A Severe Case of Diabetic Lumbosacral Radiculoplexus Neuropathy: A Diagnosis of Exclusion.

Diabetic lumbosacral radiculoplexus neuropathy (DLSRPN), also known as diabetic amyotrophy, is a rare disease of exclusion that is difficult to diagnose due to its non-specific clinical presentation of neuropathy, autonomic symptoms, and potential weight loss. Due to this, many differential diagnoses are raised before making a diagnosis of such an uncommon disease. However, once the diagnosis is made, the management of this disease can vary. Here, we would like to discuss the etiology, pathophysiology, diagnosis, and management of this disease, as well as present a rare case of diabetic lumbosacral radiculoplexus neuropathy in a 50-year-old male.

Hourglass-like constrictions of the radial nerve in the neuralgic amyotrophy: A case report.

Neuralgic amyotrophy (NA) is a peripheral nerve disorder that has a classical presentation as motor deficit after severe pain, but it is still overlooked or misdiagnosed. Formerly, the diagnosis was based on the clinical picture and electrophysiology; however, sophisticated imaging and surgical modalities showed structural abnormalities such as hourglass-like constrictions of the nerves. In this article, we present a case presenting with drop hand mimicking radial nerve entrapment. The patient was diagnosed with NA and surgery revealed hourglass-like constrictions. The clinical findings were improved after neurorrhaphy and physical therapy. In conclusion, hourglass-like constrictions can be prognostic factors of NA and should be searched carefully.

A case report of neuralgic amyotrophy.

Neuralgic muscular atrophy is not uncommon in clinical practice. Due to the different branches of brachial plexus involved in the lesion, the clinical symptoms are different, and there is a lack of clear imaging diagnostic criteria, so the diagnosis of this disease brings great challenges to clinicians. We have certain experience in the diagnosis and treatment of this disease, and hereby select a representative case of neuralgic muscular atrophy to share its diagnosis and treatment process, focusing on analyzing the characteristic symptoms of this disease, valuable imaging data and targeted treatment, so as to enable clinicians to better understand this disease.

Analysis of surgical treatment of cervical spondylotic amyotrophy.

Background: Cervical spondylotic amyotrophy (CSA) is a special type of cervical spondylosis based on cervical degeneration, which is mainly manifested by weakness and atrophy of upper limb muscles without obvious sensory impairment. Various diagnostic and treatment strategies used; however, discrepancies exist. We tried to discuss diagnosing and treating CSA. Methods: 15 patients with CSA were diagnosed in the Orthopedics Department of the First Affiliated Hospital of Zhengzhou University, aged 42-70 years old. The duration of preoperative symptoms of amyotrophy was 6 to 240 months. 12 patients received surgical treatment, and 3 patients received conservative treatment. The patients were divided into two groups according to the site of preoperative amyotrophy. The manual muscle test was used to evaluate the patients' muscle strength pre-and postoperatively. Results: During postoperative follow-up, the muscle strength of 12 patients improved to different degrees compared to before surgery. The improvement effect was excellent in 2 cases, good in 6, and moderate in 4. There was no decrease in postoperative muscle strength compared with that before surgery. The satisfaction rate of the effect was 66.7%. The two groups had no statistically significant difference in preoperative muscle strength. The postoperative muscle strength of the proximal group was significantly better than that of the distal group. Conclusion: The surgical effect of CSA of the proximal type is significantly better than that of the distal type. The recovery effect of amyotrophy after surgery for distal type CSA is poor; thus, surgical treatment should be carefully considered.

Neurolysis of the Long Thoracic Nerve for Scapular Winging due to Isolated Serratus Anterior Palsy: Early and Midterm Results in 29 Patients.

Objective: This study aimed to report our surgical outcomes of thoracic long thoracic nerve (LTN) decompression in patients with isolated LTN palsy, using a clinical scoring system designed to facilitate the grading of scapular winging severity. Methods: This was a retrospective review of patients who had undergone decompression and neurolysis of the LTN for scapular winging. Each patient underwent needle electromyography of the serratus anterior for confirmation of diagnosis and were refractory to a minimum of 6 months of nonoperative management. Preoperatively and at final follow-up, shoulder range of motion and the Wrightington Winging Score (WWS) was used to objectively grade the dynamic and static components of winging. Results: Between 2014 and 2020, 29 patients who underwent thoracic neurolysis for scapular winging were analyzed. These were 16 males and 13 females with a mean age of 37 years. The injury mechanism was due to trauma in 19 cases and neuralgic amyotrophy in 10. The median duration between winging onset and surgery was 30 months. There were significant improvements in mean active shoulder abduction and forward flexion. Winging was noticeably improved in 22 patients. At presentation, the median WWS was 3, which improved to 1 at final follow-up. Conclusion: In patients with isolated LTN palsy causing persistent scapular winging which is not responsive to conservative treatment, neurolysis of the thoracic portion of the LTN can be considered. Level of Evidence: IV.

Bilateral Phrenic Nerve Palsy Associated With Neuralgic Amyotrophy.

Neuralgic amyotrophy (NA) is a multifocal inflammatory neuropathy accompanied by acute pain and muscle atrophy. NA commonly affects the upper extremities, but rarely affects the phrenic nerve. Here, we report a male with neck pain, orthopnea, difficulty sleeping in the supine position, and inability to slurp. His saturated oxygen level decreased from 97% to 86% in the supine position. His right shoulder showed muscle atrophy. Chest X-ray examination in the supine position and a nerve conduction study showed phrenic palsy. We diagnosed it as bilateral phrenic nerve palsy associated with NA. NA sometimes causes phrenic nerve palsy and may cause slurping difficulty.

Parsonage-Turner Syndrome following COVID-19 Vaccination: A Systematic Review.

BACKGROUND: Parsonage-Turner syndrome (PTS) is an inflammatory condition of the brachial plexus, with more than half of patients presenting a trigger, such as infection or vaccination. Our objective was to synthesize the clinical and paraclinical features, therapeutic responses, and outcomes of PTS post-COVID-19 vaccination. METHODS: We systematically reviewed two databases (LitCOVID and the WHO database on COVID-19) up to January 2024 following a published protocol (OSF registries). RESULTS: We included 59 cases. PTS occurred more frequently in males (61.1% mRNA group, 83.3% viral vector group). Patients in the mRNA group were younger (41.7% between 41 and 50 years vs. 38.9% between 61 and 70 years). Most cases had sudden pain within two weeks. Unilateral PTS was present in 94.4% of mRNA and all viral vector-vaccinated cases. Symptoms included pain (97.1% and 92.3%, respectively), usually followed within two weeks by motor deficits (97.2% and 94.1%, respectively), amyotrophy (30% and 81.8%, respectively), paresthesia (50% and 27.3%, respectively), and sensory loss (33.3% and 38.5%, respectively). Viral vector vaccine recipients had nerve involvement outside the brachial plexus. Ancillary investigations revealed CSF albuminocytological dissociation (33.3% and 100%, respectively) and ipsilateral axillary lymphadenopathy. Two PTS cases worsened after the second mRNA dose, and another recurred after influenza vaccination. One patient well tolerated the second dose of the viral vector vaccine, but symptoms reemerged in another. CONCLUSIONS: Current evidence suggests PTS may occur after all COVID-19 vaccine types, with some subgroup differences. Also, PTS might recur with subsequent similar or unrelated vaccines.

Anesthetic challenges in a patient with Hirayama disease with quadriparesis and autonomic dysfunction undergoing cervical spine surgery.

Hirayama disease is a rare neurological disorder, characterized by muscular atrophy of the distal upper extremities. The occurrence of spastic quadriparesis and autonomic dysfunction is rarely reported and has important perioperative considerations during cervical spine surgery for the treatment of this disorder. The role of the anesthesiologist is vital in the thorough assessment of the patient for the involvement of the pyramidal tract, autonomic dysfunction, gastroparesis, hyperreactive airway disease, and documentation of neurological deficits. Intraoperative concerns include safe manipulation of the airway during mask ventilation and the use of a flexible fibreoptic bronchoscope during endotracheal intubation to prevent neck flexion. It is also essential to avoid drugs, leading to histamine release. The use of multimodal monitoring including bispectral index and neuromuscular monitoring is crucial to prevent delayed recovery. Anticipation and management of exaggerated hypotension in response to anesthetic induction agents and prone position is the key to a successful outcome in patients with autonomic dysfunction.

Long term quality of life follow-up and functional impairment study in patients with Hirayama disease.

Hirayama Disease (HD) is a focal motor neuron disorder generally affecting young adults with a male predominance who experience weakness and atrophy in distal upper extremity muscles in an asymmetric or unilateral pattern. Progression is insidious though significant weakness occurs during a progressive phase of the disease over 2-5 years. The long-term outcome of HD is not as well-known and, thus, this study presents self-reported outcomes from HD patients years after a diagnosis. Thirty HD patients reported quality of life (QOL) and other functional outcome measures after a mean of just over 11 years from diagnosis. Variables that predicted better or worse outcome were analyzed. Overall, QOL was affected by HD though most patients were functional with limitations. No clear attributes of patients or their disease predicted outcome.

Neuralgic amyotrophy with multiple hourglass-like constrictions of anterior interosseous nerve: a case report.

Hourglass-like constrictions (HLCs) of peripheral nerves in the upper extremity were a rare form of neuralgic amyotrophy, often characterized by the sudden onset of pain in the shoulder or arm, followed by muscle weakness and amyotrophy, with limited sensory involvement. We present a case of multiple HLCs of the anterior interosseous nerve (AIN) in a 22-year-old female with left upper arm pain, finger numbness, and limited activity for 1 month. Physical examination showed weakness of the left index flexor digitorum profundus and flexor pollicis longus, with mild hypoesthesia in the first three fingers and the radial half of the ring finger. Electromyography suggested a median nerve (mainly AIN) lesion. Ultrasonographic imaging of the median nerve shows AIN bundle swelling and multiple HLCs at left upper arm. Despite conservative treatment, which included 15 days of steroid pulse therapy, Etoricoxib, and oral mecobalamin, the patient still complained of extreme pain at night without relief of any symptoms. Operation was recommended for this patient with thorough concerns of surgical advantages and disadvantages. During surgery, a total of 7 HLCs were found in her median nerve along and above the elbow joint. Only Interfascicular neurolysis was performed because the nerve constrictions were still in the early stage. The pain was almost relieved the next day. One month after surgery, she could bend her thumb and index fingers, although they were still weak. 4 months after the surgery, she was able to bend affected fingers, with muscle strength M3 level. At the same time, her fingers had fewer numbness symptoms. There was still controversy regarding treatment strategy; however, early diagnosis and surgical treatment for nerve HLCs might be a better choice to promote nerve recovery.

Surgical Outcomes of Full Endoscopic Posterior Cervical Foraminotomy for Proximal Cervical Spondylotic Amyotrophy.

STUDY DESIGN: Retrospective analysis of case series. PURPOSE: This study aimed to clarify the effects of full endoscopic posterior cervical foraminotomy (FPCF) on cervical spondylotic amyotrophy (CSA). OVERVIEW OF LITERATURE: The method for decompressing the ventral nerve root and anterior horn (AH) in CSA is controversial. METHODS: Patients without myelopathy who underwent FPCF for proximal CSA between 2017 and 2022 were analyzed. The outcome measure was the results of the manual muscle testing (MMT) of the deltoid and biceps. Preoperative nerve root and AH compression were evaluated by magnetic resonance imaging. The intervertebral foramen morphology and bony decompression extent were evaluated by computed tomography. RESULTS: FPCF was performed at the C4/5 level and at the C4/5 and C5/6 levels in 14 and 11 patients, respectively. The width of the narrowest intervertebral foramen was significantly narrower on the affected side than on the healthy side at the C4/5 (2.5 mm vs. 3.6 mm) and operated C5/6 (1.9 mm vs. 3.1 mm) levels. AH compression occurred at the C4/5 and C5/6 levels in 28% and 21% of the patients, respectively. Bony decompression was performed laterally beyond the narrowest foramen at the C4/5 and C5/6 levels in 96% and 91% of the patients, respectively. Compared with patients without AH compression, in those with AH compression, the lamina was resected medially by an average of >1.7 mm and >3.6 mm at the C4/5 and C5/6 levels, respectively. Furthermore, 76% and 81% of the facet joint surfaces were preserved at the C4/5 and C5/6 levels, respectively. Postoperative MMT grade improvement was excellent, good, and fair in 64%, 20%, and 16% of the patients, respectively. CONCLUSIONS: FPCF was effective for treating proximal CSA.

Characteristics of Hirayama Disease in Young South Korean Soldiers.

BACKGROUND AND PURPOSE: The purpose of this study was to describe the clinical presentation and features in electrodiagnostic and imaging investigations of young South Korean males diagnosed with Hirayama disease (HD). METHODS: We reviewed the electronic medical records of South Korean enlisted soldiers who were diagnosed with HD and discharged from military service during 2011-2021. We investigated the clinical characteristics and results of electrodiagnostic and magnetic resonance imaging (MRI) investigations. We analyzed laterality and identified the involved muscles using needle electromyography (EMG). Loss of lordosis, localized cervical cord atrophy, loss of attachment between the posterior dura and subjacent lamina, asymmetric flattening of the cord, crescent-shaped mass in the posterior epidural space, and noncompressive intramedullary T2-weighted high signal intensity were investigated using neutral- or flexion-position MRI. RESULTS: Forty-two male patients aged 20.2±0.8 years (mean±standard deviation) were identified. All patients complained of hand weakness, and 10 complained of hand tremor (23.8%). Four patients (9.5%) had symptoms in both upper limbs, and five (11.9%) had sensory disturbances. Needle EMG revealed that muscles in the C7-T1 myotome were commonly involved, and C5-C6 involvement of the deltoid (10.5%) and biceps brachii (12.5%) was also observed. In cervical MRI, localized cord atrophy (90.0%) was the most characteristic finding, and cord atrophy was most severe at the C5-C6 level (58.3%). CONCLUSIONS: This is the first description of a large number of patients with HD in South Korea. The clinical presentation and features found in electrodiagnostic and imaging investigations will improve the understanding of HD in the young South Korean male population.

Neuralgic Amyotrophy and Hourglass Nerve Constriction/Nerve Torsion: Two Sides of the Same Coin? A Clinical Review.

Neuralgic amyotrophy, also called Parsonage-Turner syndrome, in its classic presentation is a brachial plexopathy or a multifocal neuropathy, involving mainly motor nerves of the upper limb with a monophasic course. Recently, a new radiological entity was described, the hourglass constriction, which is characterized by a very focal constriction of a nerve, or part of it, usually associated with nerve thickening proximally and distally to the constriction. Another condition, which is similar from a radiological point of view to hourglass constriction, is nerve torsion. The pathophysiology of neuralgic amyotrophy, hourglass constriction and nerve torsion is still poorly understood, and a generic role of inflammation is proposed for all these conditions. It is now widely accepted that nerve imaging is necessary in identifying hourglass constrictions/nerve torsion pre-surgically in patients with an acute mononeuropathy/plexopathy. Ultrasound and MRI are useful tools for diagnosis, and they are consistent with intraoperative findings. The prognosis is generally favorable after surgery, with a high rate of good motor recovery.

Subscapularis impairment on magnetic resonance imaging is correlated with functional limitations in neonatal brachial plexus palsy.

PURPOSE: The relationship between functional shoulder deficits in children with neonatal brachial plexus palsy (NBPP) and magnetic resonance imaging (MRI) shoulder abnormalities was evaluated. METHODS: Shoulder function was assessed in 16 children (mean age: 5.8 years; range: 3-12 years) with NBPP based on shoulder rotator muscle strength, as measured using an isokinetic dynamometer and the modified Mallet score. The thickness and fatty infiltration of the subscapularis and infraspinatus muscles, and the morphology of the glenoid on MRI, were also determined. RESULTS: The highest subscapularis fatty infiltration subgroup of NBPP patients promoted the highest alteration muscle thickness and modified Mallet score. CONCLUSIONS: In NBPP children, subscapularis impairments play a major role in the functional limitations. This study of pediatric NBPP patients highlighted the value of adding an examination of the muscles to routine MRI assessment of bone parameters in the shoulders of NBPP children. TRIAL REGISTRATION: NCT03440658.

[MR neurography reveals fascicular constriction of the median nerve in a patient with neuralgic amyotrophy].

Diagnosing neuralgic amyotrophy can be challenging in clinical practice. Here, we report the case of a 37-years old Japanese woman who suddenly developed neuropathic pain in the right upper limb after influenza vaccination. The pain, especially at night, was severe and unrelenting, which disturbed her sleep. However, X-ray and MRI did not reveal any fractures or muscle injuries, and brain MRI did not reveal any abnormalities. During neurological consultation, she was in a posture of flexion at the elbow and adduction at the shoulder. Manual muscle testing suggested weakness of the flexor pollicis longus, pronator quadratus, flexor carpi radialis (FCR), and pronator teres (PT), while the flexor digitorum profundus was intact. Medical history and neurological examination suggested neuralgic amyotrophy, particularly anterior interosseous nerve syndrome (AINS) with PT/FCR involvement. Innervation patterns on muscle MRI were compatible with the clinical findings. Conservative treatment with pain medication and oral corticosteroids relieved the pain to minimum discomfort, whereas weakness remained for approximately 3 months. For surgical exploration, lesions above the elbow and fascicles of the median nerve before branching to the PT/FCR were indicated on neurological examinations; thus, we performed high-resolution imaging to detect possible pathognomonic fascicular constrictions. While fascicular constrictions were not evident on ultrasonography, MR neurography indicated fascicular constriction proximal to the elbow joint line, of which the medial topographical regions of the median nerve were abnormally enlarged and showed marked hyperintensity on short-tau inversion recovery. In patients with AINS, when spontaneous regeneration cannot be expected, timely surgical exploration should be considered for a good outcome. In our case, MR neurography was a useful modality for assessing fascicular constrictions when the imaging protocols were appropriately optimized based on clinical assessment.

Bibrachial amyotrophy as a rare manifestation of intraspinal fluid collection: a case report and systematic review.

INTRODUCTION: Intraspinal cerebrospinal fluid (CSF) collection has been reported as a rare cause of lower motor neuron (LMN) disorder. We report a case of bibrachial diplegia associated with intraspinal CSF collection and perform a systematic literature review. PATIENT AND METHODS: A 52-year-old man developed a bibrachial amyotrophy over 6 years, confirmed by the presence of cervical subacute neurogenic changes at electromyography (EMG). Brain magnetic resonance imaging (MRI) revealed cerebral siderosis, while spine MRI showed a ventral longitudinal intraspinal fluid collection (VLISFC) from C2 to L2. No CSF leakage was localized at myelography; a conservative treatment was chosen. We searched for all published cases until 30th April 2023 and extrapolated data of 44 patients reported in 27 publications. RESULTS: We observed a male predominance, a younger disease onset compared to amyotrophic lateral sclerosis, and a quite long disease duration, highlighting a slow disease progression. LMN signs were more frequently bilateral, mostly involving C5-C6 myotomes. Around 61% of patients presented additional symptoms, but only three referred to a history of headache. Accordingly, CSF opening pressure was mostly normal. Spinal MRI revealed the presence of VLISFC and in some cases myelomalacia. EMG patterns displayed both chronic and subacute neurogenic change in the cervical region. The disease course mainly depended on the treatment choice, which was mostly represented by a surgical approach when a specific dural defect was detected by imaging. CONCLUSION: Bibrachial diplegia due to VLISFC can be a treatable cause of focal amyotrophy and presents some clinical and radiological "red flags" which cannot be missed by a clinical neurologist.

Monomelic Amyotrophy/Hirayama Disease: Surgical Outcome in a Large Cohort of Indian Patients.

BACKGROUND: Hirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD. METHODS: Between 2015 and 2019, 126 patients with HD underwent ACDF. Contrast magnetic resonance imaging of the cervical spine in full flexion was performed. Clinical examination and preoperative/postoperative assessment of hand function using Fugl-Meyer assessment, Jebsen-Taylor hand function test, and handheld dynamometry were performed at 3-monthly intervals for 1 year. Surgical outcomes were assessed as per the Odom criteria and Hirayama outcome questionnaire. RESULTS: Age at onset and duration of illness were 12-31 years (mean, 18 ± 2.7) and 1-96 months (32.7 ± 24.4), respectively. All patients had progressive weakness and wasting of the affected limb. Cord atrophy was seen in 97.1%, with epidural detachment and engorgement of the posterior epidural venous plexus in all. All patients underwent ACDF. Of these patients, 54% had an excellent/good outcome and 39% had a satisfactory outcome as per the Odom scale at last follow-up (mean, 44.9 ± 16.5 months) after surgery. Handheld dynamometry showed improvement from preoperative values to 1 year follow-up. Duration of illness and age at onset had a negative correlation and the preoperative Fugl-Meyer score had a positive correlation with improvement. CONCLUSIONS: ACDF resulted in remarkable improvement or stabilization in neurologic deficits in many patients with HD. Because motor disability ensues over time, early surgical intervention during the progressive phase is advocated.

Neurological manifestation of HEV infection: still a rare disease entity?

Hepatitis E virus (HEV) infection is the most common form of viral hepatitis and is reported to cause neurological manifestation in up to 30% of diagnosed infections. We evaluated the medical reports of all patients (n = 29,994) who were discharged from the Department of Neurology of Ulm University between 01.01.2015 and 30.09.2022 to detect neurological manifestations of HEV. In addition, we retrospectively analyzed the serum samples of n = 99 patients representing different neurological diseases possibly related to HEV for anti-HEV-IgM and anti-HEV-IgG. At the time of discharge from hospital, the etiology of neurological symptoms in these patients was unclear. Overall, five cases of extrahepatic neurological manifestation of HEV (defined as anti-HEV-IgM and HEV-IgG positive) could be detected. An increase of both, anti-IgM- and anti-IgG-serum levels was significantly more common in neuralgic amyotrophy/plexus neuritis/radiculitis than in AIDP/CIDP (P = 0.01), meningitis/encephalitis (P = 0.02), idiopathic peripheral facial paralysis (P = 0.02) and tension headache (P = 0.02). In 15% (n = 15 out of 99) of retrospectively analyzed serum samples, conspicuous positive anti-HEV-IgG levels were detected. This finding was most common in AIDP/CIDP. In conclusion, results of this study indicate neurological manifestation of HEV to be a rare but still underestimated course of disease, occurring at any age and gender. Therefore, testing for HEV should be considered in patients with neurological symptoms of unknown origin, especially in those with neuralgic amyotrophy/plexus neuritis.

Neuropathies related to hepatitis E virus infection: A prospective, matched case-control study.

BACKGROUND: Acute hepatitis E virus (HEV) infection has recently emerged as a potential trigger for acute dysimmune neuropathies, but prospective controlled studies are lacking. AIMS: To compare the frequency of concomitant acute HEV infection in patients with neuralgic amyotrophy (NA), Guillain-Barré syndrome (GBS), and Bell's palsy with a matched control population. METHODS: Swiss multicenter, prospective, observational, matched case-control study over 3 years (September 2019-October 2022). Neurological cases with NA, GBS, or Bell's palsy were recruited within 1 month of disease onset. Healthy controls were matched for age, sex, geographical location, and timing of blood collection. Diagnostic criteria for acute hepatitis E were reactive serum anti-HEV IgM and IgG assays (ELISA test) and/or HEV RNA detection in serum by real-time polymerase chain reaction (RT-PCR). RT-PCR was performed on sera to confirm IgM positivity. RESULTS: We included 180 patients (59 GBS, 51 NA, 70 Bell's palsy cases) and corresponding matched controls (blood donors) with median age 51 years for both groups and equal gender distribution. Six IgM+ cases were detected in the NA, two in the GBS, and none in the Bell's palsy group. Two controls were anti-HEV IgM-positive. At disease onset, most cases with acute HEV infection had increased liver enzymes. A moderate association (p = 0.027, Fisher's exact test; Cramér's V = -0.25) was observed only between acute HEV infection and NA. CONCLUSION: This prospective observational study suggests an association between concomitant acute HEV infection and NA, but not with GBS or Bell's palsy.