Cervical Cord Atrophy Research Articles

Progressive cervical cord atrophy parallels cognitive decline in Alzheimer’s disease

Alzheimer’s disease (AD) is characterized by progressive episodic memory dysfunction. A prominent hallmark of AD is gradual brain atrophy. Despite extensive research on brain pathology, the understanding of spinal cord pathology in AD and its association with cognitive decline remains understudied. We analyzed serial magnetic resonance imaging (MRI) scans from the ADNI data repository to assess whether progressive cord atrophy is associated with clinical worsening. Cervical cord morphometry was measured in 45 patients and 49 cognitively normal controls (CN) at two time points over 1.5 years. Regression analysis examined associations between cord atrophy rate and cognitive worsening. Cognitive and functional activity performance declined in patients during follow-up. Compared with controls, patients showed a greater rate of decline of the anterior–posterior width of the cross-sectional cord area per month (− 0.12%, p = 0.036). Worsening in the mini-mental state examination (MMSE), clinical dementia rating (CDR), and functional assessment questionnaire (FAQ) was associated with faster rates of cord atrophy (MMSE: r = 0.320, p = 0.037; CDR: r = − 0.361, p = 0.017; FAQ: r = − 0.398, p = 0.029). Progressive cord atrophy occurs in AD patients; its rate over time being associated with cognitive and functional activity decline.

Evaluation of cervical spinal cord atrophy using a modified SIENA approach

Spinal cord (SC) atrophy obtained from structural magnetic resonance imaging has gained relevance as an indicator of neurodegeneration in various neurological disorders. The common method to assess SC atrophy is by comparing numerical differences of the cross-sectional spinal cord area (CSA) between time points. However, this indirect approach leads to considerable variability in the obtained results. Studies showed that this limitation can be overcome by using a registration-based technique.The present study introduces the Structural Image Evaluation using Normalization of Atrophy on the Spinal Cord (SIENA-SC), which is an adapted version of the original SIENA method, designed to directly calculate the percentage of SC volume change over time from clinical brain MRI acquired with an extended field of view to cover the superior part of the cervical SC.In this work, we compared SIENA-SC with the Generalized Boundary Shift Integral (GBSI) and the CSA change. On a scan-rescan dataset, SIENA-SC was shown to have the lowest measurement error than the other two methods. When comparing a group of 190 Healthy Controls with a group of 65 Multiple Sclerosis patients, SIENA-SC provided significantly higher yearly rates of atrophy in patients than in controls and a lower sample size when measured for treatment effect sizes of 50%, 30% and 10%.Our findings indicate that SIENA-SC is a robust, reproducible, and sensitive approach for assessing longitudinal changes in spinal cord volume, providing neuroscientists with an accessible and automated tool able to reduce the need for manual intervention and minimize variability in measurements.

Optimization of cervical cord synthetic T1-weighted MRI for enhancing clinical application in neurodegenerative spinal cord disorders

Abstract Synthetic MRI offers the advantage of reducing acquisition time and enhancing flexibility through the reconstruction of various contrast weightings from a single set of MRI scans. However, the use of synthetic T1-weighted (synT1-w) MRI can lead to potentially biased measurements of the cross-sectional area (CSA) in the spinal cord when compared to conventionally acquired T1-weighted MRI. This disparity can have implications for comparability and sensitivity of MRI in assessing disease progression or treatment effects in neurodegenerative spinal cord disorders. Thus, this study aimed at improving the accuracy (i.e., difference between synthetic and acquired MRI) of cervical cord CSA measurements (C1-C3 level) based on synT1-w MRI implementing a longitudinal data set acquired from 23 acute spinal cord injury (SCI) patients and 21 healthy controls over 2 years. Moreover, the validity of using synT1-w MRI for tracking cervical cord atrophy following SCI over 2 years was verified. SynT1-w images were reconstructed from quantitative maps of proton density, longitudinal, and effective transverse relaxation rates derived from a multi-parameter mapping protocol. The results showed a minimal bias of -0.31 mm2 (-0.5%) in CSA measurements based on synT1-w compared to acquired MRI. Estimates of atrophy rates and average CSA were comparable between synthetic and acquired MRI. A sample size estimation for detecting treatment effects on CSA atrophy after 2 years following SCI revealed that the required sample size is reduced by 13.5% using synT1-w instead of acquired MRI. This study shows high accuracy of synT1-w MRI and demonstrates its applicability in clinical studies for optimizing long MRI protocols.

Brain and cervical spinal cord MRI correlates of sensorimotor impairment in patients with multiple sclerosis.

Cervical spinal cord (cSC) lesions and atrophy contribute to disability in multiple sclerosis (MS), but associations with specific sensorimotor dysfunction require further exploration. To investigate the associations of brain and cSC magnetic resonance imaging (MRI) measures with sensorimotor impairment in MS. One hundred fifty-one MS patients and 69 healthy controls underwent 3T MRI and clinical assessments including Expanded Disability Status Scale (EDSS), 9-hole peg test (9-HPT), finger tapping test (FTT), timed 25-foot walk test (T25FWT), and vibration detection threshold (VDT). Random forest ranked brain (T2-hyperintense lesion volume (T2-LV) and normalized deep gray matter (GM), cortical and white matter (WM) volumes) and cSC (T2-LV and total, GM, and WM cross-sectional areas (CSAs) at C2/C3 level) MRI measures relevance in explaining EDSS milestones (EDSS ⩾3.0, ⩾4.0, and ⩾6.0), VDT, pyramidal and sensory functional systems (P-FS and S-FS ⩾2), and motor tests impairment. Various combinations of brain and cSC MRI measures explained EDSS milestones (area under the curve (AUC) =0.879-0.900), VDT (R2 = 0.194), and impaired P-FS (AUC = 0.820), S-FS (AUC = 0.795), 9-HPT (AUC = 0.793), FTT (AUC = 0.740), and T25FWT (AUC = 0.825). cSC GM CSA was the most informative feature for all outcomes, except 9-HPT. cSC MRI measures, especially GM CSA, explain EDSS and sensorimotor dysfunction better than brain measures in MS.

The relationship between the structural changes in the cervical spinal cord and sensorimotor function of children with thoracolumbar spinal cord injury (TLSCI)

Study designCross-sectional study.ObjectivesTo study the relationship between the structural changes in the cervical spinal cord (C2/3 level) and the sensorimotor function of children with traumatic thoracolumbar spinal cord injury (TLSCI) and to discover objective imaging biomarkers to evaluate its functional status.SettingXuanwu Hospital, Capital Medical University, China; Beijing Key Laboratory of Magnetic Resonance Imaging and Brain Informatics, China.Methods30 children (age range 5–13 years) with TLSCI and 11 typically developing (TD) children (age range 6–12 years) were recruited in this study. Based on whether there is preserved motor function below the neurological level of injury (NLI), the children with TLSCI are divided into the AIS A/B group (motor complete) and the AIS C/D group (motor incomplete). A Siemens Verio 3.0 T MR scanner was used to acquire 3D high-resolution anatomic scans covering the head and upper cervical spinal cord. Morphologic parameters of the spinal cord at the C2/3 level, including cross-sectional area (CSA), anterior-posterior width (APW), and left-right width (LRW) were obtained using the spinal cord toolbox (SCT; https://www.nitrc.org/projects/sct). Correlation analyses were performed to compare the morphologic spinal cord parameters and clinical scores determined by the International Standard for Neurological Classification of Spinal Cord Injuries (ISNCSCI) examination.ResultsCSA and LRW in the AIS A/B group were significantly lower than those in the TD group and the AIS C/D group. LRW was the most sensitive imaging biomarker to differentiate the AIS A/B group from the AIS C/D group. Both CSA and APW were positively correlated with ISNCSCI sensory scores.ConclusionsQuantitative measurement of the morphologic spinal cord parameters of the cervical spinal cord can be used as an objective imaging biomarker to evaluate the neurological function of children with TLSCI. Cervical spinal cord atrophy in children after TLSCI was correlated with clinical grading; CSA and APW can reflect sensory function. Meanwhile, LRW has the potential to be an objective imaging biomarker for evaluating motor function preservation.

Upper Cervical Cord Atrophy Predicts Disability Through Motor Clinical Impairment in Multiple Sclerosis Patients (P8-11.017)

Upper Cervical Cord Atrophy Predicts Disability Through Motor Clinical Impairment in Multiple Sclerosis Patients (P8-11.017)

Systemic inflammation associates with and precedes cord atrophy in progressive multiple sclerosis.

In preclinical models of multiple sclerosis, systemic inflammation has an impact on the compartmentalized inflammatory process within the central nervous system and results in axonal loss. It remains to be shown whether this is the case in humans, specifically whether systemic inflammation contributes to spinal cord or brain atrophy in multiple sclerosis. Hence, an observational longitudinal study was conducted to delineate the relationship between systemic inflammation and atrophy using magnetic resonance imaging: the SIMS (Systemic Inflammation in Multiple Sclerosis) study. Systemic inflammation and progression were assessed in people with progressive multiple sclerosis (n = 50) over two and a half years. Eligibility criteria included: (i) primary or secondary progressive multiple sclerosis; (ii) age ≤ 70; and (iii) Expanded Disability Status Scale ≤ 6.5. First morning urine was collected weekly to quantify systemic inflammation by measuring the urinary neopterin-to-creatinine ratio using a validated ultra-performance liquid chromatography mass spectrometry technique. The urinary neopterin-to-creatinine ratio temporal profile was characterized by short-term responses overlaid on a background level of inflammation, so these two distinct processes were considered as separate variables: background inflammation and inflammatory response. Participants underwent MRI at the start and end of the study, to measure cervical spinal cord and brain atrophy. Brain and cervical cord atrophy occurred on the study, but the most striking change was seen in the cervical spinal cord, in keeping with the corticospinal tract involvement that is typical of progressive disease. Systemic inflammation predicted cervical cord atrophy. An association with brain atrophy was not observed in this cohort. A time lag between systemic inflammation and cord atrophy was evident, suggesting but not proving causation. The association of the inflammatory response with cord atrophy depended on the level of background inflammation, in keeping with experimental data in preclinical models where the effects of a systemic inflammatory challenge on tissue injury depended on prior exposure to inflammation. A higher inflammatory response was associated with accelerated cord atrophy in the presence of background systemic inflammation below the median for the study population. Higher background inflammation, while associated with cervical cord atrophy itself, subdued the association of the inflammatory response with cord atrophy. Findings were robust to sensitivity analyses adjusting for potential confounders and excluding cases with new lesion formation. In conclusion, systemic inflammation associates with, and precedes, multiple sclerosis progression. Further work is needed to prove causation since targeting systemic inflammation may offer novel treatment strategies for slowing neurodegeneration in multiple sclerosis.

Characteristics of Hirayama Disease in Young South Korean Soldiers.

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). 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. 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%). 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.

Investigation of perfusion impairment in degenerative cervical myelopathy beyond the site of cord compression

The aim of this study was to determine tissue-specific blood perfusion impairment of the cervical cord above the compression site in patients with degenerative cervical myelopathy (DCM) using intravoxel incoherent motion (IVIM) imaging. A quantitative MRI protocol, including structural and IVIM imaging, was conducted in healthy controls and patients. In patients, T2-weighted scans were acquired to quantify intramedullary signal changes, the maximal canal compromise, and the maximal cord compression. T2*-weighted MRI and IVIM were applied in all participants in the cervical cord (covering C1–C3 levels) to determine white matter (WM) and grey matter (GM) cross-sectional areas (as a marker of atrophy), and tissue-specific perfusion indices, respectively. IVIM imaging resulted in microvascular volume fraction (F\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$F$$\\end{document}), blood velocity (D∗\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$D^{*}$$\\end{document}), and blood flow (F·D∗\\documentclass[12pt]{minimal} \\usepackage{amsmath} \\usepackage{wasysym} \\usepackage{amsfonts} \\usepackage{amssymb} \\usepackage{amsbsy} \\usepackage{mathrsfs} \\usepackage{upgreek} \\setlength{\\oddsidemargin}{-69pt} \\begin{document}$$F \\cdot D^{*}$$\\end{document}) indices. DCM patients additionally underwent a standard neurological clinical assessment. Regression analysis assessed associations between perfusion parameters, clinical outcome measures, and remote spinal cord atrophy. Twenty-nine DCM patients and 30 healthy controls were enrolled in the study. At the level of stenosis, 11 patients showed focal radiological evidence of cervical myelopathy. Above the stenosis level, cord atrophy was observed in the WM (− 9.3%; p = 0.005) and GM (− 6.3%; p = 0.008) in patients compared to healthy controls. Blood velocity (BV) and blood flow (BF) indices were decreased in the ventral horns of the GM (BV: − 20.1%, p = 0.0009; BF: − 28.2%, p = 0.0008), in the ventral funiculi (BV: − 18.2%, p = 0.01; BF: − 21.5%, p = 0.04) and lateral funiculi (BV: − 8.5%, p = 0.03; BF: − 16.5%, p = 0.03) of the WM, across C1–C3 levels. A decrease in microvascular volume fraction was associated with GM atrophy (R = 0.46, p = 0.02). This study demonstrates tissue-specific cervical perfusion impairment rostral to the compression site in DCM patients. IVIM indices are sensitive to remote perfusion changes in the cervical cord in DCM and may serve as neuroimaging biomarkers of hemodynamic impairment in future studies. The association between perfusion impairment and cervical cord atrophy indicates that changes in hemodynamics caused by compression may contribute to the neurodegenerative processes in DCM.

Association of Spinal Cord Atrophy and Brain Paramagnetic Rim Lesions With Progression Independent of Relapse Activity in People With MS.

Progression independent of relapse activity (PIRA) is a crucial determinant of overall disability accumulation in multiple sclerosis (MS). Accelerated brain atrophy has been shown in patients experiencing PIRA. In this study, we assessed the relation between PIRA and neurodegenerative processes reflected by (1) longitudinal spinal cord atrophy and (2) brain paramagnetic rim lesions (PRLs). Besides, the same relationship was investigated in progressive MS (PMS). Last, we explored the value of cross-sectional brain and spinal cord volumetric measurements in predicting PIRA. From an ongoing multicentric cohort study, we selected patients with MS with (1) availability of a susceptibility-based MRI scan and (2) regular clinical and conventional MRI follow-up in the 4 years before the susceptibility-based MRI. Comparisons in spinal cord atrophy rates (explored with linear mixed-effect models) and PRL count (explored with negative binomial regression models) were performed between: (1) relapsing-remitting (RRMS) and PMS phenotypes and (2) patients experiencing PIRA and patients without confirmed disability accumulation (CDA) during follow-up (both considering the entire cohort and the subgroup of patients with RRMS). Associations between baseline MRI volumetric measurements and time to PIRA were explored with multivariable Cox regression analyses. In total, 445 patients with MS (64.9% female; mean [SD] age at baseline 45.0 [11.4] years; 11.2% with PMS) were enrolled. Compared with patients with RRMS, those with PMS had accelerated cervical cord atrophy (mean difference in annual percentage volume change [MD-APC] -1.41; p = 0.004) and higher PRL load (incidence rate ratio [IRR] 1.93; p = 0.005). Increased spinal cord atrophy (MD-APC -1.39; p = 0.0008) and PRL burden (IRR 1.95; p = 0.0008) were measured in patients with PIRA compared with patients without CDA; such differences were also confirmed when restricting the analysis to patients with RRMS. Baseline volumetric measurements of the cervical cord, whole brain, and cerebral cortex significantly predicted time to PIRA (all p ≤ 0.002). Our results show that PIRA is associated with both increased spinal cord atrophy and PRL burden, and this association is evident also in patients with RRMS. These findings further point to the need to develop targeted treatment strategies for PIRA to prevent irreversible neuroaxonal loss and optimize long-term outcomes of patients with MS.

MRI assessment of cervical spinal cord cross-sectional area in patients with multiple sclerosis.

Spinal cord abnormalities including cervical cord atrophy are common in multiple sclerosis (MS). This study aimed to assess the cervical spinal cord cross-sectional area (CSA) using magnetic resonance imaging (MRI) in MS patients. Sixty participants were enrolled in this study (16 male and 44 female), 30 patients with MS, diagnosed according to the revised McDonald criteria, and 30 apparently healthy individuals as the control group. CSA of the spinal cord was measured on axial T2-weighted images of the cervical MRI studies from C2 to C7 vertebral levels. There was a significant difference between MS patients and the control group in mean CSA at a different level. The mean CSA at C2, in MS cases, was significantly lower than controls (67.7 ± 9.4 mm2 vs. 81.3 ± 4.6 mm2). Similarly, the mean CSA at C7 (64.4 ± 9.9 mm2) and average C2-7 (68 ± 9.1 mm2) of MS cases were significantly lower than the control. There was a strong inverse correlation between mean cervical cord CSA and duration of the disease and disability score. The reduction in cervical cord CSA was more prominent in patients with secondary progressive MS. There was no significant difference regarding age, gender, type of treatment, or the number of cervical cord lesions. The mean CSA was significantly lower in patients with MS than in the control group and was lesser in progressive types. Patients with a longer duration of MS and a high disability score tend to have smaller CSA.

Association between brain and upper cervical spinal cord atrophy assessed by MRI and disease aggressiveness in amyotrophic lateral sclerosis.

To study the relative contributions of brain and upper cervical spinal cord compartmental atrophy to disease aggressiveness in amyotrophic lateral sclerosis (ALS). Twenty-nine ALS patients and 24 age- and gender-matched healthy controls (HC) were recruited. Disease duration and the Revised-ALS Functional Rating Scale (ALSFRS-R) at baseline, 3- and 6-months follow-up were assessed. Patients were clinically differentiated into fast (n=13) and slow (n=16) progressors according to their ALSFRS-R progression rate. Brain grey (GM) and white matter, brainstem sub-structures volumes and spinal cord cross-sectional area (SC-CSA) at C1-C2 vertebral levels were measured from a 3D-T1-weighted MRI. Fast progressors showed significant GM, medulla oblongata and SC atrophy compared to HC (p<0.001, p=0.013 and p=0.008) and significant GM atrophy compared to slow progressors (p=0.008). GM volume correlated with the ALSFRS-R progression rate (Rho/p=-0.487/0.007), the ALSFRS-R at 3-months (Rho/p=0.622/0.002), and ALSFRS-R at 6-months (Rho/p=0.407/0.039). Medulla oblongata volume and SC-CSA correlated with the ALSFRS-R at 3-months (Rho/p=0.510/0.015 and Rho/p=0.479/0.024). MRI measures showed high performance to discriminate between fast and slow progressors. Our study suggests an association between compartmental atrophy and disease aggressiveness. This result is consistent with the combination of upper and lower motor neuron degeneration as the main driver of disease worsening and severity in ALS. Our study highlights the potential of brain and spinal cord atrophy measured by MRI as biomarker of disease aggressiveness signature.

Cervical Spine Magnetic Resonance Imaging Findings in Hirayama Disease.

Background Hirayama disease is an uncommon type of cervical cord myelopathy seen typically in young males due to trauma from flexion movements. This study aims to assess the clinical presentations and classify the extent of various cervical spine MRI findings for the local population. Methodology A retrospective study of 13 patients diagnosed with Hirayama disease oncervical MRI was performed from January 2017 to December 2022 at Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune. Results Of the 13 patients, 12 (92%) were male, and one (8%) was female. Nine (69%) patients were in the 16-25-year age group, two (15%) were in the 26-35-year age group, and one (8%) each was in the 6-15-year and 66-75-year age groups. Upper limb weakness was the most common clinical symptom seen in 12 (92%) patients, followed by distal muscle atrophy in seven (54%) patients. Tremors in the hand were a rare symptom seen in two patients. Claw hand was an atypical symptom seen in one patient. On cervical MRI, all patients showed excessive forward shifting of the posterior dura on flexion, with resultant cord compression due to tightness of the dural sac. One (8%) patient had no signs of myelopathy, while 12 (92%) patients had developed chronic myelomalacia and showed abnormal cord hyperintensity and atrophy in the lower cervical cord. All 13 (100%) patients showed increased laminodural space on flexion; the mean thickness was 4.08 mm, with the minimum and maximum thickness being 2.4 mm and 6.7 mm, respectively. Classifying by length of the anterior bulging dura, one (8%) patient showed involvement of less than two vertebral body segments, eight (62%) patients showed involvement of two to four vertebral body segments, and four (30%) patients showed involvement of more than four vertebral body segments. Crescent-shaped post-contrast enhancement on flexion was seen in all eight (100%) patients who underwent a contrast study. Prominent epidural flow voids on flexion were seen in six (46%) patients. Conclusions Hirayama disease is an uncommon type of cervical myelopathy seen typically in juvenile males. The occult onset of distal upper limb weakness and atrophy during puberty, typical MRI features of lower cervical cord atrophy, and the presence of a crescent-shaped enhancing mass in the posterior epidural space are pathognomonic of the condition. A few atypical cases can also occur. Early diagnosis and treatment are crucial to avoiding serious dysfunction.

Clinical profile and dynamic magnetic resonance imaging in Hirayama disease: a single-centered cross-sectional study in Nepal.

Eighteen patients were assessed for clinical and radiological Hirayama disease. Clinical criteria included insidious onset nonprogressive chronic upper limb weakness and atrophy in teens or early twenties without sensory deficits and coarse tremors. MRI was done in a neutral position followed by neck flexion to evaluate cord atrophy and flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal, posterior epidural flow voids, and an enhancing epidural component with its dorsal extension. The mean age was 20.33 years, and the majority, 17 (94.4%), were male. Neutral-position MRI revealed loss of cervical lordosis in 5 (27.8%) patients, cord flattening in all patients with asymmetry in 10 (55.5%), and cord atrophy was observed in 13 (72.2%) patients with localized cervical cord atrophy in only 2 (11.1%) and extension of atrophy to dorsal cord in 11 (61.1%) patients. Intramedullary cord signal change was seen in 7 (38.9%) patients. Loss of attachment of posterior dura and subjacent lamina and anterior displacement of dorsal dura was seen in all patients. A crescent-shaped epidural intense enhancement was noted along the posterior aspect of the distal cervical canal in all patients, with dorsal level extension in 16 (88.89%) patients. The mean thickness of this epidural space was 4.38±2.26 (mean±2SD), and the mean extension was 5.5±4.6 vertebral levels (mean±2SD). The high degree of clinical suspicion can guide additional contrast studies in flexion as a set MRI protocol for early detection and avoiding false negative diagnoses of HD.

Teaching Neuro Images : A case of Hirayama disease presenting with polymyoclonus

A 17-year-old boy developed bilateral hand tremulousness, weakness, and decreased muscle bulk over 6 months. Bilateral extremity wasting with sparing of brachioradialis muscles (figure 1) was evident. With arms outstretched, distal mini-polymyoclonus (video 1) occurred. Neurophysiology suggests a pure motor neuropathy (chronic denervative changes in C7-T1 roots). MRI spine (dynamic flexion sequences)1 (figure 2) revealed cervical cord atrophy, dural sac anterior shifting, and contrast-enhancing epidural compartment. The constellation of features suggests Hirayama disease,2 postulated to be secondary to chronic segmental cord ischemia from repeated neck flexion over a tight dural sac. The polymyoclonus should not be mistaken for tremors.

Hirayama Disease: A Case Report

Hirayama disease is a rare non-progressive spinal muscular atrophy related to flexion movements of the neck. It is considered a benign motor neuron disorder with a stationary stage after a progressive course. We report a case of an 18-years old male with a history of asymmetric weakness and amyotrophy of the distal right upper extremity, suggestive of Hirayama disease. Magnetic resonance imaging (MRI) of the cervical spine was obtained both in flexion and neutral position. MRI showed loss of cervical lordosis with focal areas of lower cervical cord atrophy with increased T2 signal intensity of the spinal cervical cord in a neutral position and anterior displacement of the detached posterior dura from the underlying flexion position.

Cervical spinal cord atrophy in amyotrophic lateral sclerosis across disease stages.

Spinal cord degeneration is a hallmark of amyotrophic lateral sclerosis. The assessment of gray matter and white matter cervical spinal cord atrophy across clinical stages defined using the King's staging system could advance the understanding of amyotrophic lateral sclerosis progression. We assessed the invivo spatial pattern of gray and white matter atrophy along cervical spinal cord (C2 to C6 segments) using 2D phase-sensitive inversion recovery imaging in a cohort of 44 amyotrophic lateral sclerosis patients, evaluating its change across the King's stages and the correlation with disability scored by the amyotrophic lateral sclerosis functional rating scale revised (ALSFRS-R) and disease duration. A mathematical model inferring the potential onset of cervical gray matter atrophy was developed. In amyotrophic lateral sclerosis patients at King's stage 1, significant cervical spinal cord alterations were mainly identified in gray matter, whereas they involved both gray and white matter in patients at King's stage ≥ 2. Gray and white matter areas correlated with clinical disability at all cervical segments. C3-C4 level was the segment showing early gray matter atrophy starting about 7 to 20months before symptom onset according to our model. Our findings suggest that cervical spinal cord atrophy spreads from gray to white matter across King's stages in amyotrophic lateral sclerosis, making spinal cord magnetic resonance imaging an invivo assessment tool to measure the progression of the disease.

Role of Dynamic Magnetic Resonance Imaging in Hirayama Disease, a Rare Motor Neuron Disease.

Introduction Hirayama disease (HD) is a benign self-limiting motor neuron disease, most commonly occurring in young males. The disease has an insidious onset that affects T1, C8, and C7 myotomes. HD is more common in Japan and Asian countries, and rare in the western population. Magnetic resonance imaging (MRI) is the best technique for the diagnosis of this entity. Early diagnosis is important as the patients can be advised to limit neck flexion movements to arrest the progression of the disease. Any clinically suspected case of Hirayama disease should undergo flexion MRI as conventional neutral MRI may miss findings in a few cases. The purpose of the present study is to evaluate the usefulness of flexion MR imaging and laminodural space (LDS) measurement in young patients with clinical and electroneuromyography (ENMG) definite Hirayama disease. Materials and methods This is a retrospective observational studyof 15patients with clinical and ENMG definite Hirayama disease who were referred to the Department of Radiology. These patients underwent MRI of cervical spine in neutral position and with neck flexion of 30°-40°. In neutral MRI, atrophy and T2-weighted hyperintensities in the cord were noted. In flexion MRI, the maximum forward shifting of the posterior dural sac, also known asthe LDS, was noted along with other parameters. Observation The mean age of the study population was21 ± 3.36 years. Out of 15 patients, 14 were males and one was female; 14 patients (93.3%) had an involvement of unilateral upper extremity while one patient (6.6%) had asymmetric bilateral involvement. Straightening of cervical spinal curvature and cord atrophy was seen in 14 (93.3%) and 12 (80%) patients, respectively, on neutral position MRI.Intramedullary cervical cord T2-weighted hyperintensities were noted in eight patients (53.3%). Loss of the dural attachment and forward shifting of the posterior dural sac with prominent posterior epidural space was noted in all patients (100%). At the maximum forward shift of cord, the LDSranged from 3.1 to 7.0 mm, with a mean of 5.38 ± 1.13 mm.Epidural flow voids were noted in 86.6%of cases. Conclusion Flexion MRI plays a very important role in confirming the diagnosis of Hirayama disease in clinically suspected cases.Anterior displacement of posterior dura matter and widening of LDS is notedin all cases in our study. Even though findings like cord atrophy and T2 hyperintensities are seen in conventional neutral MRI, these findings are not seen in all cases. So flexion MRI increases diagnostic confidence by showing increased LDS, which is a characteristic finding in Hirayama disease.

Association of spinal cord lesions with cognition in multiple sclerosis

Amaç: Çalışmamızda, multipl skleroz (MS)da fiziksel özürlülük ve prognozla yakın ilişkisi olan spinal kord tutulumunun, kognitif işlevler üzerine olası etkisi incelenmiştir.&#x0D; Gereç ve Yöntem: Çalışmaya 29 MS hastası (2 klinik izole sendrom, 23 relaps ve remisyonlarla seyreden MS, 2 sekonder progresif MS, 2 primer progresif MS) alındı. Demografik özellikleri, genişletilmiş özürlülük durum skalası (EDSS) skoru, Rao’nun kısa tekrarlanabilir nöropsikolojik test bataryası ile değerlendirilen kognitif durumu, beyin ve spinal kord T2 lezyon sayısı değerlendirildi. Benzer demografik özelliklere sahip 17 sağlıklı kontrol ile kognitif test sonuçları karşılaştırıldı. &#x0D; Bulgular: İleri yaş, hastalık başlangıç yaşının ileri olması, kısa eğitim süresi kognitif test sonuçlarını olumsuz etkileyen özelliklerdir. Beyin ve spinal kord lezyon sayılarının kognitif testler üzerine anlamlı etkisi saptanmamıştır. Spinal kord lezyonu olan hastalar olmayanlara göre daha yüksek EDSS’ye sahiptir, ancak iki grup arasında kognitif test sonuçları açısından anlamlı farklılık saptanmamıştır. &#x0D; Sonuç: Spinal kord lezyonları, EDSS ile değerlendirilen fiziksel özürlülük ile ilişkilidir. Kord plak varlığının ya da sayısının kognitif test sonuçları üzerine etkisini, servikal kord atrofisi ile birlikte değerlendirecek, daha geniş örneklemli boylamsal çalışmalara ihtiyaç vardır.

Structural MRI Reveals Cervical Spinal Cord Atrophy in the P301L Mouse Model of Tauopathy: Gender and Transgene-Dosing Effects.

In primary tauopathies, the deposition of tau neurofibrillary tangles and threads as well as neurodegenerative changes have been found within the brain and spinal cord. While degenerative changes have been intensively studied in the brain using structural magnetic resonance imaging (MRI), MRI studies investigating the spinal cord are still scarce. In the present study, we acquired ex vivo high resolution structural MRI of the cervical spinal cord of 8.5–9 month old hemizygous and homozygous P301L mice and non-transgenic littermates of both genders. We assessed the total cross-sectional area, and the gray and white matter anterior-posterior width and left-right width that are established imaging marker of spinal cord degeneration. We observed significant tissue-specific reductions in these parameters in female P301L mice that were stronger in homozygous than in hemizygous P301L mice, indicating both an effect of gender and transgene expression on cervical spinal cord atrophy. Moreover, atrophy was stronger in the gray matter than in the white matter. Immunohistochemical analysis revealed neurodegenerative and neuroinflammatory changes in the cervical spinal cord in both the gray and white matter of P301L mice. Collectively, our results provide evidence for cervical spinal cord atrophy that may directly contribute to the motor signs associated with tauopathy.