[Clinical characteristics analysis of 4 cases with acute flaccid myelitis in children].

Objective: To summarize the clinical manifestations, diagnosis, treatment and prognosis of acute flaccid myelitis (AFM) in children. Methods: Clinical characteristics of 4 AFM cases from Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from September 2018 to November 2022, were analyzed retrospectively. Results: The age of 4 children with AFM was 7 years, 4 years and 3 months, 7 years and 1 month, 6 years and 5 months, respectively. There were 2 boys and 2 girls. Prodromal infection status showed 3 children of respiratory tract infection and 1 child of digestive tract infection. The main manifestation was asymmetrical limb weakness after infection, and the affected limb range was from monoplegia to quadriplegia. Cranial nerve injury was involved in 1 child, no encephalopathy. Magnetic resonance imaging in the spinal cord of all 4 children showed long T1 and T2 signals, mainly involving gray matter. Cerebrospinal fluid cell-protein separation was observed in 2 children. Pathogen detected in 1 child pharyngeal swab was enterovirus D68. Antibody IgM to adenovirus was positive in the blood of 1 child. Antibody IgG against Echo and Coxsackie B virus were positive in the blood of another child. After glucocorticoid, human immunoglobulin or simple symptomatic treatment and at the same time under later rehabilitation training, muscle strength recovered to different degrees, but there were disabilities left in 3 children. Conclusions: AFM should be considered in children with acute and asymmetrical flaccid paralysis accompanied by abnormal magnetic resonance imaging signal in the central region of spinal cord, especially post-infection. The effective treatment is limited and the prognosis is poor.

Grasp and Release Test for Tetraplegic Hand Assessment: an update of the Grasp and Release Test.

The Grasp and Release Test (GRT) was originally developed to measure effectiveness of an implanted neuroprosthesis in people with tetraplegia. Its ease of use and lack of floor and ceiling effects culminated in recommendations for inclusion in a battery of tests to measure outcome following upper limb reconstructive surgery. However, the length of time taken to administer the GRT in a clinical setting, lack of instructions of accepted grasp patterns in the upper limb reconstructive surgery population and scoring procedures lead to differences in reporting outcomes using this measure. In order to ensure clinical utility for the upper limb reconstructive surgery population, revisions of the original test instructions have been made and are reported in this article. Further testing of the psychometric properties of the new measure are currently underway.

Progressive motor quadriparesis with wasting: a diagnostic conundrum.

The case of a man in his early 30s, presenting with slowly progressive, asymmetrical onset, pure motor quadriparesis over 9 months with bulbar involvement, absence of upper motor neuron features and significant weight loss, is reported. In view of presentation with diffuse lower motor neuron involvement of short duration, the diagnostic possibilities are discussed. Apart from motor neuron disease, its mimics including muscle and neuromuscular junction disorders were also considered and evaluated for. Carefully assessed clinical and electrophysiological markers which finally clinched the diagnosis of the rare disorder Lambert-Eaton myasthenic syndrome are elaborated. In the present context, recognition of the oddities on clinical and laboratory evaluation is of paramount importance to rule in causes of pure motor quadriparesis.

Acute non-traumatic tetraparesis - Differential diagnosis.

INTRODUCTION: Potentially life-threatening disorders may present in the emergency department with acute tetraparesis, and their recognition is crucial for an appropriate management and timely treatment. Our review aims to systematize the differential diagnosis of acute non-traumatic tetraparesis. RESULTS: Causes of tetraparesis can be classified based on the site of defect: upper motor neuron (UMN), peripheral nerve, neuromuscular junction or muscle. History of present illness should include the distribution of weakness (symmetric/asymmetric or distal/proximal/diffuse) and associated clinical features (pain, sensory findings, dysautonomia, and cranial nerve abnormalities such as diplopia and dysphagia). Neurological examination, particularly tendon reflexes, helps further in the localization of nerve lesions and distinction between UMN and lower motor neuron. Ancillary studies include blood and cerebral spinal fluid analysis, neuroaxis imaging, electromyography, muscle magnetic resonance and muscle biopsy. CONCLUSIONS: Acute tetraparesis is still a debilitating and potentially serious neurological condition. Despite all the supplementary ancillary tests, the neurological examination is the key to achieve a correct diagnosis. The identification of life-threatening neurologic disorders is pivotal, since failing to identify patients at risk of complications, such as acute respiratory failure, may have catastrophic results.

Quadriplegia and quadriparesis after endovascular aortic procedures: a catastrophic and under-reported complication?

In this study are presented three cases of spinal cord ischemia (SCI) involving the cervical-dorsal level and leading to quadriplegia and quadriparesis, following thoraco-abdominal aortic aneurysm (TAAA) endovascular repair. A 79-year-old woman with an extent III TAAA was scheduled for a multi-step fenestrated/branched endovascular aortic repair. Immediately after the first step, consisting of standard proximal thoracic stent-graft implantation, she developed quadriplegia that did not resolve despite all therapeutic actions, and died therefore on postoperative day 32. A 72-year old male with an extent IV TAAA underwent endovascular repair, using a customized fenestrated aortic stent-graft. Five hours after the procedure, he developed an asymmetric quadriparesis, that progressively resolved after spinal fluid drainage and arterial pressure increase, even if signs of SCI were documented at magnetic resonance imaging (MRI). A 79-year old man, referred for a type II TAAA with rapid enlargement, underwent a one-stage endovascular repair, using a customized branched aortic stent-graft. As soon as the procedure was completed, the patient presented inferior limbs paralysis and upper limbs paresis. Although no signs of SCI were documented at MRI, the patient did not recover and died therefore three months after the procedure. Although rare, cervical-dorsal SCI may develop during TAAA endovascular aortic repair. This possibly catastrophic event should be considered in the decisional process of TAAA repair and considered to allow prompt recognition and treatment.

Motor Point Topography of Fundamental Grip Actuators in Tetraplegia: Implications in Nerve Transfer Surgery.

The differentiation between an upper motoneuron (UMN) lesion and lower motoneuron (LMN) lesion of forearm muscles in patients with tetraplegia is critical for the choice of treatment strategy. Specifically, the M. pronator teres (PT), M. flexor digitorum profundus III (FDPIII), and M. flexor pollicis longus (FPL) were studied since they represent key targets in nerve transfer surgery to restore grasp function. Forearm muscles of 24 patients with tetraplegia were tested bilaterally with electrical stimulation (ES) to determine whether UMN or LMN lesion was present. For detecting and testing the nerve stimulation points, a standardized mapping was developed and clinically applied. The relationship between the anatomical segmental spinal innervation and the innervation pattern tested by ES was determined. The data of 44 arms were analyzed. For PT, 19 arms showed an intact UMN, 18 arms an UMN lesion, and seven arms partial denervation. For FDPIII, three arms demonstrated an intact UMN, 26 arms an UMN lesion, 10 arms partial denervation, and five arms denervation. For FPL, two arms presented an intact UMN, 16 arms an UMN lesion, 12 arms partial denervation, and 14 arms denervation. A total of 20.1% ES tested muscles were partially denervated. In four patients, only one arm could be tested because of surgery-related limitations. According to the level of lesion and the segmental spinal innervation, most denervated muscles were present in the patient group C6 to C8. The ES, together with the developed mapping system, is reliable and can be recommended for standardized testing in surgery and rehabilitation. It offers the possibility to detect if and to what extent UMN and LMN lesions are present for the target muscles. It allows for refined pre-operative diagnostics and prognostics in spinal cord injury neurotization surgery.

Five years of follow-up of immediate quadriparesis caused by a large calcified mass in the ligamentum flavum: A case report.

INTRODUCTION: Calcification of ligamentum flavum (CLF) is an important cause of spinal stenosis and spinal cord compression. CLF does not usually induce immediate quadriparesis. Here we describe a rare case of immediate quadriparesis due to a large calcified mass containing liquids in the ligamentum flavum, which was easily confused with gout crystals. PATIENT CONCERNS: A 74-year-old Asian male felt progressive bilateral arm and leg weakness. On the fourth day, acute quadriparesis occurred. DIAGNOSIS: Coronal and sagittal computerized tomography (CT) and magnetic resonance imaging (MRI) showed a large circular mass in the left posterior part of the cervical 3/4 spinal canal, protruding into the canal, and occupying one-half of the spinal canal. INTERVENTIONS: Emergency laminectomy was performed at C3/4 level. The huge cyst was excised and 1 ml of white viscous liquid flowed out. OUTCOMES: After operation, CT and MRI showed a full laminectomy of C3/4 and complete decompression of the cervical spinal cord. Hematoxylin-eosin (HE) staining showed that large amounts of calcium was deposited around cystic tissues. Five-year follow-up after laminectomy showed good recovery. CONCLUSION: This case of immediate quadriparesis, caused by a large calcified mass containing fluid, is very rare. It should be at the earliest stage of calcification. Laminectomy is an effective treatment. This calcification was deceptive and was easily confused with gout crystals. It can help to understand the exact pathophysiology of CLF.

Permanent lesion to the corticospinal tract after therapy with capecitabine.

Capecitabine is an oral fluoropyrimidine used to treat solid tumours such as colorectal and breast cancer. A rare but severe side effect is capecitabine-induced leukoencephalopathy, including bilateral lesion to the corticospinal tract. However, neurological symptoms due to capecitabine treatment are usually reported to be reversible after discontinuation of capecitabine. Here, we present the case of a patient with bilateral degeneration of the corticospinal tract and progressive spastic tetraplegia after chemotherapy with capecitabine mimicking primary lateral sclerosis. Although therapy with capecitabine was ended, symptoms substantially worsened over the following years and the patient finally died from aspiration pneumonia almost 3 years after the application of capecitabine.

The Modified Keystone Flap for Pressure Ulcers: A Modification of the Keystone Flap With Rotation and Advancement.

INTRODUCTION: The reconstruction of extensive soft tissue defects from pressure ulcers is a great challenge. Resurfacing such defects with like tissues and minimizing morbidities are important. Here we present our surgical experience using a novel modified keystone flap for pressure ulcer patients. METHODS: We retrospectively reviewed the data of 13 consecutive cases reconstructed with the modified keystone flaps between March and December, 2017. The mean dimensions of the reconstructed defect were 7.7 × 6.5 cm, while the mean dimensions of the flap were 12.1 × 8.3 cm. RESULTS: Time efficient reconstructions with the modified keystone flap were performed without any following major complications. Minor wound dehiscence occurred in only one case, which soon healed with conservative management. CONCLUSIONS: Considering its simple design, reliable flap survival, minimal donor-site morbidity, optimal flap thickness, and evenly distributed surgical tension, our novel technique of the modified keystone flap is as an excellent surgical option for the reconstruction of pressure ulcers in the gluteal region.

Current Best Peripheral Nerve Transfers for Spinal Cord Injury.

LEARNING OBJECTIVES: After reviewing this article, the participant should be able to: 1. Understand the anatomy and pathophysiology of spinal cord injury and the resulting upper and lower motor neuron syndromes. 2. Recognize who may benefit from nerve transfers. 3. Understand the role of history, examination, imaging, and electrodiagnostics in the determination of time-sensitive lower motor neuron injury versus non-time-sensitive upper motor neuron injury. 4. Outline the surgical options and perioperative care for those undergoing nerve transfer and the expected outcomes in restoring shoulder, elbow, wrist, and hand function. SUMMARY: This article outlines how to localize and differentiate upper motor neuron from combined upper and lower motor neuron injury patterns in spinal cord injury by means of detailed history, physical examination, imaging, and electrodiagnostic studies to formulate appropriate surgical plans to restore function in this complex population.

Activity gains after upper limb surgery for spasticity in patients with spinal cord injury.

Spasticity is a common and increasingly prevalent secondary complication of spinal cord injury. The aim of the study was to evaluate patient-experienced gains in prioritized activities after surgery to reduce the effects of spasticity in upper limbs in tetraplegia. The study includes evaluation of 30 operations for 27 patients performed on hypertonic tetraplegic hands during 2007-2015 using the Canadian Occupational Performance Measure. Activity performance increased at both 6 months and 12 months by a mean of 3.0 and 2.9 points, respectively. Satisfaction increased by 3.3 and 3.4, respectively. All types of activities improved, with wheelchair manoeuvring as one of the highest rated. The intervention increased prioritized activity performance and persisted at least 12 months after surgery. Patients with mild upper limb impairment showed greater improvement after surgery. After operation, patients were able to perform 71% of their prioritized activities, which they could not perform before. Patients' satisfaction with the performance was high. LEVEL OF EVIDENCE: IV.

Delayed diagnosis of xanthogranulomatous pyelonephritis in a quadriplegic patient with uncontrolled cutaneous fistula: A case report.

RATIONALE: Xanthogranulomatous pyelonephritis (XGP) is a chronic destructive granulomatous inflammation that is characterized by urinary tract obstruction and invasion of the renal parenchyma. Although rare, XGP can lead to fatal complications, including perinephric inflammation, psoas abscess, and cutaneous fistula. PATIENT CONCERNS: A quadriplegic patient initially presented to the hospital with a chronic open wound and cutaneous fistula. DIAGNOSES: Abdominal computed tomography revealed a renal obstructing stone and enlarged right kidney with a perinephric fluid collection that communicated with the cutaneous fistula. INTERVENTIONS: The patient underwent a right nephrectomy at the department of urology. OUTCOMES: Two months after surgery, the patient was clinically well with no discharging fistula. LESSONS: The XGP accompanied by complications requires an immediate evaluation and early diagnosis. In this case, the diagnosis was delayed because the state of quadriplegia rendered no symptoms of XGP.

Quadriplegia post-coronary artery bypass graft surgery: an unusual case.

A 74-year-old Chinese man with triple-vessel coronary artery disease, developed quadriplegia following coronary artery bypass graft surgery, secondary to cervical disc anterior herniation and a C5-6 to T1 acute spinal hematoma. Preoperatively, no neurological signs or symptoms had been reported.

Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review.

INTRODUCTION: Autoantibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor and N-methyl-d-aspartate (NMDA) receptor are known to be the causes of autoimmune encephalitis particularly limbic encephalitis. The involvement of the peripheral nervous system is rarely reported. METHODS: We analyzed the serial nerve conduction studies of a previously reported case of anti-AMPA receptor encephalitis, who was presented with conscious disturbance and quadriplegia. Initial nerve conduction studies (NCS) revealed motor axonal polyneuropathy with active denervation. We also performed systematic review of similar cases with overlapped peripheral neuropathy and glutamate receptor encephalitis through Embase, PubMed, and MEDLINE. RESULTS: Follow-up NCS of the patient with anti-AMPA receptor encephalitis found reverse of the acute neuropathy, which was compatible with clinical recovery of quadriplegia. The systematic review identified 10 cases with overlapping peripheral neuropathy with anti-AMPA or NMDA receptor encephalitis. Motor or sensorimotor neuropathies were more common than pure sensory neuropathies. Anti-Hu, anti-amphiphysin, or anti-gnaglioside antibodies coexisted in some cases and might be associated with the peripheral symptoms. CONCLUSIONS: Both anti-AMPA and anti-NMDA receptor encephalitis could overlap with acute peripheral neuropathy. It is important to consider peripheral symptoms and perform diagnostic tests.

Nonsurgical management of an extensive spontaneous spinal epidural hematoma causing quadriplegia and respiratory distress in a choledocholithiasis patient: A case report.

RATIONALE: Spontaneous spinal epidural hematoma (SSEH) manifests from blood accumulating in the epidural space, compressing the spinal cord, and leading to acute neurological deficits. The disease's cloudy etiology and rarity contribute to dangerously suboptimal therapeutic principles. These neural deficits can be permanent, even fatal, if the SSEH is not treated in a timely and appropriate manner. Standard therapy is decompressive laminectomy, though nonsurgical management is a viable course of action for patients who meet a criterion that is continuously being refined. PATIENT CONCERNS: A 76-year-old woman on warfarin for a past pulmonary embolism presented to the emergency room with jaundice, myalgia, hematuria, neck pain, and an International Normalized Ratio (INR) of 14. Upon admission, she rapidly developed quadriplegia and respiratory distress that necessitated intubation. DIAGNOSES: T2-weighted magnetic resonance imaging (MRI) revealed an epidural space-occupying hyperintensity from C2 to S5 consistent with a spinal epidural hematoma. An incidental finding of dilated intrahepatic and common bile ducts prompted an endoscopic retrograde cholangiopancreatography, which demonstrated choledocholithiasis. INTERVENTIONS: The patient's INR was normalized with Vitamin K and Beriplex. Upon transfer to the surgical spine team for assessment of a possible intervention, the patient began to demonstrate recovery of neural functions. The ensuing sustained motor improvement motivated the team's preference for close neurologic monitoring and continued medical therapy over surgery. Thirteen hours after the onset of her symptoms, the patient was extubated. A sphincterotomy was later performed, removing 81 common bile duct stones. OUTCOMES: MRI demonstrated complete resorption of the SSEH and the patient maintained full neurological function at final follow-up. LESSONS: Nonsurgical management of SSEH should be considered in the context of early and sustained recovery. Severe initial neural deficit does not necessitate surgical decompression. Choledocholithiasis and subsequent Vitamin K deficiency, particularly when coupled with anticoagulant use, can increase INR and is a novel proposed risk factor for SSEH. Furthermore, coagulopathies should be medically corrected before surgical intervention within a given timeframe, as spontaneous recovery may manifest. This should be favored over surgery in patients demonstrating early and sustained recovery, as nonsurgical management is 25% more effective in achieving full recovery.

Permanent Quadriplegia Following Replacement of Voice Prosthesis.

The authors presented a patient with quadriplegia caused by cervical spine abscess following voice prosthesis replacement. The authors present the first reported permanent quadriplegia patient caused by voice prosthesis replacement. The authors wanted to emphasize that life-threatening complications may be faced during the replacement of voice prosthesis. Care should be taken during the replacement of voice prosthesis and if some problems have been faced during the procedure patients must be followed closely.

Rapidly Progressive Quadriplegia and Encephalopathy.

A woman aged 77 years was transferred to our neurocritical care unit for evaluation and treatment of rapidly progressive motor weakness and encephalopathy. Examination revealed an ability to follow simple commands only and abnormal movements, including myoclonus, tongue and orofacial dyskinesias, and opsoclonus. Imaging study findings were initially unremarkable, but when repeated, they demonstrated enhancement of the cauda equina nerve roots, trigeminal nerve, and pachymeninges. Cerebrospinal fluid examination revealed mildly elevated white blood cell count and protein levels. Serial electrodiagnostic testing demonstrated a rapidly progressive diffuse sensory motor axonopathy, and electroencephalogram findings progressed from generalized slowing to bilateral periodic lateralized epileptiform discharges. Critical details of her recent history prompted a diagnostic biopsy. Over time, the patient became completely unresponsive with no further abnormal movements and ultimately died. The differential diagnosis, pathological findings, and diagnosis are discussed with a brief review of a well-known yet rare diagnosis.