Symptomatic spinal epidural lipomatosis (SEL) is very rare and frequently associated to chronic exogenous steroid use, obesity and Cushing syndrome. The idiopathic cases where no identifiable association with SEL are found constitute only 17% of all cases. The usual clinical manifestations of this entity consist of dorsal or lumbar pain with paresthesias and weakness in lower limbs, but acute symptoms of myelopathy are exceptional. We report a case of acute paraparesis and urinary retention caused by thoracic SEL in a 55-year-old male who did not have any recognized predisposing factor for this condition. Urgent surgical decompression was performed in order to relieve the symptoms. Slow but progressive improvement was assessed after surgery. We consider this case to be exceptional due to the needing to perform an urgent decompressive laminectomy to treat a rapidly progressive myelopathy caused by idiopathic SEL.
Epidural lipomatosis; Paraparesis; Spinal cord compression; Surgical decompression; Myelopathy
Spinal epidural lipomatosis (SEL) results from an abnormal accumulation of unencapsulated fat within the epidural space and is a rare cause of spinal cord compression, which needs to be considered with a high index of suspicion. It most commonly occurs secondary to chronic corticosteroid use and endocrinopathies. Idiopathic cases are highly associated with obesity. We report an unusual case of idiopathic thoracic SEL in a 69-year-old male, with an adjacent infiltrative Hodgkin's lymphoma and associated vertebral crush fracture, which resulted in ataxia and sensory loss. Magnetic resonance imaging scans displayed extensive SEL and an infiltrative disease process causing thoracic cord compression. Surgical decompression confirmed the presence of extensive epidural lipomatosis and Hodgkin's lymphoma and subsequently led to improvement in neurological symptoms. To our knowledge, this is the first reported case of concomitant SEL with an adjacent Hodgkin's lymphoma resulting in cord compression.
Spinal epidural lipomatosis (SEL) is a rare condition characterized by overgrowth of normal adipose tissue in the extradural space within the spinal canal that can lead to significant spinal cord compression. It is most commonly reported in patients receiving chronic glucocorticoid therapy. Other causes can include obesity and hypercortisolism. Occasionally, idiopathic SEL will occur in patients with no known risk factors, but cases are more generally reported in obesity and males. We present the case of a 35 year-old non-obese woman found to have rapidly progressive SEL that was not associated with any of the common causes of the disorder.
lipomatosis; laminectomy; hypercortisolism
Spinal epidural lipomatosis (SEL) is a rare disorder, regarded in literature as a consequence of administration of exogenous steroids, associated with a variety of systemic diseases, endocrinopathies and the Cushing's syndrome. Occasionally, SEL may occur in patients not exposed to steroids or suffering from endocrinopathies, namely, idiopathic SEL. Thus far, case studies of SEL among Korean have been published rather sporadically. We reviewed the clinical features of SEL cases, among Koreans with journal review, including this report of three operated cases. According to this study, there were some differences between Korean and western cases. Koreans had higher incidences of idiopathic SEL, predominant involvement in the lumbar segments, very few thoracic involvement and lower MBI, as opposed to westerners.
Spinal epidural lipomatosis; Korean; Idiopathic
Abnormal fat deposition in the epidural space or spinal epidural lipomatosis (SEL) due to corticosteroid treatment or obesity may cause obstruction to cerebrospinal fluid flow. Little is known about SEL in patients with hematologic malignancies who require frequent lumbar punctures and corticosteroid treatment that places them at risk.
Patients and methods
Records and radiologic images of patients with SEL and leukemia or non Hodgkin lymphoma (NHL) treated at a single institution from 1999–2009 were reviewed. Risk factors were compared with 405 control patients with leukemia.
Fourteen patients with leukemia or NHL were diagnosed with SEL. The majority of patients underwent diagnostic imaging after unsuccessful lumbar punctures within one month of their primary diagnosis. Prior to SEL diagnosis, all patients received systemic and/or intrathecal corticosteroids. SEL diagnosis led to modification of intrathecal administration in 8 patients, including Ommaya reservoir placement in 4 patients. All patients completed protocol-specified chemotherapy without neurologic symptoms or surgical intervention. Risk factors for developing SEL include older age and high body mass index.
Investigation for SEL in leukemia or lymphoma patients with difficult lumbar punctures is warranted. Placement of an Ommaya reservoir may facilitate safe CNS-directed therapy in severely affected patients.
spinal epidural lipomatosis; lumbar canal obstruction; difficult lumbar puncture; leukemia; lymphoma
Spinal epidural lipomatosis (SEL) is a rare condition that presents as a back pain with progressive neurologic symptoms. Most affected patients are obese and receiving steroid therapy, or have an endocrinopathies. We report a rare case of cauda equina syndrome caused by SEL in a non-obese healthy young man without any evident traumatic episode. A healthy 19-year-old man, who had experienced lower back pain for two months, visited our emergency room because of the sudden development of motor weakness and voiding difficulty. Lumbar magnetic resonance image revealed extradural fat compressing the cauda equina. Urgent decompression via posterior laminectomy and excision of excess epidural fat resulted in an immediate symptom improvement.
Cauda equina syndrome; Epidural; Lipomatosis
There are very few reports of spinal epidural lipomatosis (SEL) in association with scoliosis. A 49-year-old man presented with persistent back pain and right hip lump. The lumbar spine X-rays showed scoliosis (Lenke classification 5BN). Lumbar MRI demonstrated circumferential epidural fat deposit from L1 to lower S2 level. There was no obvious etiology of SEL except mild increased body mass index (BMI). The patient was managed with conservative treatment. After 6 months medication (limaprost and ginkgo), his symptoms were relieved.
Epidural lipomatosis; scoliosis
Spinal epidural lipomatosis (SEL) is an abnormal localized accumulation of fat tissues in the epidural space. It is strongly related with steroid administration. The symptoms of SEL are various and range from back pain to paraplegia. In severe cases, decompressive laminectomy is the choice of treatment. A 32-year-old woman who had been under long-term steroid administration after suprasellar tumor resection was admitted for both leg radiating pain and weakness. She was diagnosed with SEL and had a decompressive laminectomy. During the operation, we found the nerve roots were compressed by epidural fat tissues and engorged vessels. After the operation, her radiating pain was relieved and motor weakness was improved.
Spinal epidural lipomatosis; Suprasellar tumor; Steroid
Epidural lipomatosis is a rare entity to cause spinal cord compression and neurological deficits. This is usually associated with excess of steroids in the body either because of endogenous source as in Cushings disease or exogenous intake as in some diseases like systemic lupus erythematosus, in some endocrinopathies or in morbid obesity. But in some cases no cause has been found. Such idiopathic cases of spinal epidural lipomatosis have also been reported. Here, we report a case of idiopathic spinal epidural lipomatosis with relapsing and remitting paraparesis which is quite unusual. Treatment depends upon neurological status of the patient. We operated the patient as he had significant neurological compromise and he improved significantly.
Idiopathic cord compression; myelopathy; paraparesis; spinal epidural lipomatosis
Spinal epidural lipomatosis (SEL) is a rare but well-recognized condition. In general, the onset of its symptoms is insidious and the disease progresses slowly. We report two cases of rapid progression of SEL with no history of steroid intake in non-obese individuals after epidural steroid injection. These SEL patients developed neurologic symptoms after less than 5 months; these symptoms were confirmed to be due to SEL by serial MR images. After the debulking of the epidural fat, their symptoms improved.
Spinal epidural lipomatosis; Epidural steroid injection
The authors report a case of a thoracic epidural spinal lipomatosis causing severe neurological deficits along the review of pertinent literature. The patient is a 56-year-old woman who presented with acute onset of severe paraparesis; she was investigated with cervical and thoracic MRI and then surgically managed because of an intraspinal mass compressing the cord. The operation consisted in the excision of the mass confirmed to be a fibrolipoma by pathological analysis. The patient attained complete neurological recovery and at 18 months follow-up she reported a generalised well-being. Thoracic lipomas are rare lesions that presenting mostly with back pain; however, in rare instances they may cause progressive and/or abrupt neurological dysfunction. Appropriate imaging can help in the diagnosis and management of such cases.
Introduction. Epidural lipomatosis is most frequently observed in patients on chronic steroid treatment. Only a few idiopathic epidural lipomatosis cases have been described. Material and Methods. 64-year-old male patient presented with low back pain and left leg pain. Later, the patient experienced neurogenic claudication and radicular pain in the left leg without urinary dysfunction. Plain radiography and magnetic resonance imaging demonstrated an abnormal fat tissue overgrowth in the epidural space with compression of the dural sac, degenerative disc disease at L4-L5 level, and instability at L5-S1. Endocrinopathic diseases and chronic steroid therapy were excluded. If conservative treatment failed, surgical treatment can be indicated. Results. After surgery, there was a gradual improvement in symptoms and signs, and six months later the patient returned to daily activities and was neurologically normal. Conclusion. In the absence of common causes of neurogenic claudication, epidural lipomatosis should be considered. The standard test for the diagnosis of epidural lipomatosis is magnetic resonance (MR). At first, conservative treatment must be considered; weight loss and the suspension of prior corticosteroid therapy are indicated. In the presence of neurological impairment, the operative treatment of wide surgical decompression must be performed soon after diagnosis.
A 68-year-old male presented with rapidly progressive paraplegia. MR images of the thoracic spine were interpreted as being consistent with an abscess within an epidural lipomatosis compressing the spinal cord. Laminectomy was performed, and a large amount of pus was drained from the epidural lipomatosis, from which Staphylococcus aureus was isolated. This is the first reported case of an abscess involving an epidural lipomatosis.
Epidural lipomatosis; Spinal abscess; Magnetic resonance imaging
Epidural lipomatosis (EL) is an increase of adipose tissue, normally occurring in the epidural space, sufficient to distort the thecal sac and compress neural elements. There is a lack of knowledge of risk factors, impact on patient’s symptoms, and its possible association with epidural steroid injections.
History, physical examination, patient chart, and MRI were analyzed from 856 outpatients referred for epidural steroid injections. Seventy patients with signs of EL on MRI comprised the study group. Thirty-four randomly selected patients comprised the control group. The severity of EL was determined by the MRI assessment. The impact of EL was determined by the patient’s history and physical examination. Logistic regression was used to correlate the probability of developing EL with BMI and epidural steroid injections.
EL was centered at L5 and S1 segments. The average BMI for patients with EL was significantly greater than that of control group (36.0 ± 0.9 vs. 29.2 ± 0.9, p <0.01). The probability of developing EL with increasing BMI was linear up to the BMI of 35 after which it plateaued. Triglycerides were significantly higher for the EL group as compared to controls (250 ± 30 vs. 186 ± 21 mg/dL p < 0.01). The odds of having EL were 60% after two epidural steroid injections, 90% after three epidural steroid injections and approached 100% with further injections, independent of BMI. Other risk factors considered included alcohol abuse, use of protease inhibitors, levels of stress, hypothyroidism and genetic predisposition. However there were insufficient quantities to determine statistical significance with a degree of confidence. The impact of EL on patient’s symptoms correlated with EL severity with Spearman correlation coefficient of 0.73 at p < 0.01 significance level.
The BMI and triglycerides levels were found to be significantly elevated for the EL group, pointing to an increased risk of EL occurrence in progressively more obese US population. The data also revealed a strong correlation between the number of subsequent epidural steroid injections and EL occurrence calling for caution with the use of corticosteroids.
Epidural lipomatosis; Epidural steroid injection; Obesity; Cortisol; Sciatica
Selenoprotein S (SelS) is a transmembrane protein that is expressed in the liver, skeletal muscle, adipose tissue, pancreatic islets, kidney, and blood vessels. In addition to its transmembrane localization, SelS is also secreted from hepatoma HepG2 cells (but not L6 skeletal muscle cells, 3T3-L1 adipocytes, Min6 pancreatic β cells and human embryonic kidney 293 cells) and has been detected in the serum of some human subjects, with a detection rate of 31.1 %. These findings prove that serum SelS is secreted by hepatocytes. However, whether vascularly expressed SelS can be secreted has not been reported. Transmembrane SelS has been suggested to play different roles in the pathogenesis and progression of diabetes mellitus (DM) and atherosclerosis (AS), but the association of secreted SelS with DM and macroangiopathy remains unclear.
Research design and methods
Supernatants were collected from human umbilical vein endothelial cells (HUVECs), human aortic vascular smooth muscle cells (HA/VSMCs) and human hepatoma HepG2 cells that were untransfected or transfected with the indicated plasmid and concentrated for western blotting. Serum samples were collected from 158 human subjects with or without type 2 DM (T2DM) and/or AS. Serum SelS levels were measured using an enzyme-linked immunosorbent assay.
Secreted SelS was only detected in the supernatants of hepatoma HepG2 cells. The SelS detection rate among the 158 human serum samples was 100 %, and the average SelS level was 64.81 ng/dl. The serum SelS level in the isolated DM subjects was lower than the level in the healthy control subjects (52.66 ± 20.53 vs 70.40 ± 21.38 ng/dl). The serum SelS levels in the DM complicated with SAS subjects (67.73 ± 21.41 ng/dl) and AS subjects (71.69 ± 27.00 ng/dl) were significantly increased compared with the serum SelS level in the isolated DM subjects. There was a positive interaction effect between T2DM and AS on the serum SelS level (P = 0.002). Spearman correlation analysis showed that the serum SelS level was negatively correlated with fasting plasma glucose.
Vascular endothelial and vascular smooth muscle cells could not secrete SelS. Serum SelS was primarily secreted by hepatocytes. SelS was universally detected in human serum samples, and the serum SelS level was associated with T2DM and its macrovascular complications. Thus, regulating liver and serum SelS levels might become a new strategy for the prevention and treatment of DM and its macrovascular complications.
Electronic supplementary material
The online version of this article (doi:10.1186/s12933-016-0388-3) contains supplementary material, which is available to authorized users.
Secreted selenoprotein S; Type 2 diabetes mellitus; Atherosclerosis
Spinal epidural lipomatosis is a rare complication of chronic corticosteroid treatment. We report a new pediatric case and an analysis of this and 19 pediatric cases identified in the international literature. The youngest of these combined 20 patients was 5 years old when lipomatosis was diagnosed. Lipomatosis manifested after a mean of 1.3 (+/- 1.5) years (SD) (median, 0.8 years; range, 3 weeks - 6.5 years) of corticosteroid treatment. The corticosteroid dose at the time of presentation of the lipomatosis ranged widely, between 5 and 80 mg of prednisone/day. Back pain was the most common presenting symptom. Imaging revealed that lipomatosis almost always involved the thoracic spine, extending into the lumbosacral region in a subset of patients. Predominantly lumbosacral involvement was documented in only two cases. Although a neurological deficit at presentation was documented in about half of the cases, surgical decompression was not performed in the cases reported after 1996. Instead, reducing the corticosteroid dose (sometimes combined with dietary restriction to mobilize fat) sufficed to induce remission. In summary, pediatric spinal epidural lipomatosis remains a potentially serious untoward effect of corticosteroid treatment, which, if recognized in a timely manner, can have a good outcome with conservative treatment.
Case report: To report a case of epidural lipomatosis in a patient with abnormal adipose tissue distribution, glucose intolerance and mixed hyperlipidemia. A 63-year-old male patient presented with low back pain radiating to the left calf on standing and walking (walking distance <100 m). He weighed 97.5 kg, was 1.73 m tall (BMI 32.6 kg/m2) and had a waist circumference of 113 cm. He had a glucose intolerance after a 75-g glucose oral load test. CT-Myelography revealed voluminous epidural lipomatosis around L4–L5 and L5–S1. Low calorie diet and reduction in alcohol intake achieved a weight loss of 17.5 kg in 7 months (80 kg, BMI 25.8 kg/m2, waist circumference 94 cm) and dramatic improvement in low back pain, walking distance (>500 m) and reduction of lipomatosis on CT-scan. Our case suggests a relationship between central obesity phenotype and epidural lipomatosis. Specific insulin resistance treatment might be proposed for these patients if this hypothesis is confirmed in further studies.
Lipomatosis; Epidural; Obesity; Hyperinsulinism
We intend to report the largest series of spinal epidural cavernomas (SEC), discuss their clinical features, imaging characteristics, surgical findings, outcome analysis and compare them with similar reports in the literature.
Among the cases of spinal tumors treated surgically by the authors, there have been nine cases of SEC’s. All the data were collected prospectively and the cases have been followed after surgery up to the time of this analysis.
There were six female and three male patients with the ages ranging between 13 and 74 years. The lesions were located in the thoracic spine (4 cases), lumbar spine (4 cases) and one at the sacral level. Clinical presentations included acute spinal pain and paraparesis in two, low back pain and radiculopathy in five, and slowly progressive myelopathy in the other two cases. The lesion was iso-intense with the spinal cord in T1W images and hyperintense in T2W images and showed strong homogeneous enhancement after contrast medium injection in most of our cases. In the presence of hemorrhage inside the lesion, it was hyperintense in both T1W and T2W MR sequences as in our case 6. In the single case presenting with acute hemorrhage, epidural hematoma was the only finding, our case 1. Complete surgical removal was achieved in all our cases, and confirmed by postoperative MRI.
SEC is hard to be differentiated from other epidural spinal lesions before intervention but should be considered in the list of differential diagnosis regarding its favorable outcome.
Cavernous angioma; Magnetic resonance imaging; Spinal epidural cavernoma; Spinal epidural lesion
We report a case of paraplegia without neurologic deficit of upper extremities following cervical epidural catheterization using air during the loss of resistance technique. A 41-year-old woman diagnosed with complex regional pain syndrome had upper and lower extremity pain. A thoracic epidural lead was inserted for a trial spinal cord stimulation for treating lower extremity pain and cervical epidural catheterization was performed for treating upper extremity pain. Rapidly progressive paraplegia developed six hours after cervical epidural catheterization. Spine CT revealed air entrapment in multiple thoracic intervertebral foraminal spaces and surrounding epidural space without obvious spinal cord compression before the decompressive operation, which disappeared one day after the decompressive operation. Her paraplegia symptoms were normalized immediately after the operation. The presumed cause of paraplegia was transient interruption of blood supply to the spinal cord through the segmental radiculomedullary arteries feeding the spinal cord at the thoracic level of the intervertebral foramen caused by the air.
Air; Cervical; Complication; Paraplegia; Spinal cord ischemia
•In this article, reporting on the case of a huge 10 levels spontaneous spinal subdural hematoma treated with decompressive thoracic no-instrumented laminectomy in a 45-year-old woman with good neurological recovery, we would like to underline the importance of a timely surgical decompression as the mainstay option in the management of strongly symptomatic spontaneous idiopathic acute spinal subdural hematomas.•To our knowledge, 10 levels thoracic laminectomy for a SSDH removal have never been described. We performed “conservative” laminectomy by sparing of articular processes with no need to posterior fixation also considering the intrinsic stability of thoracic chest.
Spontaneous idiopathic acute spinal subdural hematoma (SSDH) is a rare cause of acute back pain followed by signs and symptoms of nerve root and/or spinal cord compression, frequently associated with coagulopathies, blood dyscrasias and arterio-venous malformations. Standard management includes non-operative treatment and timely (within 24 h) surgical decompression.
Presentation of case
We report on the case of a huge 10 levels SSDH treated with decompressive thoracic no-instrumented laminectomy in a 45-year-old woman with good neurological recovery (from ASIA A to D).
Spontaneous SSDHs without detectable structural lesion or anticoagulant therapy are very rare. Among 26 cases documented the literature harbouring SSDHs, the thoracic spine was found to be the preferred site, and the compression was usually extending over several vertebral levels. Nonoperative treatment for SSDH may be justified in presence of minimal neurologic deficits, otherwise, early decompressive laminectomy along with evacuation of hematoma are considered the treatment of choice in presence of major deficits.
To our knowledge, the present case is the most extensive laminectomy for a SSDH removal never described before. No postoperative instability occurs in 10 levels thoracic laminectomy in case the articular processes are spared. When major neurological deficits are documented, early decompressive laminectomy with evacuation of hematoma should be considered the best treatment for SSDH.
Complications after lumbar anaesthesia and epidural blood patch have been described in patients with congenital small spinal canal and increased epidural fat or epidural lipomatosis. These conditions, whether occurring separately or in combination, require magnetic resonance imaging for diagnosis and grading, but their clinical significance is still unclear.
A 35-year-old Caucasian woman who was undergoing a Caesarean section developed a longstanding L4-L5 unilateral neuropathy after the administration of spinal anaesthesia. There were several attempts to correctly position the needle, one of which resulted in paraesthesia. A magnetic resonance image revealed that the patient's bony spinal canal was congenitally small and had excess epidural fat. The cross-sectional area of the dural sac was then reduced, which left practically no free cerebrospinal fluid space.
The combination of epidural lipomatosis of varying degrees and congenital small spinal canal has not been previously discussed with spinal anaesthesia. Due to the low cerebrospinal fluid content of the small dural sac, the cauda equina becomes a firm system with a very limited possibility for the nerve roots to move away from the puncture needle when it is inserted into the dural sac. This constitutes risks of technical difficulties and neuropathies with spinal anaesthesia.
Mediastinal widening is a common finding on chest radiograph, and can be caused by numerous conditions. Most of these diseases have grave prognosis if accompanied by chest pain, and require immediate attention. However, mediastinal lipomatosis is a very benign condition caused by deposition of adipose tissue in the mediastinum.
We present a case of a morbidly obese female patient who presented to emergency department with a fall. She had mediastinal widening on chest radiograph, and borderline low voltage on electrocardiogram. On computed tomography, mediastinal lipomatosis was evident.
Obesity is a major epidemic in United States, and can lead to deposition of fat in the chest. Mediastinal lipomatosis is very benign condition, which rarely causes grave consequences.
Epidural venous plexus enlargement, presenting with low back pain and radiculopathy, is an uncommon cause of nerve roots impingement. This condition commonly mimics a herniated nucleus pulposus radiologically. The radiological diagnosis is often missed and the diagnosis is made during the surgery. We are hereby presenting 2 such cases of epidural varices mimicking intervertebral disc prolapse with lumbar radiculopathy.
Case 1: 43 yr old female presented with acute exacerbation of low back ache and significant right L5–S1 radiculopathy without neurological deficit. MRI reported as L5-S1 disc prolapse. Intra-operatively engorged dilated epidural vein seen compressing S1 nerve root. Associated Disc bulge removed and Coagulative ablation of the dilated epidural vein was performed Case 2: 45 year old male manual labourer presented with backache with left sided sciatica since 8 months, increased in severity since past 1month associated with sensory blunting in L5 and S1 dermatomes. Neurologic examination revealed normal muscle power in his lower extremities. Sensations was blunted in L5 and S1 dermatomes. MRI was reported as L5-S1 disc prolapsed compressing left S1 nerve root. Decompression of the L5–S1 intervertebral space was performed through a left –sidelaminotomy. Large, engorged serpentine epidural veins was found in the axilla of S1 nerve root, compressing it. Coagulative ablation of the dilated epidural vein was performed. Retrospectively, features of epidural varices were noted in the preoperative magnetic resonance imaging scans. Both patients had significant improvement in radiculopathy immediate postoperatively, and sensory symptoms resolved over the next 6 weeks in second case. At recent follow up, both patients had significant relief of symptoms and no recurrent radicular symptoms.
An abnormal dilated epidural venous plexus that mimics a herniated lumbar disc is a rare entity. This pathology should be always kept in mind during lumbar disc surgery. Preoperative misdiagnosis is common. When faced with this Situation, microsurgical coagulation and decompression of the nerve root are adequate.
Epidural varices; Disc prolapse; Radiculopathy; Decompression
Osteoporosis is the most common contributing factor of spinal fractures, which characteristically are not generally known to produce spinal cord compression symptoms. Recently, an increasing number of medical reports have implicated osteoporotic fractures as a cause of serious neurological deficit and painful disabling spinal deformities. This has been corroborated by the present authors as well. These complications are only amenable to surgical management, requiring instrumentation. Instrumenting an osteoporotic spine, although a challenging task, can be accomplished if certain guidelines for surgical techniques are respected. Neurological deficits respond equally well to an anterior or posterior decompression, provided this is coupled with multisegmental fixation of the construct. With the steady increase in the elderly population, it is anticipated that the spine surgeon will face serious complications of osteoporotic spines more frequently. With regard to surgery, however, excellent correction of deformities can be achieved, by combining anterior and posterior approaches. Paget's disease of bone (PD) is a non-hormonal osteometabolic disorder and the spine is the second most commonly affected site. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12–24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients, back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. In this context, one must be certain before attributing low back pain to PD exclusively, and antipagetic medical treatment alone may be ineffective. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Five classes of drugs are available for the treatment of PD. Bisphosphonates are the most popular antipagetic drug and several forms have been investigated.
Osteoporosis; Fractures; Neurological deficit; Deformity; Paget's disease; Back pain; Spinal stenosis; Myelopathy; Treatment
Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.
Idiopathic hypertrophic spinal pachymeningitis; Spinal cord compression; Chronic nonspecific inflammation; Dural thickening