MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio-Brailsford syndrome)

Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto-axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri-odontoid infiltration. Transverse/alar ligament incompetence causes instability. Atlanto-axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre-existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1–2 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal-body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1–2 level cord compression was evident in the canal-cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis.     

Is continuation of anti-tumor necrosis factor-α therapy a safe option for patients who have developed pulmonary mycobacterial infection?

Continuation of anti-tumor necrosis factor-α (TNFα) therapy generally has not been recommended for patients who have developed nontuberculous mycobacterial (NTM) diseases; in daily practice, however, we often encounter patients with refractory rheumatoid arthritis (RA) who experience uncontrollable flares following withdrawal of anti-TNFα agents. Here, we report a case of pulmonary NTM disease caused by Mycobacterium intracellulare occurring in a patient with refractory RA undergoing etanercept therapy. Since there was the concern of an exacerbation of RA symptoms, etanercept was continued during anti-NTM therapy. The patient’s pulmonary symptoms and radiological abnormalities were found to have markedly improved in a relatively short time period after beginning the anti-NTM therapy. Additionally, her RA symptoms were adequately controlled without the occurrence of any unexpected adverse events. The continuation of etanercept therapy may be a safe option during anti-NTM therapy if patients’ underlying diseases would otherwise be difficult to control. Strictly supervised anti-NTM therapy and patients’ informed consent are mandatory. We review the medical literature on NTM disease associated with anti-TNFα therapy for rheumatic diseases and discuss the safety of simultaneous use of anti-TNFα agents in patients during anti-NTM therapy.     

Correction of kyphosis in tuberculous spondylitis in children]

Various modifications of radical reparative surgery were made in 145 children with tuberculous spondylitis were comparatively analyzed. Greatest correction of kyphosis was achieved by combining anterior spinal repair with posterior plunged contractors, in additional resection of the apical vertebral arch in particular. There is a direct correlation between the degree of compression of the dural sac and the severity of neurological disorders. Anterior decompression of the spinal cord is an obligatory surgical component in tuberculous spondylitis, eliminates central stenosis of the spinal canal; however, it is insufficient to eliminate neurological disorders caused by myelic ischemia.     

Treatment of upper cervical spine involvement in rheumatoid arthritis patients

The cervical spine, especially the upper cervical spine, is a common focus of destruction by rheumatoid arthritis (RA). Because of its potentially debilitating and life-threatening sequelae, cervical spine involvement remains a priority in the diagnosis and treatment of RA. Many studies show that early surgical intervention gives a more satisfactory outcome. Surgery aims to establish spinal stability and to prevent neurological deterioration and injury to the spinal cord, leading to improved neurological function. The recent sophisticated screw-rod-plate technique allows one to obtain a solid fixation of the upper cervical spine with a high possibility of bone union even in RA patients. Although surgery of the occipitoatlantoaxial region is a challenge with many possibilities of serious complications, recent advances in the surgical technique, complete understanding of the anatomy, and precise preoperative evaluation have decreased complication rates. Early consultation with a specialized spine surgeon is mandatory once cervical involvement is suspected in an RA patient because once the patient becomes myelopathic, the rate of long-term mortality increases and the chance of neurological recovery decreases.     

Radiological changes in the cervical spine in rheumatoid arthritis -- prognostic factors obtained by a cross-sectional study

OBJECTIVE: The involvement of the cervical spine is the most serious skeletal manifestation of rheumatoid arthritis (RA). Instabilities of the upper cervical spine can lead to neurological complications and signs of vertebrobasilar insufficiency. The study investigates the relationship between the course of the RA and the degree of radiological changes in the cervical spine and describes prognostic factors of cervical spine involvement. MATERIAL AND METHODS: Clinical data were gathered from 205 patients with RA. Standardized X-ray examination of the cervical spine was performed and arthritic changes were assessed by a semiquantitative score. The association of clinical symptoms with defined radiological changes was evaluated. RESULTS: Radiological changes in the cervical spine related to RA were found in 67.8% of the patients. Anterior atlantoaxial dislocations were detected in 25.4%, subaxial dislocations in 55.6% and a vertical displacement of the dens in 21.5% of the cases. The degree of radiological changes in the cervical spine was not directly correlated with neurological symptoms. Age above 55 years, disease duration of more than 10 years, an onset of cervical pain later than 11 years after the diagnosis of RA, steroid therapy for more than 5 years, bilateral shoulder involvement and the presence of severe erosive changes in at least one peripheral joint were positively associated with the development of severe changes in the cervical spine. In patients with basilar impression, paraesthesia in the upper extremities was significantly more common. CONCLUSION: Prognostic factors are helpful for the assessment of risk of cervical spine involvement in RA. Since definite clinical hints for cervical involvement are absent, the cervical spine has to be included in the routine examination of patients with RA.     

Severe low back pain in patients with rheumatoid arthritis is associated with Disease Activity Score but not with radiological findings on plain X-rays

Abstract

Objective. To investigate the prevalence and associated factors of severe low back pain (LBP) among patients with rheumatoid arthritis (RA).

Methods. This cross-sectional study included 201 patients with RA without prior spinal surgery. Severe LBP was defined as that with a visual analog scale (VAS) score of ≥ 50 mm within the previous 4 weeks. Lumbar lesions, sagittal alignment, and disc degeneration were evaluated by plain standing X-rays and magnetic resonance imaging. Associated factors of severe LBP were evaluated using multiple logistic regression analysis.

Results. Forty-eight patients (23.8%) had LBP with a VAS score of ≥ 50 mm. Multivariate analysis indicated that the associated factors for severe LBP were female, smoking, and moderate and high disease activity on the Disease Activity Score in 28 joints–erythrocyte sedimentation rate (DAS28-ESR). There was no relationship between severe LBP and any radiological findings. Among DAS28-ESR subscores, patients with severe LBP had significantly higher tender joint counts and VAS scores for general health.

Conclusions. The prevalence of severe LBP was relatively high in patients with RA. The factor most closely associated with severe LBP was Disease Activity Score, but not radiological findings. Severe LBP was related to the tender joint count or subjective complaints of RA.     

Classification of neurological disorders in tuberculous spondylitis

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.     

Pathologie vasculaire de la moelle

Compared to cerebral ischemia, spinal cord ischemia remains rare, and results in various types of neurological deficiencies such as abrupt and complete paraplegia. Magnetic resonance imaging is a valuable tool to rule out other causes of acute spinal cord syndromes and to show the infarct. Most spinal cord infarcts are due to aortic diseases (atherosclerosis, aneurysm, dissection), or are of iatrogenic origin (surgery of the aorta and aortography). Spinal hemorrhagic strokes are usually the consequence of arterio-venous malformations or coagulation disorders. These malformations require interventional radiology.     

Neck pain in the elderly: a management review. Part II

Rheumatoid arthritis and metastatic cancer occur commonly in the elderly, and may cause neck pain. Rheumatoid arthritis may produce cervical radiculopathy and myelopathy resulting from vertebral body subluxation, although radiological manifestations of subluxation are much more common than neurological dysfunction. Cervical spinal cord compression is a neurological emergency and may produce cervical radiculopathy as well as myelopathy. Careful neurological and radiological assessments are required to minimize pain and preserve neurological function in elderly patients suffering from neck pain complicating rheumatoid arthritis or cervical spinal metastasis.     

Live/Real Time Three‐Dimensional Transesophageal Echocardiographic Assessment of the Spinal Cord

We present an adult patient in whom live/real time three‐dimensional transesophageal echocardiography (3DTEE) provided incremental value in the assessment of the spinal cord as compared to two‐dimensional transesophageal echocardiographic (2DTEE) findings published in the literature. It improved accurate identification and assessment of the anterior radiculomedullary spinal arteries which may have an important clinical application in monitoring for spinal cord ischemia during thoracic aortic surgery. Because the spinal cord and spinal canal could be examined using not only transverse but also coronal (frontal), sagittal, and oblique planes, 3DTEE further allowed for three‐dimensional measurements of the dimensions and volumetric analysis of the visualized spinal cord and spinal canal. These may have implications in the assessment of spinal cord edema due to trauma and other conditions which result in increase in the size and volume of the spinal cord.     

Anorectal physiologic testing for bowel dysfunction in patients with spinal cord lesions

PURPOSE: Bowel dysfunction is common in patients with spinal cord lesions. This study aims to determine whether there are any discriminatory findings at anorectal physiologic testing in patients with spinal cord lesions. METHODS: Twelve consecutive patients (6 females) with significant spinal cord lesions who had mixed symptoms of constipation, fecal impaction, and fecal incontinence were evaluated by perfusion manometry and pudendal nerve terminal motor latency. None of the patients had a sphincter defect as evaluated by endoanal ultrasonography. RESULTS: The median age was 54 (range, 40-87) years. Eight (67 percent) of them had had traumatic spinal cord injuries. Other spinal cord lesions included spina bifida, syringomyelia, arachnoid cyst, and spinal cord ischemia after abdominal aortic aneurysm repair. In patients with spinal cord lesions, the mean (range) resting anal canal pressure and maximum squeeze anal canal pressure were 46 (10-100) mmHg and 76 (30-120) mmHg respectively compared with 62 (50-70) mmHg, and 138 (100-180) mmHg, respectively, in healthy controls. Eleven (92 percent) patients had prolonged pudendal nerve terminal motor latency (9 bilateral and 2 unilateral) whereas rectoanal inhibitory reflex was abolished in all 9 patients tested. CONCLUSIONS: Spinal patients with severe bowel symptoms tended to have lower anal canal pressures than healthy controls. Pudendal netropathy and impaired rectoanal inhibitory reflex are common and may be important in the pathogenesis of bowel dysfunction in patients with spinal cord lesions.     

有限减压内固定治疗胸腰段脊柱爆裂骨折(附46例报告)

对46例胸腰段脊柱爆裂骨折采用后方手术入路，在彻底有效减压的前提下应用锤击、推顶等方法进行复位，最大限度保留椎体的骨量及椎板附件。结果：46例术后获得良好的治疗效果，椎管矢状径由平均6mm增至12mm，压缩后的椎体高度与上、下椎体高度平均值比由术前65％增至90％，突入椎管骨量占椎管容积由平均60％减至20％。神经损伤恢复按ASIA分级有显著的提高。认为只要术前定位准确，术中操作仔细，有限减压同样能达到全椎板切除环状减压效果。     

A case of small cell lung cancer with intramedullary spinal cord metastasis

An 83-year-old man was admitted with paraplegia and loss of all sensation below the level of umbilicus, with bowel and bladder dysfunction. Stage IV small cell lung cancer had been diagnosed two years ago and had received several courses of chemotherapies. A magnetic resonance imaging revealed an enhanced mass in the intramedullary spinal cord at the level of Th10-L1. Metastatic spinal tumor was diagnosed by clinical and radiological examinations. This is a rare case of small cell lung cancer with intramedullary spinal cord metastasis which caused various neurological symptoms.     

Pelvic Pain as an Unusual First Presentation of a Demyelinating Disease

Pelvic pain as the presenting symptom of demyelinating disease is rare. We report on a 49-year-old female patient that initially had symptoms of pain and anesthesia in the perineum. Symptoms later evolved to include both lower and upper extremity weakness and were associated with enhancing spinal cord lesions on MRI. Recognizing that the patient’s disease was localized only to the spinal cord led to an eventual serological diagnosis of neuromyelitis optica (Devic’s disease), a demyelinating syndrome that is now considered distinct from multiple sclerosis and that primarily affects the spinal cord and optic nerves. Pelvic pain is an unusual first presentation of this illness. Additionally, this case illustrates the challenges of establishing a diagnosis of neuromyelitis optica. Recognizing the distinct clinical features of this rare illness, referring specifically from a spinal cord or ophthalmogical etiology, is essential for its rapid diagnosis, and hence for initiation of appropriate therapy.     

MRI of cervical spine with flexion and extension used in patients with rheumatoid arthritis

Patients with rheumatoid arthritis (RA) often have involvement of the cervical spine. The most common abnormality is atlanto-axial subluxation (AAS). The more serious vertical subluxation (VS) is thought to develop at a later stage. Direct cord compression may occur, but the symptoms may be vague and difficult to interpret. In addition to clinical follow up, RA patients undergo several conventional radiographs of the cervical spine, with addition of flexion and extension images. This, in spite of the fact that the cervical cord and soft tissue do not show. Magnetic resonance imaging (MRI), is the modality of choice to visualize soft tissue and the cervical medulla, but is rarely performed in the follow up of RA patients. Five patients with long-standing RA, episodes of neck pain, and known AAS were asked to volunteer for a MRI study of the cervical spine, consisting of sagittal T2 weighted images of the cervical spine during flexion and extension of the neck. Compared to clinical examinations and cervical radiographs, MRI gave valuable information not otherwise obtained. The importance of MRI with the neck in a flexed and extended position is stressed. This is possible to obtain within a conventional quadrature neck coil in many RA patients.     

Serum concentrations of formation (PINP) and resorption (Ctx) bone turnover markers in rheumatoid arthritis

Joint inflammation in rheumatoid arthritis (RA) induces local periarticular osteoporosis. Generalised bone mineral density (BMD) decrease concerns approximately 50% of rheumatic patients. Both types of bone mass depletion can issue from cytokine-induced (TNF-α, IL-1, IL-6) osteoclasts’ activation, osteoprotegerin and its ligand’s (RANKL) function disorders, patients’ immobilisation and glucocorticosteroid (GCS) intake, as well as from hormonal alterations in postmenopausal women, predominate among RA individuals. The aim of the study was to compare serum concentrations of marker of bone formation—serum aminoterminal propeptide of type I collagen (PINP), and bone resorption, carboxy (C) terminal telopeptide (Ctx), bone turnover markers in RA and osteoarthritis (OA) patients and in RA groups of different disease activity, different degree of joint damage and the history of GCS intake. A total of 50 RA female patients and 50 women with knee OA were included in the study. Blood for morphology and biochemistry laboratory tests was taken. Joint X-rays to establish OA and RA diagnosis and the degree of RA progression, as well as DEXA BMD measurements were performed. PINP and Ctx concentrations were assessed. In RA patients the number of swollen and painful joints, the duration of morning stiffness, visual analogue scale values and Waaler–Rose’s test activity were recorded. The Disease Activity Index (DAS 28) was counted from the appropriate formula. No differences in bone turnover markers’ concentrations were noted neither between RA and OA patients nor between the RA group when compared to the one without the history of GCS use. Bone turnover markers’ concentrations in RA were proportional to the number of swollen and painful joints. However, no correlation was found between the markers’ concentrations and RA activity assessed by DAS 28 or by laboratory means. Ctx concentrations were higher in patients at II degree joint damage according to Larsen and Dale’s than at more advanced stages. Ctx concentrations decreased with the disease duration. Serum morphogenesis and resorption markers’ concentrations change in course of RA indicating the decrease in bone metabolic activity with the disease duration and progression. High RA activity and severity correlate with increased markers’ levels—the resorption one. The influence of GCS on bone metabolism in RA requires further study.     

A patient with spinal metastasis from hepatocellular carcinoma discovered from neurological findings

Although spinal tumors are uncommon, they may reduce survival or cause serious functional disorders in the extremities. Metastatic spinal tumors from malignant tumors can induce symptoms of spinal cord compression, such as paraplegia, quadriplegia, and vesicorectal disturbance, which are aggravated with progression of the diseases and time. We report a patient with hepatocellular carcinoma (HCC) who was suspected of having spinal lesions based on neurological findings, and a metastatic spinal tumor was found by imaging examination. Assuming that metastasis had occurred at the time lumbar pain developed, the patient reached the level of gait disturbance within only 4 mo, showing a rapid advancement of symptoms. If early diagnosis had been possible, treatment could be performed before acute myelopathy progressed to complete paralysis. We speculate that the terminal stage of HCC is not only liver failure associated with intrahepatic lesions but also metastasis to other regions, treatment for individual pathologies therefore, will be needed, which constitutes an important issue.     

Radiographic progression of cervical lesions in patients with rheumatoid arthritis receiving infliximab treatment

We evaluated radiographic change in the cervical lesions of 47 RA patients receiving continuous infliximab therapy for at least 1 year. Infliximab treatment had been initiated between November 2003 and December 2007. Patients who were progressive and non-progressive in terms of RA cervical lesions were compared. Matrix metalloproteinase 3 (MMP3) values improved significantly only in non-progressive patients within the 1-year treatment window. Cervical lesion progression was suppressed in 19 of the 23 patients (83%) showing a good response to infliximab treatment and occurred in 16 of the 24 patients (67%) showing a moderate response. This difference was shown to be significant by the Fisher’s exact test (p = 0.002). In the well-responding patients (n = 23) and moderately responding patients (n = 24), the respective changes in the cervical lesion parameters within 1 year were: atlanto-dental interval, 0.17 ± 0.49 and 0.54 ± 0.58 mm (p = 0.013); spinal cord, −0.17 ± 0.49 and −0.54 ± 0.59 mm (p = 0.025); Ranawat value, −0.09 ± 0.29 and −0.42 ± 0.65 mm (p = 0.032). Based on these results, we conclude that infliximab treatment can be used to suppress the progression of rheumatoid arthritis (RA) cervical lesions. It is possible that response to infliximab and MMP3 values can be used to predict the progression of these cervical lesions.     

Failure of conservative treatment for lumbar spinal stenosis in elderly patients

Spinal stenosis syndrome affects mainly patients at their 5th-6th decades of life. There is a strong debate in the literature whether patients with spinal stenosis should be operated or treated conservatively. Our aim was to evaluate patients with lumbar spinal stenosis who were treated conservatively. All patients over 65 years of age who were handled conservatively in the private clinic of the senior author due to spinal stenosis syndrome were evaluated. There were 21 males and 15 females aged between 65 and 88 years. Intermittent claudication was the dominant clinical presentation. The radiological examination showed a frequent narrowing at the level L4-L5. All patients had a severe stenosis of less than 10mm diameter of the spinal canal. All patients underwent a conservative therapy which included physical therapy with ultrasound waves, short waves, and flexion exercises. All patients were offered to undergo an epidural or nerve root injection. Twenty-four patients agreed also to have one of these procedures to relieve their symptoms. All patients were followed between 6 and 10 months after the initial diagnosis. We used the Oswestry index category for evaluation. In all parameters the conservative treatment failed to improve the symptoms in the vast majority of patients. None of the patients was very satisfied with the results of conservative treatment while 33 patients (92%) were not so satisfied or unsatisfied with the results. We conclude that conservative treatment for lumbar spinal stenosis is not a success for elderly patients. These patients should be convinced to undergo operative treatment.