A critical reappraisal of corticospinal tract somatotopy and its role in traumatic cervical spinal cord syndromes.

The corticospinal tract (CST) is the preeminent voluntary motor pathway that controls human movements. Consequently, long-standing interest has focused on CST location and function in order to understand both loss and recovery of neurological function after incomplete cervical spinal cord injury, such as traumatic central cord syndrome. The hallmark clinical finding is paresis of the hands and upper-extremity function with retention of lower-extremity movements, which has been attributed to injury and the sparing of specific CST fibers. In contrast to historical concepts that proposed somatotopic (laminar) CST organization, the current narrative summarizes the accumulated evidence that 1) there is no somatotopic organization of the corticospinal tract within the spinal cord in humans and 2) the CST is critically important for hand function. The evidence includes data from 1) tract-tracing studies of the central nervous system and in vivo MRI studies of both humans and nonhuman primates, 2) selective ablative studies of the CST in primates, 3) evolutionary assessments of the CST in mammals, and 4) neuropathological examinations of patients after incomplete cervical spinal cord injury involving the CST and prominent arm and hand dysfunction. Acute traumatic central cord syndrome is characterized by prominent upper-extremity dysfunction, which has been falsely predicated on pinpoint injury to an assumed CST layer that specifically innervates the hand muscles. Given the evidence surveyed herein, the pathophysiological mechanism is most likely related to diffuse injury to the CST that plays a critically important role in hand function.

Numerical investigation of the relative effect of disc bulging and ligamentum flavum hypertrophy on the mechanism of central cord syndrome.

BACKGROUND: The pathogenesis of the central cord syndrome is still unclear. While there is a consensus on hyperextension as the main traumatic mechanism leading to this condition, there is yet to be consensus in studies regarding the pathological features of the spine (intervertebral disc bulging or ligamentum flavum hypertrophy) that could contribute to clinical manifestations. METHODS: A comprehensive finite element model of the cervical spine segment and spinal cord was used to simulate high-speed hyperextension. Four stenotic cases were modelled to study the effect of ligamentum flavum hypertrophy and intervertebral disc bulging on the von Mises stress and strain. FINDINGS: During hyperextension, the downward displacement of the ligamentum flavum and a reduction of the spinal canal diameter (up to 17%) led to a dynamic compression of the cord. Ligamentum flavum hypertrophy was associated with stress and strain (peak of 0.011 Mpa and 0.24, respectively) in the lateral corticospinal tracts, which is consistent with the histologic pattern of the central cord syndrome. Linear intervertebral disc bulging alone led to a higher stress in the anterior and posterior funiculi (peak 0.029 Mpa). Combined with hypertrophic ligamentum flavum, it further increased the stress and strain in the corticospinal tracts and in the posterior horn (peak of 0.023 Mpa and 0.35, respectively). INTERPRETATION: The stenotic typology and geometry greatly influence stress and strain distribution resulting from hyperextension. Ligamentum flavum hypertrophy is a main feature leading to central cord syndrome.

Idiopathic tumoral calcinosis-like lesion in the lower cervical spine causing acute central cord syndrome: case report.

A 57-year-old male presented with recurrent falls, bilateral lower-limb paresthesia, and severe neck pain. Imaging revealed a mass compressing his spinal cord. He was admitted for further workup for spinal cord compression. Within 24 hours of admission, he developed upper-extremity weakness while maintaining lower-extremity function. He underwent urgent decompression of his spinal cord. During exposure, a white, creamy odorless substance was noted. This same substance was found under pressure within the spinal canal. The mass was grossly removed, and the patient's weakness improved postoperatively. Based on the clinical picture, intraoperative presentation, and final histological examination, idiopathic tumoral calcinosis-like lesion was considered as the most appropriate diagnosis.

Recurrent Neurological Deterioration after Conservative Treatment for Acute Traumatic Central Cord Syndrome without Bony Injury: Seventeen Operative Case Reports.

The mechanisms of late recurrent neurological deterioration after conservative treatment for acute traumatic central cord syndrome (ATCCS) remain unclear. Seventeen operative cases sustaining late recurrent neurological deterioration after conservative treatment for ATCCS were reviewed to investigate the mechanisms. The assessment of neurological status was based on International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI). Gender, age, cause of injury, results of image, conservative treatment and operative data, and neurological status at different time points were recorded. The mean age of 17 patients was 43.8 ± 2.3 years old, and the causes of the cervical injury were 14 vehicle accidents and 3 falls. The neurological deficits of 17 patients on admission were not serious, and patients recovered quickly after conservative treatment. No fractures or dislocation were found in any patient's radiographs or CT scan images. All 17 patients performed first MRI test in 4 days and there was a slight or mild compression on the spinal cord in 16 patients. Eight patients had a second MRI scan ∼6 weeks later, which showed that there was aggravated compression on the spinal cord in six patients. All patients underwent an anterior approach to cervical decompression and internal fixation operation. During the operation, there were loose discs found in all 17 patients, obvious ruptures of disks found in 3 patients, obvious ruptures of anterior longitudinal ligaments (ALLs) found in 8 patients, and obvious ruptures of posterior longitudinal ligaments (PLLs) found in 7 patients. There was serious adhesion between PLLs and cervical disks in 12 patients. In five patients, partial ossification of PLLs was detected. All patients had a good neurological outcome at 6 month follow-up. Ruptures of ALLs, PLLs, and discs resulting in cervical instability and secondary compression on the spinal cord were important causes for recurrent neurological deterioration after conservative treatment for ATCCS. With timely spinal decompression after recurrent neurological deterioration, patients could achieve a good neurological outcome.

The effect of vertebral fracture on the early neurologic recovery in patients with central cord syndrome.

PURPOSE: To compare early changes in the ASIA Motor Score (AMS) between patients with central cord syndrome (CCS) from an acute fracture to patients without a fracture. METHODS: Patients with CCS were identified and stratified based on the presence of a fracture. The AMS through the first week of the patients' hospitalization was obtained. Initial injury severity as well as early neurologic recovery was measured using the AMS. Analysis of variance was performed to determine if age, gender, rectal tone at presentation, congenital stenosis, or surgery within 24 h significantly effected the change in AMS. RESULTS: A strong trend (p = 0.0504) towards a more severe initial neurologic injury in patients with a fracture (AMS 59.7) than in patients without a fracture (AMS 70.2) was identified. However, in the week after injury, patients with a fracture had an improvement in their neurologic function (ΔAMS +4.8) while patients without a fracture demonstrated neurologic decline (ΔAMS -5.9). The change in AMS between patients with and without a fracture was nearly significant (p = 0.06). CONCLUSION: Patients with central cord syndrome present with similar symptoms, but injuries with and without a fracture may be associated with a different early neurologic recovery. Patients with a fracture have a more severe injury at initial presentation, but tend to have neurologic improvement in the first week; conversely patients without a fracture have a less severe initial neurologic injury, but tend to have a slight decline in neurologic function over the first week.

Hyperextension cervical spine injuries and traumatic central cord syndrome.

Traumatic central cord syndrome (TCCS), regardless of its biomechanics, is the most frequently encountered incomplete spinal cord injury. Patients with TCCS present with disproportionate weakness of the upper extremities, and variable sensory loss and bladder dysfunction. Fractures and/or subluxations, forced hyperextension, and herniated nucleus pulposus are the main pathogenetic mechanisms of TCCS. Nearly 50% of patients with TCCS suffer from congenital or degenerative spinal stenosis and sustained their injuries during hyperextension as originally described by Schneider in 1954. Immunohistochemical and imaging studies indicate mild to moderate insult to axons and their ensheathing myelin in the lateral funiculi culminating in cytoskeletal injury and impaired conduction. More than one-half of these patients enjoy spontaneous recovery of motor weakness; however, as time goes on, lack of manual dexterity, neuropathic pain, spasticity, bladder dysfunction, and imbalance of gait render their activities of daily living nearly impossible. Based on the current level of evidence, there is no clear indication of the timing of decompression for relief of sustained spinal cord compression in hyperextension injuries. Future research, taking advantage of validated digital imaging data such as maximum canal compromise, maximum spinal cord compression, and lesion length on the CT and MR images, as well as more sensitive measures of bladder and hand function, spasticity, and neuropathic pain may help tailor surgery for a specific group of these patients.

Acute traumatic central cord syndrome: analysis of clinical and radiological correlations.

AIM: In patients with traumatic spinal cord injury, several studies correlate neurological impairment and radiological findings. However, little information is available about this correspondence in the particular group of acute traumatic central cord syndrome. The object of the present work was to describe the clinical and radiological features of a series of patients presenting with acute traumatic central cord syndrome and to analyze clinical and radiological correlations on admission and at last follow-up. METHODS: Retrospective review of 15 patients diagnosed of acute traumatic central cord syndrome between 1995 and 2005. Global motor score and motor score in upper extremities were determined on admission and at last follow-up (6 months-4 years, mean 16 months). Plain films, cervical computed tomography and magnetic resonance (MR) were performed in every patient and retrieved for the study. In seven patients, serial MR studies were performed during follow-up. Clinical and radiological correlations were statistically analyzed with non-parametric tests. RESULTS: Cervical spondylosis appeared associated with older age, falls, and absence of fracture. Spinal cord edema was the most common finding in MR studies but hemorrhage was also observed. The length of spinal cord edema significantly correlated with initial motor score. The decrease in T2-weighted hyperintensity in serial MR studies correlated with the gain of motor power in upper limbs at last follow-up. CONCLUSION: Elderly patients with more degenerated cervical spines commonly develop acute traumatic central cord syndrome after incidental falls. Length of spinal cord edema correlates with neurological impairment on admission and may provide significant prognostic information.

Surgery for acute subaxial traumatic central cord syndrome without fracture or dislocation.

Twenty-two patients with subaxial acute traumatic central cord syndrome (CCS) without fracture or dislocation who underwent surgery between 1995 and 2002 were reviewed, retrospectively. There were 13 males and nine females ranging in age from 24 to 84 years (mean 61.2). Falls were the most common injury (68%), followed by motor vehicle accidents (32%). All patients had dynamic cervical lateral radiographs and magnetic resonance imaging (MRI). Cord compression was present in all cases and cervical instability in 11. Associated pathology included disc herniation in seven patients, cervical spondylosis (CS) in 11 and ossification of the posterior longitudinal ligament (OPLL) in four. Anterior decompression and fusion was performed in 12 patients with 1- or 2-level lesions. Posterior decompression and fusion was performed for multilevel lesions in 11 patients, including one patient who required re-operation. The interval between injury and surgery ranged from 1 to 37 days (mean 8.0). Postoperatively, all patients improved clinically. We conclude that surgical management of subaxial acute traumatic CCS without fracture or dislocation improved neurological status and prevented delayed neurological deterioration in our patients.

Traumatic central cord syndrome: analysis of factors affecting the outcome.

BACKGROUND: The indications and timing of treatment, as well as the best treatment method for traumatic central cord syndrome (CCS), remain controversial. The aims of this study are to determine the prognostic factors of traumatic CCS and to determine appropriate surgical indications. METHODS: We reviewed the clinical and radiological data of 47 patients with this syndrome. The data collected included age, neurological status as measured on a scale defined by the Japanese Orthopaedic Association (JOA), anteroposterior (AP) diameter of the spinal canal on computed tomography, signal intensity change of the spinal cord on T2-weighted magnetic resonance imaging (MRI), associated spinal diseases, and the type of treatment received. The correspondence between the clinical and radiological findings and the neurological outcome was investigated. RESULTS: The patient's age, JOA score on admission, signal intensity change of the spinal cord on MRI, and associated spinal diseases were not significant in predicting the patient's recovery. On the other hand, the AP diameter of the spinal canal (P = .0402) and the interval between injury and surgery (P < .0001) were factors predictive of excellent recovery. In the surgical treatment group, timely surgery was found to improve the outcome, while conservative treatment did not improve the outcome of patients with a low JOA score, a relatively small AP diameter of the spinal canal, or a positive signal intensity change of the spinal cord on T2-weighted MRI. CONCLUSION: The AP canal diameter of the spinal canal and the interval between injury and surgery may be reliable predictors of excellent recovery in patients with CCS. We recommend timely surgery, preferably within 2 weeks of injury, to achieve a better functional outcome in selected patients.

Cutaneous silent periods in intramedullary spinal cord lesions.

OBJECTIVE: The neurophysiological assessment of intramedullary spinal cord lesions has been unsatisfactory. Previous studies in patients with syringomyelia suggest that testing of cutaneous silent periods (CSPs) may be useful to assess centromedullary lesions. METHODS: The authors studied nine patients with intramedullary spinal cord lesions of different etiologies. Eight patients with cervical lesions presented with hypalgesia, hypothermesthesia, or pain in at least one upper extremity; five of them had also upper limb weakness or sensory impairment. One patient with a thoracic lesion had normal upper limb function. The authors recorded CSPs in abductor pollicis brevis muscle following digit II and digit V stimulation. Somatosensory evoked potentials (SEPs) were obtained following median and tibial nerve stimulation. Motor evoked potentials (MEPs) were obtained in biceps brachii, abductor digiti minimi and tibialis anterior muscles following transcranial magnetic or electrical stimulation. RESULTS: CSP abnormalities were found in all patients with cervical lesions, but not in the patient with a thoracic lesion. Cortical median nerve SEPs had normal latencies in all patients, while tibial nerve SEPs, upper limb MEPs, and lower limb MEPs were delayed in five patients each. In one patient, abnormal CSP were the only neurophysiological finding. CSP abnormalities were associated with hypalgesia and hypothermesthesia in 95% of the studies. CONCLUSION: Upper extremity CSP testing is a sensitive neurophysiological technique for the assessment of cervical intramedullary lesions. In particular, abnormal CSPs are highly associated with spinothalamic dysfunction.

Treatment of chronic neuropathic pain after traumatic central cervical cord lesion with gabapentin.

Central cord syndrome may be associated with severe pain, resistant to conventional pain therapy regimens. Chronic pain may be a persistent problem in rehabilitation of spinal cord injuries. These pain syndromes are long lasting and challenging to treat. Gabapentin has been shown to be useful in treatment of different conditions which may be caused by increased neuronal excitability. This report describes a case where central cord syndrome and its chronic neuropathic pain associated with allodynia was successfully treated with gabapentin.

Selective paralysis of the upper extremities after odontoid fracture: acute central cord syndrome or cruciate paralysis?

A patient presented with selective paralysis of the arms after having sustained a fall. X-ray of the cervical spine showed a type II odontoid fracture with posterior atlantoaxial dislocation. The diagnosis in the emergency room was cruciate paralysis, which is frequently associated with fractures of axis and/or atlas. However, magnetic resonance imaging (MRI) of the cervical spine revealed a lesion consistent with the acute central cord syndrome (CCS) at the C2-C6 level. The patient underwent posterior atlantoaxial arthrodesis to correct instability and was discharged, without much neurological improvement. Cruciate paralysis has been reported to be associated with fractures of axis and/or atlas, and acute CCS has rarely been associated with the fractures. However, this case illustrates that the lesion responsible for selective paralysis of the upper extremities is not as specific as it had been thought to be, and that it is difficult to accurately identify the level of the cervical cord injury by neurological diagnosis and X-rays alone. Supplementary diagnostic modalities, particularly MRI, are required to make a correct diagnosis and develop a therapeutic strategy.

A histopathological analysis of the human cervical spinal cord in patients with acute traumatic central cord syndrome.

STUDY DESIGN: We have applied conventional histochemical and morphometric techniques to study the changes within the human spinal 'hand' motor neuron pool after spinal cord injury in patients who presented with acute traumatic central cord syndrome (ATCCS). OBJECTIVE: To determine whether a reduction of large alpha motor neurons at the C7, C8 and T1 spinal cord levels underlies the mechanism which causes hand dysfunction seen in patients with (ATCCS). BACKGROUND: The etiology of upper extremity weakness in ATCCS is debated and injury and/or degeneration of motor neurons within the central gray matter of the cervical enlargement has been advanced as one potential etiology of hand weakness. METHODS: The spinal cords of five individuals with documented clinical evidence of ATCCS and three age-matched controls were obtained. The ATCCS spinal cords were divided into acute/sub-acute (two cases) and chronic (three cases) groups depending on the time to death after their injury; the chronic group was further subdivided according to the epicenter of injury. We counted the motor neurons using light microscopy in 10 randomly selected axial sections at the C7, C8 and T1 spinal cord levels for each group. We also analyzed the lateral and ventral corticospinal tracts (CST) in all groups for evidence of Wallerian degeneration and compared them to controls. RESULTS: A primary injury to the lateral CST was present in each case of ATCCS with evidence of Wallerian degeneration distal to the epicenter of injury. There was minimal Wallerian degeneration within the ventral corticospinal tracts. In the chronic low cervical injury group, there was a decrease in motor neurons supplying hand musculature relative to the other injury groups where the motor neurons sampled at the time of death were not reduced in number when compared to the control group. CONCLUSIONS: We hypothesize that hand dysfunction in ATCCS can be observed after spinal cord injury without any apparent loss in the number of motor neurons supplying the hand musculature as seen in our acute/sub-acute (n=2) and our chronic high injury (n=1) groups. The motor neuron loss seen in the chronic low level injury was felt to be secondary to the loss of C7, C8, and T1 neurons adjacent to the injury epicenter.