自发性硬脊膜外血肿发病的解剖因素探讨

目的　探讨自发性脊髓硬膜外血肿（SSEH）发病中的解剖学因素。 方法　分析1995~2010年10例接受手术的SSEH患者临床表现、诊治经过及病理结果。 结果　10例患者均行随访,时间6-18个月,手术治疗的患者中2例患者受累肢体神经功能完全恢复;8例患者除大小便功能不同程度改善外,肢体的运动感觉功能无明显恢复。术中所见并结合病理检查,血肿组织者10例,血管畸形者。 结论　SSEH病因不明确,多发病急,发展迅速,预后差。自发性的出血可能与硬脊膜外隐匿性血管瘤或静脉血管扩张等有关,解剖学因素在发病中起到重要作用。     

Spinal epidural granulocytic sarcoma preceding acute myelogenous leukemia.

A rare case of spinal epidural granulocytic sarcoma (GS) preceding acute myelogenous leukemia is described. A 10-year-old boy presented with lower leg weakness. The initial diagnosis was a histiocytic lymphoma, and he was treated accordingly. No evidence of bone marrow involvement was found at that time. The correct diagnosis of epidural GS was made possible in retrospect by using immunoperoxidase staining for lysozyme fourteen months later when the patient showed the full-blown features of leukemia. This rare tumor should be considered in the differential diagnosis of an epidural mass with cord compression in patients with or even without acute leukemia, because early diagnosis followed by appropriate combined chemotherapy and radiation may obviate surgical intervention and eventually prevent leukemic transformation.     

The poor outcome of the delayed diagnosis of acute spontaneous spinal epidural hematoma: two cases report

We present two patients who had acute paraplegia with sensory loss due to spontaneous spinal epidural hematoma (SSEH). One had myocardial infraction and the other had deep vein thrombosis, and the former was treated with anticoagulants and the latter was treated with thrombolytic agent. We analyzed the neurological status of our two cases each between its preoperative and postoperative state. Postoperatively both showed no improvement of neurologic symptom, and on follow-up of 12 months, one showed no neurologic improvement and the other showed a insignificant improvement of lower extremity muscle power (trace knee extensor/ankle dorsi-flexor). We thought that this poor outcome was due to delayed operation, which was done more than 24 hr after the symptom onset. The outcome in SSEH is essentially determined by the time taken from symptom onset to operation. Therefore, early and precise diagnosis such as careful history taking and MRI evaluation is necessary.     

Pure epidural cavernous hemangioma of the cervical spine that presented with an acute sensory deficit caused by hemorrhage

Pure epidural cavernous hemangioma of the spine without vertebral involvement is rare. Due to the slow growth of this lesion, the most common symptoms are chronic pain, myelopathy, and radiculopathy. In our case, the patient complained of an acute onset sensory deficit of the C4 dermatome. An MRI revealed an epidural mass with an acute hematoma. Here, we report a case of a pure epidural cavernous hemangioma that presented with acute neurologic symptoms caused by intralesional hemorrhage and an acute epidural hematoma, which were demonstrated on the patient's MRI.     

Cervical spinal cord compression in infants with achondroplasia: should neuroimaging be routine?

PurposeTo examine results of magnetic resonance imaging (MRI), polysomnograms (PSG), and patient outcomes in patients with achondroplasia in light of recent screening recommendations for infants with achondroplasia.MethodsWe reviewed medical records of 49 patients with achondroplasia followed at our institution between September 1997 and January 2017, including physical exams, MRIs, PSGs (when available), and surgical histories. Appropriate PSG data were available for 39 of these patients.ResultsTwenty-seven of 49 patients had cervical cord compression on MRI, and 20 of those patients required surgery. Central apnea was detected in 2/23 patients with cervical cord compression in whom PSG data was available. Physical exam revealed depressed deep-tendon reflexes in two patients with cord compression and one patient without cord compression. Besides hypotonia in some, the neurological exams of these patients were unremarkable.ConclusionsCervical cord compression is a common occurrence in infants with achondroplasia and necessitates surgical intervention in some patients. Physical exam and PSG are poor predictors of the presence of cord compression or the need for surgery. All infants with achondroplasia should have MRIs of the craniocervical junction in the first 6 months of life.     

Foramen magnum arachnoid cyst induces compression of the spinal cord and syringomyelia: case report and literature review

It is very rare that a foramen magnum arachnoid cyst induces compression of the spinal cord and syringomyelia, and currently there are few treatment experiences available. Here we reported the case of a 43-year-old male patient who admitted to the hospital due to weakness and numbness of all 4 limbs, with difficulty in urination and bowel movement. MRI revealed a foramen magnum arachnoid cyst with associated syringomyelia. Posterior fossa decompression and arachnoid cyst excision were performed. Decompression was fully undertaken during surgery; however, only the posterior wall of the arachnoid cyst was excised, because it was almost impossible to remove the whole arachnoid cyst due to toughness of the cyst and tight adhesion to the spinal cord. Three months after the surgery, MRI showed a reduction in the size of the arachnoid cyst but syrinx still remained. Despite this, the symptoms of the patient were obviously improved compared to before surgery. Thus, for the treatment of foramen magnum arachnoid cyst with compression of the spinal cord and syringomyelia, if the arachnoid cyst could not be completely excised, excision should be performed as much as possible with complete decompression of the posterior fossa, which could result in a satisfying outcome.     

腰椎间盘突出症合并椎管内硬膜外血管畸形的解剖与临床特点

目的研究腰椎间盘突出症合并椎管内硬膜外血管畸形的解剖与临床特点。方法2001-2006年期间在我科手术治疗的7例腰椎间盘突出症合并椎管内硬膜外血管畸形,术前行MRI和选择性脊髓血管造影检查,均行椎间盘髓核摘除及畸形血管烧灼切除术。结果术中发现5例硬膜前方畸形血管,2例椎管内大量陈旧积血。术后症状消失或逐渐减轻,随访4年均无复发。根据Macnab疗效评定标准,优5例、良2例,无可及差。结论MRI、脊髓造影、手术探查是腰椎间盘突出症合并椎管内硬膜外血管畸形的主要诊断方法,充分认识其解剖与临床特点,有助于提高诊断准确率及手术疗效。     

Treatment of spontaneous cervical spinal subdural hematoma with methylprednisolone pulse therapy

We report herein a case of hyperacute onset of spontaneous cervical spinal subdural hematoma treated with methylprednisolone pulse therapy that showed good results. A 57-year-old man was admitted for posterior neck pain and paraparesis which occurred an hour ago. MRI revealed a ventral subdural hematoma distributed from the level of C1 down to T3, compressing the spinal cord. Conservative management with methylprednisolone pulse therapy was administered considering the patient's poor general condition. Although emergent surgical decompression is necessary in most cases of spinal subdural hematoma, conservative management with steroid therapy could be effective.     

Vascular anatomy of the spinal epidural space: Considerations on the etiology of the spontaneous spinal epidural hematoma

The anatomy of the blood vessels in the spinal epidural space is an important basis for the understanding of the etiology of the spontaneous spinal epidural hemorrhage (SSEH). The structure of the internal vertebral venous plexus and epidural arteries, in particular the poorly recognised “cluster-type” arteries, has been studied. The cluster arteries are easily mistaken for vascular anomalies. The literature is reviewed, and the different theories on the etiology of the SSEH are discussed.     

Successful medical treatment of spinal epidural abscess.

Spinal epidural abscess is a rare but potentially fatal disease. A 67-year-old female suffered fever and painful swelling of the right knee and lower leg for one week. Both synovial fluid and blood cultures yielded methicillin-sensitive Staphylococcus aureus. Low back pain developed and fever was sustained despite the administration of intravenous oxacillin. Magnetic resonance imaging (MRI) of the thoracolumbar spine revealed spinal epidural abscess from T12 to S1. Because of severe hypoalbuminemia and general anasarca and followed by exploratory laparotomy for massive duodenal bleeding, she did not receive surgical intervention for the spinal epidural abscess. After intravenous administration of oxacillin 2 g 4-hourly for 12 weeks, she recovered and follow-up MRI confirmed the efficacy of the medical treatment. She remained well at 1-year follow-up. In a patient with minimal neurological deficit or surgical contraindication, spinal epidural abscess can be successfully treated with a medical regimen.     

A Korean case of juvenile muscular atrophy of distal upper extremity (Hirayama disease) with dynamic cervical cord compression

We present a Korean case of Hirayama disease with its typical neuroradiological findings of forward displacement of cervical dural sac and compression of the lower cervical cord during neck flexion. A 15-yr-old boy was presented with a one-year history of progressive weakness and atrophy affecting bilateral hands and forearms. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. Presumably, this disease might have been prevalent in Korea frequently under the diagnosis of "benign focal amyotrophy". In this regard, we discuss the clinical importance of cervical MRI with neck flexion and anticipate the increasing reports of the case substantiated by its characteristic radiological features.     

A novel model for biomechanical behavior of human brain in epidural hematoma injuries

To obtain an appropriate model for the simulation of the biomechanical behavior of brain tissue and the deformation of ventricles, in particular, we have developed a novel computerized plain strain finite element model. For optimum results, a multiple loading solutions approach using various tissue parameters for the simulation of epidural hematoma have been tested. For this purpose, CT-Scan of a patient with traumatic epidural hematoma has been modeled. By changing the tissue parameters (E and nu) and increasing intraventricular pressure gradient, the displacement of similar points in the modeled ventricle was compared with the true values obtained from patient's CT-Scan taken 3 months later after the resolution of hematoma. The magnitudes leading to least errors were determined. Best solutions were obtained with E=11-12 kPa and DeltaP=1.25-1.5 kPa (7.5-9.4 mmHg), which were consistent with the patient's clinical condition. Biomechanical modeling of unilateral displacement loadings, which are the conditions similar to surgical navigation systems, without considering ventricular geometry and their internal pressure resulted in unacceptable results.     

New onset focal weakness in children with Down syndrome

New onset focal weakness is relatively common in patients with Down syndrome (DS), and has broad differential diagnosis. Ten cases of new onset focal weakness in patients with DS were encountered or are currently being followed in two DS clinics, with a combined population of patients of approximately 850, for a clinic population prevalence of 1.2%. The median age at presentation was 4 years old (range 1 month-44 years). The causes of new onset focal weakness were: stroke from Moyamoya disease (two patients); stroke from vaso occlusive disease (one patient); stroke from venus sinus thrombosis (one patient); traumatic subdural hematoma (one patient); brain abscess (one patient); spinal cord injury (SCI) from cervical spinal stenosis (two patients); SCI from atlantoaxial instability (AAI) (one patient); and brachial plexus injury (one patient). Of the 10 patients with focal weakness, 8 had potentially treatable conditions, and 5 had surgery. The differential diagnosis of new onset focal weakness in DS is broad, with diseases reported involving all levels of the nervous system from brain to muscle. For some diagnoses, expeditious diagnosis may improve outcome.     

Corticospinal tract compression by hematoma in a patient with intracerebral hemorrhage: a diffusion tensor tractography and functional MRI study

The purpose of this study was to demonstrate corticospinal tract compression that was due to a hematoma by using diffusion tensor tractography (DTT) and functional MRI (fMRI) in a patient with an intracerebral hemorrhage (ICH). A 23-year-old right-handed woman presented with severe paralysis of her right extremities at the onset of a spontaneous ICH. Over the first three days from onset, the motor function of the affected upper and lower extremities rapidly recovered to the extent that she was able to overcome applied resistance to the affected limbs, and her limbs regained normal function 3 weeks after onset. The tract of the right hemisphere originated from the primary sensori-motor cortex (SM1) and it passed through the known corticospinal tract pathway. However, the tract of the left hemisphere was similar to that of the right hemisphere except that it was displaced to the antero-medial side by the hematoma at the cerebral peduncle. Only the contralateral SM1 area centered on the precentral knob was activated during affected (right) or unaffected (left) hand movements, respectively. In conclusion, fMRI and DTT demonstrated a corticospinal tract compression due to hematoma in this patient. We conclude that the combined use of these two modalities appears to improve the accuracy of investigating the state of the corticospinal tract.     

A 15-year-old female with progressive myelopathy

March 2005. A 15-year-old female presented with urinary retention, inability to walk and low back pain. MRI disclosed an elongated epidural mass in the thoracic spine with cord compression and invasion into the surrounding bone and soft tissue. Cytologic preparation at intraoperative consultation disclosed a rather monotonous small tumor cells with hyperchromatic nuclei and vacuolated cytoplasm. These vacuoles were best visualized with DiffQuick stain. Pathologic studies revealed an Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) with typical features including periodic acid Schiff positive diastase sensitive cytoplasmic substance; strong membranous pattern of immunoreactivity for CD99, and a reciprocal translocation of t(11;22)(q24;q12) that was demonstrated by fluorescent in situ hybridization (FISH). The vacuolated cytoplasm was produced by glycogen as demonstrated by electron microscopy. Although primary vertebral EWS/pPNETs are uncommon, they should be considered in the differential diagnoses of extradural/epidural mass of the spine in young patients.     

Chronic idiopathic myelofibrosis presenting as cauda equina compression due to extramedullary hematopoiesis: a case report

Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow- up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea.     

Morphology of the human internal vertebral venous plexus: a cadaver study after latex injection in the 21-25-week fetus

The morphology of the anterior and posterior internal vertebral venous plexus (IVVP) in human fetuses between 21-25 weeks of gestational age is described. The results are compared to the findings of a previous morphological study of the IVVP in the aged. The morphological pattern of the anterior IVVP in the fetus is very similar with the anterior IVVP in the aged human. In contrast, the posterior IVVP in the fetus lacks the prominent transverse bridging veins that are present in the aged lower thoracic and the lumbar posterior IVVP. The background of these morphological differences is unclear. Maybe the thoracolumbar part of the posterior IVVP is subject to "developmental delay," or the observed differences in the aged may result from functional and age-related factors that trigger this part of the vertebral venous system during (erect) life. The observed age related morphological differences of the posterior IVVP support the concept of the venous origin of the spontaneous spinal epidural hematoma (SSEH).     

Collet-Sicard syndrome associated with occipital condyle fracture and epidural hematoma

A 31-year-old male was presented with a very rare case of ipsilateral palsies of the nerves IX through XII (Collet-Sicard syndrome) after a closed head injury. An occipital condyle fracture that was associated with epidural hematoma was diagnosed by computed tomography. The patient was conservatively managed, and following the treatment, partial neurological recovery ensued. The phenomenon of occipital condyle fracture involving the last four cranial nerve palsies is relatively rare. Although 3 cases of Collet-Sicard syndrome that were caused by an occipital condyle fracture has been reported, the association between condyle fracture and epidural hematoma has never been described before.     

以脊髓压迫为首发症状的髓系肉瘤5例临床分析

目的 探讨以脊髓压迫为首发症状脊柱髓系肉瘤患者的临床表现、影像学特征及治疗方法。方法 采用回顾性横断面研究方法。纳入2014年1月—2017年12月长征医院骨肿瘤科收治的经骨髓穿刺及病理证实的脊柱髓系肉瘤患者5例,其中男3例、女2例,年龄15~54岁;肿瘤位于胸椎3例、腰椎2例。4例采取开放性外科手术治疗,出院后根据骨髓穿刺及病理结果,予以化疗及血液系统肿瘤规范化治疗;另1例行内科保守治疗(消炎镇痛、营养支持、化疗等)。分析患者治疗前后各项观察项目。结果 5例患者均有腰背部疼痛,伴下肢无力3例、双下肢不全瘫1例;X线检查均未见异常,CT和MRI表现为椎体骨质破坏或占位性病变。骨髓穿刺及术后肿瘤病理结果显示为急性粒细胞白血病4例,慢性粒细胞白血病1例;5例患者均经治疗后症状有所缓解。5例患者均获随访:1例术后行去甲氧柔红霉素+阿糖胞苷方案化疗5个疗程,并行异基因造血干细胞移植治疗,目前已随访至术后28个月,患者一般状态良好、无肿瘤复发;另外4例经化疗后出现复发,均死于感染,生存期5~26.5个月。结论 以脊髓压迫为首发症状的髓系肉瘤病例临床罕见,其影像学表现缺乏特异性,易出现误诊,骨髓穿刺及病理检查可确诊;当出现脊髓压迫表现时,建议早期行肿瘤切除神经减压术,术后辅以异基因造血干细胞移植和全身化疗。