'White Cord Syndrome': A Rare Catastrophic Complication Following Anterior Cervical Discectomy and Fusion.

Background: 'White-cord syndrome' is an extremely rare entity following decompression of cervical cord in which post-operative reperfusion injury results in worsening of patient's neurology and MRI reveals signal changes in spinal cord in absence of cord compression. We wish to report a case of 'white-cord syndrome' following a 'routine' ACDF. Case Description: A 39-year-old woman with paresthesias and spastic quadriparesis was found to have C5-C6 PIVD on MRI. ACDF was performed at C5-C6, after which worsening of quadriparesis was noted, for which intravenous high-dose steroids were started. An urgent MRI was done, which revealed findings of white-cord syndrome, without compression on underlying cord. With conservative management, her ASIA grade improved from C to D and the features of white-cord syndrome disappeared on follow-up imaging. Conclusion: It is important for surgeons and patients to be aware of this rare but potentially catastrophic entity as this needs to be discussed while taking consent for surgery.

Rare case of real-time observation of paralytic deterioration after cervical dislocation in the hyperacute phase.

BACKGROUND: There have been no prior reports of real-time detailed records leading to complete quadriplegia immediately after fracture dislocation in high-energy trauma. Here, we report a case of cervical dislocation in which the deterioration to complete motor paralysis (modified Frankel B1) and complete recovery (Frankel E) could be monitored in real time after reduction in the hyperacute phase. CASE PRESENTATION: A 65-year-old man was involved in a car accident and sustained a dislocation at the C5/6 level (Allen-Ferguson classification: distractive flexion injury stage IV). His paralysis gradually deteriorated from Frankel D to C 2 hours after the injury and from Frankl C to B 5 hours after the injury. His final neurological status immediately before reduction was Frankel B1 (complete motor paralysis with sensation only in the perianal region). Reduction was completed within 6 h and 5 min after injury, and spinal fusion was subsequently performed. The patient exhibited rapid motor recovery immediately after surgery, and was able to walk independently on postoperative day 14. CONCLUSIONS: This case suggests that there is a mixture of cases in which the spinal cord has not been catastrophically damaged, even if the patient has complete motor paralysis. Prompt reduction has the potential to improve neurological function in such cases.

[Untreated os odontoideum may cause tetraplegia].

This review summarises the present, limited, knowledge of os odontoideum (OO). The prevalence is unknown, and the aetiology is widely debated, though irrelevant to management of the disease. Surgery of symptomatic patients is advocated, as there is more uncertainty about the treatment of asymptomatic patients. Diagnosis is obtained with plain X-ray. However, nowadays MRI and CT scanning are recommended for further clarification and judgement of the severity. Knowledge of OO is important to minimise misjudgement and consequently inappropriate treatment. It is recommended, that patients are examined by highly specialised spine doctors before further cervical manipulation due to the high risk of instability with potentially severe consequences.

Spinal cord injury in infancy: activity-based therapy impact on health, function, and quality of life in chronic injury.

INTRODUCTION: Spinal cord injury (SCI) in infancy magnifies the complexity of a devastating diagnosis. Children injured so young have high incidences of scoliosis, hip dysplasia, and respiratory complications leading to poor health and outcomes. We report the medical history, progression of rehabilitation, usual care and activity-based therapy, and outcomes for a child injured in infancy. Activity-based therapy (ABT) aims to activate the neuromuscular system above and below the lesion through daily, task-specific training to improve the neuromuscular capacity, and outcomes for children with acquired SCI. CASE PRESENTATION: A 3-month-old infant suffered a cervical SCI from a surgical complication with resultant tetraplegia. Until age 3, her medical complications included scoliosis, kyphosis, and pneumonia. Even with extensive physical and occupational therapy, she was fully dependent on caregivers for mobility and unable to roll, come to sit, sit, stand or walk. She initiated ABT at ~3 years old, participating for 8 months. The child's overall neuromuscular capacity improved significantly, especially for head and trunk control, contributing to major advances in respiratory health, novel engagement with her environment, and improved physical abilities. DISCUSSION: From injury during infancy until 3 years old, this child's health, abilities, and complications were consistent with the predicted path of early-onset SCI. Due to her age at injury, severity and chronicity of injury, she demonstrated unexpected, meaningful changes in her neuromuscular capacity during and post-ABT associated with improved health, function and quality of life for herself and her caregivers.

Spinal Epidural Hematoma Associated with Epidural Metastasis After Minor Trauma.

BACKGROUND: Spinal epidural hematoma associated with epidural metastasis is very rare. Epidural hematoma is unusual in itself, and metastatic epidural tumors do not commonly occur. CASE DESCRIPTION: A 76-year-old man with a medical history of untreated stage III rectal cancer and chronic obstructive pulmonary disease underwent emergency spine surgery for acute development of severe quadriplegia due to cervical epidural hematoma after minor indirect trauma. Hemorrhagic lesions, such as yellow ligaments, were accompanied by hematomas that adhered to the dura mater and were confirmed on pathology to be small cell carcinoma. Some nodules were detected in the right pulmonary hilar lesion and pleural lesion on computed tomography, and stage IV small cell carcinoma was diagnosed. CONCLUSIONS: To our knowledge, spinal epidural hematoma accompanied by epidural metastasis has not previously been reported. We hypothesized that tissue invasion of malignant neoplasms may cause hemorrhagic conditions. The presence of a tumor should therefore be considered in the differential diagnosis of cases of epidural hematoma.

Bilateral Accessory C1 and C2 Facet Joints; Clinical, Neuroradiological and Peroperative Findings in an Adult with Quadriparesis

Objectives Accessory C1 and C2 facet joints are very rare. Only few cases were reported in the literature.We report a case of bilateral accessory facets in an adult with special attention to clinical, neuroradiological, as well as peroperative findings. Case report A 37-year-old male presented with progressive quadriparesis. Radiology revealed bilateral posterior accessory C1 and C2 facet joints compressing the spinal cord with craniovertebral junction (CVJ) instability. Both accessory C1 and C2 facets with the posterior arch of the C1 were removed. Lateral mass screws and plates fixation at the C1 and C2 level, as well as fusion, were performed. Postoperatively, the patient recovered well. Conclusion In accessory C1 and C2 facet joints, when symptomatic, neuroradiological findings can guide to the proper diagnosis, to pathological understanding, and, ultimately, to management strategy.

Iatrogenic spinal cord injury with tetraplegia after an elective non-spine surgery with underlying undiagnosed cervical spondylotic myelopathy: Literature review and case report.

Cervical spondylotic myelopathy (CSM) can predispose to acute spinal cord injury and post-operative quadriparesis during non-spine procedures, although few accounts of this phenomenon exist within the literature. To the best of our knowledge, there are only 18 cases reported in the literature including ours with spinal cord injury following elective non-spine surgery with undiagnosed CSM. Due to multifactorial pathophysiology, the maintenance of cervical cord neutrality is not sufficient to ensure that these patients will not sustain cord injury intraoperatively as this solely addresses the role of static factors. Vigilance to factors affecting cord perfusion and vascular compromise, such as the mean arterial pressure (MAP), is imperative. Additionally, further studies should evaluate the role of positioning in the myelopathic patient and whether the steep Trendelenburg position, commonly used in robotic surgeries, contributes to spinal cord venous congestion and resultant cord ischemia in these patients given their baseline stenotic canal. This review illustrates the importance of having a heightened awareness of this common degenerative condition in our aging patient population, often a forgotten underlying medical comorbidity.

Cervical epidural hematoma after spinal manipulation therapy: a case report.

BACKGROUND: Cervical spinal manipulation therapy is a common non-invasive treatment for neck pain and stiffness, and has been widely used in the population. However, most people do not pay attention to the potential risks of neck manipulation, such as ligament damage, fractures, and spinal cord injuries. Epidural hematoma is a disease in which blood accumulates in the epidural space of the vertebral body. This disease is usually caused by trauma or iatrogenic surgery, and may be associated with blood coagulopathies, neoplasms, or degenerative spinal disease. Reports of epidural hematoma caused by cervical spinal manipulation are rare. CASE PRESENTATION: We present the case of a patient with tetraplegia and spinal shock after neck manipulation. A physical examination of the patient on admission found tenderness in the neck and increased muscle tension in both upper limbs. The superficial sensation of the upper limb disappeared, but the deep sensation still remained. The lower extremity had 0/5 power on both sides. The sensation below the T2 level completely disappeared. A cervical magnetic resonance imaging scan showed an acute posterior epidural hematoma from the C3-T3 vertebrae. Ultimately, the patient underwent emergency hematoma removal and showed partial improvement in symptoms of paralysis during follow-up. CONCLUSIONS: Although spinal manipulation is simple and neck pain is common and recurrent in the general population, the basic condition and disease history of patients should be determined before manipulation. For high-risk patients, caution should be applied for cervical spinal manipulation or it should be prohibited. For a suspected hematoma, MRI should be used at an early stage to diagnose and locate the hematoma.

An exoskeleton controlled by an epidural wireless brain-machine interface in a tetraplegic patient: a proof-of-concept demonstration.

BACKGROUND: Approximately 20% of traumatic cervical spinal cord injuries result in tetraplegia. Neuroprosthetics are being developed to manage this condition and thus improve the lives of patients. We aimed to test the feasibility of a semi-invasive technique that uses brain signals to drive an exoskeleton. METHODS: We recruited two participants at Clinatec research centre, associated with Grenoble University Hospital, Grenoble, France, into our ongoing clinical trial. Inclusion criteria were age 18-45 years, stability of neurological deficits, a need for additional mobility expressed by the patient, ambulatory or hospitalised monitoring, registration in the French social security system, and signed informed consent. The exclusion criteria were previous brain surgery, anticoagulant treatments, neuropsychological sequelae, depression, substance dependence or misuse, and contraindications to magnetoencephalography (MEG), EEG, or MRI. One participant was excluded because of a technical problem with the implants. The remaining participant was a 28-year-old man, who had tetraplegia following a C4-C5 spinal cord injury. Two bilateral wireless epidural recorders, each with 64 electrodes, were implanted over the upper limb sensorimotor areas of the brain. Epidural electrocorticographic (ECoG) signals were processed online by an adaptive decoding algorithm to send commands to effectors (virtual avatar or exoskeleton). Throughout the 24 months of the study, the patient did various mental tasks to progressively increase the number of degrees of freedom. FINDINGS: Between June 12, 2017, and July 21, 2019, the patient cortically controlled a programme that simulated walking and made bimanual, multi-joint, upper-limb movements with eight degrees of freedom during various reach-and-touch tasks and wrist rotations, using a virtual avatar at home (64·0% [SD 5·1] success) or an exoskeleton in the laboratory (70·9% [11·6] success). Compared with microelectrodes, epidural ECoG is semi-invasive and has similar efficiency. The decoding models were reusable for up to approximately 7 weeks without recalibration. INTERPRETATION: These results showed long-term (24-month) activation of a four-limb neuroprosthetic exoskeleton by a complete brain-machine interface system using continuous, online epidural ECoG to decode brain activity in a tetraplegic patient. Up to eight degrees of freedom could be simultaneously controlled using a unique model, which was reusable without recalibration for up to about 7 weeks. FUNDING: French Atomic Energy Commission, French Ministry of Health, Edmond J Safra Philanthropic Foundation, Fondation Motrice, Fondation Nanosciences, Institut Carnot, Fonds de Dotation Clinatec.

Elongated Clivus with Deficient Anterior Atlantal Arch and Congenital Posterior Atlantooccipital Dislocation: Pathoembryology and Management Nuances of a Rare Form of Proatlas Segmentation Anomaly.

BACKGROUND: Proatlas segmentation anomalies represent a rare subset of congenital craniovertebral junction anomalies. In this condition, the structures originating from the proatlas, such as the clivus, occipital condyles, foramen magnum rim, and odontoid tip, may demonstrate congenital anomalies, usually without any spinal instability. Elongated clivus, as a result of nonsegmentation of the odontoid tip from the rest of the proatlas, has been reported before to cause ventral spinal cord compression. We report such a case with certain unreported other associations and explore the pathoembryology and management options of such complex anomalies. CASE DESCRIPTION: An 8-year old girl presented with a 2-year history of progressive spastic quadriparesis. On neuroimaging, the anterior arch of the atlas was deficient, the odontoid process was foreshortened, and the clivus was elongated, encroaching into the spinal canal leading to ventral spinal cord compression. Additionally, there was rotatory posterior dislocation of the occipital condyles onto the posterior atlantal arch and vertebral artery anomaly. This patient underwent transoral decompression followed by occipitocervical fusion using rods and screws with satisfactory results. CONCLUSIONS: Proatlas anomalies are rare, varied, and often subtle enough to go unrecognized. Knowledge of the embryology and its aberrations is necessary to understand these anomalies. Our case describes a rare form of bony anomalies pertaining to the fate of the proatlas with accompanying atlanto-occipital dislocation.

Multiple Hereditary Exostoses with Tetraparesis Due To Cervical Spine Osteochondroma.

This is a case report of a 34-year-old man with hereditary multiple exostoses who presented with gradual tetraparesis. Neuroimaging evaluation revealed an important posterior spinal cord compression by a C3 bony formation. Following posterior microsurgical decompression, the patient recovered nearly completely. Histopathologic examination of the intraspinal lesion confirmed the diagnosis of benign osteochondroma. The patient returned to a normal life without evidence of recurrence at 2-year follow-up.

Adaptive motor cortex plasticity following grip reconstruction in individuals with tetraplegia.

BACKGROUND: Tendon transfer is a surgical technique for restoring upper limb motor control in patients with cervical spinal cord injuries (SCI), and offers a rare window into cortical neuroplasticity following regained arm and hand function. OBJECTIVE: Here, we aimed to examine neuroplasticity mechanisms related to re-established voluntary motor control of thumb flexion following tendon transfer. METHODS: We used functional Magnetic Resonance Imaging (fMRI) to test the hypothesis that restored limb control following tendon transfer is mediated by activation of that limb's area of the primary motor cortex. We examined six individuals with tetraplegia who underwent right-sided surgical grip reconstruction at Sahlgrenska University Hospital, Sweden. All were right-handed males, with a SCI at the C6 or C7 level, and a mean age of 40 years (range = 31-48). The average number of years elapsed since the SCI was 13 (range = 6-26). Six right-handed gender- and age-matched control subjects were included (mean age 39 years, range = 29-46). Restoration of active thumb flexion in patients was achieved by surgical transfer of one of the functioning elbow flexors (brachioradialis), to the paralyzed thumb flexor (flexor pollicis longus). We studied fMRI responses to isometric right-sided elbow flexion and key pinch, and examined the cortical representations within the left hemisphere somatomotor cortex a minimum of one year after surgery. RESULTS: Cortical activations elicited by elbow flexion did not differ in topography between patients and control participants. However, in contrast to control participants, patients' cortical thumb flexion activations were not topographically distinct from their elbow flexion activations. CONCLUSION: This result speaks against a topographic reorganization in which the thumb region regains thumb control following surgical tendon transfer. Instead, our findings suggest a neuroplastic mechanism in which motor cortex resources previously dedicated to elbow flexion adapt to control the thumb.

Traumatic Atlantoaxial Spondyloptosis Associated with Displaced Odontoid Fracture: Complete Reduction via Posterior Approach Using "Joint Remodeling" Technique.

BACKGROUND: Atlantoaxial spondyloptosis (AAS), which is defined as complete displacement of facets of atlas anterior to the facets of axis such that there is no contact between the 2 articulating surfaces, is an extremely rare manifestation of atlantoaxial instability. The reason for an extreme rarity of traumatic AAS is probably the severity of injury in traumatic AAS that is usually incompatible to life. It represents the most severe form of atlantoaxial dislocation, and complete reduction in such a case presents a real technical challenge because of the interlocking of C1-C2 facets. Cranial traction fails to achieve reduction in such cases. CASE DESCRIPTION: In this report, we describe a case of traumatic odontoid fracture associated with AAS and discuss our technique of complete reduction of deformity via posterior approach. An 11-year-old child presented to us 4 months after a road traffic accident with progressive spastic quadriparesis. On evaluation, displaced type II odontoid fracture with AAS was detected. The patient underwent surgery via posterior approach. The techniques of "joint manipulation" and "joint remodeling" were used to achieve complete reduction of spondyloptosis. Postoperative imaging showed complete reduction of deformity. The patient also improved neurologically after surgery. CONCLUSION: This case report aims to present the ability of "joint manipulation" and "joint remodeling" techniques in achieving excellent reduction in even one of the most difficult post-traumatic deformities that affect the craniovertebral junction.

Extensive Mirror-Image Neurofibromas of Entire Spine Resulting in Spastic Tetraplegia.

Neurofibromatosis 1 (NF1) is associated with increased incidence of spinal tumors including neurofibromas. The majority of NF1-associated spine neurofibromas are asymptomatic; however, a minority of patients will experience neurologic symptoms that can range from mild paresthesia, radiculopathy, myelopathy, and focal weakness to quadriplegia in extreme cases. We present a 21-year-old male diagnosed with NF1 in infancy and followed for multiple mirror-image neurofibromas involving the entire spine. He was asymptomatic until age 14 when he developed neck pain and progressive tetraplegia with magnetic resonance imaging showing severe cord compression secondary to bilateral C2 neurofibromas. Emergent cervical decompression was performed at C1-C3 along with debulking of bilateral neurofibromas. Postoperatively he regained full strength with no signs of myelopathy several years postoperatively. This case demonstrates a dramatic neuroimaging finding and emphasizes the potential for significant neurologic deterioration in previously asymptomatic NF1 patients, highlighting the need for long-term follow-up.

Ewing Sarcoma of the Cervical Epidural Space Presenting with Tetraplegia: Case Report and Review of Literature.

BACKGROUND: Ewing sarcoma (ES) is among the most frequented extremity osseous tumor in childhood. It was first described by James Ewing as diffuse endotheliomas in 1921. The name Ewing sarcoma was coined by Oberling in 1928 as a tribute to the legend who described this disease. ES exists in osseous and extraosseous forms. It shares much of its molecular typing with primitive neuroectodermal tumor (PNET); hence, they are regarded as different ends of the same molecular spectrum. ES, extraosseous ES, PNET, and Askins tumor are the other members of this family termed the Ewing sarcoma family of tumors. Extraosseous ES has been described in various locations of the spine, but its occurrence in the cervical epidural region is uncommon. CASE DESCRIPTION: A 12-year-old child presented to us with neck pain and progressive weakness of all 4 limbs. She was investigated and found to have a highly vascular lesion in the cervical epidural region extending from C2 to the C4 region. Embolization therapy was unsuccessful. She deteriorated and eventually had to undergo emergency surgery to decompress the spinal cord. The lesion was biopsied and diagnosed as extraosseous ES. After radiotherapy and chemotherapy, the patient regained most of the power in her limbs. CONCLUSION: Extraosseous ES is a rare tumor of the cervical cord in this age group. We report this case to highlight the difficulties encountered in the management of this variant.

Is neck massage safe? A rare case of tetraplegia and spinal shock after neck manipulation.

Neck manipulation is associated with spinal cord injury. However, occurrence of such cases is infrequent. This article presents a 33-year-old gentleman who sustained acute tetraplegia after neck manipulation. The aim of this case report is to create awareness that vigorous neck manipulation could cause injury to the spinal cord.

Antimalarial myopathy in a systemic lupus erythematosus patient with quadriparesis and seizures: a case-based review.

Weakness, seizures, and encephalopathy have a broad differential diagnosis in patients with systemic lupus erythematosus (SLE). We present a case of a 26-year-old female with a recent diagnosis of SLE who experienced a clinical deterioration with quadriparesis, seizures, and encephalopathy. Her quadriparesis was found to be secondary to biopsy-proven hydroxychloroquine-induced myopathy with concomitant inflammatory myopathy. Her seizures and encephalopathy were suspected to be multifactorial in the setting of sepsis and critical illness with possible contributions from neuropsychiatric manifestations of SLE and macrophage activation syndrome. She experienced a dramatic clinical recovery with discontinuation of hydroxychloroquine, treatment of lupus disease activity with mycophenolate mofetil and prednisone, and antibiotic treatment for methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia. This case-based review provides a systematic approach to quadriparesis, seizures, and encephalopathy in patients with SLE and an evidence-based discussion of antimalarial myopathy, which is of critical importance given the widespread use of antimalarial medications for rheumatologic diseases.

Quadriplegia caused by an epidural abscess occurring at the same level of cervical destructive spondyloarthropathy: a case report.

BACKGROUND: Destructive spondyloarthropathy (DSA) is one of the major complications in patients undergoing long-term hemodialysis. To the best of our knowledge, an epidural abscess occurring at the level of preexisting cervical DSA has not been well described in the literature. We report a unique case of quadriplegia caused by an epidural abscess occurring at the same level of preexisting cervical DSA. CASE PRESENTATION: A 49-year-old woman was transferred to our emergency department with 5 days of sepsis, drowsy mental status, and quadriplegia below the C5 level. The patient had a medical history of hemodialysis for 10 years. Magnetic resonance imaging showed spinal cord compression by an epidural abscess at the level of preexisting cervical DSA. Blood culture revealed methicillin-sensitive Staphylococcus aureus. Infection of the arteriovenous (AV) shunt was considered as the primary focus of sepsis and pyogenic spondylitis. We performed an emergent open door laminoplasty and the vascular team debrided the infected AV shunt site. Approximately 8 months after surgery, the patient was able to perform activities of daily living somewhat independently. CONCLUSIONS: Emergent surgical decompression and intensive medical care led to successful recovery from a septic and quadriplegic state in this patient. When diagnosing a patient who has undergone long-term hemodialysis presenting with neurologic deficits, the possibility of infectious spondylitis at the same level as DSA should be considered.