Spinal cord demyelination, cervical cord demyelination

Multi-parametric MRI (mpMRI) technology enables non-invasive and quantitative assessments of the structural, molecular, and functional characteristics of various neurological diseases. Despite the recognized importance of studying spinal cord pathology, mpMRI applications in spinal cord research have been somewhat limited, partly due to technical challenges associated with spine imaging. However, advances in imaging techniques and improved image quality now allow longitudinal investigations of a comprehensive range of spinal cord pathological features by exploiting different endogenous MRI contrasts...

Background and Purpose: Evidence has emerged for an association between degenerative disc disease (DDD) and multiple sclerosis (MS). The purpose of the current study is to determine the presence and extent of cervical DDD in young patients (age <35) with MS, an age cohort that is less well studied for these changes. Methods: Retrospective chart review of consecutive patients aged <35 referred from the local MS clinic who were MRI scanned between May 2005 and November 2014. 80 patients (51 female and 29 male) with MS of any type ranging between 16 and 32 years of age (average 26) were included...

Neural activity and learning lead to myelin sheath plasticity in the intact central nervous system (CNS), but this plasticity has not been well-studied after CNS injury. In the context of spinal cord injury (SCI), demyelination occurs at the lesion site and natural remyelination of surviving axons can take months. To determine if neural activity modulates myelin and axon plasticity in the injured, adult CNS, we electrically stimulated the contralesional motor cortex at 10 Hz to drive neural activity in the corticospinal tract of rats with sub-chronic spinal contusion injuries...

STUDY DESIGN: A retrospective case-control study. OBJECTIVE: To differentiate neurodegenerative diseases from compressive cervical myelopathy (CCM) using motor evoked potentials (MEPs). SUMMARY OF BACKGROUND DATA: When considering surgery for CCM, it may be necessary to differentiate the condition from a neurodegenerative disease. METHODS: A total of 30 healthy volunteers, 52 typical CCM patients with single-level compression of the spinal cord at C4-5 or C5-6, seven patients with amyotrophic lateral sclerosis (ALS), and 12 patients with demyelinating disease of the central nervous system (DDC), including 11 patients with multiple sclerosis and one patient with neuromyelitis optica spectrum disorder, formed our study population...

The anti-CV2/CRMP5 antibody is a well-characterized biomarker of paraneoplastic neurological syndrome. The anti-NF186 antibody is a recently discovered antibody associated with central or peripheral demyelination. The co-occurrence of these two antibodies has not been reported. Herein, we report a case with anti-CV2/CRMP5 and anti-NF186 antibodies in a 57-year-old male presenting with progressive numbness and weakness in his four limbs. At first admission, the spinal cord MRI showed a cervical cord demyelinating lesion and electrophysiological examination showed a mixed demyelinating and axonal polyneuropathy...

INTRODUCTION: Tract-specific myelopathies with distinctive imaging features are uncommon and typically occur with metabolic or paraneoplastic syndromes. We report a unique case of tract-specific myelopathy with neurosyphilis. CASE PRESENTATION: A 53-year-old male presented with a four-month history of flaccid quadriparesis, sensory loss, and bladder dysfunction. His MRIs revealed striking symmetric T2-weighted hyperintensities in the lateral corticospinal tracts and dorsal columns of the cervical spinal cord that extended rostrally into the pyramidal decussation and medial lemnisci of the medulla oblongata...

The progression and remission of cervical spondylotic myelopathy (CSM) are quite unpredictable due to the ambiguous pathomechanisms. Spontaneous functional recovery (SFR) has been commonly implicated in the natural course of incomplete acute spinal cord injury (SCI), while the evidence and underlying pathomechanisms of neurovascular unit (NVU) compensation involved in SFR remains poorly understood in CSM. In this study, we investigate whether compensatory change of NVU, in particular in the adjacent level of the compressive epicenter, is involved in the natural course of SFR, using an established experimental CSM model...

STUDY DESIGN: Systematic review. BACKGROUND: Although degenerative cervical myelopathy (DCM) is the most prevalent spinal cord condition worldwide, the pathophysiology remains poorly understood. Our objective was to evaluate existing histological findings of DCM on cadaveric human spinal cord tissue and explore their consistency with animal models. METHODS: MEDLINE and Embase were systematically searched (CRD42021281462) for primary research reporting on histological findings of DCM in human cadaveric spinal cord tissue...

Acute disseminated encephalomyelitis (ADEM), which is a disease that causes multifocal inflammatory demyelination of the central nervous system, occurs predominantly in children and young adults. We report an autopsy case of an elderly man with brainstem ADEM that progressed over a period of about 3 months. An 82-year-old man developed disturbance of consciousness, dysphagia, and ataxic gait over a period of about 3 months. He was admitted to another hospital for aspiration pneumonia and recovered but was transferred to our hospital due to prolonged disturbance of consciousness...

BACKGROUND: Following spinal cord injury (SCI), disease processes spread gradually along the spinal cord forming a spatial gradient with most pronounced changes located at the lesion site. However, the dynamics of this gradient in SCI patients is not established. OBJECTIVE: This study tracks the spatiotemporal dynamics of remote anterograde and retrograde spinal tract degeneration in the upper cervical cord following SCI over two years utilizing quantitative MRI...

While demyelination is the most common etiology of longitudinally extensive myelopathy, other causes are important to recognize. In this study, we present the case of a longitudinal cervical lesion with a very rare cause. We discuss the approach to the differential diagnosis and workup for longitudinal myelopathy. This clinical reasoning case also illustrates the anatomical relationship between symptomatic spinal cord lesions and nonsymptomatic intracranial etiologies.

Infection with COVID-19 (SARS-CoV-2) is associated with a variety of generalized and specific symptoms, including neurological complications of both the peripheral and central nervous systems. In this case report, we present the case of a previously healthy 55-year-old woman who was diagnosed with transverse myelitis following a previous infection with COVID-19. MRI showed progressive demyelination of the cervical and thoracic spinal cord, and cerebrospinal fluid (CSF) showed increased levels of protein and red blood cells and no markers of infection, including negative polymerase chain reaction (PCR) for COVID-19 antibodies...

Charcot-Marie-Tooth (CMT) disease is the most common inherited neuromuscular disorder causing a symmetric, slowly progressive distal neuropathy of the legs and arms. Tethered cord syndrome (TCS) encompasses a constellation of neurological, gastrointestinal, musculoskeletal, and urinary abnormalities resulting from spinal cord traction. The signs and symptoms of CMT and TCS may be very similar. Electrodiagnostic (EDX) studies are crucial in differentiating between these two conditions. We describe a 20-year-old woman with a history of low back pain, right leg tingling, and difficulty in walking...

BACKGROUND: Remote activity monitoring has the potential to evaluate real-world, motor function, and disability at home. The relationships of daily physical activity with spinal cord white matter and gray matter (GM) areas, multiple sclerosis (MS) disability and leg function, are unknown. OBJECTIVE: Evaluate the association of structural central nervous system pathology with ambulatory disability. METHODS: Fifty adults with progressive or relapsing MS with motor disability who could walk >2 minutes were assessed using clinician-evaluated, patient-reported outcomes, and quantitative brain and spinal cord magnetic resonance imaging (MRI) measures...

BACKGROUND: Spinal cord (SC) gray and white matter pathology plays a central role in multiple sclerosis (MS). OBJECTIVE: We aimed to investigate the extent, pattern, and clinical relevance of SC gray and white matter atrophy in vivo. METHODS: 39 relapsing-remitting patients (RRMS), 40 progressive MS patients (PMS), and 24 healthy controls (HC) were imaged at 3T using the averaged magnetization inversion recovery acquisitions sequence. Total and lesional cervical gray and white matter, and posterior (SCPH) and anterior horn (SCAH) areas were automatically quantified...

BACKGROUND: We evaluated imaging features suggestive of neurodegeneration within the brainstem and upper cervical spinal cord (UCSC) in non-progressive multiple sclerosis (MS). METHODS: Standardized 3-Tesla three-dimensional brain magnetic resonance imaging (MRI) studies were prospectively acquired. Rates of change in volume, surface texture, curvature were quantified at the pons and medulla-UCSC. Whole and regional brain volumes and T2-weighted lesion volumes were also quantified...

Neuromyelitis optica spectrum disorders are characterized by severe demyelination and axonal damage with autoimmune mechanisms, predominantly targeting the optic nerves and the spinal cord. Patients often test positive for anti-AQP4 antibodies, while some have anti-MOG antibodies. Double seropositivity has been described, with a variable prevalence (0 to 26%) dependent on the testing method. The clinical significance of double seropositivity remains unclear. We present the case of a 65-year-old patient, admitted to our clinic with optical neuritis, followed up approximately 10 days later by cervical myelitis, who tested positive for both anti-AQP4 and anti-MOG antibodies...

BACKGROUND AND PURPOSE: Around 5% of all Neuromyelitis Optica Spectrum Disorders (NMOSD) cases start before 18 years of age. Clinical and radiological manifestations of AQP4-IgG positive NMOSD were revised in 2015, and the importance of neuroimaging in the diagnosis is well recognized. Neuroimaging findings in pediatric-onset NMOSD were scarcely described, and longitudinal evaluation of NMOSD lesions was only accessed in a few adult-onset cohorts. METHODS: This study evaluated brain, spinal cord, and optic nerve MRI of sixteen pediatric-onset AQP4-IgG positive NMOSD through a qualitative evaluation of lesion evolution...

A 28-year-old man with Hirayama disease presented with a peculiar hand posture called "horn hand" ("main en corne"). The patient noticed that he could not extend his 3rd and 4th fingers when extending the fingers of his right hand 1 year prior to presentation. On neurological examination, the strength of the finger extension is severely weak in the 1st, 3rd and 4th fingers, causing a drop finger posture, but it is only moderately weak in the 2nd and 5th fingers, enabling him to sustain the extension finger posture...

We describe 3 cases of solitary sclerosis (SS), a rare condition characterized by a single inflammatory demyelinating lesion in the white matter of the brain or spinal cord. All patients had progressive limb motor impairment (patient 1, 66-year-old female: left spastic hemiparesis; patient 2, 39-year-old male: right spastic hemiparesis; patient 3, 42-year-old female: proximally predominant left upper limb weakness with amyotrophy and fasciculations). In all patients, MRI disclosed a single small T2-hyperintense demyelinating lesion: in the right anterior paramedian upper medulla, in the median-left paramedian anterior lower medulla, and in the left paramedian anterior cervical spinal cord at C4 level, respectively...