Snake Eyes Appearance Research Articles

Anterior horn damage in brachial multisegmental amyotrophy with superficial siderosis and dural tear: an autopsy case report

BackgroundPatients with superficial siderosis (SS) rarely show brachial multisegmental amyotrophy with ventral intraspinal fluid collection accompanied with dural tear.Case presentationWe describe spinal cord pathology of a 58-year-old man who developed brachial multisegmental amyotrophy with ventral intraspinal fluid collection from the cervical to lumbar spinal levels accompanied with SS, dural tear, and snake-eyes appearance on magnetic resonance imaging (MRI). Radiological and pathological analyses detected diffuse and prominent superficial deposition of hemosiderin in the central nervous system. Snake-eyes appearance on MRI expanded from the C3 to C7 spinal levels without apparent cervical canal stenosis. Pathologically, severe neuronal loss at both anterior horns and intermediate zone was expanded from the upper cervical (C3) to middle thoracic (Th5) spinal gray matter, and these findings were similar to compressive myelopathy.ConclusionExtensive damage of the anterior horns in our patient may be due to dynamic compression induced by ventral intraspinal fluid collection.

Lower Motor Syndrome Limited to the Upper Extremities with a Snake-eyes Appearance on the Cervical Cord

Lower Motor Syndrome Limited to the Upper Extremities with a Snake-eyes Appearance on the Cervical Cord

Reader Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction.

We thank Zhao et al.1 for presenting an interesting case of a longitudinally extensive spinal cord lesion in a patient with a mitochondrial sequence variant. The authors suggest that the neuroimaging mimics spinal cord infarction (SCI). However, the initial MRI shows a swollen, expanded, and enhancing spinal cord lesion. This appearance is typical for neuromyelitis optic spectrum disorders or other inflammatory myelopathies, rather than SCI. While acute snake eye appearance suggests SCI, it is usually nonenhancing, and an early MRI could be normal.2 In addition, evidence for the efficacy of idebenone for the treatment of optic neuropathy in Leber Hereditary Optic neuropathy (LHON) is modest at best,3 making it difficult to attribute this patient's recovery to idebenone. Was the patient treated with steroids or another immunotherapy acutely? In our opinion, it is likely that this patient, who harbors a familial mitochondrial sequence variant, presented with inflammatory myelopathy. Association of LHON and multiple sclerosis or antiaquaporin-4 disease is known and likely responsible for the aggressive presentation here4,5; however, causality is not proven. While we agree with the authors that consideration of underlying mitochondrial disorders in the right clinical scenario is important, ongoing neuroimmunology surveillance is prudent, and on relapse, the case may warrant immunotherapy.

Differences between spinal cord injury and cervical compressive myelopathy in intramedullary high-intensity lesions on T2-weighted magnetic resonance imaging: A retrospective study.

Increases in aging populations have raised the number of patients with cervical spinal cord injury (SCI) without fractures due to compression of the cervical spinal cord. In such patients, it is necessary to clarify whether SCI or cervical compressive myelopathy (CCM) is the cause of disability after trauma. This study aimed to clarify the differences in magnetic resonance imaging (MRI) features between SCI and CCM.Overall, 60 SCI patients and 60 CCM patients with intramedullary high-intensity lesions on T2-weighted MRI were included in this study. The longitudinal lengths of the intramedullary T2 high-intensity lesions were measured using sagittal MRI sections. Snake-eye appearance on axial sections was assessed as a characteristic finding of CCM. The T2 values of the high-intensity lesions and normal spinal cords at the first thoracic vertebra level were measured, and the contrast ratio was calculated using these values.The longitudinal length of T2 high-intensity lesions was significantly longer in SCI patients than in CCM patients. Snake-eye appearance was found in 26 of the 60 CCM patients, but not in SCI patients. On both the sagittal and axial images, the contrast ratio was significantly higher in the SCI group than in the CCM group. Based on these results, a diagnostic scale was created. This scale made it possible to distinguish between SCI and CCM with approximately 90% accuracy.

Impact of various MRI signal intensity changes on radiological parameters, the neurological status, and surgical outcomes in degenerative cervical myelopathy

Study designA retrospective study of prospectively collected date. PurposeThe present study aimed to identify patient characteristics, radiological factors, the neurological status, and postoperative outcomes associated with MRI signal intensity changes in degenerative cervical myelopathy (DCM). Material and methodOne hundred and fourteen DCM patients who underwent posterior decompression surgeries were enrolled. Pre- and postoperative (12 months) Japanese Orthopedic Association (JOA) scores, cervical alignment and balance on X rays, the cross-sectional area of the spinal cord (CSA) in kinematic CT myelography (CTM). The recovery rate (RR) of the total JOA score and those specific for the upper and lower limb items were evaluated. MRI included T1-weighted imaging (WI) and T2WI. Patients were classified into two groups for a univariate analysis according to the presence or absence of a high signal intensity (HIS) in the sagittal view on T2WI, a low signal intensity (LSI) in the sagittal view on T1WI, the distribution of HSI at a single or multiple levels, and the presence or absence of the snake eye appearance. A multiple logistic regression analysis (MLRA) was performed to identify factors associated with signal intensity changes on MRI. ResultsNinety-six patients (84.3%) were classified into HIS changes in the sagittal view on T2WI. The group with multilevel HSI showed significantly lower RR specific for the lower extremity JOA score (30.8% vs 47.7%; P = 0.016). The snake eye-positive group showed a significantly inferior RR specific for the upper extremity JOA score (40.1% vs 53.2%; P = 0.04).In MLRA, LSI changes on T1WI revealed small CSAF (P = 0.02, Odds ratio; 0.77, 95%CI:0.61–0.97), while HSI changes on T2WI revealed a small CSAF (P = 0.008, Odds ratio;1.36, 95%CI:1.08–1.72) and large C2–7 angle during extension (P = 0.009, Odds ratio;0.73, 95%CI:0.58–0.92). CSAF correlated with multilevel HSI changes (P = 0.006, Odds ratio;0.78, 95%CI:0.66–0.93). The snake eye appearance correlated with CSAF (P = 0.006, Odds ratio;0.78, 95%CI:0.66–0.93) and the duration of disease (P = 0.05, Odds ratio;1.01, 95%CI:1.0–1.03). ConclusionSevere and poorly compensated spinal cord compression during neck flexion may cause signal intensity changes. The snake eye appearance may be useful as a sign of the inferior postoperative recovery of upper limb function, and multilevel HSI as a sign of the inferior postoperative recovery of lower limb function.

Snake Eye Appearance; A Rare Radiology Presentation in Acute Flaccid Paralysis: A Case Report

Background: Acute flaccid paralysis (AFP) is defined by the acute onset of weakness or paralysis with reduced muscle tone in children. There are many non-infectious and infectious causes. Snake eye appearance (SEA) is a rare radiologic appearance and helps narrow down differential diagnoses in flaccid paralysis. Case Presentation: Here, we reported a 6 months-old girl who was admitted with sudden onset flaccid paralysis. She was lethargic and ill without any detectable deep tendon reflexes. She had a high fever that had started 3 days earlier with malaise, poor feeding and coryza. The first child of the family was a boy who expired with similar symptoms; however, the reason is still unknown. Her parents were relatives (cousins). The laboratory and cerebrospinal fluid tests analysis were normal. The brain MRI analysis revealed T1 dim Hypo intensity and T2 hyperintensity along with obvious ADC map hyperintensity in the brain stem. At first, the PCR tests analysis of stool samples for poliovirus and enterovirus were normal. Spinal MRI showed snake eye appearance and helped us narrow our differential diagnosis. We repeated the PCR tests of stool because of snake eye appearance in cervical MRI that was positive for poliovirus and indicated vaccine-associated Paralytic Poliomyelitis (VAPP). Unfortunately, she expired from vaccine associated poliomyelitis. Conclusion: Snake eye appearance is a rare radiologic appearance that can be seen in several pathological conditions; however, it is very rare in patients with acute flaccid paralysis. Radiology signs, especially in spinal cord MRI, can help recognizing abnormalities in images, and narrow the list of differential diagnosis in acute flaccid paralysis. Therefore, spinal cord MRI has an important role in the evaluation of patient with brain stem involvement in acute flaccid paralysis.

Anti-Myelin Oligodendrocyte Glycoprotein Syndrome with Findings Resembling “Snake-Eye Appearance”: a Case Report

Anti-Myelin Oligodendrocyte Glycoprotein Syndrome with Findings Resembling “Snake-Eye Appearance”: a Case Report

Residual numbness of the upper extremity after cervical surgery in patients with cervical spondylotic myelopathy.

Although numbness is one of the chief complaints of patients with cervical spondylotic myelopathy (CSM), preoperative factors relating to residual numbness of the upper extremity (UE) and impact of the outcomes on cervical surgery are not well established. The authors hypothesized that severe preoperative UE numbness could be a risk factor for residual UE numbness after surgery and that the residual UE numbness could have a negative impact on postoperative outcomes. Therefore, this study aimed to identify the preoperative factors that are predictive of residual UE numbness after cervical surgery and demonstrate the effects of residual UE numbness on clinical scores and radiographic parameters. The study design was a retrospective cohort study. The authors analyzed data of 103 patients who underwent cervical laminoplasty from January 2012 to December 2014 and were followed up for more than 2 years postoperatively. The patients were divided into two groups: the severe residual-numbness group (postoperative visual analog scale [VAS] score for UE numbness > 40 mm) and the no/mild residual-numbness group (VAS score ≤ 40 mm). The outcome measures were VAS score, Japanese Orthopaedic Association scores for cervical myelopathy, physical and mental component summaries of the 36-Item Short-Form Health Survey (SF-36), radiographic film parameters (C2-7 sagittal vertical axis, range of motion, C2-7 lordotic angle, and C7 slope), and MRI findings (severity of cervical canal stenosis, snake-eye appearance, severity of foraminal stenosis). Following univariate analysis, which compared the preoperative factors between groups, the variables with p values < 0.1 were included in the multivariate linear regression analysis. Additionally, the changes in clinical scores and radiographic parameters after 2 years of surgery were compared using a mixed-effects model. Among 103 patients, 42 (40.8%) had residual UE numbness. In the multivariate analysis, sex and preoperative UE pain were found to be independent variables correlating with residual UE numbness (p = 0.017 and 0.046, respectively). The severity of preoperative UE numbness did not relate to the residual UE numbness (p = 0.153). The improvement in neck pain VAS score and physical component summary of the SF-36 was significantly low in the severe residual-numbness group (p < 0.001 and 0.040, respectively). Forty-one percent of the CSM patients experienced residual UE numbness for at least 2 years after cervical posterior decompression surgery. Female sex and preoperative severe UE pain were the predictive factors for residual UE numbness. The patients with residual UE numbness showed less improvement of neck pain and lower physical status compared to the patients without numbness.

234. Residual arm numbness after laminoplasty for the patients with cervical spondylotic myelopathy

BACKGROUND CONTEXT Laminoplasty has been widely accepted as a standard treatment option for the patients with cervical spondylotic myelopathy (CSM) with good clinical outcomes. However, some patients suffered from the residual arm numbness postoperatively. But the preoperative factor relating to residual arm numbness and its impact on the outcomes of laminoplasty are not well established. PURPOSE To identify the preoperative factor relating the residual arm numbness after laminoplasty, and to demonstrate the effect of the residual numbness on the change of clinical scores and radiographic parameters. STUDY DESIGN/SETTING Retrospective cohort study PATIENT SAMPLE One hundred three patients who underwent laminoplasty for CSM and were followed up over 2 years after surgery. OUTCOME MEASURES Japanese Orthopedic Association (JOA) score including detailed component score (finger motion, upper extremity motion, lower extremity motion, upper extremity sensory, trunk sensory, lower extremity sensory, bladder function), visual analogue scale (VAS; arm pain, arm numbness and neck pain), 36-Item Short-Form Health Survey (SF-36; physical component summary and mental component summary), preoperative radiographic x-ray film parameters (C7 slope, C2-C7 lordotic angle, C2-C7 sagittal vertical axis and range of cervical motion) and preoperative Magnetic Resonance Imaging (MRI) findings (the severity of stenosis and the existence of snake-eye appearance). METHODS Patients were divided into two groups; residual arm numbness group (postoperative VAS >40mm) and control group (≤40mm). Following to univariate analysis comparing the preoperative factors between groups, the variables of p RESULTS The residual arm numbness group included 42 patients (23 female, 67.5±11.3 years old) and the no numbness group included 61 patients (42 female, 65.1±12.7 years old). In the multivariate analysis after univariate comparison, gender and preoperative arm pain were demonstrated as the independent variables (p=0.017 and 0.046) correlating with the postoperative residual numbness. Contrary to our hypothesis, the preoperative arm numbness and intensity change of MRI were not significant factors relating to the residual arm numbness. In the comparison of the change after surgery, the improvement of physical component summary of SF-36 was significantly low in residual arm numbness group (0.040). CONCLUSIONS In our data set, up to 40% of the CSM patients had residual arm numbness 2 years after laminoplasty. Female and preoperative severe arm pain was the independent predictor for the residual arm numbness. The patients with residual arm numbness showed the lower improvement of quality of life compared to the patient without numbness. These results can help physicians to provide adequate information to treat the CSM patients and help surgeons to predict the patients with poor surgical outcomes preoperatively. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.

Snake-Eyes Appearance on MRI Occurs during the Late Stage of Hirayama Disease and Indicates Poor Prognosis.

Purpose Because Hirayama disease is stereotyped as a self-limited disease in the absence of a definite pathology, we investigated the potential relationship between snake-eyes appearance (SEA) and Hirayama disease to bring a new perspective in the pathological process of Hirayama disease based on relevant radiological and clinical evidence. Methods A total of 30 cases observed SEA were selected from 293 patients with Hirayama disease to constitute the SEA group, and an equal number of cases were randomly selected from the remaining patients to form the non-SEA group. Cervical magnetic resonance imaging (MRI) was performed and subsequently used to measure the anteroposterior diameter and anterior shifting of the spinal cord. Additionally, clinical data, such as age, sex, duration of symptoms, symptoms, and signs, were collected and analyzed. Results Of 293 patients, 10.6% appeared with the SEA, which was mainly multisegmental (86.7%), particularly at the C5-6 segment (73.3%), and intense with a well-defined border (70.0%). The SEA group was an older population (p < 0.0001) with a longer duration (p < 0.0001) and a higher incidence of Hoffmann signs and knee hyperreflexia (p < 0.0001, p = 0.0038, respectively). The degree of spinal cord atrophy demonstrated a close association with the SEA, as it was significantly worse in the SEA group and SEA segment (p = 0.0008, p < 0.0001, respectively). The degree of spinal cord atrophy was positively related to both age and duration (p = 0.0095, p = 0.0176, respectively). Conclusions Confirmed as an irreversible lesion and an indication of poor prognosis, SEA appears during the late stage of Hirayama disease and is closely related to pyramidal signs and spinal cord atrophy.

Monomelic Amyotrophy (Hirayama Disease) with Proximal Upper Limb Involvement: A Case Report

Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. A 28-year-old man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his shoulder girdle and arm muscles on right side. A neurological examination revealed weakness and atrophy in his right deltoid, infraspinatus, supraspinatus and triceps muscles. Electromyography demonstrated an active and chronic neurogenic pattern affecting his right C5 and C6 myotomes; magnetic resonance imaging of his cervical spine showed snake eye appearance within the spinal cord. Upper limb proximal form of monomelic amyotrophy is a rare clinical entity with a wide differential diagnosis. Physicians, especially neurologists, should be familiar with this benign condition to avoid inappropriately labeling patients as having amyotrophic lateral sclerosis and other disorders with less favorable outcomes.Journal of National Institute of Neurosciences Bangladesh, 2018;4(1): 63-66

Snake Eye Sign

Clinical Image: Snake eye appearance has been described in disorders like Amyotrophic lateral sclerosis (ALS), Spondylotic amyotrophy, Hirayama disease and ossification of posterior longitudinal ligament. On axial T2 weighted images of spinal cord anterior horn of grey matter appears hyperintense giving appearance of snake eyes (Figure 1 ).

Sporadic lower motor neuron disease with a snake eyes appearance on the cervical anterior horns by MRI

ObjectiveLower motor neuron disease (LMND) is the term generally used to describe diseases in which only lower motor neuron signs are detected. A snake eyes appearance on magnetic resonance imaging (MRI) is associated with a wide spectrum of neurological conditions including LMND. The author reports on three unique LMND patients with upper limb muscle weakness and atrophy who show a snake eyes appearance by MRI. Patients and methodsThe patients were aged 18, 40 and 52 years, respectively, at the onset of the disease and had a longstanding clinical course (more than 10 years for two patients and 8 years for one patient). They were followed up for more than 6 years. ResultsClinical manifestations were characterized by (1) longstanding slow progression or delayed spontaneous arrest of asymmetric lower motor neuron signs localized exclusively in the upper extremities with unilateral predominance and distal or proximal preponderance; (2) the absence of upper motor neuron signs, bulbar signs, sensory disturbances and respiratory involvement; (3) a snake eyes appearance on the anterior horns of the cervical cord over more than 3 vertebrae by axial T2-weighted MRI and a longitudinal linear-shaped T2-signal hyperintensity by sagittal MRI; (4) neurogenic change with fasciculation and denervation potentials (fibrillation and a positive sharp wave) confined to the affected muscles by needle electromyogram; and (5) normal cerebrospinal fluid and a normal creatine kinase level. These cases did not fall into any existing category of LMND, such as progressive muscular atrophy, flail arm syndrome or Hirayama disease. ConclusionsThese patients should be classified as sporadic LMND with snake eyes on MRI with a relatively benign prognosis.

Revisiting the spectrum of lower motor neuron diseases with snake eyes appearance on magnetic resonance imaging.

The 'snake eyes' sign refers to bilateral hyperintensities of the anterior horns on axial spinal cord imaging. Based on sporadic reports, it has been associated with a range of lower motor neuron (LMN) syndromes, such as spondylotic amyotrophy and Hirayama disease, as well as spinal cord infarction. The objective of our study was to comprehensively characterize the full diagnostic spectrum of LMN syndromes with this radiological clue and discuss potential aetiological factors. A large patient cohort with snake eyes sign and upper limb LMN degeneration was recruited from three French neuromuscular units. Patients underwent detailed electrophysiological, radiological, clinical and anamnestic profiling. Twenty-nine patients were ascertained and followed up for 9.5 ± 8.6 years. The majority of the patients were male (86.2%) with a mean age of 37.3 ± 14.4 years. Symptoms were bilateral in most cases (86.2%). Patients with predominantly proximal and distal deficits were equally represented (44.8% and 55.2%, respectively). A history of preceding trauma or intense physical activity was confirmed in 58.6% of the cases; 27.6% of the patients were given an initial clinical diagnosis of amyotrophic lateral sclerosis (ALS), and 51.7% were originally suspected to have multifocal motor neuropathy. None of the patients developed ALS on longitudinal follow-up. The snake eyes sign on magnetic resonance imaging is associated with a wide spectrum of neurological conditions and is more common in young men with a history of strenuous activity or antecedent trauma. The recognition of this syndrome is crucial as many of these patients are initially misdiagnosed with ALS.

Is the "snake-eye" MRI sign correlated to anterior spinal artery occlusion on CT angiography in cervical spondylotic myelopathy and amyotrophy?

The goal of this study was to identify anterior spinal artery (ASA) occlusion by CT angiography in cervical spondylotic myelopathy (CSM) and amyotrophy (CSA) with T2-weighted hyperintensity of MR image of documented small intramedullary high signal intensity known as "snake-eye appearance" (SEA). One hundred and six patients with CSM were admitted to the investigator group between June 2010 and June 2013. Intramedullary high signal intensity was found in 42 cases and was divided into two types, SEA and non-SEA. SEA was observed in 10 patients, including seven CSM patients and three CSA patients. All SEA patients were performed CT angiography of ASA after admission. The ASA was visualized in all 10 patients. ASA incomplete occlusion was found in one CSA patient and one CSM patient. No ASA occlusion was found in other CSA and CSM patients with SEA. ASA occlusion is not commonly seen in CSM and CSA patients with SEA. Pathological changes about SEA in CSM and CSA have no close correlation with ASA occlusion, but may be with anterior radiculomedullary arteries.

Teaching Neuro images : Snake eyes appearance in MRI in patient with ALS

Sharma et al.1 presented an interesting radiologic finding—the “snake eyes” appearance—which has been reported in several neurologic disorders. However, I question the diagnosis of amyotrophic lateral sclerosis (ALS) since the patient was only 17 years old. Classic ALS does not occur in this age group. An ALS-like complication of HIV or human T-cell lymphotrophic virus 1 has been reported.2 In addition, we have seen teenage girls who have diffuse …

UNUSUAL COMPLICATION OF VERTEBRAL ARTERIAL DISSECTION

IntroductionVertebral artery dissection (VAD) is a common cause of ischemic stroke in patients younger than 45 yrs of age. VAD is rarely life–threatening but we here present a patient with...

Teaching Neuro Images : Snake eyes appearance in MRI in patient with ALS

A 17-year-old girl presented with 6 months of progressive asymmetrical distal weakness of upper limbs with fasciculation. There were no bulbar symptoms, weakness in lower limbs, or sensory symptoms. There was no history of neck injury or pain. Deep tendon reflexes were all brisk. MRI of cervical spine revealed symmetrical T1 hypointense, T2 hyperintense signal in the anterior horns (“snake eyes” appearance) (figure, A and B) without evidence of extradural compression. EMG showed diffuse denervation changes in both upper and lower limb muscles. Sensory nerve action potentials were normal. A diagnosis of amyotrophic lateral sclerosis (ALS) was made. Snake eyes appearance has been described in disorders like ALS, cervical spondylotic amyotrophy, Hirayama disease, and ossification of posterior longitudinal ligament.1,2

Progressive post-traumatic myelopathy presenting with magnetic resonance imaging snake-eye appearance

Progressive post-traumatic myelopathy presenting with magnetic resonance imaging snake-eye appearance

Does the type of T2-weighted hyperintensity influence surgical outcome in patients with cervical spondylotic myelopathy? A review

To review the literature on different classifications of T2-weighted (T2W) increased signal intensity (ISI) on preoperative magnetic resonance (MR) images of patients with cervical spondylotic myelopathy (CSM). The authors searched the databases of PubMed and Cochrane for studies that used a categorization of T2W ISI to predict the functional outcome after decompressive surgery for CSM. Selected studies were analyzed for the type of ISI classification used, patient selection, methodology and results. The level of evidence provided by each study was determined. Twenty-two studies fulfilled our search criteria. There were 11 prospective studies and a total of 1,508 patients were studied. The majority of studies classified ISI based on either the longitudinal extent (12 studies) or the qualitative features of the ISI (10 studies). Three studies used both parameters to classify T2W ISI. Other classifications were based on the position of ISI (1 study), presence of snake-eye appearance on axial MR images (1 study) and signal intensity ratio (SIR) (1 study). Poorer functional outcomes correlated with sharp, intense ISI (6 studies) and multisegmental ISI (5 studies) (Class II evidence). Five of ten studies reported that the regression of ISI postoperatively was associated with better neurological outcomes (Class II evidence). Methodological variations in previous studies made it difficult to compare studies and results. Both multisegmental T2W ISI and sharp, intense T2W ISI are associated with poorer surgical outcome (Class II evidence). The regression of T2W ISI postoperatively correlates with better functional outcomes (Class II). Future studies on the significance of ISI should ensure use of a uniform grading system, standardized outcome measures and multivariate analyses to control for other preoperative variables.