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Chikungunya virus (CHIKV) is an arbovirus endemic to South Asia with frequent outbreaks. A wide spectrum of neurological complications has been described in Chikungunya infections. Myeloneuropathy is a rare complication seen in Chikungunya and is proposed to have an underlying immune mediated pathogenesis. We report a case of a 45-year-old man presenting to the emergency services with acute onset of quadriparesis, breathlessness, urinary retention, profound pain, and sensory disturbances 6 weeks after the onset of high-grade fever and arthralgia. On examination, the patient had Medical Research Council grade 1 flaccid quadriparesis with prominent wasting and areflexia with distinct sensory level at T4. Immunoglobulin M CHIKV antibodies were positive, tested twice at a 1-week interval. He had notable magnetic resonance imaging (MRI) findings in the form of patchy T2 hyperintensities involving the entire length of the cervical and thoracic cord with normal brain imaging and extensive short tau inversion recovery hyperintense signal changes on muscle MRI. He was treated with five cycles of plasmapheresis and intravenous methylprednisolone followed by oral steroids for 8 weeks. At 20-week follow-up, the patient had improvement in upper limb weakness, but paraparesis persisted. The case highlights the presence of unusual MRI findings and also the importance of early recognition of after infective neurological complications, and prompt treatment with immunomodulation may be beneficial.

BACKGROUND The aim of this study was to verify whether the combined classification of increased signal intensity (ISI) on magnetic resonance imaging is more closely related to surgical outcomes than signal quality changes or signal longitudinal extent changes alone and to evaluate whether the combined classification ISI method could be used to predict surgical outcomes in cervical spondylotic myelopathy. MATERIAL AND METHODS Eighty-four patients (61 men and 23 women) who underwent surgery for cervical spondylotic myelopathy were included in this retrospective study. The patterns of ISI were classified into 3 categories based on (1) the quality of ISI into Grade 0: none, Grade 1: faint (fuzzy), and Grade 2: intense (sharp); (2) the longitudinal extent of ISI into none, focal, and multisegmental; and (3) the combined classification of the quality and longitudinal extent into Type 1 (none/none), Type 2 (focal/faint), Type 3 (focal/intense), Type 4 (multisegmental/faint), and Type 5 (multisegmental/intense). The multifactorial effects of variables were studied. A stepwise regression analysis was performed to verify whether this combined classification could predict outcome. RESULTS Of the 3 categories, the combined classification type of ISI was most closely related to recovery rate. Stepwise regression analysis confirmed the significance of combined classification of ISI as a predictor for surgical outcome. CONCLUSIONS A combined classification of ISI is more closely related to surgical outcomes than either signal quality changes or signal longitudinal extent changes alone and it could be used as a meaningful indicator for predicting surgical outcomes. We recommend further studies to confirm this finding.

The objective of this study was to summarize the available literature describing the presentation, diagnostic evaluation, and management for adults with Type 1 and Type 2 split spinal cord malformations. A review of the literature was performed using the CINAHL, PubMed, Embase, and Web of Science database, alongside all associated bibliographies, to include studies describing Type 1 and Type 2 split cord malformations diagnosed in patients above the age of 18. All relevant studies of split cord malformations were included, regardless of the year published and terminology used to describe the dysraphism. Clinical case series (≥ 2 patients), cohort studies, and review articles comprising adult patients with radiographically diagnosed diastematomyelia, diplomyelia, or dimyelia were included (Class of Evidence I-IV). A total of 17 unique articles, describing 146 unique adult spinal cord malformation subjects, were included. The most common associated condition was tethered cord syndrome (59.8 %). Operative management for symptomatic split cord malformation was performed in 72.3 % of cases. For those with preoperative neurologic deficits, operative management resulted in symptomatic improvement in 96.6 %, compared to 0 % conservative management (p < 0.05). For those with pain alone, operative management resulted in improvement of 91.1 %, compared to 12.5 % conservative management (p < 0.05). To date, this is the only literature review to include all split cord malformations (SCM Types I and II) presenting in adulthood, with clinical characteristics, associated conditions, and long-term treatment outcomes., Competing Interests: Declaration of Competing Interest The authors have no disclosures relevant to the current work, nor any true/perceived conflicts of interest. Disclosures unrelated to the current work include: A. Karim Ahmed, BS: None. Elizabeth P. Howell, BS: None. Stephen Harward, MD, PhD: None. Eric W. Sankey, MD: None. Jeffrey Ehresman, BS: None. Andrew Schilling, BA: None. Timothy Wang, MD: None. Zachary Pennington, BS: None. Linda Gray, MD: None Daniel M. Sciubba, MD: Consultant for Orthofix, Globus, K2M, Medtronic, Stryker, Baxter. C. Rory Goodwin, MD, PhD: Received grants from the Burroughs Wellcome Fund, North Carolina Spine Society, and the NIH/NINDS K12 NRCDP Physician Scientist Award, Robert Wood Johnson Harold Amos Medical Faculty Development Program., (Copyright © 2020. Published by Elsevier B.V.)

Background: Patients with mild degenerative cervical myelopathy (DCM) represent a heterogeneous population, and indications for surgical decompression remain controversial., Objective: To dissociate patient phenotypes within the broader population of mild DCM associated with degree of impairment in baseline quality of life (QOL) and surgical outcomes., Methods: This was a post hoc analysis of patients with mild DCM (modified Japanese Orthopedic Association [mJOA] 15-17) enrolled in the AOSpine CSM-NA/CSM-I studies. A k-means clustering algorithm was applied to baseline QOL (Short Form-36 [SF-36]) scores to separate patients into 2 clusters. Baseline variables and surgical outcomes (change in SF-36 scores at 1 yr) were compared between clusters. A k-nearest neighbors (kNN) algorithm was used to evaluate the ability to classify patients into the 2 clusters by significant baseline clinical variables., Results: One hundred eighty-five patients were eligible. Two groups were generated by k-means clustering. Cluster 1 had a greater proportion of females (44% vs 28%, P = .029) and symptoms of neck pain (32% vs 11%, P = .001), gait difficulty (57% vs 40%, P = .025), or weakness (75% vs 59%, P = .041). Although baseline mJOA correlated with neither baseline QOL nor outcomes, cluster 1 was associated with significantly greater improvement in disability (P = .003) and QOL (P < .001) scores following surgery. A kNN algorithm could predict cluster classification with 71% accuracy by neck pain, motor symptoms, and gender alone., Conclusion: We have dissociated a distinct patient phenotype of mild DCM, characterized by neck pain, motor symptoms, and female gender associated with greater impairment in QOL and greater response to surgery., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Study Design: This study retrospectively analyzes prospectively collected data., Objective: Here, we aim to determine the influence of preoperative and 12-month modified Japanese Orthopedic Association (mJOA) on satisfaction and understand the change in mJOA severity classification after surgical management of degenerative cervical myelopathy (DCM)., Summary of Background Data: DCM is a progressive degenerative spine disease resulting from cervical cord compression. The natural progression of DCM is variable; some patients experience periods of stability, while others rapidly deteriorate following disease onset. The mJOA is commonly used to grade and categorize myelopathy symptoms, but its association with postoperative satisfaction has not been previously explored., Methods: The quality and outcomes database (QOD) was queried for patients undergoing elective surgery for DCM. Patients were divided into mild (≥14), moderate (9 to 13), or severe (<9) categories on the mJOA scores. A McNemar-Bowker test was used to assess whether a significant proportion of patients changed mJOA category between preoperative and 12 months postoperative. A multivariable proportional odds ordinal logistic regression model was fitted with 12-month satisfaction as the outcome of interest., Results: We identified 1963 patients who underwent elective surgery for DCM and completed 12-months follow-ups. Comparing mJOA severity level preoperatively and at 12 months revealed that 55% remained in the same category, 37% improved, and 7% moved to a worse category. After adjusting for baseline and surgery-specific variables, the 12-month mJOA category had the highest impact on patient satisfaction (P < 0.001)., Conclusion: Patient satisfaction is an indispensable tool for measuring quality of care after spine surgery. In this sample, 12-month mJOA category, regardless of preop mJOA, was significantly correlated with satisfaction. Given these findings, it is important to advise patients of the probability that surgery will change their mJOA severity classification and the changes required to achieve postoperative satisfaction., Level of Evidence: 3.

Purpose: Neurointerventional approaches have improved myelopathy in patients with spinal vascular lesions by providing effective management, particularly when surgical approaches are difficult. However, there have been challenges in describing and comparing recovery status during the post-treatment period., Methods: We evaluated 43 patients with venous congestive myelopathy (VCM) using Aminoff-Logue Disability Scale for gait (AL-G) and micturition (AL-M) scores. These results were compared with our new PSMS grading system that evaluates four categories (grades 0-3): pain, sensory symptoms, motor deficit, and sphincter change. Simple linear regression was used to identify the association or trend among the scales. We also calculated an overall area under the receiver operating characteristic curve to compare the predictive ability of the PSMS system with that of the previous grading system (AL-G and AL-M)., Results: Compared with other grading system, the PSMS system was more sensitively correlated with patient status and the results were easy to compare with previous clinical statuses during follow-up. The PSMS system also measured pain, which is commonly associated with spinal dural arteriovenous fistula and not precisely evaluated by other grading system., Conclusions: The new PSMS grading system for patients with VCM correlated well with the previously used systems and included pain evaluation. This new grading system is an easy tool for the evaluation and comparison of outcomes before and after endovascular treatment.

Objective: To assess the predictive value of the initial clinical and paraclinical features in the differentiation of inflammatory myelopathies from other causes of myelopathy in patients with initial diagnosis of transverse myelitis (TM)., Methods: We analyzed the clinical presentation, spinal cord MRI, and CSF features in a cohort of 457 patients referred to a specialized myelopathy center with the presumptive diagnosis of TM. After evaluation, the myelopathies were classified as inflammatory, ischemic/stroke, arteriovenous malformations/fistulas, spondylotic, or other. A multivariable logistic regression model was used to determine characteristics associated with the final diagnosis and predictors that would improve classification accuracy., Results: Out of 457 patients referred as TM, only 247 (54%) were confirmed as inflammatory; the remaining 46% were diagnosed as vascular (20%), spondylotic (8%), or other myelopathy (18%). Our predictive model identified the temporal profile of symptom presentation (hyperacute <6 hours, acute 6-48 hours, subacute 48 hours-21 days, chronic >21 days), initial motor examination, and MRI lesion distribution as characteristics that improve the correct classification rate of myelopathies from 67% to 87% (multinomial area under the curve increased from 0.32 to 0.67), compared to only considering CSF pleocytosis and MRI gadolinium enhancement. Of all predictors, the temporal profile of symptoms contributed the most to the increased discriminatory power., Conclusions: The temporal profile of symptoms serves as a clinical biomarker in the differential diagnosis of TM. The establishment of a definite diagnosis in TM requires a critical analysis of the MRI and CSF characteristics to rule out non-inflammatory causes of myelopathy., Classification of Evidence: This study provides Class IV evidence that for patients presenting with myelopathy, temporal profile of symptoms, initial motor examination, and MRI lesion distribution distinguish those with inflammatory myelopathies from those with other causes of myelopathy., (Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Background: The clinical significance of pathologies of the spinal dura is often unclear and their management controversial., Objective: To classify spinal dural pathologies analogous to vascular aneurysms, present their symptoms and surgical results., Methods: Among 1519 patients with spinal space-occupying lesions, 66 patients demonstrated dural pathologies. Neuroradiological and surgical features were reviewed and clinical data analyzed., Results: Saccular dural diverticula (type I, n = 28) caused by defects of both dural layers, dissections between dural layers (type II, n = 29) due to defects of the inner layer, and dural ectasias (type III, n = 9) related to structural changes of the dura were distinguished. For all types, symptoms consisted of local pain followed by signs of radiculopathy or myelopathy, while one patient with dural ectasia presented a low-pressure syndrome and 10 patients with dural dissections additional spinal cord herniation. Type I and type II pathologies required occlusion of their dural defects via extradural (type I) or intradural (type II) approaches. For type III pathologies of the dural sac no surgery was recommended. Favorable results were obtained in all 14 patients with type I and 13 of 15 patients with type II pathologies undergoing surgery., Conclusion: The majority of dural pathologies involving root sleeves remain asymptomatic, while those of the dural sac commonly lead to pain and neurological symptoms. Type I and type II pathologies were treated with good long-term results occluding their dural defects, while ectasias of the dural sac (type III) were managed conservatively., (Copyright © 2017 by the Congress of Neurological Surgeons)

Predicting the functional outcomes of spinal cord disorder patients after medical treatments, such as a surgical operation, has always been of great interest. Accurate posttreatment prediction is especially beneficial for clinicians, patients, care givers, and therapists. This paper introduces a prediction method for postoperative functional outcomes by a novel use of Gaussian process regression. The proposed method specifically considers the restricted value range of the target variables by modeling the Gaussian process based on a truncated Normal distribution, which significantly improves the prediction results. The prediction has been made in assistance with target tracking examinations using a highly portable and inexpensive handgrip device, which greatly contributes to the prediction performance. The proposed method has been validated through a dataset collected from a clinical cohort pilot involving 15 patients with cervical spinal cord disorder. The results show that the proposed method can accurately predict postoperative functional outcomes, Oswestry disability index and target tracking scores, based on the patient's preoperative information with a mean absolute error of 0.079 and 0.014 (out of 1.0), respectively.

Study Design: Analysis of the prospective AOSpine CSM-International and North America datasets and survey of AO Spine International., Objective: This study aims to define the minimum clinically important difference (MCID) of the modified Japanese Orthopaedic Association (mJOA) in patients with degenerative cervical myelopathy (DCM)., Summary of Background Data: The mJOA is the most frequently used clinician-administered tool to assess functional status in patients with DCM. By defining its MCID, clinicians can better evaluate treatment outcomes for this condition., Methods: Three methods were used to determine the MCID of the mJOA: (1) distribution-based, (2) anchor-based and receiver operating characteristic (ROC) analysis, and (3) professional opinion. Distribution-based methods were used to estimate the MCID by computing the half standard deviation and standard error of measurement. Using anchor-based methods, mJOA at 12 months after surgery was compared between patients who were "slightly improved" on the Neck Disability Index (NDI) and those who were "unchanged." ROC analysis was performed to compute a discrete integer value for the MCID that yielded the smallest difference between sensitivity and specificity. We repeated anchor-based methods for patients with mild (mJOA: 15-17), moderate (mJOA: 12-14), and severe disease (mJOA <12)., Results: The half standard deviation of the baseline mJOA was 1.36 and the standard error of measurement was 1.21. The difference in mJOA between patients who "slightly improved" on the NDI and "unchanged" patients was 1.11. ROC analysis yielded a value of 2 for the MCID. The survey of 416 spine professionals confirmed these estimates: the mean response was 1.65 ± 0.66. The MCID significantly varied depending on myelopathy severity: ROC analysis yielded a threshold of 1 for mild, 2 for moderate, and 3 for severe patients., Conclusion: The MCID of the mJOA is estimated to be between 1 and 2 points and varies with myelopathy severity. This knowledge will enable clinicians to identify meaningful functional improvements in DCM patients., Level of Evidence: N/A.

The prevalence of arachnoid cysts in children is 1-3%. They are more frequent in boys. They can be located intracranially or in the spine. Intracranial cysts are classified as supratentorial, infratentorial, and supra-infratentorial (tentorial notch). Supratentorial are divided into middle cranial fossa, convexity, inter-hemisferic, sellar region, and intraventricular. Infratentorial are classified into supracerebellar, infracerebellar, hemispheric, clivus, and cerebellopontine angle. Finally spinal arachnoid cysts are classified taking into account whether they are extra- or intradural, and nerve root involvement., (Copyright © 2015 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.)

Purpose: To investigate the use of a newly designed machine learning-based classifier in the automatic identification of myelopathic levels in cervical spondylotic myelopathy (CSM)., Materials and Methods: In all, 58 normal volunteers and 16 subjects with CSM were recruited for diffusion tensor imaging (DTI) acquisition. The eigenvalues were extracted as the selected features from DTI images. Three classifiers, naive Bayesian, support vector machine, and support tensor machine, and fractional anisotropy (FA) were employed to identify myelopathic levels. The results were compared with clinical level diagnosis results and accuracy, sensitivity, and specificity were calculated to evaluate the performance of the developed classifiers., Results: The accuracy by support tensor machine was the highest (93.62%) among the three classifiers. The support tensor machine also showed excellent capacity to identify true positives (sensitivity: 84.62%) and true negatives (specificity: 97.06%). The accuracy by FA value was the lowest (76%) in all the methods., Conclusion: The classifiers-based method using eigenvalues had a better performance in identifying the levels of CSM than the diagnosis using FA values. The support tensor machine was the best among three classifiers., (© 2014 Wiley Periodicals, Inc.)

The term 'acute myelopathies'--referred to a spinal cord dysfunction--represent a heterogeneous group of disorders with distinct etiologies, clinical and radiologic features, and prognoses. The objective of this review is to discuss the non-traumatic acute myelopathies. Acute myelopathy can be due to several causes as infective agents or inflammatory processes, such as in acute myelitis, compressive lesions, vascular lesions, etc. The clinical presentation is often dramatic with tetraparesis or paraparesis, sensory disturbances and bladder and/or bowel dysfunction. History and physical examination are used to localize the lesion to the root or specific level of the cord, which can guide imaging. Different syndromes are recognized: complete transverse lesion, central grey matter syndrome, anterior horn syndrome, anterior spinal artery syndrome, etc). The first priority is to rule out a compressive lesion. If a myelopathy is suspected, a gadolinium-enhanced MRI of the spinal cord should be obtained as soon as possible. If there is no structural lesion such as epidural blood or a spinal mass, then the presence or absence of spinal cord inflammation should be documented with a lumbar puncture. The absence of pleocytosis would lead to consideration of non inflammatory causes of myelopathy such as arteriovenous malformations, fibrocartilaginous embolism, or possibly early inflammatory myelopathy. In the presence of an inflammatory process (defined by gadolinium enhancement, cerebrospinal fluid pleocytosis, or elevated cerebrospinal fluid immunoglobulin index), one should determine whether there is an inflammatory or an infectious cause. Different virus, bacterias, parasites and fungi have to be considered as autoimmune and inflammatory diseases that involve the central nervous system.

Objective: Spinal digital subtraction angiography (SpDSA) continues to be the imaging gold standard for the evaluation of spinal cord vascular disorders. The safety of this procedure has a poor historical reputation and there are few current reports on complication rates. We hypothesized that modern SpDSA can be performed with an acceptably low risk of iatrogenic complications., Methods: In this retrospective series, we reviewed 302 consecutive spinal angiograms performed during a 10-year period at our institution for the frequency of intraprocedural and postprocedural neurologic, non-neurologic, and local complications. Indications for SpDSA, prior noninvasive imaging findings, and angiographic diagnoses for each case were assessed to evaluate the diagnostic contribution of the procedure., Results: There were no intraprocedural or postprocedural neurologic complications in the studied cohort. Systemic complications occurred in 2 cases (0.7%), in the form of back spasms in one patient and pulmonary edema in the other. Both recovered promptly and were discharged at baseline status. Access-site complications included 3 groin hematomas (1.0%), all managed conservatively. MRI findings showed 51% sensitivity and 83% specificity for spinal vascular malformations. A total of 31% of patients with the preangiographic diagnosis of transverse myelitis were found to have a vascular malformation., Conclusion: SpDSA carries very low risks of neurologic and systemic complications, while offering a gold-standard level of diagnostic confidence for the evaluation of the normal and pathologic vasculature of the spinal cord.

Background: Considerable attention has focused on concerns of increased complications with recombinant human bone morphogenetic protein-2 (rhBMP-2) use for anterior cervical fusion, but few reports have assessed its use for posterior cervical fusions., Objective: To assess the safety, efficacy, and dosing of rhBMP-2 as an adjunct for instrumented posterior cervical arthrodesis., Methods: All patients treated by the senior author with posterior cervical or cervicothoracic instrumented fusion using rhBMP-2 from 2003 to 2008 with a minimum of 2 years of follow-up were included. Diagnosis, levels fused, rhBMP-2 dose, complications, and fusion were assessed., Results: Fifty-three patients with a mean age of 55.7 years (range, 2-89 years) and an average follow-up of 40 months (range, 25-80 months) met inclusion criteria. Surgical indications included basilar invagination (n = 6), fracture (n = 6), atlantoaxial instability (n = 16), kyphosis/kyphoscoliosis (n = 22), osteomyelitis (n = 1), spondylolisthesis (n = 1), and cyst (n = 1). Fifteen patients had confirmed rheumatoid disease. The average rhBMP-2 dose was 1.8 mg per level, with a total of 282 levels treated (average, 5.3 levels; SD, 2.8 levels). Among 53 patients, only 2 complications (3.8%) were identified: a superficial wound infection and an adjacent-level degeneration. No cases of dysphagia or neck swelling requiring treatment were identified. At the last follow-up, all patients had achieved fusion., Conclusion: Despite many of the patients in the present series having complex pathology and/or rheumatoid arthritis, a 100% fusion rate was achieved. Collectively, these data suggest that use of rhBMP-2 as an adjunct for posterior cervical fusion is safe and effective at an average dose of 1.8 mg per level.

Spinal cord contusion and transection models are widely used for studying spinal cord injury (SCI). Clinically, however, other biomechanical injury mechanisms such as vertebral dislocation and distraction frequently occur, but these injuries are difficult to produce in animals. We mechanically characterize a vertebral clamping strategy that enables the modeling of vertebral dislocation and distraction injuries--in addition to the standard contusion paradigm--in the rat cervical spine. These vertebral clamps have a stiffness of 83.6+/-18.9 N/mm and clamping strength 64.7+/-10.2N which allows injuries to be modeled at high-speed (approximately 100 cm/s). Logistic regression indicated that a moderate-to-severe injury, with an acute mortality rate of 10%, occurs at 2.6 mm of C4/5 dorso-ventral dislocation and 4.1 mm of rostro-caudal distraction between C4 and C5. Injuries produced by dislocation and distraction exhibited features of axonal damage that were absent in contusion injuries. We conducted morphometric analysis at the nodes of Ranvier using immunohistochemistry for potassium channels (Kv1.2) in the juxtaparanodal region. Following distraction injuries, elongated nodes of Ranvier were observed up to 4mm rostral to the lesion. In contrast, contusion injuries produced distortions in nodal geometry which were restricted to the vicinity of the lesion. The greatest deformations in node of Ranvier geometry occurred at the dislocation epicenter. Given the importance of white matter damage in SCI pathology, the distinctiveness of these injury patterns demonstrate that the dislocation and distraction injury models complement existing contusion models. Together, these three animal models span a broader clinical spectrum for more reliably gauging the potential human efficacy of therapeutic strategies.

From the earliest observations of spinal vascular malformations, successful management has been challenging. Initially the challenges were diagnosing, understanding, and treating these lesions. They were originally considered all to be the same, or at least to be a single general type, of disease. With the introduction of selective spinal arteriography in the 1960s and more recently with the introduction and widespread use of MR imaging, the initial challenge of diagnosing spinal vascular malformations was overcome, and significant progress has been made in understanding their anatomy as well as the pathophysiology that underlies the myelopathy commonly associated with them. The anatomical features defined by selective arteriography and the observations permitted with the operating microscope ultimately led to distinctions between the major categories of the vascular lesions affecting the spinal cord; these distinctions were based on the lesions' anatomy, epidemiology, and the mechanism of spinal cord injury.

Typical Lhermitte phenomenon (tingling sensations moving down the limbs or trunk on neck flexion) is a sign of intrinsic spinal cord pathology, most commonly cervical spinal cord demyelination. The phenomenon has several variant forms, and each has a different pathological significance. A delayed typical Lhermitte phenomenon can follow contusion of the spinal cord from neck trauma. Reverse Lhermitte phenomenon induced by neck extension is usually produced by extrinsic compression of the cervical spinal cord. Upward moving paraesthesia with neck flexion (inverse Lhermitte phenomenon) is relatively rare, and can be a sign of myelopathy from nitrous oxide inhalation.

The ventriculus terminalis is a small cavity inside the conus medullaris that is formed during the embryonic development. Previous reports regarding cystic lesion of the ventriculus terminalis (CLVT) in adults have detailed a broad and diversified distribution in terms of clinical symptoms, clinical evolution, neurological findings, and treatment. Therefore, nonstandardized management has led to unsatisfactory outcomes. Thus, the authors propose a new classification system in which the clinical presentation is taken into account to standardize the cases and facilitate the proper management of these lesions. Two more cases are described. The literature was reviewed, dividing the patients into 3 groups by clinical presentation as follows: CLVT Type I, patients with nonspecific neurological symptoms or nonspecific complaints; CLVT Type II, presence of focal neurological deficit; and CLVT Type III, presence of sphincter disturbances (bowel or bladder dysfunction). Two patients were classified as CLVT Type I, 3 as CLVT Type II, and 12 as CLVT Type III. In Type I, no improvement was observed in clinical evaluation after surgery, and stable symptoms were achieved with clinical management. In Type II, 2 patients had total improvement and 1 had subtotal improvement after surgery. Finally, in Type III, 92% of the patients improved postoperatively; among these 33% presented with total improvement. Only 1 case in this group was handled conservatively and no improvement was documented. This new classification is useful to group the patients into 3 clinical types to provide guidance as to the best management options. Treatment for the Type I lesion seems to be best conducted conservatively, whereas Types II and III seem to be best handled surgically.

Spinal dural arteriovenous fistula (SDAVF) is a rare and enigmatic disease entity. The clinical features and structural changes have been recognized since 1926, and the pathophysiology and the essentials of treatment since 1974, but up to the present day it is unknown why these fistulas develop. The fistula between a radicular artery and the corresponding radicular vein within the dural root sleeve leads to congestion of the venous outflow of the spinal cord and eventually ischaemia. Patients, who are mostly middle-aged men, develop a progressive myelopathy, which at the early stages of the disease often mimics a polyradiculopathy or anterior horn cell disorder. By the time involvement of upper motoneurons or sacral segments makes the diagnosis of SDAVF inescapable, patients suffer from considerable neurological deficits. The diagnosis relies on MRI, which shows swelling of the spinal cord, with a centrally located hyperintense signal on T2-weighted images, and with hypointense 'flow void' phenomena dorsal to the cord, representing enlarged and tortuous veins. Catheter angiography is required to determine the exact location of the fistula as well as the angio-architecture, on which the mode of treatment depends. If the arterial feeder of the fistula is a tributary of the anterior spinal artery, embolization is not possible. After embolization recanalization may occur, but this is rarely seen after filling of the draining vein with glue. Alternatively, operation is a safe and permanent mode of treatment. No prognostic factors have been reliably established. Muscle strength and gait disturbances respond better to treatment than pain and symptoms related to damage of sacral segments. In any middle aged male patient with ascending motor or sensory deficits in the legs, SDAVF should be considered in order to prevent irreversible handicap.

Among 168 cases with neurologic findings of cervicothoracic syringomyelia and MRI findings of Chiari 1 malformation and/or underdevelopment of the posterior cranial fossa, 15 patients (9.1 %) had collapsed, flat syrinxes and 14 patients (8.3 %) did not have syrinxes. Both groups of patients had clinical findings of central myelopathy that had been stable for at least 3 years. Magnetic resonance imaging detected atrophy of the cervical spinal cord in both groups and spontaneous communications between the syrinx and the subarachnoid space in 3 patients of the group with collapsed syrinxes. Analysis of these results and review of the literature suggest that patients with clinical signs of syringomyelia and Chiari 1 malformation or underdeveloped posterior fossa, but with small or absent syringomyelitic cavities, have the "postsyrinx" state as a result of spontaneous collapse of distended syrinxes.

Embolization of type I perimedullary spinal arteriovenous fistulas (AVFs) can be difficult, because of tortuosity and the small diameter of the feeder and distal location of the fistula site. The 1.5F flow-directed catheter in conjunction with a hydrophilic guidewire has been used in fistula embolization with cyanoacrylate glue for spinal vascular malformations at our institution. This combination has improved our success rate in achieving superselective catheterization of the fistula. Thus, 4 of 5 patients with type I perimedullary AVFs could be cured with this technique. Like type II and type III perimedullary AVFs, the endovascular approach may also be the first line of treatment in type I perimedullary spinal AVF.

The Catz-Itzkovich Spinal Cord Independence Measure was found to be reliable and more sensitive than the FIM to functional changes, when used by a multidisciplinary team. This study was performed to find out whether assessment may be similar when done by a single rater. Twenty-eight patients with spinal cord lesions participated in the study, in which examinations performed within a week by a single nurse or a team were compared for correlation, differences and agreement. The team members scored their relevant fields. A significant correlation was found between the nurse's scoring and that of physiotherapists and occupational therapists (r = 0.82-0.94; p < 0.0001), and the differences between the mean scores were small. The agreement between raters was modest, however (total agreement 38-90%, Kappa 0.17-0.73). It was concluded that although disability assessment performed by a single nurse may not be as accurate as by a multidisciplinary team, it could be reliable and valid.

Diagnosis of acute myelopathy applies to subjects with acute motor involvement, segmental sensory loss and sphincter dysfunction, reaching a maximum no later than 4 weeks after onset. Among the different causes of acute myelopathy (e.g. compressive, vascular, traumatic or toxic lesions, paraneoplastic involvement and electrical injury), acute myelitis due to infective agents or to an autoimmune-inflammatory process frequently causes problems relating to the differential diagnosis of multiple sclerosis (MS) and its potential risk of developing MS.

The paper summarizes surveys of over 2500 patients with tuberculous spondylitis complicated by neurological disorders of the spinal cord. The authors identified 5 grades of these disorders: from reflex disorders to limb plegia and pelvic dysfunctions. The classification proposed made it possible to clearly systematize spinal cord disorders in patients with tuberculous spondylitis, to establish a topical diagnosis and the severity of spinal cord lesions, to choose surgical accesses and method for stabilizing the spine, and to make a research processing of clinical and neurological data.

Cancer can affect the peripheral nervous system by non-metastatic, sometimes immune-mediated mechanisms. Recognition of these paraneoplastic syndromes is important because it can lead to the detection of the tumor, and also helps to avoid unnecessary studies to determine the cause of the neurologic symptoms in patients with cancer. Many paraneoplastic syndromes of the peripheral nervous system are not associated with serum antibodies that serve as markers of paraneoplasia. For this group of disorders the diagnosis depends on the clinician's index of suspicion and conventional electrophysiologic and laboratory tests. Treatment of the tumor, immunotherapy, or both may improve some of these syndromes. This review focuses on paraneoplastic syndromes of the spinal cord, peripheral nerve, and muscle., (Copyright 2000 John Wiley & Sons, Inc.)

Purpose: To document the changes observed at sequential magnetic resonance (MR) imaging of sarcoidosis lesions of the central nervous system (CNS) during treatment with corticosteroids., Materials and Methods: The abnormalities detected in 24 patients (mean follow-up, 36 months) were compared before and after therapeutic periods (n = 75) that were divided into attack (high-dose), upkeep (decreased-dose), and minimal (low-dose) periods. Parenchymal lesions were classified as type 1 (enhanced with gadolinium), type 2 (demyelinating), or type 3 (lacunar) and were assessed as regressing, stable, or progressing., Results: Seven of the 24 patients had several types of lesions. Isolated type 3 lesions (six patients) were the only lesions not associated with neurologic deficit. Type 1 lesions (13 patients) regressed in 22 of 22 attack periods and progressed in nine of 27 upkeep and minimal periods. MR imaging depicted relapses in patients with multifocal CNS involvement or long-standing CNS impairment or in those who had previously received steroid therapy. Type 2 (seven patients) and type 3 (13 patients) lesions remained stable in 68 of 68 therapeutic periods. Type 1 lesions appeared in three patients with type 2 and type 3 lesions during two upkeep and three minimal periods. Findings at follow-up MR imaging contributed to the reintroduction of high-dose corticosteroid therapy in eight patients., Conclusion: MR imaging can be used to differentiate between reversible and irreversible lesions in CNS sarcoidosis. MR imaging can be a useful tool for adjusting treatment to prevent irreversible CNS damage.

Hirayama's disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. We report clinical and magnetic resonance imaging findings in nine patients with Hirayama's disease. Cervical imaging of seven patients revealed spinal cord changes consisting of focal atrophy and foci of signal alterations. On neck flexion a forward movement and mild reduction in the anteroposterior diameter of the lower cervical cord against the vertebral bodies was noted in affected individuals as well as in five normal controls. In contrast to earlier reports, none of our patients showed complete obliteration of the posterior subarachnoid space. Measurement of the anteroposterior spinal cord diameter in each vertebral segment (C4-C7) revealed no significant differences in the degree of spinal cord flattening between the two groups. Furthermore, two of our patients had significant degenerative changes in the cervical spine (disc herniation, retrospondylosis) contralateral to the clinically affected side. These degenerative changes resulted in a marked cord compression on neck flexion but were not associated with ipsilateral clinical abnormalities or spinal cord alterations. Our results argue against a flexion-induced cervical myelopathy and support the view that Hirayama's disease is an intrinsic motor neuron disease.

The candidate region for spinal muscular atrophy (SMA) has been defined as a 750 kb interval on 5q13. In this study, we performed allelic association studies in 154 German SMA families with the multicopy markers Ag1-CA (D5S1556); C212 (D5F149S1/S2) and correlated genotype data with deletion of candidate genes. Both multicopy markers recognize 0-3 alleles pro chromosome. Deletions were detected for all copies of the markers Ag1-CA (C272) and C212 in 13 of 88 (15%) type I SMA patients and three of 48 (6%) type II patients. In all informative cases, the deletion was inherited from one parent. In two further cases (one type I and one type III SMA), de novo deletions of only one copy of Ag1-CA and C212 were found. In both cases the patients were homozygously deleted for the survival motor neuron (SMN) gene (exons 7 and 8) but only the type I SMA patient was deleted for the neuronal apoptosis inhibitory protein (NAIP) gene (exons 5 and 6). A third case (type II SMA) showed de novo deletion of SMN, but not of Ag1-CA, C212 and NAIP. Specific alleles of Ag1-CA and C212 showed significant association with SMA, particularly in type I SMA. When the number of marker copies defines genotypes, 1,1 (one allele on each chromosome) is found to be increased in type I SMA (50%) and 1,2 (one allele on one chromosome and two alleles on the other one) in type II SMA (60%). The 2,2 genotype (two alleles on each chromosome) was found in 4% of type I and II patients. By comparison, pooled normal genotype frequencies were 20, 44 and 36%, respectively. These results suggest a strong correlation between genotype and severity of disease. Based on these data we propose a model which indicates that type I SMA patients are composed of two severe alleles, type II of a mild and a severe, and type III of two mild alleles. Correlation of Ag1-CA genotype with deletion of the XS2G3/NAIP genes indicates that most patients with a deletion have a 1,1 genotype. Owing to the physical proximity of these markers, we propose that a large deletion occurs on type I SMA chromosomes that removes DNA between C212 and XS2G3/NAIP and that type II SMA results from compound heterozygosity for mild (small deletion) and severe mutations.

Between 1987 and 1991 the spine was stabilised in 205 patients using corundum ceramic implants. The radiological results were assessed with a follow up of from 6 to 24 months and were graded as good in 81%. Poor results were not due to the implanted material, but were usually caused by failure to obtain full correction of the deformity at operation. Porous ceramic implants produce better radiological results, with a decrease in operating time and a reduced risk of infection.

To investigate possible etiologic influences on the development of constipation, the present study examines the concordant occurrence of constipation with other diseases. Using 8.8 million Medicare patients hospitalized in the United States during 1987, the frequency distribution of all three-digit International Classification of Diseases (ICD) codes was compared in constipated patients and patients without constipation. Most associations and the closest associations were observed between constipation and neurologic diseases, the majority of which resulted from damage to the central nervous system. Associations between constipation and disorders of the spine also involved neuronal damage. The influence of potential confounding factors such as immobility or pain on the development of constipation was assessed and found to be minimal. The frequent association of constipation with disorders involving neuronal malfunction suggests that disruption of the neural modulation of colonic motility may play an important role in the development of constipation.

To ascertain the relationship between the clinical neurological level, and bladder and sphincter behavior, the video-urodynamic studies of 489 patients with spinal cord lesions due to a variety of causes were retrospectively analyzed. Patients were classified based on the clinical neurological level, etiology of the lesion and presence or absence of signs of sacral cord involvement. Urodynamic findings were classified as either detrusor hyperreflexia, detrusor-external sphincter dyssynergia, detrusor areflexia or normal. The results indicate that although there was a general correlation between the neurological level of injury and the expected vesicourethral function, it was neither absolute nor specific. For example, 20 of 117 cervical cord lesions had detrusor areflexia, 42 of 156 lumbar cord lesions had detrusor-external sphincter dyssynergia and 26 of 84 sacral cord had either detrusor hyperreflexia or detrusor-external sphincter dyssynergia. However, if one considers the presence of neurological abnormalities, 84% of the suprasacral cord lesions with detrusor areflexia have sacral cord signs. In contrast, all suprasacral cord lesions with no evidence of sacral cord involvement have either detrusor hyperreflexia or detrusor-external sphincter dyssynergia. The positive predictive value for positive sacral cord signs and detrusor areflexia was 87%. The positive predictive value for negative sacral cord signs and detrusor hyperreflexia/detrusor-external sphincter dyssynergia was 81%. These data suggest that the clinical neurological examination alone is not an adequate barometer to predict neurourological dysfunction and that video-urodynamic evaluation provides a more precise diagnosis for each patient.

The postoperative progress of 3 patients with spinal epidural hemorrhage, but without spinal fracture or dislocation, is presented. From the literature, 158 cases were collected of spontaneous spinal epidural hematoma treated surgically. Postoperative return of motor function was noted in 95.3%, 87%, and 45.3% of the patients with incomplete sensorimotor, incomplete sensory but complete motor, and complete sensorimotor lesions, respectively. Complete sensorimotor recovery occurred in 41.9%, 26.1%, and 11.3% of these 3 groups of patients, respectively. Recovery following surgical treatment depends on the severity of neurological deficits before treatment. However, the absence of motor or sensorimotor functions preoperatively does not necessarily indicate a poor prognosis.

A patient presenting with progressive paraparesis was found to have a spinal arteriovenous fistula at the T3-4 vertebral level. The lesion consisted of a direct communication of the anterior spinal artery with a very distended venous varix that drained mostly superiorly to the posterior fossa and simulated a posterior fossa arteriovenous malformation (AVM) on vertebral angiography. The patient was treated by surgical ligation of the fistula through an anterior transthoracic approach. He deteriorated abruptly on the 4th postoperative day, probably because of retrograde thrombosis of the enlarged anterior spinal artery. Over the next few months, he improved to the point of being able to walk with crutches. He has also regained sphincter control. The different types of spinal AVM's are reviewed. Our case does not fit into any of these groups. A new category, Type IV, is proposed to designate direct arteriovenous fistulas involving the intrinsic arterial supply of the spinal cord.

In recent years the name of Brown-Séquard has been assigned to syndromes caused by spinal cord damage differing widely in extension. On the basis of a review of more than 600 published cases, we propose that Brown-Séquard-plus syndromes be distinguished from the pure Brown-Séquard syndrome. This distinction is shown to be justified on clinical, anatomic, and historical grounds. Two case histories are reported herein to illustrate the essential differences between these syndromes.