First Manifestation of AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder Associated with the COVID-19 mRNA Vaccine BNT162b2

BNT162b2 is one of the effective COVID-19 vaccines. However, some researchers have also reported that the vaccines caused some neurological complications. Here, we present a case of a 52-year-old female who developed aquaporin (AQP) 4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) fourteen days after the first dose of BNT162b2. She experienced pain of the neck, weakness of the left arm and leg, numbness of the left hand, and impaired temperature sensation of the right leg. MRI showed T2WI hyperintense lesions in the area postrema and cervical spinal cord ranging from C1 to C6 level, and Gd-enhanced lesions from C3 to C5 level; especially left lateral column was predominantly enhanced. Cell-based assays showed anti-AQP4 antibody (AQP4Ab) was positive. We diagnosed AQP4-IgG-positive NMOSD. After high-dose glucocorticoid therapy, she is showing improved symptoms. The present case was characterized by the findings that a Gd-enhanced lesion in the cervical cord localized dominantly at the left lateral column, consistent with the side of the shoulder where the vaccine was injected. Many studies suggested that AQP4-IgG-positive NMOSD development has multistep mechanisms following the blood-brain barrier (BBB) breakdown. We suspected that BNT162b2-associated immune responses lead to BBB disruptions. Through the limitedly damaged BBB, the plasma cells producing AQP4Abs might be recruited to CNS, and AQP4Abs might bind to the cervical cord and the area postrema. A large population-based study revealed that BNT162b2-associated complications were less likely to be observed than COVID-19 infectious symptoms. However, considering the present case, neurologists need to observe the conditions following vaccination.

Compressive Myelopathy Secondary to TRPV4 Skeletal Dysplasia: Spondylometaphyseal Dysplasia, Kozlowski Type

Transient receptor potential vanilloid 4 channel (TRPV4) gene mutations have been described in skeletal system and peripheral nervous system pathology. The case described here is a 9-year-old male child patient, born to a nonconsanguineous marriage with normal birth history who had difficulty in walking and stiffness of joints for the last 7 years, and progressive weakness of all four limbs and urine incontinence for 1 year following falls. Physical examination showed below-average weight and height and short trunk. Musculoskeletal examination revealed bony prominence bilaterally in the knee joints and contractures in knee and elbow joints with brachydactyly; muscle tone was increased, with brisk deep tendon reflexes. Skeletal survey showed platyspondyly with anterior beaking with metaphyseal dysplasia. Magnetic resonance imaging of the spine revealed atlantoaxial instability with hyperintense signal changes at a cervicomedullary junction and upper cervical cord with thinning and spinal canal stenosis suggestive of compressive myelopathy with platyspondyly and anterior beaking of the spine at cervical, thoracic and lumbar vertebrae. Exome sequencing revealed a heterozygous de novo variant c.2389G > A in exon 15 of TRPV4, which results in the amino acid substitution p.Glu797Lys in the encoded protein. The characteristics observed indicated spondylometaphyseal dysplasia, Kozlowski type (SMD-K). The child underwent surgical intervention for compressive myelopathy by reduction of atlantoaxial dislocation with C1 lateral mass and C2 pars fusion using rib graft and fixation using screws and rods. To conclude, for any child presenting with progressive kyphoscoliosis, short stature, platyspondyly, and metaphyseal changes, a diagnosis of SMD-K should be considered and the patient and family should be advised to avoid spinal injuries.

Brain, Spinal Cord and Labyrinthine Concussion in Sports

In this paper, we discuss the 3 types of concussion that occur in sports; brain concussion, spinal concussion (spinal cord neurapraxia), and labyrinthine (inner ear) concussion. Brain concussion data was collected from professional ice hockey players (Tohoku Free Blades, Japan) during 9 consecutive seasons. Spinal cord concussion and Labyrinthine concussion data was collected from athletes who sustained the injury in various sports including ice hockey. Material and methods The average incidence of brain concussion in professional ice hockey players was 2.0 per season. All 13 cases of spinal cord concussion were cervical cord concussion. Seven cases showed evidence of spinal cord compression; six cases had no radiological abnormality observed. All cases of labyrinthine concussion were sustained through a traumatic blow to the lateral aspect of the head. Results In any sports injury, all three of these concussions can co-exist and requires the team doctor to be at the site in order to fully assess the injury.

Hypoplasia of C1’s posterior arch: Is there an ideal anatomical classification?

Background: Congenital anomalies of the atlas are rare and usually occur in conjunction with other congenital variants. They include a wide spectrum of anomalies ranging from clefts to hypoplasia or aplasia of its arches that may contribute to spinal cord compressive syndrome. Case Description: A 54-year-old male presented with the sudden onset of a severe quadriparesis and loss of proprioception after a minor fall. The magnetic resonance (MR) scan showed cord compression at the C1 level attributed to C1 arch hypoplasia. Two months following a decompressive C1 laminectomy without fusion, and the patient was symptom free. Conclusion: Posterior C1 arch hypoplasia is a rare anomaly that can contribute to cervical cord compression and myelopathy. The optimal surgical management may include, as in this case, a posterior decompression without fusion.

Paralysis from an ear infection: a severe case of otitis externa leading to acute complete cervical cord syndrome

We report a case of a generally fit and well 54-year-old man who presented with a 2-day history of worsening left-sided otorrhea, headache, neck stiffness, vomiting and fever on the background of a 7-week history of otitis externa (OE). His condition progressed dramatically as he developed symptoms consistent with acute complete cervical cord syndrome with radiological evidence of skull base osteomyelitis, parapharyngeal, retropharyngeal and paravertebral abscesses and sigmoid sinus thrombus. Ultimately, he made a significant, although not complete, recovery. This case is unique in demonstrating how OE can develop into a potentially life threatening condition. It emphasises the importance of early diagnosis and treatment of OE, the recognition of ‘red flag’ symptoms and highlights the importance of a multidisciplinary team approach when managing complex complications of OE.

Spinal Cord Parenchyma Vascular Redistribution Underlies Hemodynamic and Neurophysiological Changes at Dynamic Neck Positions in Cervical Spondylotic Myelopathy

Cervical spondylotic myelopathy (CSM) is a degenerative condition of the spine that caused by static and dynamic compression of the spinal cord. However, the mechanisms of motor and somatosensory conduction, as well as pathophysiological changes at dynamic neck positions remain unclear. This study aims to investigate the interplay between neurophysiological and hemodynamic responses at dynamic neck positions in the CSM condition, and the pathological basis behind. We first demonstrated that CSM patients had more severe dynamic motor evoked potentials (DMEPs) deteriorations upon neck flexion than upon extension, while their dynamic somatosensory evoked potentials (DSSEPs) deteriorated to a similar degree upon extension and flexion. We therefore generated a CSM rat model which developed similar neurophysiological characteristics within a 4-week compression period. At 4 weeks-post-injury, these rats presented decreased spinal cord blood flow (SCBF) and oxygen saturation (SO2) at the compression site, especially upon cervical flexion. The dynamic change of DMEPs was significantly correlated with the change in SCBF from neutral to flexion, suggesting they were more sensitive to ischemia compared to DSSEPs. We further demonstrated significant vascular redistribution in the spinal cord parenchyma, caused by angiogenesis mainly concentrated in the anterior part of the compressed site. In addition, the comparative ratio of vascular densities at the anterior and posterior parts of the cord was significantly correlated with the perfusion decrease at neck flexion. This exploratory study revealed that the motor and somatosensory conductive functions of the cervical cord changed differently at dynamic neck positions in CSM conditions. Compared with somatosensory conduction, the motor conductive function of the cervical cord suffered more severe deteriorations upon cervical flexion, which could partly be attributed to its higher susceptibility to spinal cord ischemia. The uneven angiogenesis and vascular distribution in the spinal cord parenchyma might underlie the transient ischemia of the cord at flexion.

Cervical Cord Compression With Lower Limbs Sensory Disturbance Presentation: Three Case Reports and Literature Review

Background: Lower limb sensory disturbance presentation can be a false localizing cervical cord compressive myelopathy (CSM). It may lead to delayed or missed diagnosis, resulting in the wrong management plan, especially in the presence of concurrent lumbar lesions.Case presentation:Three Asian patients with lower limb sensory disturbances presentation were treated ineffectively in the lumbar. Magnetic resonance imaging (MRI) showed cervical disc herniation and cervical level spinal cord compression. Anterior cervical discectomy surgery and zero-p interbody fusion were performed. After operations, imagings showed that the spinal cord compression were relieved, and the lower limbs sensory disturbances were also relieved. Three-months follow-up after operation showed good recovery.Conclusions:These three cervical cord compression cases of lower limb sensory disturbance presentation were easily misdiagnosed with lumbar spondylosis. Anterior cervical discectomy and fusion operation had a good therapeutic effect. Therefore, cases that present with lower limb sensory disturbance, but in a non-radicular classical pattern, should always alert a suspicion of a possible cord compression cause at a higher level.

Intubation Biomechanics: Clinical Implications of Computational Modeling of Intervertebral Motion and Spinal Cord Strain during Tracheal Intubation in an Intact Cervical Spine

Background In a closed claims study, most patients experiencing cervical spinal cord injury had stable cervical spines. This raises two questions. First, in the presence of an intact (stable) cervical spine, are there tracheal intubation conditions in which cervical intervertebral motions exceed physiologically normal maximum values? Second, with an intact spine, are there tracheal intubation conditions in which potentially injurious cervical cord strains can occur? Methods This study utilized a computational model of the cervical spine and cord to predict intervertebral motions (rotation, translation) and cord strains (stretch, compression). Routine (Macintosh) intubation force conditions were defined by a specific application location (mid-C3 vertebral body), magnitude (48.8 N), and direction (70 degrees). A total of 48 intubation conditions were modeled: all combinations of 4 force locations (cephalad and caudad of routine), 4 magnitudes (50 to 200% of routine), and 3 directions (50, 70, and 90 degrees). Modeled maximum intervertebral motions were compared to motions reported in previous clinical studies of the range of voluntary cervical motion. Modeled peak cord strains were compared to potential strain injury thresholds. Results Modeled maximum intervertebral motions occurred with maximum force magnitude (97.6 N) and did not differ from physiologically normal maximum motion values. Peak tensile cord strains (stretch) did not exceed the potential injury threshold (0.14) in any of the 48 force conditions. Peak compressive strains exceeded the potential injury threshold (–0.20) in 3 of 48 conditions, all with maximum force magnitude applied in a nonroutine location. Conclusions With an intact cervical spine, even with application of twice the routine value of force magnitude, intervertebral motions during intubation did not exceed physiologically normal maximum values. However, under nonroutine high-force conditions, compressive strains exceeded potentially injurious values. In patients whose cords have less than normal tolerance to acute strain, compressive strains occurring with routine intubation forces may reach potentially injurious values. Editor’s Perspective What We Already Know about This Topic What This Article Tells Us That Is New