Background: In Japan, familial studies utilize human leukocyte antigen (HLA) or other genetic markers, morphological/biochemical studies, and plain X-rays to identify simple recessive, simple dominant, and multifactorial modes of genetic inheritance for ossification of the posterior longitudinal ligament (OPLL) [2,3,5-8]. Methods and Results: Two members of four North American Caucasian families presented with OPLL; two sets of brothers, one sister/brother pair, and one father/daughter combination. Each member exhibited radiographic (MR/CT) and surgical confirmation of early and mature OPLL or mature OPLL alone. Patients averaged 50 years of age, and exhibited mild to severe myeloradiculopathy requiring multilevel diskectomies/corpectomies with fusion. Original operations were performed between 1990-1997, and patients were followed an average of 10.25 years (range 7-14 years). Conclusion: It is now recognized that OPLL contributes to myeloradiculopathy in the North American Caucasian population, with evidence for familial inheritance factors and predisposition to this pathologic entity. This is the first report suggesting the inheritance of OPLL with myeloradiculopathy in 4 Caucasian North American Families. The presence of OPLL in one individual family member may presage an increased susceptibility to this disorder in other immediate relatives.

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Background Context: Posterior cervical fusion techniques have evolved from spinous process or facet wiring methods to the application of plates or rods utilizing lateral mass or pedicle screws. However, the morbidity associated with screw placement has prompted many surgeons to revert back to wiring constructs. Purpose: To document the efficacy of posterior cervical fusion employing a rod-eyelet and braided cable construct (Vertex System, Atlas Cables, Medtronic, Sofamor Danek, Memphis, TN). Cables were passed around rods, through eyelets and the base of spinous processes or facet joints in a “cerclage” fashion. Study Design/Setting: Eight posterior cervical Vertex fusions were prospectively performed. Patient Sample: Three patients exhibited ossification of the posterior longitudinal ligament (OPLL), 2 demonstrated stenosis with ossification of the yellow ligament (OYL), while 3 showed stenosis with spondylosis. Outcome Measures: Outcomes were measured one year postoperatively utilizing Nurick Grades (preoperative/postoperative) and Odom's Criteria. Medical Outcomes Trust Short-Form 36 questionnaires were administered preoperatively, and 3 months, 6 months and 1 year postoperatively. Methods: Three patients with OPLL had multilevel anterior corpectomy/fusion (ACF) with Vertex spinous process fusions (PF) performed under one anesthetic. Two patients with stenosis and OYL underwent focal 1-2 level laminectomies with Vertex spinous process fusions, while 3 with stenosis and spondylosis had multilevel laminectomies with Vertex spinous process/facet fusions. Results: Eight patients demonstrated dynamic X-ray and 2D-CT evidence of fusion an average of 4.5 months postoperatively. One year following surgery, patients improved an average of 3.6 Nurick Grades, and Odom's Criteria revealed 3 good and 5 excellent outcomes. Maximal improvement occurred 1 year postoperatively on the SF-36 Role Physical (0 to 30), Bodily Pain (27.2 to 65), and Role Emotional (13.3 to 40) Health Scales. Conclusions: Posterior spinous process/facet fusions utilizing the Vertex rods and eyelets with Atlas cables resulted in successful fusions in 8 patients.

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Between the end of 1979 and the end of 2002, the author operated 24 cases of intramedullary astrocytomas, 34 ependymomas, 12 hemangioblastomas and 30 vascular tumors. The long-term results were investigated at the end of March 2002. Nearly half of the patients with astrocytoma died in 5 years without regard to historogical malignancy except for the pilocytic astrocytoma and astrocytoma grade I. About 6 months after the operation, 91.3% of the patients with ependymoma in cervical including cranio-cervical and cervico-thoracic level went back to work. On the contrary 75% of the patients, who had tumor in thoracic level indicated no significant recovery and had to reconcile themselves to wheelchair life. The functional prognosis of the patient with hemangioblastomas is good so far as he was operated properly. Just half of the causes of the intramedullary hematoma were the cavernous angioma, and the clinical and functional prognosis were generally excellent. During the same period the author experienced 10 miscellaneous tumors and also 10 nontumorous intramedullary lesions.

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To evaluate neuroradiological characters affecting postoperative shrinkage of syringes associated with Chiari type 1 malformation, we assessed 56 patients who underwent foramen magnum decompression (FMD) and were followed-up at least one year. In 48 cases (85.4%), the postoperative courses were quite satisfactory. The cerebellar tonsils have moved upward during surgery and the syringes have decreased in size on MRI taken one or three months after surgery (the prompt group). In eight of 56 patients (14.3%), however, the syringes were slow to shrink and remained over one year after surgery (the delayed group). Basilar impression was more common in the delayed group (p=0.026). These patients had a wider basal angle (142.6 degrees for the delayed group compared with 135.4 degrees for the prompt group), narrower clivo-axial angle (CAA; 121.7 and 142.8 degrees, respectively) and severely lordotic cervical spine (8.0mm and 4.5mm, respectively) on plain X ray films. Those with CAA narrower than 130 degrees showed significant delay in shrinking of the syringes. The CAA might be a good indicator to predict the time course of syrinx shrinkage after FMD.

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The authors describe a rare case of hemangiopericytoma with cervical metastasis. A 34 year-old man with a history of intracranial hemangiopericytoma complained of a weakness of his right hand. Both CT scan and MR imaging demonstrated homogeneous enhanced tumor in his C5 vertebral body, right facet joint and lamina. Preoperative embolization was performed in order to decrease bleeding during the operation. A two-stage operation was performed because the tumor was considered to be too large to remove all at once. First, from the anterior, partial tumor resection and C5 corpectomy were done followed by anterior fusion with a cervical plate. After which, a secondary surgery was carried out from the posterior. The gross total tumor removal was accomplished successfully by these procedures. Histological findings revealed typical hemangiopericytoma. Despite fifty-four Gy of local irradiation subsequently being performed for cervical lesion to prevent tumor recurrence, multiple metastases have been revealed twenty-five months after the surgery. Careful and long follow up for hemangiopericytoma is essential even after gross total surgical removal and postoperative radiotherapy.

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The aim of the present study is to better understand the histological alterations of arterialized medullary veins (MV) in spinal dural arteriovenous fistulas (SDAVFs) and its relationship to clinical status before and after surgical treatment of SDAVFs. Three patients presented with progressive congestive myelopathy of varying duration. In all three cases, magnetic resonance imaging (MRI) demonstrated patchy cord enhancement on postcontrast Tl-weighted images, and extension of spinal intramedullary high signal on T2-weighted images. Spinal angiography demonstrated SDAVFs with retrograde filling of the medullary vein (MV). Surgical obliteration was achieved by direct intradural interruption of the arterialized MV in all cases. After surgical interruption, a segment of the arterialized MV was obtained from each patient for histological examination. The three patients were stabilized or improved neurologically after surgery, with a gradual decrease of spinal intramedullary high signal on T2-weighted MR images. Histological examination showed hyperplasia of the elastic fibers of the MV, which varied between the three cases. These histological alterations of the MV appeared to reflect the preoperative evolution of retrograde venous hypertension of the spinal cord circulation, and may correlate with neurological recovery. The histological alterations of the spinal cord circulation in cases of SDAVF may reflect the progressive and irreversible nature of spinal cord function. Although the relationship between the spinal cord circulation and function needs to be further explored, better understanding of the histological nature of SDAVFs is important for their early and appropriate treatment.

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The craniovertebral junction is the greatest mobile region in the whole spinal column and often associated with instability. Therefore, one must consider stability of this region in addition to decompression of the nervous system in the pathology of the craniovertebral junction. Two representative cases were shown here, one with Chiari I malformation associated with atlanto-axial dislocation and basilar invagination, and the other with atlantoaxial dislocation and periodontoid pannus associated with rheumatoid arthritis. The selection of surgical approaches and operative results in these patients were demonstrated to facilitate understanding of the current standards of spinal surgery in the craniovertebral junction.

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There are many non-neoplastic intramedullary lesions that mimic intramedullary neoplasm. They are consisted of infections, demyelination, granulomatous disease, radiation myelopathy, vascular lesions, and syringomyelia. In this report, the authors described its differential diagnosis in clinical and neuroradiological findings. Furthermore, we mentioned its surgical approach for several non-neoplastic lesions. Syringomyelia and spinal AV fistula are both good candidate for surgical treatments. We would like to stress that good cooperation between neurologist, neuroradiologist, and neurosurgeon is important to differentiate non-neoplastic lesions and surgical biopsy is final procedure to confirm their pathology.

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