immune-mediated neuropathies such as Guillain–Barre Syndrome (GBS), Multifocal Motor Neuropathy (MMN) and Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM). This review provides a timely update on the ultrasound findings of chronic inflammatory demyelinating polyneuropathy. INTRODUCTION Vascular ultrasound has gained a key role in the diagnostics of vascular lesions of the central nervous system. The recent development of high frequency ultrasonography (> 12MHz) provided the neurologist with a valuable tool to study peripheral nerve structures in detail. The first pathological ultrasound findings on peripheral nerve structures have been published in 1985 by Solbiati et al.1 On the other hand, Fornage and Rifkin reported for the first time the pathological findings of carpal tunnel syndrome.2 Immune-mediated neuropathies are a heterogenous group of disorders, with a frequency of 13% on consecutive patients with neuropathy seen at neuromuscular reference centres.3 The diagnosis and classification is based, in typical cases, on the time course, distribution pattern of nerve impairment (predominant involvement of motor/sensory fibers or/and autonomic nerve system), and paraclinical parameters [such as nerve conductions studies and serum antibodies]. On cases, with a typical clinical presentation, an extended diagnostic work-up, including cerebrospinal fluid examination, nerve conductions studies, sural nerve biopsy, laboratory testing, may be needed. The role of neuromuscular ultrasound in the diagnostic workup of CIDP remains in the literature less well defined and parallels the beginning of research on entrapment neuropathies. Only a few studies in the literature have used ultrasound to examine the pathological changes *Corresponding author: Antonios Kerasnoudis, MD Neuroimmunological Department St. Luke Hospital, Thessaloniki Private Practice: Ethnikis Antistasis 18, Serres, Greece Tel: 0030-6974994379 E-mail: antonis.kerasnoudis@gmail.com Article History: Received: March 19th, 2014 Accepted: March 31st, 2014 Published: April 8th, 2014 Citation: Kerasnoudis A, Yoon MS. The Diagnostic Role of Neuromusclar Ultrasound in Chronic Inflammatory Demyelinating Polyneuropathy. Neuro Open J. 2014; 1(1): 1-6. Copyright: © 2014 Kerasnoudis A. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Volume 1 : Issue 1 Article Ref. #: 1000NOJ1101 Review

25. A new mutation for asymmetrical inherited neuropathy: The help of ultrasound study in a paediatric diagnostic challenge

Paediatric neuromuscular diseases represent a diagnostic challenge often requiring invasive investigations hardly tolerate by children. We describe the case of a young athlet, male,13 years-old, presenting a subacute progressive right pes-cavus and lower limb’s hypometria. This clinical presentation was noted following an increase in height of child and also in correspondence of an accidental crash. Initially his deficit was setted as traumatic but further clinical development proposed again the diagnostic doubt. A neuromuscular-ultrasound(NMUS)-study showed a bilateral involvement of lower limb?s posterior loggias, mainly right, with echogenicity homogeneously increased and preserved architecture. The EMG-study showed a distal asymmetric polyneuropathy, predominant in the Tibial nerve. Similar EMG-results were found in his father. No encephalic-RMI alterations. In his parents, the immunological investigations resulted normal while the genetic ones showed a novel heterozygosis mutation of MFN2 gene. Further NMUS-studies were performed to follow up. This paucisymptomatic, monolateral, late-onset clinical case showed how a preliminary NMUS-study can guide further investigations. A prevalent chronic damage with muscular structure preserved and EMG-detection of asymmetric-neuropathy could exclude a traumatic genesis guiding diagnosis to an inherited asymmetrical neuropathy. The combined morpho-functional evaluation with integrated NMUS-EMG facilitates pediatric neuromuscular diagnosis, improving spatial resolution with limited costs and invasivness.

103. Neuro-muscular ultrasound study in pediatric healthy subjects: Normative data

Neuromuscular ultrasound (NMUS) is a non-invasive, painless and inexpensive technique that quickly screen large areas of muscles but only few data are reported in literature about pediatric physiological muscular structural changes. We collected, by means of a NMUS Health-Technology-Assessment (HTA) study (approved by Local Ethic Committee), pediatric quantitative and qualitative normative data. By NMUS we have bilaterally studied Anterior-Tibial, Long-Toe-Extensor, Rectus-Vastus-Femoris, Forearm-Flexors, Biceps muscles, considering thickness, echogenicity, pennation in 100 healthy children (M49-F51; range 2–16, mean 10,44 years; shared in five-age-groups: (I) 2–5; (II) 6–8; (III) 9–11; (IV) 12–14; (V) 15–16 ys); in about half of them sural and/or ulnar/median perimeter and area were measured. The muscles of I–II were less echogenic than III–IV–V groups. The muscles’ thickness (MT) increased with age especially between IV and V groups. In groups I–II the females’ MT was smaller than the males’s one. MT, BMI, weight were in relationship. The nerves’ area increased from I to V group. During childhood the muscular echogenicity and thickness increased rapidly, the latter especially during the puberty. Also the nerves’ area increased with age. NMUS is a useful tool to know physiological structural changes that are crucial to correctly interpretate and guide electromyographic and neuroimaging evaluation in clinically heterogeneous pediatric neuromuscular diseases.

108. Electromyography integrated with neuromuscular ultrasound evaluation in pediatric age: Description of two clinical cases

Pediatric neuromuscular diseases, clinically heterogeneous, are a diagnostic challenge, and can require invasive and expensive investigations. Two clinical cases studied with integrated electromyography (EMG) and Neuro-Muscular UltraSound (NMUS) are reported: Case 1: A 5 years-old female developing a left equinus-foot within the last year, without any trauma or infection. NMUS-examination shows selective Soleus muscle involvement with increased echogenicity and alterated architecture. The EMG-study is negative for neuropathic/myopathic disorders; the biopsy finds a Soleus’s vascular malformation. Case 2: A 12 years-old male, agonistic karateka, with pes-cavus and right lower limb’s hypometria noted in the last year following an increase in height. NMUS-study shows bilateral involvement of lower limb’s posterior loggias, mainly right, with echogenicity homogeneously increased and preserved architecture. The EMG shows a distal asymmetric polyneuropathy, predominant in the Tibial nerve. Genetic and immunological investigations are in progress. In both clinically similar cases, (paucisymptomatic, monolateral, late-onset presentation), integrated-NMUS-EMG study guided the diagnosis toward: a selective damage with muscle’s morphostructural alteration and negative EMG (case 1); a prevalent chronic damage with muscular structure preserved and EMG-detection of asymmetric neuropathy (case 2). The combined morpho-functional evaluation with integrated NMUS-EMG facilitates pediatric neuromuscular diagnosis, improving the spatial resolution with limited costs and invasiveness.

Neuromuscular Ultrasound in Carpal Tunnel Syndrome: A Tool for Cutting a Gordian Knot

Neuromuscular Ultrasound in Carpal Tunnel Syndrome: A Tool for Cutting a Gordian Knot

Neuromuscular ultrasound in polyneuropathies and motor neuron disease

Current standards for diagnosing polyneuropathies (PN) and motor neuron disease (MND) sometimes lack early sensitivity and result in delayed diagnosis and treatment. Neuromuscular ultrasound (NMUS), already established in the diagnosis of entrapment neuropathies, may offer another means of diagnosis and monitoring response to therapy. This review of current evidence discusses diffuse nerve hypertrophy in hereditary demyelinating neuropathies, multifocal nerve enlargement in acquired demyelinating PN and the lack of readily apparent structural change in axonal neuropathies. NMUS detection of fasciculations and muscular change in MND is also reviewed, along with the need for further research to better define the role of nerve imaging in patients with PN.

Neuromuscular Ultrasound for Diaphragm Assessment Following Gene Replacement Therapy in a Canine Model of X-Linked Myotubular Myopathy (XLMTM) (P05.083)

Neuromuscular Ultrasound for Diaphragm Assessment Following Gene Replacement Therapy in a Canine Model of X-Linked Myotubular Myopathy (XLMTM) (P05.083)

Neuromuscular Ultrasound for Evaluation of the Normal and Abnormal Diaphragm (P05.084)

Neuromuscular Ultrasound for Evaluation of the Normal and Abnormal Diaphragm (P05.084)

Reporting the results of diagnostic neuromuscular ultrasound: An educational report

Neuromuscular ultrasound (NMUS), an emerging diagnostic subspecialty field, has become an important extension of the electrodiagnostic examination. However, there are no formal guidelines on how to appropriately report NMUS results. The AANEM convened an expert panel to develop recommendations for reporting NMUS findings. Providers should describe the reason for referral, the nerves or muscles studied, and normal values as well as numerical values for the results of imaging. Muscle imaging reports should also include a description of how gray-scale values were calculated. NMUS-guided needle placement reports should include a description of the length and gauge of needle, the type of probe used, and an indication of how well the patient tolerated the procedure. All reports should clearly state whether the findings were normal or abnormal and include a definitive diagnosis. NMUS reports should provide comprehensive information along with a succinct conclusion, mirroring guidelines for electrodiagnostic reports.

Ultrasound Is Superior to Magnetic Resonance Imaging for Detecting Peripheral Nerve Pathology in Mononeuropathies and Brachial Plexopathies (P06.149)

Objective: Compare accuracy of magnetic resonance imaging (MRI) and ultrasound (US) in detecting peripheral nerve pathology. Background It is unknown whether MRI or US is superior for detecting peripheral nerve pathology. Design/Methods: We reviewed charts of 141 cases referred for neuromuscular US between 6/2007 and 10/2011 for suspected upper and lower extremity mononeuropathy(ies) or brachial plexopathy, excluding idiopathic carpal tunnel syndrome and ulnar neuropathy at the elbow, and identified cases who underwent MRI of the same limb for the same indication as the US. Imaging results were compared to the gold standard of surgical or, if unavailable, clinical/electrodiagnostic evaluation. Results: We identified 52 cases evaluated with both MRI and US. Focal nerve pathology was established in 46 and excluded in six by surgical (39) or clinical/electrodiagnostic evaluation (13). Diagnoses included peripheral nerve sheath tumors (18), traumatic (10) or idiopathic (8) neuropathy, fibrosis (4), compression by ganglion/synovial cysts (4) or other soft-tissue structures (3), non-nerve soft tissue tumor (3), intraneural granuloma (1) and vasculitis (1). US was more sensitive than MRI (93 vs. 70%) and equally specific (both 83%). Pathology accurately identified on US but not MRI included nerve enlargement (7), peripheral nerve sheath tumor (3), compression by anomalous muscle (1), and a hemangioma identified as non-nerve pathology on US but not MRI. Of these 12 cases with inaccurate MRI but not US, eight (67%) had lesions larger than two centimeters; only two lesions were outside the field of view of the MRI. In three cases classified as accurate for a single lesion on both MRI and US, US identified 11 additional lesions (multifocal neurofibromas, nerve enlargements, and fibrous thickening) not seen on MRI. Conclusions: Ultrasound is more sensitive, has equivalent specificity, and may better identify multifocal lesions than MRI in suspected mononeuropathies and brachial plexopathies. Supported by: This publication was made possible in part by the Washington University Neuromuscular Research Fund and the Grant Number UL1RR024992 from the NIH-National Center for Research Resources (NCRR), a component of the National Institutes of Health (NIH), and NIH Roadmap for Medical Research. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of NCRR or NIH. Disclosure: Dr. Zaidman has nothing to disclose. Dr. Seelig has nothing to disclose.

Thoracic Outlet Syndrome—Postural Type: Ultrasound Imaging of Pectoralis Minor and Brachial Plexus Abnormalities

Thoracic outlet syndrome (TOS) is a controversial diagnosis that sometimes is referred to as disputed or nonspecific neurogenic TOS because diagnostic test results typically are negative or inconclusive [1-10]. A review of the pathomechanics and associated dysfuncional body habitus suggests that use of the term “postural TOS” is more appropriate for this ommon type of presentation [10]. Postural TOS is thus recommended in cases without bjectifiable neurologic or vascular abnormality. In contrast, the less-common type of TOS nvolves compromise of the neurovascular bundle (NVB), which includes the brachial lexus (BP), the axillary or subclavian artery, and the axillary or subclavian vein [1-8,10]. Compression in all types of TOS may occur superiorly at the interscalene space, the costoclavicular space, or beneath the pectoralis minor muscle (PMM). The PMM location is recognized as a common and important site of compression in both the postural and nonpostural types of TOS [1-3,9-13]. The findings on an electrodiagnostic examination (EDX) typically are normal in patients with postural TOS because there is no axonal damage and no significant conduction block or demyelination [1,8,10]. Results of blood-flow studies usually are normal as well, and although they may reveal mild vascular compromise during stress maneuvers [1,2], this finding may not be clinically significant because it also can be seen in asymptomatic persons [1,7-9]. In addition, it is important to consider differential diagnoses and rule out the following conditions: cervical radiculopathy, peripheral nerve entrapment (median or ulnar), peripheral polyneuropathy, cervical cord lesion, or superior sulcus (Pancoast) tumor. The EDX may reveal the first 3 categories, but cervical magnetic resonance imaging (MRI) and a chest radiograph will be needed if the other conditions are considered. Neuromuscular ultrasound (NMUS) is a new imaging modality for examining nerve structures within the thoracic outlet area [14,15]. NMUS has unique applications for the postural type of TOS; as some researchers have noted, “evaluation of proximal nerves is often problematic when using electrodiagnostic techniques and may be more easily studied with ultrasound” [16]. My experience with this type of postural TOS [1,2,9,12,17,18] suggests that the PMM is contributory, and NMUS now appears to support these prior clinical impressions. Palpation of increased tension and trigger points in the PMM, combined with shoulder protraction, suggests that the PMM has shortened. The hypothesis herein is that the effects of a tight PMM can be observed to affect the NVB and explain the symptoms. The goal of this article is to present and analyze imaging findings in patients with TOS who were examined with NMUS while in neutral and stress positions, while monitoring for symptoms.

The Role of Neuromuscular Ultrasound in the Diagnostic of the Chronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune-mediated inflammatory disorder of the peripheral nervous system. The diagnosis is based in classic cases, on the distribution pattern of the neurological semiology and pathological changes of nerve conduction studies (NCS). However, in cases with subtle clinical presentation, an extended diagnostic workup may be needed (cerebrospinal fluid examination, laboratory tests, nerve biopsy). NCS remain fundamental for the diagnosis, follow-up and measurement of response to immunetreatment in CIDP. However, new challenges arose on how best to acquire a static and dynamic imaging of the peripheral nerves, with the aim of providing a holistic approach to the nerve impairment. According to the literature, neuromuscular ultrasound is able to detect in cases of CIDP thickened or swollen roots, peripheral nerves or brachial plexus, findings that are consistent with ongoing inflammation. This review provides a timely update on the nerve ultrasound findings of CIDP and future possibilities of neuromuscular ultrasound are also discussed.

Ultrasound imaging of the carpal tunnel during median nerve compression

Median nerve (MN) compression is a recognized component of carpal tunnel syndrome (CTS). In order to document compressive changes in the MN during hand activity, the carpal tunnel was imaged with neuromuscular ultrasound (NMUS). Ten patients with CTS and five normal controls underwent NMUS of the MN at rest and during dynamic stress testing (DST). DST maneuvers involve sustained isometric flexion of the distal phalanges of the first three digits. During DST in the CTS patients, NMUS demonstrated MN compression between the contracting thenar muscles ventrally and the taut flexor tendons dorsally. The mean MN diameter decreased nearly 40%, with focal narrowing in the mid-distal carpal canal. Normal controls demonstrated no MN compression and a tendency towards MN enlargement, with an average diameter increase of 17%. Observing the pathologic mechanism of MN injury during common prehensile hand movements could help better understand how to treat and prevent CTS.