Myelopathy associated with melorheostosis: a case report

A man in his mid thirties presented with lower-extremity weakness and spasticity because of a myelopathy caused by a rare disorder of bone known as melorheostosis. The primary pathology involved was compression of the cord at the cervicothoracic levels by dystrophic osseous formation within the vertebral bodies. Based on a review of existing literature, it was evident that the spine is an uncommon location to find melorheostosis, making this disease entity a unique cause of myelopathy. The patient's progress was closely observed during his inpatient rehabilitation program, after he underwent spinal decompression surgery. Starting from the level of complete paralysis, he was able to regain functional strength in his legs by the end of his 2-month course. Despite the lack of reported outcomes in cases of myelopathy associated with melorheostosis, our report describes a favorable prognosis with good recovery of both strength and function.     

前路三种手术方式治疗脊髓型颈椎病的疗效分析

目的分析脊髓型颈椎病前路三种手术方式的优缺点。方法回顾性分析2002-01～2007-01治疗的150例脊髓型颈椎病患者。男82例,女68例;年龄40～75岁,平均53岁;单节段病变65例,双节段病变85例;前路减压＋植骨融合术45例（A组）、前路减压＋cage融合术30例（B组）、前路减压＋钛网内植骨＋钢板内固定术75例（C组）。所有病例均随访1年以上,采用日本骨科学会制定的JOA评分系统评定不同手术方法的疗效。结果所有患者术后JOA评分均有改善,单纯植骨融合组的JOA评分改善率为72.8%,椎间融合器组为85.6%,钛网内植骨并钢板内固定组为80.6%,椎间融合器组和钛网内植骨并钢板内固定组JOA评分改善率无显著性差异（P〉0.05）,椎间融合器组和钛网内植骨并钢板内固定组与单纯植骨融合组JOA评分改善率有显著性差异（P〈0.05）。结论脊髓型颈椎病的治疗关键在于充分减压及有效植骨融合,单纯减压植骨融合术后并发症多;减压加椎间融合器植入术具有操作简单、出血少、术后恢复好等优点;钛网内植骨加钢板固定牢固是治疗脊髓型颈椎病的较好方法。     

Unusual neurological complications following tetanus toxoid administration

Zusammenfassung Es wird über eine Poly-neuro-myelo-encephalopathie, die fünf Tage nach einer Tetanus-Toxoid-Gabe auftrat, berichtet. Komplikationen, speziell neurologische Symptome, sind bei der aktiven Tetanusimmunisierung sehr selten. Sie verlaufen meist unter dem Bilde einer Armplexus-oder Hirnnervenneuropathie. Eine derart schwere neurologische Symptomatik mit Befall des peripheren und des zentralen Nervensystems wurde bisher bei der Tetanus-Toxoid-Impfung nicht beschrieben.     

An ISO-effect table for radiation tolerance of the human spinal cord

Available literature on radiation injury to the human spinal cord was collected into a comprehensive data set relating the incidence of myelopathy to dosage, number of fractions and total treatment time. The data was analyzed using a search program (RAD3) to derive best-fitting cell kinetic parameters on the assumption that radiation myelopathy arises from cellular depletion in the irradiated tissues. From these parameters iso-effect tables were constructed for a wide range of treatment schedules, including daily treatment as well as fractionation at longer intervals. The tables provide a set of limiting doses, above which the risk of radiation injury to the spinal cord becomes substantial. Iso-effect functions derived from the multi-target and linear-quadratic models were essentially similar and compatible with a linear function having an origin at 11 Gy and a slope of about 0.45. This is distinctly different from the conventional nominal standard dose (NSD) equation. General application of NSD tolerance limits could lead to systematic overdosage of the spinal cord, expecially with large individual fractions or short treatment times. We conclude that the computed iso-effect tables provide a more reliable clinical guide than conventional time-dose equations.     

Paget's Disease of the spine and its management

A review of the literature was conducted to study the pathomechanics by which Paget's Disease of bone (PD) alters the spinal structures that result in distinct spinal pathologic entities such as pagetic spinal arthritis, spinal stenosis, and other pathologies, and to assess the best treatment options and available drugs. The spine is the second most commonly affected site with PD. 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. 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. Since, in the majority of cases with pagetic spinal involvement, there are also coexisting osteoarthritic changes, antipagetic medical treatment alone may be disappointing. Therefore, one must be careful before attributing low back pain to PD alone. Five classes of drugs are available for the treatment of PD: bisphosphonates, calcitonins, mithramycin (plicamycin), gallium nitrate, and ipriflavone. Bisphosphonates are the most popular, and several forms have been investigated, but only the following forms have been approved for clinical use: disodium etidronate, clodronate, aledronate, risedronate, neridronate, pamidronate, tiludronate, ibadronate, aminohydroxylbutylidene bisphosphonate, olpadronate, and zoledronate. Several of these forms are still under investigation.     

Spinal dysraphism in an elderly patient

Spinal dysraphisms are diagnosed more frequently at birth or in infancy. We report a spinal malformation compatible with lipomyeloschisis in an elderly patient presenting with symptoms and signs of myelopathy. Magnetic resonance imaging revealed an intraspinal mass continuous with a subcutaneous lipoma. Three-dimensional computed tomography reconstructions better showed the spinal dysraphism; dermal sinus was also evident. Neuroimaging can define the precise diagnosis also in elderly patients presenting with myelopathy and can provide valuable structural details. Received: 23 May 2001 / Accepted in revised form: 20 September 2001     

Syphilitic meningomyelitis misdiagnosed as spinal cord tumor: Case and review

Context: Syphilitic meningomyelitis is a rare manifestation of neurosyphilis, not well described in the literature.Methods: We reported a rare case of a 29-year-old female with syphilitic meningomyelitis. Her clinical manifestations and imaging findings were discussed with the related literatures reviewed.Results: The patient presented with progressive bilateral lower extremities numbness and weakness for months. Laboratory tests revealed positive serum Treponema pallidum Hemagglutinin Test (TPHA) and rapid plasma reagin test (RPR). The cerebral spinal fluid (CSF) was positive with TPHA but negative for RPR with lymphocytic pleocytosis and elevated protein. Spinal MRI showed swelling and high-signal intensity of thoracic spinal cord except T6-7 level with associated gadolinium enhancement (“flip-flop sign”) and peripheral strip-like enhancement on T1WI (“candle guttering appearance”). She was initially diagnosed as spinal cord tumor due to the chronic clinical onset and cord swelling with central enhancement found on thoracic MRI. After dramatic clinical and radiographic improvement with dexamethosone and serological tests of syphilis, she was diagnosed as probable syphilitic meningomyelitis. Till now, there are 12 cases of syphilitic myelitis reported with spinal cord MR images. Thoracic cord is the predominant involved segment (10/12), “candle guttering appearance” is the most common enhancing characteristics of the lesion (7/12), “flip-flop sign” may be seen in the stage with significant inflammation (3/12).Conclusion: Syphilitic meningomyelitis can occur at early or late stage of syphilis, the onset may be acute, subacute or chronic. The imaging findings suggested focal inflammation of the spinal cord. Prognosis is relatively good after proper treatment.     

Modeling chronic cervical spinal cord injury in aged rats for cell therapy studies

With an expanding elderly population, an increasing number of older adults will experience spinal cord injury (SCI) and might be candidates for cell-based therapies, yet there is a paucity of research in this age group. The objective of the present study was to analyze how aged rats tolerate behavioral testing, surgical procedures, post-operative complications, intra-spinal cell transplantation and immunosuppression, and to examine the effectiveness of human iPSC-derived Neural Progenitor Cells (IMR90-hiPSC-NPCs) in a model of SCI. We performed behavioral tests in rats before and after inducing cervical hemi-contusions at C4 level with a fourth-generation Ohio State University Injury Device. Four weeks later, we injected IMR90-hiPSC-NPCs in animals that were immunosuppressed by daily cyclosporine injection. Four weeks after injection we analyzed locomotor behavior and mortality, and histologically assessed the survival of transplanted human NPCs. As rats aged, their success at completing behavioral tests decreased. In addition, we observed high mortality rates during behavioral training (41.2%), after cervical injury (63.2%) and after cell injection (50%). Histological analysis revealed that injected cells survived and remained at and around the grafted site and did not cause tumors. No locomotor improvement was observed in animals four weeks after IMR90-hiPSC-NPC transplantation. Our results show that elderly rats are highly vulnerable to interventions, and thus large groups of animals must be initially established to study the potential efficacy of cell-based therapies in age-related chronic myelopathies.