[Neuralgic amyotrophy: an inflammatory neuropathy and its surgical treatment]. / Neuralgische Amyotrophie ­ eine entzündliche Neuropathie und ihre operative Behandlung.

BACKGROUND: Neuralgic amyotrophy (NA) is a monofocal or oligofocal inflammatory neuropathy whose incidence has been significantly underestimated. A connection between constrictions and torsions of peripheral nerves with this disease has been increasingly established in recent years. Modern imaging techniques such as high-resolution nerve ultrasound and MR neurography have contributed to a better understanding of the pathophysiology and a better assessment of the prognosis of the disease. This has led to the concept of treating patients with such focal changes surgically in order to improve the prognosis. This review presents current ideas on the pathophysiology, clinical presentation, diagnosis and treatment of the disease. PATIENTS AND METHODS: In a retrospective study, pre-, intra- and postoperative findings of 22 patients with 23 constrictions/torsions of peripheral nerves of the upper extremity were analysed. The patients underwent surgery at a nerve surgery centre over a period of 3.5 years (Dec. 2019-May 2023). The median nerve was most frequently affected (N=9), followed by the suprascapular nerve (N=6) and radial nerve (N=4). The axillary nerve (N=3) and the accessory nerve (N=1) were also involved. Surgical exploration revealed nerve torsions (N=9), nerve constrictions (N=5), fascicular torsions (N=12) and fascicular constrictions (N=9). Depending on the intraoperative findings, epineuriotomies (N=1), epi- and perineuriotomies (N=33), end-to-end sutures (N=2), and one epi- and one perineural suture were performed. RESULTS: After an average follow-up of 10 months (3-28 months), 17 patients were re-examined. All of them reported a clear subjective improvement in motor deficits. Clinically and electromyographically, a reinnervation and significant increase in strength from a pre-existing strength grade of M0 to at least M3 in the vast majority of affected muscles was demonstrated in these patients. SUMMARY: The incidence of NA continues to be underestimated and, in a significant proportion of patients, leads to permanent motor deficits, most likely due to constrictions and torsions of affected nerves. Surgical treatment is recommended as early as possible. Very good results can usually be achieved with epi- and perineuriotomy. In rare cases, end-to-end neurorrhaphy or nerve grafting is required.

Effects of indirect foraminal decompression during anterior cervical disc fusion procedure: preliminary results of a prospective study with clinical and radiological outcomes.

INTRODUCTION: The respective effects of direct and indirect decompression in the clinical outcome after anterior cervical disc fusion (ACDF) is still debated. The main purpose of this study was to analyze the effects of indirect decompression on foraminal volumes during ACDF performed in patients suffering from cervico-brachial neuralgias due to degenerative foraminal stenosis, i.e. to determine whether implant height was associated with increased postoperative foraminal height and volume. METHODS: A prospective follow-up of patients who underwent ACDF for cervicobrachial neuralgias due to degenerative foraminal stenosis was conducted. Patient had performed a CT-scan pre and post-operatively. Disc height, foraminal heights and foraminal volumes were measured pre and post operatively. RESULTS: 37 cervical disc fusions were successfully performed in 20 patients, with a total of 148 foramina studied. Foraminal height and volume were measured bilaterally on the pre- and post-operative CT scans (148 foramina studied). After univariate analysis, it was found a significant improvement for every radiological parameter, with a significant increase in disc height, foraminal height and foraminal volume being respectively +3,22 mm (p < 0,001), +2,12 mm (p < 0,001) and +54 mm3 (p < 0,001). Increase in disc height was significantly associated with increase in foraminal height (p < 0,001) and foraminal volume (p < 0,001). At the same time, increase in foraminal height was significantly correlated with foraminal volume (p < 0,001), and seems to be the major component affecting increasing in foraminal volume. CONCLUSION: Indirect decompression plays an important part in the postoperative foraminal volume increase after ACDF performed for cervicobrachial neuralgias.

Persistent Hourglass Constriction in Recovered Anterior Interosseous Nerve Palsy Due to Neuralgic Amyotrophy: A Case Report.

The current articles recommended the interfascicular neurolysis for anterior interosseous nerve (AIN) palsy with hourglass-like fascicular constrictions (FCs) detected by ultrasonography or surgical exploration to realign to the fascicular torsion for those who failed to recover spontaneously. We present the case report of spontaneous AIN palsy recovered after conservative treatment; however, ultrasonographic findings showed persistent FCs of AIN in the arm at the beginning, at 6 weeks, and subsequent 3-year follow-ups, even after complete clinical recovery of palsy. This finding calls into question the current notion that AIN paralysis is due to FCs and that neurolysis is the best surgical treatment when spontaneous recovery does not occur for a considerable observation period. Level of Evidence: Level V (Therapeutic).

Clinical Outcomes of Upper Extremity Nerve Transfers in Neuralgic Amyotrophy.

SUMMARY: Neuralgic amyotrophy (NA) is a disease affecting peripheral nerves. Treatment has historically been conservative, as the natural course of the disease was thought to be self-limiting. Recent work has demonstrated that as many as two-thirds of people with NA have persistent pain, fatigue, or weakness. At the authors' center, supercharged end-to-side (SETS) nerve transfers are commonly performed in patients with NA to optimize motor recovery while allowing for native axonal regrowth. The authors describe the technique and clinical outcomes of patients with NA affecting the anterior interosseous nerve (AIN) who were treated with SETS nerve transfer from extensor carpi radialis brevis to AIN. Ten patients (90% male; mean age, 51.3 ± 9.7 years) underwent extensor carpi radialis brevis-to-AIN transfer at a mean period of 6.4 ± 1.4 months after onset of symptoms. Mean postoperative follow-up duration was 14.8 ± 3.2 months. Before surgery, all patients demonstrated clinically significant weakness in the flexor pollicis longus (FPL), flexor digitorum profundus muscle to the index finger (FDP2), or both. FPL strength improved from a median Medical Research Council (MRC) grade of 1.5 to 4 ( P = 0.011) and FDP2 strength improved from a median MRC grade of 1 to 5 ( P = 0.016). A postoperative MRC grade of 4 or greater was achieved in nine of 10 (90%) FPL and 10 of 10 (100%) FDP muscles. This is the first report of SETS nerve transfer for the treatment of NA. The outcomes of this work suggest that SETS nerve transfers may be an option to optimize motor outcomes in patients with NA. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

A standardized ultrasound approach in neuralgic amyotrophy.

Neuralgic amyotrophy (NA), also referred to as idiopathic brachial plexitis and Parsonage-Turner syndrome, is a peripheral nerve disorder characterized by acute severe shoulder pain followed by progressive upper limb weakness and muscle atrophy. While NA is incompletely understood and often difficult to diagnose, early recognition may prevent unnecessary tests and interventions and, in some situations, allow for prompt treatment, which can potentially minimize adverse long-term sequalae. High-resolution ultrasound (HRUS) has become a valuable tool in the diagnosis and evaluation of NA. Pathologic HRUS findings can be grouped into four categories: nerve swelling, swelling with incomplete constriction, swelling with complete constriction, and fascicular entwinement, which may represent a continuum of pathologic processes. Certain ultrasound findings may help predict the likelihood of spontaneous recovery with conservative management versus the need for surgical intervention. We recommend relying heavily on history and physical examination to determine which nerves are clinically affected and should therefore be assessed by HRUS. The nerves most frequently affected by NA are the suprascapular, long thoracic, median and anterior interosseous nerve (AIN) branch, radial and posterior interosseous nerve (PIN) branch, axillary, spinal accessory, and musculocutaneous. When distal upper limb nerves are affected (AIN, PIN, superficial radial nerve), the lesion is almost always located in their respective fascicles within the parent nerve, proximal to its branching point. The purpose of this review is to describe a reproducible, standardized, ultrasonographic approach for evaluating suspected NA, and to share reliable techniques and clinical considerations when imaging commonly affected nerves.

Surgical Treatment of Parsonage Turner Syndrome With Primary Nerve Transfers: A Case Series and Cadaver Dissection.

ABSTRACT: Parsonage Turner syndrome (PTS) is the development of severe, spontaneous pain with subsequent nerve palsy. Unfortunately, many patients never achieve full functional recovery, and many have chronic pain. The use of nerve transfers in PTS has not been reported in the literature. We present 4 cases of PTS treated surgically with primary nerve transfer and neurolysis of the affected nerve following the absence of clinical and electrodiagnostic recovery at 5 months from onset. In addition, we present a cadaver dissection demonstrating an interfascicular dissection of the anterior interosseous nerve (AIN) into its components to enable a fascicular transfer in partial AIN neuropathy. Two patients with complete axillary neuropathy underwent a neurorrhaphy between the nerve branch to the lateral head of the triceps and the anterior/middle deltoid nerve branch of the axillary nerve. Two patients with partial AIN neuropathy involving the FDP to the index finger (FDP2) underwent a neurorrhaphy between an extensor carpi radialis brevis nerve branch and the FDP2 nerve branch. All patients had neurolysis of the affected nerves. All subjects recovered at least M4 motor strength. The cadaver dissection demonstrates 3 separate nerve fascicles of the AIN into FPL, FDP2, and pronator quadratus that can be individually selected for reinnervation with a fascicular nerve transfer. Functional recovery for patients with PTS with neurolysis alone is variable. Surgical treatment with neurolysis and a nerve transfer to improve functional recovery when no recovery is seen by 5 months is an option.

Parsonage-Turner posterior a vacunación antirrábica por mordedura de murciélago: comunicación de un caso / Parsonage-Turner after rabies vaccination due to bat bite: case report

Introducción:Paciente adulto masculino que consulta por mordedura de murciélago. Se realiza aplicación de vacuna antirrábica y toxoide tetánico, tras lo cual presenta alteraciones motoras, sensitivas y dolor de difícil manejo en el miembro superior derecho. Siendo un reto diagnóstico de manera inicial, posterior a estudios electrofisiológicos se consideró un síndrome de Parsonage-Turner. Este caso es relevante por la asociación de vacuna antirrábica no descrita en antecedentes de inmunización en revisiones previas.Caso clínico:Se le realizaron varias pruebas diagnósticas e imágenes como ecografías, resonancia magnética y estudios electrofisiológicos los cuales confirmaron el diagnóstico de neuritis braquial (síndrome de Parsonage-Turner). El paciente recibió intervenciones quirúrgicas con fascitomías y neurólisis, además de múltiples manejos farmacológicos para dolor, incluyendo opioides fuertes, neuromoduladores, antidepresivos y medicamentos por bomba intratecal, con disminución solo del 50 % de los síntomas de dolor y afectación a su calidad de vida.Conclusión:El caso nos muestra cómo el síndrome de Parsonage-Turner, una enfermedad neurológica con etiología desconocida, se puede cronificar en un síndrome doloroso al no tener un diagnóstico temprano o someter al paciente procedimientos no indicados. Este síndrome debe considerarse en la atención de urgencias y consulta externa por sus consecuencias a largo plazo y el difícil manejo de los síntomas crónicos. Hay una relación causal reportada en la literatura con el toxoide tetánico, pero este se aplicó previo a los síntomas; no hay referencias de inicio de síntomas posterior a la vacuna antirrábica, lo cual puede generar a futuro, una relación causal si se encontraran nuevos casos.(AU)

Introduction:Adult male patient who suffers a bat bite, after which rabies vaccine and tetanus toxoid are administered; later, he presents on the right upper limb severe motor, sensory and pain disorders. Initially a diagnostic challenge, after electrophysiological studies, Parsonage-Turner syndrome was considered. This case is relevant since the association between rabies vaccine and this syndrome has not been described.Clinical case:Several diagnostic tests and images were performed, including ultrasound, magnetic resonance and electrophysiological studies, which confirmed the diagnosis of brachial neuritis (Parsonage-Turner syndrome). Even though the patient received surgical interventions with fasciotomies and neurolysis as well as multiple pharmacological pain management with strong opioids, neuromodulators, antidepressants and intrathecal pump medications, there was a 50 % decrease in pain symptoms and an impairment of their quality of life.Conclusion:The case shows a Parsonage-Turner syndrome, a neurological disease with unknown etiology, with difficult diagnosis which can lead to chronic pain syndrome or unnecessary surgical procedures. This syndrome should be considered in emergency care and outpatient care due to its long-term consequences and the difficult management of chronic symptoms. There is a causal relationship reported in the literature with tetanus toxoid, but it was applied prior to symptoms; there are no reports of onset of symptoms after the rabies vaccine, which may generate a causal relationship in the future if new cases are found.(AU)

Anterior Interosseous Nerve Syndrome Reconsidered: A Critical Analysis Review.

Anterior interosseous nerve syndrome (AINS) represents a form of neuralgic amyotrophy (Parsonage-Turner syndrome). AINS does not originate from external compression of the AIN in the forearm. Fascicular constrictions (FCs) of the median nerve are identified within the anterior interosseous fascicular group at or above the medial epicondyle. Spontaneous recovery is not ensured, leaving up to 30% of patients with permanent weakness or palsy. Fascicular microneurolysis of the median nerve, performed at or above the elbow, is a treatment option for patients who do not recover spontaneously.

Neuralgic amyotrophy: a paradigm shift in diagnosis and treatment.

Neuralgic amyotrophy (NA), also known as Parsonage-Turner syndrome, is characterised by sudden pain attacks, followed by patchy muscle paresis in the upper extremity. Recent reports have shown that incidence is much higher than previously assumed and that the majority of patients never achieve full recovery. Traditionally, the diagnosis was mainly based on clinical observations and treatment options were confined to application of corticosteroids and symptomatic management, without proven positive effects on long-term outcomes. These views, however, have been challenged in the last years. Improved imaging methods in MRI and high-resolution ultrasound have led to the identification of structural peripheral nerve pathologies in NA, most notably hourglass-like constrictions. These pathognomonic findings have paved the way for more accurate diagnosis through high-resolution imaging. Furthermore, surgery has shown to improve clinical outcomes in such cases, indicating the viability of peripheral nerve surgery as a valuable treatment option in NA. In this review, we present an update on the current knowledge on this disease, including pathophysiology and clinical presentation, moving on to diagnostic and treatment paradigms with a focus on recent radiological findings and surgical reports. Finally, we present a surgical treatment algorithm to support clinical decision making, with the aim to encourage translation into day-to-day practice.

A nationwide analysis of 30-day adverse events, unplanned readmission, and length of hospital stay after peripheral nerve surgery in extremities and the brachial plexus.

BACKGROUND: Little is known on adverse events and their timing after peripheral nerve surgery in extremities. The aim of this study is to identify predictors and typical timing of complications, unplanned readmission, and length of hospital stay for patients undergoing peripheral nerve surgery in the extremities. METHODS: Data were extracted from the National Surgical Quality Improvement Program (NSQIP) registry from 2005 to 2015. Adult patients undergoing peripheral nerve surgery in the extremities were included. A subgroup analysis was performed for brachial plexus operations. Multivariable logistic regression was performed to identify predictors of any complication, surgical site infection, unplanned readmission, and reoperation. RESULTS: A total of 2,840 patients were identified; 628 were brachial plexus operations. Overall complications were 4.4% and 7.0%, respectively. Median time for occurrence of any complication was 8 days. The most common complications were wound-related (1.7%), which occurred at a median of 15 days postoperatively. Reoperation occurred in 1.8% of all cases; most commonly for musculoskeletal repair (16.7%). Unplanned readmissions occurred in 2.3% and were most often due to wound-related problems (24.1%). Preoperatively contaminated wounds, inpatient procedures, and longer operative time seemed to have the most influence on all adverse events. In brachial plexus pathology, insulin-dependent diabetes and emergency cases also negatively affected outcomes. CONCLUSIONS: Complications usually occur one to two weeks postoperatively. Preoperatively contaminated wounds, inpatient procedures, and longer operative times influence outcome. Anatomical level of operation results in significantly different lengths of hospital stay; brachial plexus pathology has the longest length of stay.

Giant Cell Ependymoma of Cervicomedullary Junction: A Case Report of a Long-Term Survivor and Literature Review.

BACKGROUND: Ependymoma accounts for 3%-9% of all neuroepithelial tumors. Giant cell ependymoma (GCE) is a rare and distinct variant, with only 22 cases described in the literature. The 2007 World Health Organization classification first acknowledged this rare subtype. The cytologic features of GCE include the presence of pleomorphic giant cells with several cellular atypias, which at intraoperative frozen diagnosis may appear to be high-grade glial lesions. Despite its apparently malignant histology, GCE seems to be a neoplasm with a relatively good prognosis. Extended tumor removal is the gold standard without adjuvant treatment. CASE DESCRIPTION: We describe the first case, to our knowledge, of GCE situated at the cervicomedullary junction in a 62-year-old patient. Surgery was performed with combined intraoperative monitoring of motor evoked potentials and somatosensory evoked potentials. Intraoperative frozen diagnosis revealed a high-grade glial neoplasm; however, gross total resection was achieved. The definitive diagnosis was GCE. At follow-up evaluation 11 years after surgery, the patient did not present with any tumor recurrence. CONCLUSIONS: As the intraoperative diagnosis can be misleading, whenever a cleavage plane is recognized, it is essential to perform a gross total resection with the aid of intraoperative neurophysiologic monitoring, to improve prognosis and neurologic outcome. Data reported in the literature show that prognosis is mainly influenced by grade of resection.

Hourglass-Like Constriction of the Brachial Plexus in the Posterior Cord: A Case Report.

BACKGROUND AND IMPORTANCE: Hourglass-like constrictions are fascicular conditions confirmed definitively by interfascicular neurolysis. Certain peripheral nerves have vulnerable areas such as around the elbow in the posterior interosseous nerve. We report the first hourglass-like constriction in the brachial plexus supplying the radial innervated forearm musculature. Preoperative magnetic resonance imaging (MRI) findings of the brachial plexus were consistent with neuralgic amyotrophy (NA). CLINICAL PRESENTATION: A 9-yr-old boy experienced worsening left arm pain and difficulty in elevating the shoulder. Sequentially, severe palsy emerged when extending the wrist, thumb, and fingers. Based on the clinical picture, we diagnosed him with NA. The oblique coronal T2-weighted short-tau inversion recovery images showed mildly diffuse enlargement and hyperintensity of the brachial plexus. He showed few signs of improvement and interfascicular neurolysis was performed 11 mo after the onset. One of the fascicles in the posterior cord had developed an hourglass-like constriction. Electrical stimulation confirmed that the fascicle supplied forearm muscles. His wrist and finger extension had almost recovered at the 12-mo postoperative visit. CONCLUSION: Hourglass-like constrictions can occur in the brachial plexus. Although surgical approaches for the constrictions are still controversial, several reports demonstrated their effectiveness. Meanwhile, concerning NA treatment, evidence on the surgical intervention is lacking. Brachial plexus MRI might help in discerning the lesion and planning treatment options including surgical interventions. Hourglass-like constrictions are a possible etiology for certain NA patients with residual symptoms or paresis.

MRI bullseye sign: An indicator of peripheral nerve constriction in parsonage-turner syndrome.

INTRODUCTION: The role of MRI in identifying hourglass constrictions (HGCs) of nerves in Parsonage-Turner syndrome (PTS) is largely unknown. METHODS: Six patients with PTS and absent or minimal recovery underwent MRI. Surgical exploration was performed at identified pathologic sites. RESULTS: The time between symptom onset and surgery was 12.4 ± 6.9 months; the time between MRI and surgery was 1.3 ± 0.6 months. Involved nerves included suprascapular, axillary, radial, and median nerve anterior interosseous and pronator teres fascicles. Twenty-three constriction sites in 10 nerves were identified on MRI. A "bullseye sign" of the nerve, identified immediately proximal to 21 of 23 sites, manifested as peripheral signal hyperintensity and central hypointensity orthogonal to the long axis of the nerve. All constrictions were confirmed operatively. CONCLUSIONS: In PTS, a bullseye sign on MRI can accurately localize HGCs, a previously unreported finding. Causes of HGCs and the bullseye sign are unknown. Muscle Nerve 56: 99-106, 2017.

Restoration of prehensile function for motor paralysis in Hopkins syndrome: case report.

Hopkins syndrome is a rare cause of poliomyelitis-like paralysis affecting 1 or more extremities after an acute attack of asthma. The exact etiology of Hopkins syndrome is not known. A 4-year-old girl developed acute asthma followed by complete flaccid paralysis of the left upper extremity. She underwent staged reconstruction using the double free muscle transfer technique. Rigorous postoperative physiotherapy was carried out to achieve a good functional outcome. At recent follow-up, 27 months after the first procedure, the patient was able to effectively use the reconstructed hand for most daily activities. She had good control and could perform 2-handed activities. The selection of a suitable operative treatment and suitable donor nerves is critical, and there are no clear guidelines in the literature. The double free muscle transfer can be effectively employed in similar cases to restore grasping function.

Total disc replacement.

Total disc replacement (TDR) (partial disc replacement will not be described) has been used in the lumbar spine since the 1980s, and more recently in the cervical spine. Although the biomechanical concepts are the same and both are inserted through an anterior approach, lumbar TDR is conventionally indicated for chronic low back pain, whereas cervical TDR is used for soft discal hernia resulting in cervicobrachial neuralgia. The insertion technique must be rigorous, with precise centering in the disc space, taking account of vascular anatomy, which is more complex in the lumbar region, particularly proximally to L5-S1. All of the numerous studies, including prospective randomized comparative trials, have demonstrated non-inferiority to fusion, or even short-term superiority regarding speed of improvement. The main implant-related complication is bridging heterotopic ossification with resulting loss of range of motion and increased rates of adjacent segment degeneration, although with an incidence lower than after arthrodesis. A sufficiently long follow-up, which has not yet been reached, will be necessary to establish definitively an advantage for TDR, particularly in the cervical spine.