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Post-herpetic lumbar plexopathy (post-herpetic segmental paresis), immune-mediated lumbar plexopathy and diabetic radiculoplexopathy (diabetic amyotrophy) have similar and seemingly parallel semiologies. The latter two conditions have an underlying microvasculitic pathological substrate that has shown potential (yet unproven) amelioration with immunodulatory therapy. These observations gave us the motivation to treat our patient with intravenous methylprednisolone for a profound proximal left leg weakness due to post-herpetic segmental paresis. In this case report, we outline in detail the clinical and electophysiolgical phenotype of this disease and we demonstrate the spectacular improvement of left leg power that had previously remained static for three months. In the discussion section, we review the vascular invasiveness of the varicella-zoster virus.

Prone positioning improves oxygenation in adult respiratory distress syndrome. This procedure has been widely used during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic. However, this procedure can also be responsible for nerve damage and plexopathy.We retrospectively reviewed a series of 7 infectious patients with coronavirus disease 2019 who underwent prone positioning ventilation at the San Raffaele Hospital of Milan, Italy, during the SARS-CoV-2 pandemic.Clinical and neurophysiological data of 7 patients with nerve compression injuries have been reported.Health care workers should take into consideration the risk factors for prone positioning-related plexopathy and nerve damage, especially in patients with coronavirus disease 2019, to prevent this type of complication.

Focal weakness of the limbs can be organized by affected body region. Shoulder weakness is most commonly due to C5–6 radiculopathy or brachial plexopathy. Scapular winging is usually secondary to long thoracic or accessory neuropathy. Hand weakness is most commonly the result of ulnar neuropathy, C8–T1 radiculopathy, motor neuron disease, or cerebral processes. Wrist drop is a special kind of hand weakness. The two most common sources of wrist drop are radial neuropathy and stroke, and can be differentiated from each other with focused examination. Proximal leg weakness is usually the result of upper lumbar radiculopathy or lumbar plexopathy. Postpartum leg weakness has multiple potential causes that can be distinguished by careful examination. Foot drop may be due to lesions of the peroneal nerve, sciatic nerve, L5 nerve root, or central nervous system. Multifocal weakness is due to multifocal central or peripheral nervous system disease. Relevant peripheral nervous system causes of multifocal weakness include mononeuropathy multiplex, multifocal motor neuropathy with conduction block, and hereditary neuropathy with liability to pressure palsies.

We present the case of a 77-year-old man who developed shingles over the cervical C8 dermatome followed by post-herpetic medial cord brachial plexopathy, with hand weakness and difficulty performing the pinch " O " sign. This is the very first case, to our knowledge, of a detailed presentation of a medial cord plexopathy following shingles. We review the literature of post-herpetic brachial plexopathies and discuss the magnetic resonance imaging (MRI) findings of the brachial plexus in this group of patients. We also speculate on the intriguing finding that despite frequent abnormalities on MRI such as T2 signal hyperintensity and nerve hypertrophy, contrast enhancement of nerves is exceedingly rare. Furthermore, we adumbrate on the localization of brachial plexus lesions by proposing a user-friendly diagram and table, which simplifies the diagnostic algorithm.

Vertebral arteriovenous fistulas (VAVF) are uncommon, high-flow communications between a vertebral artery and surrounding venous plexus that occur spontaneously or secondary to trauma. A 57-year-old female presented with a multi-day history of rapidly progressive numbness and weakness in the left C5-C6 dermomyotomes. Her physical exam findings and subsequent electrophysiological testing were suggestive of a brachial radiculo-plexopathy. Noninvasive imaging demonstrated venous congestion with multilevel compromise of the left-sided cervical foramina, and subsequent vertebral angiography confirmed a VAVF, which was treated with trapping of the involved VA segment. Her numbness and weakness progressively improved with concurrent involution of the dilated veins. An exceptional case of VAVF manifesting as a brachial radiculo-plexopathy is presented. VAVF are rare, though they may be considered as a potential underlying cause in patients with comparable symptoms. Endovascular embolization has been demonstrated as a safe and efficacious method in treating VAVFs, though multiple patient-specific factors must be contemplated. Disclosures A. Larson: None. L. Rinaldo: None. C. Arnold Fiebelkorn: None. N. Young: None. G. Lanzino: None.

In the Chap. 5, we describe other neurological conditions without research on postherpetic neuralgia, CRPS, diabetic and other neuropathies, and phantom limb pain, or the unique issues associated with them. Hence, Chaps. 1– 4, written by Antoun Nader, Taruna Penmetcha, Michiaki Yamakage, and Wolfgang Hamann are described in detail. Depending on the site to be impaired, neuropathic pain can be divided into peripheral neuropathic pain or central neuropathic pain [1] and cancer-associated neuropathic pain. The four diseases mentioned in Chaps. 1– 4 are classified as peripheral neuropathic pain: carpal tunnel syndrome, meralgia paresthesia, trigeminal neuralgia, and post-thoracotomy pain and so on are residual diseases of peripheral neuropathic pain to be described in this chapter. Central neuropathic pain includes central post-stroke pain, multiple sclerosis pain, Parkinson’s disease pain, spinal cord injury pain, and syringomyelia. Cancer-associated neuropathic pain contains chemotherapy-induced polyneuropathy, neuropathy secondary to tumor infiltration or nerve compression, phantom breast pain, postmastectomy pain, and post-radiation plexopathy and myelopathy. Classifications of “definite,” “possible,” and “unlikely” have been proposed when neuropathic pain is clinically diagnosed [2].

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant inherited disorder commonly presenting with acute-onset, non-painful focal sensory and motor mono neuropathy. In 80% of cases, the genetic defect is a 1.5 Mb deletion on chromosome 17p11.2, including PMP22. Only few cases of partial deletion and point mutations in PMP22 are involved in HNPP. We investigated a 62-years-old man with lower limb plexopathy first considered as Garland’s syndrome. A month later, his 29 years old son also consulted for paresthesia on the peroneal nerve. Targeted sequencing of the PMP22 gene identified a c.370delT (p.Trp124Glyfs*31) in both affected patients. We report a new PMP22 point mutation associated with an atypical clinical phenotype of HNPP, a painful plexopathy of the lower limb worsenen by diabetes and a mere paresthesia, but a typical ENMG. This study illustrates the large spectrum of the disease, and emphasizes the importance of a complete ENMG and family history.

Neuropathic cancer pain (NCP) is caused by nerve damage attributable to the cancer per se, and/or treatments including chemotherapy, radiotherapy, and surgery; the prevalence is reported to be as high as 40%. The etiologies of NCP include direct nerve invasion or nerve compression by the cancer, neural toxicity, chemotherapy, and radiotherapy. NCP is subdivided into plexopathy, radiculopathy, and peripheral neuropathies, among several other categories. The clinical characteristics of NCP differ from those of nociceptive pain in terms of both the hypersensitivity symptoms (burning, tingling, and an electrical sensation) and the hyposensitivity symptoms (numbness and muscle weakness). Recovery requires several months to years, even after recovery from injury. Management is complex; NCP does not usually respond to opioids, although treatments may feature both opioids and adjuvant drugs including antidepressants, anticonvulsants, and anti-arrhythmic agents, all of which improve the quality-of-life. This review addresses the pathophysiology, clinical characteristics and management of NCP, and factors rendering pain control difficult.

Background Segmental zoster paresis (SZP) of limbs, characterized by focal weakness of extremity, is recognized as a rare complication of herpes zoster (HZ). The following study analyzes the clinical characteristics and data from electromyography and MRI scans in patients with motor weakness after zoster infection. Methods One thousand three hundred ninety-three patients from our database (Shandong Provincial Qianfoshan Hospital) suffering from HZ were retrospectively reviewed from June 2015 to July 2017. Patients who fulfilled the diagnostic criteria for SZP were included in the analysis. The clinical characteristics, as well as electromyography findings and MRI scans were analyzed. Results SZP was present in 0.57% of patients with HZ (8/1393). The average age of symptom onset in 8 SZP patients was 69 years old (SD: 13, range 47–87). The severity of muscle weakness ranged from mild to severe. The electrophysiological testing revealed the characteristics of axonopathy. Radiculopathy (2/8), plexopathy (2/8), radiculoplexopathy (3/8) and combined radiculopathy and mononeuropathy (1/8) were also identified. MRI revealed hyperintensity of the affected spinal dorsal horns, nerve roots or peripheral nerves. Conclusions SZP is associated with obvious limb weakness, nerve axons lesions and localization to nerve roots, plexus or peripheral nerves.

The aim of this study was to evaluate whether high-resolution brachial plexus (BP) magnetic resonance neurography (MRN) is capable of (1) distinguishing patients with compressive neuropathy or noncompressive plexopathy from age- and sex-matched controls, (2) discriminating between patients with compressive neuropathy and noncompressive plexopathy, and (3) detecting spatial lesion patterns suggesting somatotopic organization of the BP.Thirty-six patients (50.9 ± 12.7 years) with clinical symptoms, nerve conduction studies, and needle electromyography findings suggestive of brachial plexopathy and 36 control subjects matched for age and sex (50.8 ± 12.6 years) underwent high-resolution MRN of the BP. Lesion determination and localization was performed by 2 blinded neuroradiologists at the anatomical levels of the plexus trunks and cords.By applying defined criteria of structural plexus lesions on high-resolution MRN, all patients were correctly rated as affected, whereas 34 of 36 controls were correctly rated as unaffected by independent and blinded reading from 2 neuroradiologists with overall good to excellent interrater reliability. In all cases, plexopathies with a compressive etiology (n = 12) were correctly distinguished from noncompressive plexopathies with inflammatory origin (n = 24). Pathoanatomical contiguity of lesion from trunk into cord level allowed recognition of distinct somatotopical patterns of fascicular involvement, which correlated closely with the spatial distribution of clinical symptoms and electrophysiological data.Brachial plexus MRN is highly accurate for differentiating patients with symptomatic plexopathy from healthy controls and for distinguishing patients with compressive neuropathy and noncompressive plexopathy. Furthermore, BP MRN revealed evidence for somatotopic organization of the BP. Therefore, as an addition to functional information of electrodiagnostic studies, anatomical information gained by BP MRN may help to improve the efficiency and accuracy of patient care.

Denervation of muscle usually leads to muscle atrophy with fatty replacement but, uncommonly, also results in muscle hypertrophy or pseudohypertrophy with fatty replacement. We report the ultrasonographic and magnetic resonance imaging (MRI) findings of a patient with diffuse fatty infiltration of calf muscles as a result of denervation pseudohypertrophy. The elevated fasting glucose, neurogenic electromyographic changes, and muscle atrophy with adipose tissue infiltration are consistent with diabetic neuropathy as the cause of denervation pseudohypertrophy. Lumbosacral radiculopathy and plexopathy were excluded by MRI. The imaging features reported in the literature are reviewed. The important differential diagnosis of infiltrating lipoma and denervation hypertrophy, as well as other causes of monomelic hypertrophy or swelling, is discussed. This case report demonstrates the importance of MRI, with clinical, biochemical, electrophysiological, and histologic correlation in the diagnosis of denervation pseudohypertrophy. Correct diagnosis of denervation pseudohypertrophy has an important role in guiding further investigations and treatment of the disease and the underlying cause.

The objective of this study is to analyze clinical manifestations, features of imaging, and laboratory assessment of patients with neuropsychiatric SLE (NPSLE) for better diagnosis and outcome prediction. One hundred eighteen NPSLE patients admitted to the Anhui Provincial Hospital in Hefei, China, between January 2006 and December 2016 were enrolled and analyzed retrospectively. All patients fulfilled the American College of Rheumatology revised classification criteria for SLE. Patients with NPSLE fulfilled the American College of Rheumatology (ACR) nomenclature and case definitions. All NPSLE patients underwent neurological investigations including MRI of nervous system, electroencephalograms, or CSF examination as part of the diagnostic evaluation of nervous system involvement. All statistical analyses were performed. According to different types of data, different statistical methods were used to determine factors associated with abnormal MRI among NPSLE patients. Statistical significance was defined as P value

Neuralgia and neuritis (plexitis) are lesions of the peripheral nerves caused by hypothermia, infection, trauma, intoxication, and metabolic disorders. Neuralgia is characterized by pain arising along the course of a nerve or its branches. Neuritis is accompanied by sensitivity disorders, trophic disorders, paresis and paralysis, as well as those that perceive the nerve impulse (sensitivity) and motor functions. All this is associated with varying degrees of degeneration of nerve fibers until their breakdown.

High-resolution manometry (HRM) and new analysis algorithms, summarized in the Chicago Classification, have led to a restructured classification of oesophageal motility disorders. This advance has led to increased detection of clinically relevant disorders, in particular achalasia. It has become apparent that the cardinal feature of achalasia — impaired lower oesophageal sphincter (LES) relaxation — can occur in several disease phenotypes: without peristalsis (type I), with pan-oesophageal pressurization (type II), with premature (spastic) distal oesophageal contractions (type III), or with preserved peristalsis (outlet obstruction). Furthermore, no manometric pattern is perfectly sensitive or specific for achalasia caused by a myenteric plexopathy, and there is no biomarker for this pathology. Consequently, physiological testing reveals other syndromes not meeting achalasia criteria that also benefit from therapies formerly reserved for achalasia. These findings have become particularly relevant with the development of a minimally invasive technique for performing a long oesophageal myotomy, the per-oral endoscopic myotomy (POEM). Optimal management is to render treatment in a phenotype-specific manner; that is, POEM calibrated to patient-specific physiology for spastic achalasia and the spastic disorders, and more conservative strategies such as pneumatic dilation for the disorders limited to the LES. This Consensus Statement examines the effect of HRM on our understanding of oesophageal motility disorders, with a focus on the diagnosis, epidemiology and management of achalasia and achalasia-like syndromes.

Clavicular fractures are common injuries that traditionally are managed nonsurgically without clinically significant sequelae. However, they may develop hypertrophic callus formation that compresses the brachial plexus. These cases may present months to years after initial injury with varying degrees of pain, paresthesia, and weakness on the affected side and usually are treated by surgical resection of the hypertrophic callus. We present a case of brachial plexopathy due to hypertrophic clavicular callus causing weakness and paresthesia. The plexopathy was confirmed with imaging and electrodiagnostic studies. This case was unusual in that resolution of symptoms was achieved nonsurgically. Level of Evidence V

MRI without and with contrast is the most accurate imaging method to determine whether a process is intrinsic or extrinsic to a nerve of the brachial or lumbosacral plexus. However, there are no Current Procedural Terminology codes to correspond to imaging studies of the brachial or lumbar plexus discretely. This assessment uses "MRI of the brachial plexus" or "MRI of the lumbosacral plexus" as independent entities given that imaging acquisition for the respective plexus differs in sequences and planes compared with those of a routine neck, chest, spine, or pelvic MRI, yet acknowledges the potential variability of ordering practices across institutions. In patients unable to undergo MRI, CT offers the next highest level of anatomic evaluation. In oncologic patients, PET/CT imaging can identify the extent of tumor involvement and be beneficial to differentiate radiation plexitis from tumor recurrence but provides limited resolution of the plexus itself. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Herpes zoster is a reactivation of the latent varicella zoster virus. Among the complications of herpes zoster is zoster-associated limb paresis. The clinical and imaging features of patients with zoster-associated limb paresis due to plexopathies (zoster-associated plexopathy) have had limited description in the literature. The Mayo Clinic patient data base was searched by diagnostic code for patients diagnosed with herpes zoster between January 1, 1996, and September 30, 2012. Patients who met the inclusion criteria for zoster-associated limb paresis or herpes zoster with MRIs obtained were reviewed. Ten patients with zoster-associated plexopathy were identified. Imaging abnormalities were found in 70% of patients. Secondary denervation changes in shoulder girdle muscles and nerve T2 signal hyperintensity were the most frequent abnormalities (50%), followed by nerve enlargement (20%). Enhancement was not evident in any cases despite early imaging in 80% of the cohort. These results demonstrate the clinical utility of MR imaging in confirming the diagnosis of zoster-associated plexopathy.

This case report describes a 29-year-old male who presented with immediate onset of Horner's syndrome and ipsilateral brachial plexopathy after sleeping with his arm dangling outside a car window for 8 h. Outside workup and imaging revealed rhabdomyolysis of the left neck musculature. Subsequent electrodiagnostic testing and high-resolution brachial plexus magnetic resonance imaging at the authors' institution attributed the Horner's syndrome and concurrent brachial plexopathy to rhabdomyolysis of the longus colli and scalene musculature, which had compressed-and consequently scar tethered-the cervical sympathetic trunk and brachial plexus. This case of co-existent Horner's syndrome and brachial plexopathy demonstrates the role of high-resolution brachial plexus MRI in diagnosing plexopathy and the importance of being familiar with plexus and paravertebral muscle anatomy.

The authors report the clinical case of a 7-year-old child with gradual development in left leg weakness considering by the neurologist as the unknown lesion of the sciatic nerve. According to the results of clinical and instrumental examinations, the diagnosis was established as a focal form of chronic inflammatory demyelinating polyneuropathy (CIDP). The dysimmune cause of the disease was confirmed by MRI of the lumbosacral plexus with contrast enhancement of neural structures and response to therapy with high-dose intravenous immunoglobulin in combination with corticosteroids.Представлен клинический случай у ребенка 7 лет с постепенным развитием слабости в левой ноге, которая была расценена неврологом как поражение седалищного нерва неуточненного генеза. По результатам проведенного клинико-инструментального обследования верифицирован диагноз: фокальная форма хронической демиелинизирующей полинейропатии. Дизиммунный характер болезни подтвержден данными МРТ пояснично-крестцового сплетения с контрастным усилением невральных структур и ответом на терапию высокодозными внутривенными иммуноглобулинами в комбинации с кортикостероидами.

Varicella zoster reactivation (“shingles” or “herpes zoster”) usually presents as a self-limiting, unilateral, dermatomal vesicular rash in older adults. We present the case of a 73 year-old woman with unilateral brachial plexopathy, an unusual but debilitating complication of shingles. Despite treatment with intravenous acyclovir and immunoglobulin she had a marked residual motor paresis that required an upper limb rehabilitation program after discharge.

Interpreting imaging studies of peripheral nerve diseases and especially the brachial plexus (BP) and lumbosacral plexus (LSP) can be challenging. It requires a firm knowledge of the anatomy. It is just as important to be familiar with the end-organ innervations and the surrounding structures, the latter serving as important landmarks.

Peripheral nervous system complications such as acute demyelinating polyradiculopathy and mononeuropathy may rarely develop after substance use. A 27-year-old man used illegal drugs the day before his admission to the emergency service. Initially, he was suspected for rhabdomyolysis, due to elevated blood urea nitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, myoglobin, and creatine kinase levels. On Day 4, generalized edema and flask paralysis were noted in both upper limbs. The patient was diagnosed with bilateral brachial pan-plexopathy based on electrophysiological study results. He underwent a rehabilitation program. After eight months, repeated electrophysiological study revealed a significant improvement in all bilateral upper limb muscles, except for the right abductor pollicis brevis and abductor digiti minimi muscles. The underlying cause of bilateral brachial pan-plexopathy was rhabdomyolysis secondary to substance use. In conclusion, substance use in patients with non-traumatic plexopathy should always be questioned.

Background Varicella zoster virus is a Deoxyribonucleic acid (DNA) virus exclusively affecting humans. Reactivation of varicella zoster virus causes herpes zoster with vesicular eruptions in a restricted dermatomal distribution. Peripheral motor neuropathy is a very rare complication of varicella zoster virus. Case presentation A 57-year-old previously well Sri Lankan female presented with acute onset painful weakness of the left upper limb with a preceding history of a febrile illness. Subsequently she developed vesicular eruptions in the dermatomal distribution of cervical 5, 6, and 7. Electromyography was suggestive of acute denervation of cervical 5, 6, and 7 myotomes. Diagnosis of zoster-associated brachial plexopathy was made, and the patient was treated with acyclovir, steroids, and analgesics. She made a good recovery. Conclusion Brachial plexus neuritis due to varicella zoster infection should be considered in an acute monoparesis of a limb as it is a treatable and reversible condition

Objectives Peripheral neuropathic pain is a common and disabling problem after traumatic brachial plexus injury with global plexopathy secondary to C5 rupture and avulsion of C6, C7, C8, and T1. The purpose of this paper is to evaluate restoration of sensation and pain levels before and after targeted muscle reinnervation to the hand using contralateral C7 nerve transfers in patients who have traumatic brachial plexus injuries with C5 rupture and avulsion of C6, C7, C8, and T1. Patients and Methods This paper presents a retrospective review of the outcomes of the surgical management of 11 patients with global plexopathy with C5 rupture and avulsion from C6 to T1 roots. Ten of the patients had peripheral neuropathic pain. Mean follow-up was 6.9 years. Results Sensory recovery to the median nerve territory was returned in 10 out of 11 patients. Pain and quality of life were improved in the majority. Conclusion Contralateral C7 nerve transfers may restore sensation to the median nerve territory with concurrent improvement in pain and some function in a limited number of patients for whom no other treatment options exist.

Summary Revascularisation of the brachial plexus is controversial. Traditional techniques use free tissue transfer of omentum, groin fat or muscle, on the principle of supplying rich quantities of vascularised tissue to wrap around the nerves permitting neural gliding and revascularising the scarred nerves. However, the complexity of an additional microsurgical procedure coupled with uncertainty of their effectiveness have curtailed their clinical application. We describe a local pedicled transposition flap that is a novel modification of the deltopectoral (DP) flap using only the subcutaneous adipofascial tissue vascularised by the medial pectoral perforators. This flap is harvested through the supraclavicular brachial plexus access incision. This avoids free tissue transfer and additional donor scarring, yet provides adequate volumes of well-vascularised tissue for mechanical protection and revascularisation of the plexus. We reviewed sixteen consecutive patients who underwent a pedicled adipofascial DP flap to protect the brachial plexus over the 20-year study period. Inclusion criteria were patients with recurrent thoracic outlet compression and patients with radiation plexitis. At latest follow-up (average 3.6 years), 75% of patients reported the improvement or resolution of symptoms. The majority of patients reported improved pain scores (82%) with an average pain visual analogue scale (VAS) score of 5.1. Patients were very satisfied with scar outcomes, reporting low Vancouver Scar Scale Scores and low scar VAS scores. Post-operative MR imaging, available in 31% of the cohort, demonstrates the maintenance of flap position and vascularity at an average of 2.1 years. This novel and simple technique is recommended in aiding revascularisation and cover of the brachial plexus in recurrent and recalcitrant plexopathy.