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PubMed2006

Brachytelephalangic chondrodysplasia punctata with severe spinal cord compression: report of four new cases.

Brachytelephalangic chondrodysplasia punctata (CDPX1, OMIM: #302950) is a rare congenital skeletal dysplasia caused by arylsulfatase E deficiency (OMIM: #300180). Although the symptoms are usually mild, severe spinal cord compression by dysplastic vertebras may develop. We report four new cases with severe cervical spinal canal narrowing documented by radiography, magnetic resonance imaging (MRI), and autopsy. In all, nine cases of CDPX1 with severe cervical spinal cord compression have now been described. Because these cases account for a large proportion of all reported CDPX1 cases, we believe that an antenatal suspicion of CDPX1 should lead to genetic counseling and to investigations for spinal cord compression. After birth, this complication must be routinely anticipated, and we suggest spinal MRI in all CDPX1 infants. Unless spinal cord compression is confidently ruled out, we recommend that these newborns receive the same care as trauma patients suspected of craniocervical junction disruption.

Abnormalities, Multiple↗
PubMed1990

Recovery of gait after radiotherapy in paralytic patients with metastatic epidural spinal cord compression.

We report on 15 patients with paralysis of the legs caused by metastatic epidural spinal cord compression. After radiation therapy 6 patients regained motor function, 5 of whom recovered ambulatory function, but the recovery was delayed for 3 months or more. The duration of the paralysis prior to treatment varied from 20 hours to 10 days with no significant difference between the group with and the group without recovery. The median time from the initial motor symptoms to total paralysis was longer (45 days) in the patients who recovered compared with those permanently paralyzed (9 days). The response to radiation therapy seems to depend on the rate of loss of spinal cord function rather than the length of total paralysis. We recommend active treatment of patients with paraplegia due to metastatic epidural spinal cord compression, even when the paralysis has been present for over a week.

Female↗
PubMed1998

Malignant spinal cord compression: prospective study of delays in referral and treatment.

OBJECTIVES: To examine the delay in presentation, diagnosis, and treatment of malignant spinal cord compression and to define the effect of this delay on motor and bladder function at the time of treatment. DESIGN: Prospective study of all new patients presenting to a regional cancer centre with this condition. SETTING: Regional cancer centre. SUBJECTS: 301 consecutive patients. MAIN OUTCOME MEASURES: Interval from onset of symptoms to presentation and treatment, delay at each stage of referral, and functional deterioration. RESULTS: The median (range) delay from onset of symptoms of spinal cord compression to treatment was 14 (0-840) days. Of the total delay, 3 (0-300) days were accounted for by patients, 3 (0-330) days by general practitioners, 4 (0-794) days by the district general hospital, and 0 (0-114) days by the treatment unit. Initial presentation to the regional cancer centre with symptoms of malignant spinal cord compression led to a significant reduction in delay to treatment and improved functional status at the time of treatment. Deterioration of motor or bladder function >=1 grade occurred at the general practice stage in 28% (57) and 18% (36) of patients, the general hospital stage in 36% (83) and 29% (66), and the treatment unit stage in 6% (19) and 5% (15), respectively. CONCLUSIONS: Unacceptable delay in diagnosis, investigation, and referral occurs in most patients with malignant spinal cord compression and results in preventable loss of function before treatment. Improvement in the outcome of such patients requires earlier diagnosis and treatment.

Cancer Care Facilities↗
PubMed2005

Spinal reirradiation after short-course RT for metastatic spinal cord compression.

PURPOSE: To investigate the feasibility and effectiveness of reirradiation (re-RT) for in-field recurrence of metastatic spinal cord compression after primary RT with 1 x 8 Gy or 5 x 4 Gy. METHODS AND MATERIALS: A total of 62 patients, treated with 1 x 8 Gy (n = 34) or 5 x 4 Gy (n = 28) between January 1995 and August 2003, received re-RT for in-field recurrence of metastatic spinal cord compression. The median time to recurrence was 6 months (range, 2-40 months). Re-RT was performed with 1 x 8 Gy (after 1 x 8 Gy or 5 x 4 Gy, n = 34), 5 x 3 Gy (after 1 x 8 Gy or 5 x 4 Gy, n = 15), or 5 x 4 Gy (after 1 x 8 Gy, n = 13). The cumulative biologically effective dose (primary RT plus re-RT) was 80-100 Gy2. The median follow-up after re-RT was 8 months (range, 2-42 months). Motor function was evaluated up to 6 months after re-RT. RESULTS: After re-RT, 25 patients (40%) showed improvement of motor function, 28 (45%) had no change, and 9 (15%) had deterioration. Of the 16 previously nonambulatory patients, 6 (38%) regained the ability to walk. No second in-field recurrence in the same spinal region was observed after re-RT. The outcome was not significantly influenced by the radiation schedule. Radiation myelopathy was not observed. CONCLUSIONS: Spinal re-RT with 1 x 8 Gy, 5 x 3 Gy, or 5 x 4 Gy for in-field recurrence of metastatic spinal cord compression appears safe and effective. Myelopathy seems unlikely, if the cumulative biologically effective dose is < or = 100 Gy2.

Adult↗
PubMed2002

Thoracic paraganglioma presenting with spinal cord compression and metastases.

We report a case of a 41-year-old man with a primary thoracic spine paraganglioma presenting with spinal cord compression and distant metastasis. Paragangliomas arising within the spinal canal are rare and when they occur are usually found in the intradural, extramedullary compartment and in the lumbosacral region. Thoracic paragangliomas are very rare, with only five prior cases reported in the literature and with distant metastasis seen in only one of these. In this case, the tumor was entirely extradural and produced extensive sclerosis of the surrounding vertebrae. The clinical, radiographic, and pathologic features of this case are detailed and the relevant literature is reviewed.

Adult↗
PubMed1982

The effect of acute spinal cord compression injury on thyroid function in the rat.

The effect of acute spinal cord injury on thyroid function was studied in rats subjected to severe spinal cord compression at T1. Serum thyroxine (T4), effective thyroxine index (ETI), and thyroid stimulating hormone (TSH) were measured at 1 and 100 minutes at one, three, and seven days after laminectomy and spinal cord injury. Control animals were subjected to laminectomy only. T4 was decreased at 1 minute after laminectomy with or without spinal cord injury, though the animals with cord injury had a much more profound reduction. The effects on TSH at 1 minute were dramatically different: laminectomy alone caused an elevation of TSH, while spinal cord injury produced a marked decline. At the later time intervals both groups showed gradual normalization of T4 and TSH levels, and at seven days there were no significant differences between the groups. Thus, acute spinal cord compression injury produced a major alteration in thyroid function during the acute phase.

Acute Disease↗
PubMed2006

Metastatic epidural spinal cord compression: update on management.

Until recently, patients with metastatic epidural spinal cord compression (MESCC) were routinely treated with corticosteroids and radiotherapy (RT). However, major advances in imaging, recognition of new prognostic factors, and new techniques in RT and surgery have led to a number of management choices that need to be considered when treating a patient with MESCC. In our view, the management should be individualized taking into account many variables. We present here some of the advances we believe are among the most important. A historical background to the modern management of this condition is first presented.

Epidural Neoplasms↗
PubMed1997

Cervical spinal cord compression in hereditary multiple exostoses. Report of a case and a review of the literature.

Spinal cord compression is an extremely serious complication of hereditary multiple exostoses (HME). A case of HME with compression of the cervical spinal cord is reported. Complete recovery following surgery was achieved. A review of the relevant literature revealed 51 previous cases of HME with cord/cauda equina compression. Most patients were under 30 years of age with more men affected than women. The family history was positive in 60%. The cervical and thoracic areas were predominantly affected, with the symptoms usually developing slowly. Recovery following surgery is to be expected in the majority of cases. In patients with HME and suffering from neurological symptoms, the possibility of spinal cord compression should be considered. Prompt diagnosis and surgical excision provide the best prognosis.

Adolescent↗
PubMed2005

[Surgical treatment of thoracic vertebral angioma with extraosseous extension causing spinal cord compression].

OBJECTIVE: To evaluate the therapeutical effect of surgical treatment for thoracic angioma with extraosseous extension which causes spinal cord compression and neurological dysfunction. METHODS: We retrospectively analyzed 5 cases of thoracic vertebral body angioma with extraosseous extension and spinal cord compression. The operation were performed through anterior approach to resect the involved vertebral body and intracanal angioma in 5 patients. Bone graft and internal fixation were also completed in the meantime. Two patients accepted radiotherapy for 2 periods of treatment postoperatively, and the other 3 patients didn't accept the other supportive therapy. RESULTS: The blood loss during the operation was 1000 mL to 1500 mL. All the patients recovered their neurological function rapidly after the operation. The patients were followed up for 1 to 4 years, and no patient recurred. The bone graft was solidly fused in all patients. No hardware failure occurred. CONCLUSION: The surgical treatment for thoracic vertebral body and extraosseous angioma with spinal cord compression is effective, reliable, and workable. Good long-term result can be obtained.

Adult↗
PubMed1990

Spinal cord compression due to metastatic neoplasm.

A retrospective study of 73 assessable patients with spinal cord compression due to metastatic tumor was conducted. Fifty-five patients had paraparesis and 18 were paraplegic. Treatment consisted of surgical decompression in 22 patients, radiotherapy in 31 patients, and a combination of both modalities in 20 patients. The three groups were comparable in their pretreatment characteristics. Of patients treated with surgical decompression followed by radiotherapy, 45% showed improvement in motor deficit; of patients treated with either surgery or radiotherapy, 18% (P = .06) and 16% (P = .02) showed improvement, respectively. These results were most significant in patients with paraparesis, where 47% of those who received combined therapy improved compared to 17% (P = .06) and 9% (P = .009) in the surgical and radiotherapy groups, respectively. In our series of patients with spinal cord compression due to metastatic tumor, surgical decompression followed by radiotherapy was superior to either surgical decompression or radiotherapy in improving motor deficit.

Combined Modality Therapy↗
PubMed2004

Metastatic spinal cord compression by solid tumors.

As the survival of cancer patients continues to improve, physicians in the 21st century face the challenge of early detection of metastatic spinal cord compression. Prompt diagnosis and intervention increase the likelihood of functional recovery. Because the epidural space is the most common site of spinal cord metastasis from solid tumors, this article will review the epidemiology, relevant anatomy, pathophysiology, clinical presentation, diagnostic evaluation, treatment, and prognosis for metastatic epidural spinal cord compression. Special attention will be given to the various modalities available for management of metastatic epidural spinal cord compression to maintain or restore normal spinal cord function and relieve pain. These treatment options will be considered according to patients' disease burden, life expectancy, and values. Intramedullary metastasis will be briefly discussed.

Adrenal Cortex Hormones↗
PubMed2003

Prognostic factors for survival in metastatic spinal cord compression: a retrospective study in a rehabilitation setting.

OBJECTIVE: Metastatic spinal cord compression (MSCC) occurs in 5-10% of all patients with cancer, and it is associated with short survival durations. Patients with MSCC may also have functional loss and require rehabilitation before discharge from the hospital. The purposes of this retrospective study were to identify clinical and social variables that had a significant impact on survival of patients with MSCC who underwent inpatient rehabilitation. DESIGN: A total of 60 consecutive patients with MSCC who were admitted to the inpatient rehabilitation unit at our tertiary care cancer center between 1996 and 1998 were included. Age, discharge destination, primary tumor site, metastasis, comorbidity, hemoglobin and albumin levels, treatment rendered for MSCC, opioids used, and psychological symptoms were examined as variables, and the Kaplan-Meier survival analysis was used. RESULTS: Our study showed that most of the variables we examined had no significant influence on survival time (median, 4.1 mo), with the exception of gastrointestinal cancer; patients with gastrointestinal cancer had a poorer prognosis (median survival durations, 0.6 mo; P < 0.0001). We also found a 1-mo gap between the time of diagnosis and the time of transfer to the rehabilitation unit. CONCLUSION: This study suggests that rehabilitation programs for patients with MSCC should be of short duration and that early referral (i.e., when the patient is diagnosed of MSCC) to the rehabilitation service should be encouraged.

Gastrointestinal Neoplasms↗
PubMed2004

A patient presenting with spinal cord compression who had two distinct follicular cell type thyroid carcinomas.

A 61-yr-old woman presented with complaints of weakness and pain in her legs. A magnetic resonance imaging showed a 3 x 5.6 x 7.8 cm mass lesion destructing the T1 and T2 vertebral bodies and compressing the spinal cord. The mass was excised surgically. It was follicular carcinoma metastasis of the cervicodorsal region. Then, she underwent a total thyroidectomy. Pathological examination showed two different types of carcinomas in two different focuses; follicular carcinoma in the left lobe and follicular variant papillary carcinoma in the isthmic lobe. After the operation she was given 100 mCi 131I. This is the first report of a patient who had both metastatic follicular carcinoma and follicular variant papillary carcinoma together.

Adenocarcinoma, Follicular↗
PubMed1995

Astrocytic reaction after graded spinal cord compression in rats: immunohistochemical studies on glial fibrillary acidic protein and vimentin.

The relation between the degree of spinal cord compression and the extent of early posttraumatic reaction of astrocytes was investigated in rats using the blocking-weight technique to induce a spinal cord compression at the level of the Th8-9. Immunohistochemistry was used to detect changes in the expression of glial fibrillary acidic protein (GFAP) and vimentin up to 24 h after injury. A mild compression, which did not cause any measurable neurological deterioration, induced a mild increase of GFAP immunoreactivity at 4 h and a more marked and widespread immunoreactivity at 24 h. The greatest increase of GFAP immunoreactive astrocytes occurred in rats with moderate compression of the cord causing reversible paraparesis and in animals with severe compression leading to paraplegia. The increase of GFAP immunoreactivity was present already 4 h after injury in virtually all the segments investigated (Th5-6-Th11-12) and was most marked at 24 h. Vimentin immunoreactivity of control rats was present in the ependymal cells of the central canal, the leptomeninges, and walls of a few intramedullary vessels. Occasional astrocytes were stained. In rats surviving 24 h after moderate and severe compression vimentin immunoreactivity was increased in the walls of intramedullary blood vessels including capillaries of one rostral and one caudal segment. Many macrophages with immunoreactivity appeared and occasional glial cells with astrocyte shape were stained. This investigation shows that within 24 h after compression of the spinal cord a widespread astrocyte reaction occurs. Even a mild compression that does not produce any signs of motor dysfunction can induce widespread astrocyte alterations in the spinal cord. This astrocyte response is more marked in rats with more severe compression leading to more pronounced neurological deterioration. The increase in vimentin immunoreactivity of blood vessels is more localized and occurs in moderate and severe compression of the cord.

Animals↗
PubMed1990

Pathogenesis of vertebral metastasis and epidural spinal cord compression.

The authors have studied the sequential events in the process of vertebral metastasis that result in spinal cord compression. Different tumor cell lines were injected into the systemic arterial circulation of syngeneic or nude mice, and they were killed at timed intervals after injection or when they became paraplegic. The following observations were made. The tumor cells lodged and grew in the hematopoietic bone marrow of the vertebrae. Cancer cells in the vertebral marrow cavity invaded into the spinal canal through the foramina of the vertebral veins rather than destroying the cortical bone. Tumor cell lines that grew in an infiltrative fashion migrated toward a posterior location in the spinal canal, and compressed the spinal cord from a posterior direction. Tumor cell lines that grew as compact tumors formed a tumor mass at the same location from which the cells emerged from the vertebra, and compressed the cord predominantly from an anterior direction. Radiographic evidence of vertebral metastasis was a late event, and commonly associated with significant compression of the cord and extraosseous tumor. These experimental findings may help to establish better diagnostic and treatment strategies for patients with metastatic disease of the spine.

Animals↗
PubMed1988

Spinal cord compression by epidural metastases. Fibrosarcoma experiments in rats.

Morphologic changes were studied in 43 rats that were paralyzed by an epidural fibrosarcoma inoculated through the spinous process. By this technique, of 29 rats examined radiographically, the tumor destroyed vertebral bone in 22. Changes of the cord in the early stage of paralysis were specially analyzed by Marchi's stain and microangiograms. In compromised cord segments in the early stage, extravasation of contrast medium was observed in the gray matter and the dorsal funiculus; and in the dorsal funiculus just proximal or distal to the compressed portion, hemorrhagic areas were present. Ascending degenerated fibers in the dorsal funiculus, which were derived from the degenerated posterior nerve root or the degeneration of the dorsal funiculus in the compressed segments, were characteristically detected in rats in the early stages. In the advanced stages a transverse cord lesion was observed at the involved level. Based on the present analyses, to prevent more advanced damage to the spinal cord, the tumor should be removed at an early stage that clinically coincides with the period when radicular signs appear.

Animals↗
PubMed1997

Spinal cord compression in prostate cancer: treatment outcome and prognostic factors.

BACKGROUND AND PURPOSE: Spinal cord compression (SCC) is an important complication of metastatic prostate cancer. We have analysed patients treated at the Royal Marsden Hospital to assess treatment outcome and prognostic factors in this patients group. MATERIALS AND METHODS: We performed retrospective analysis of 69 patients with spinal cord compression and prostate cancer treated at the Royal Marsden Hospital. RESULTS: At presentation 40 (58%) patients were non-ambulant and 52% were catheterised. Diagnosis was established by myelography in 42% and magnetic resonance imaging (MRI) in 47% of patients. MRI detected significantly more patients with multiple sites of compression (51 versus 7%, P < 0.001). SCC was present at the initial diagnosis of prostatic cancer in 13 patients and 17 patients had received no hormone treatment prior to diagnosis. Following treatment 36 (52%) patients had a functional improvement of motor power with 25/40 (63%) non-ambulant patients becoming ambulant. Seventy-seven percent of patients who had eventual improvement had some improvement in power within 7 days. On multivariate analysis a single level of compression, no previous hormone therapy and a young age (<65 years) predicted for better outcome. When these factors were included an increased radiation dose (>30 Gy) or the addition of surgery did not improve the functional outcome. Following initial recovery; there was a 45% risk of developing a further episode of cord compression at the same or new site by 2 years with a median time to progression of 236 days (range 47-1215 days). The median survival was 115 days (range 5-2016 days) with 25% of patients surviving for 2 years. Patients with no prior hormone therapy had a median survival of 627 days (range 46-1516 days). Other predictors of improved survival on multivariate analysis were a single site of compression and a haemoglobin over 12 g. CONCLUSIONS: Treatment of SCC in prostate cancer results in improved motor function in the majority of patients. Long-term survival is possible, especially in good performance status patients with no prior hormone treatment. Survivors remain at high risk of subsequent neurological relapse. An early improvement in motor power is a strong predictor of subsequent functional improvement. MRI detects additional sites of asymptomatic SCC which makes it the investigation of choice.

Aged↗