Miyazaki syndrome: Cervical myelo/radiculopathy caused by overshunting. A systematic review.

OBJECTIVES: Miyazaki syndrome is a cervical myelopathy or radiculopathy caused by cervical epidural venous congestion, due to cerebrospinal fluid over-drainage by an implanted ventricular shunt. The complex pathophysiology includes CSF pressure-changes consistent with the Monro-Kellie doctrine and a non-functional Starling resistor, leading to spinal epidural venous plexus enlargement and dilation. This venous congestion may be significant enough to exert compression on the spinal cord or nerve roots. The typical clinical and imaging findings together with a history of ventricular CSF shunting may establish the diagnosis, proven by a successful treatment. The aim of treatment is the abrogation of CSF over-drainage. The eligible interventions may be the followings: the increase of the opening-pressure of the valve system by the insertion of a new programmable valve if necessary, closing or removing the shunt. AIM: We want to call attention to this rare iatrogenic condition with potentially severe consequences. PATIENTS AND METHODS: We perform a systematic literature-review and present our five cases. RESULTS: Once recognized in time, Miyazaki syndrome can be well taken care of. CONCLUSIONS: Patients with chronic ventricular shunt need monitoring for CSF over-drainage to recognise potential complications such as cervical myelopathy or radiculopathy.

Role of modified open-door laminoplasty in the treatment of multilevel cervical spinal stenosis: a retrospective analysis of 43 cases.

BACKGROUND AND PURPOSE: Symptomatic degenerative multilevel cervical spinal stenosis--beside other methods--is often treated using the open-door laminoplasty. This procedure aims to decompress the spinal cord and preserve the stability of the cervical spine. The efficiency and safety of the method was proved by numerous Japanese and American studies, also the technique related complications are well known. We treated 43 patients with symptomatic multilevel cervical spine stenosis using the open-door laminoplasty as a surgical procedure of choice in the National Institute of Clinical Neurosciences between 2009 and 2012. In this article we analyse our results and the related literature is discussed. METHODS: Symptomatic patients with a minimum of three-segment cervical spine stenosis and radiologically proved myelopathy or with electrophisiologically verified subclinical myelopathy were selected for laminoplasty. Patients in whom cervical kyphosis was present were operated on using laminectomy and posterior fusion. Postoperative control CT, MRI and/or X-ray images were made after the surgery and at six weeks, three, six and 12 months after the operation and in the same time neurological evaluation was performed. The modified Japanase Orthopaedic Association (mJOA) scale value was assigned to patients preoperatively, six weeks, three, six and 12 months after the operation. The statistical difference between the groups of data was tested by chi square test. RESULTS: The average follow-up time was 27 months (minimum seven, maximum 42). According to the mJOA scale, 26 patient's condition (61%) improved, in 13 cases (30%) remained unchanged, and in one case (2%) we detected neurological deterioration. We lost three patients during the follow up period. The median of mJOA preoperatively was 12 (minimum eight, maximum 18), while six week postoperative mJOA was 14 (minimum 10, maximum 17). Three, six and 12 months mean value of mJOA was 14 which shows that the improvement in patients' condition remained stable at one year after surgery. The difference was statistically significant (p < 0.05). The canal's average anteroposterior diameter on CT was 8.29 ± 0.92 mm at the level of C III, while after the operation we measured 15.16 ± 1.02 mm; 7.54 ± 0.62 mm at the level of C IV before, and 15.29 ± 0.2 mm after; 9.05 ± 0.48 mm at the level of C V before and 17.23 ± 0.4 mm after the surgery. The differences proved to be significant (p = 0.0001). CONCLUSION: According to our experiences the modified open-door laminoplasty is an efficient and safe method for the treatment of symptomatic multilevel cervical spinal stenosis.

[Treatment of osteoporotic vertebral compression fractures with PMMA-augmented pedicle screw fixation]. / Osteoporoticus kompressziós csigolyatörések kezelése PMMA-augmentált csavaros transpedicularis rögzítéssel.

BACKGROUND: Over the last few decades many innovative operation technique were developed due to the increase of porotic vertebral fractures. These new techniques aim to reach the required stability of the vertebral column. In case of significant instability, spinal canal stenosis or neural compression, decompressive intervention may be necessary, which results in further weakening of the column of the spine, the minimal invasive percutan vertebroplasty is not an adequate method to reach the required stability, that is why insertion of complementary pedicular screws is needed. Considering the limited screw-fixing ability of the porotic bone structure, with this new technique we are able to reach the appropriate stability of cement-augmented pedicle screws by dosing cement carefully through the screws into the vertebral body. We used this technique in our Institute in case of 12 patients and followed up the required stability and the severity of complications. METHODS: Fifteen vertebral compression fractures of 12 patients were treated in our Institute. Using the classification proposed by Genant et al. we found that the severity of the vertebral compression was grade 3 in case of 13, while grade 2 in case of two fractures. The average follow up time of the patients was 22 months (12-39), during this period X-ray, CT and clinical control examinations were taken. During the surgery the involved segments were localised by using X-ray and after the exploration the canulated screws were put through the pedicles of the spine and the vertebral body was filled through the transpedicular screws with bone cement. Depending on the grade of the spinal canal stenosis, we made the decompression, vertebroplasty or corpectomy of the fractured vertebral body, and the replacement of the body. Finally the concerned segments were fixed by titanium rods. RESULTS: In all cases the stenosis of spinal canal was resolved and the bone cement injected into the corpus resulted in adequated stability of the spine. In case of six patients we observed cement extravasation without any clinical signs, and by one patient--as a serious complication--pulmonary embolism. Neurological progression or screw loosening were not detected during the follow up period. Part of the patients had residual disability after the surgery due to their older ages and the problem of their rehabilitation process. CONCLUSION: After the right consideration of indications, age, general health condition and the chance of successful rehabilitation, the technique appears to be safe for the patients. With the use of this surgical method, the stability of the spine can be improved compared to the preoperative condition, the spinal canal stenosis can be solved and the neural structures can be decompressed. The severity of complications can be reduced by a precise surgical technique and the careful use of the injected cement. The indication of the surgical method needs to be considered in the light of the expected outcome and the rehabilitation.

[Tailored cranioplasty using CAD-CAM technology]. / Személyre szabott koponyacsontpótlások CAD-CAM technológia felhasználásával.

OBJECTIVE: The majority of cranial defects are results of surgical intervention. The defect must be covered within resonable period of time usually after 4-6 week given the fact that the replacement of bone improve the brain circulation. Number of surgical techniques and materials are available to perform cranioplasty. Due to favorable properties we chosed ultra high molecular weight polyethylene as material. In this paper the authors show a procedure which allows tailored artificial bone replacement using state of art medical and engineering techniques. METHODS: between 2004 and 2012, 19 patients were operated on cranial bone defect and a total of 22 3D custom-designed implants were implanted. The average age of patients was 35.4 years. In 12 patients we performed primary cranioplasty, while seven patients had the replacement at least once. Later the implants had to be removed due to infection or other causes (bone necrosis, fracture). All patients had native and bone-windowed 1 mm resolution CT. The 3D design was made using the original CT images and with design program. Computer controlled lathe was used to prepare a precise-fitting model. During surgery, the defect was exposed and the implant was fixed to normal bone using mini titanium plates and screws. All of our patients had control CT at 3, 6 and 12 months after surgery and at the same time neurological examination. RESULTS: Twenty-one polyethylene and one titanium implants were inserted. The average follow-up of the patients was 21.5 months, ranged from two to 96 months. We follow 12 patients (63.15%) more than one year. No intraoperative implant modifications had to be made. Each of the 22 implant exactly matched the bone defect proved by CT scan. No one of our patients reported aesthetic problems and we did not notice any kind of aesthetic complication. We had short term complication in three cases due to cranioplasty, subdural, epidural haemorrhage and skin defect. CONCLUSION: Polyethylene is in all respects suitable for primary and secondary cranioplasty. Combined with 3D CAD- CAM method excellent aesthetic and functional result was achieved. In our study no case of infection occured. Proper preoperative preparation is important.

Symptomatic subependymomas of the ventricles. Review of twenty consecutive cases.

BACKGROUND AND PURPOSE: Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. PATIENT SELECTION AND METHODS: Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005. RESULTS: Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years. CONCLUSION: Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.

Ependymoma in adults: surgery, reoperation and radiotherapy for survival.

PURPOSE: to retrospectively determine the long-term outcome of adult intracranial ependymoma patients treated with surgery, reoperation, and postoperative radiation therapy. MATERIAL AND METHODS: 61 patients were treated at our institution between 1980 and 2004. Forty patients had World Health Organization (WHO) Grade II ependymoma, and 21 patients had Grade III ependymoma. The median age was 34 years. The majority of patients were female (59%), and 35 had gross total resections (60%). Eighteen patients were reoperated, 15 only once but 2 twice and one six times. Survival times following reoperation was mostly short but some of them reached more than 5 or 10 years. Postoperative radiation therapy was delivered to 31 patients postoperative (55.4%) and to 5 after reoperation, a median total dose of 54 Gy. RESULTS: The median follow-up of surviving patients was 10.6 years. The 5-year and 10-year disease free survival rates for all patients were 50% and 32.9% respectively. The 5-year and 10-year overall survival rates for all patients were 57.1% and 39.4%, respectively. A statistically significant effect on prognosis was observed with WHO tumour grade as well as with MIB-1 labelling index. Subtotal resection predicted a worse overall survival, but this failed to reach statistical significance. No statistically significant effect on prognosis was observed with tumour location and radiation therapy. CONCLUSION: In our experience the use of radiotherapy in adult, intracranial WHO Grade II ependymoma patients had no significant effect on prognosis. Radical surgery and eventual reoperation seems to be more favorable.

Temozolomide chemotherapy of patients with recurrent anaplastic astrocytomas and glioblastomas.

INTRODUCTION: Anaplastic astrocytomas and glioblastomas are the most frequent and most malignant hemispherial tumours. Unfortunately, astrocytic tumours are of infiltrative character and radical removal is not possible. Recurrent malignant gliomas are rarely suitable for reoperation. In most of the cases of recurrent gliomas chemotherapy is the last choice. PATIENTS AND METHOD: Seventy-five consecutive patients with recurrent malignant astrocytomas and glioblastomas had been treated at our institute with per os temozolomide for five days every month. The patients received two to 16 courses of chemotherapy. The toxicity, quality of life, response to chemotherapy and survival data were analysed. RESULTS: Out of 75 patients four were excluded following the first treatment due to myelotoxicity, and allergic reactions. Among the patients treated with temozolomide in seven cases complete response, 17 partial response, 14 progressive disease were observed. In 33 cases the disease stabilized and out of them in 27% a significant neurological improvement was detected. The time to progression was 6.8 months and the median survival time 8.75 months for patients with glioblastoma and with malignant astrocytoma or malignant mixed oligoastrocytoma 9.45 and 11.15 months, respectively. The overall survival for patients with originally lower grade glioma was 70.32 and for patients with glioblastoma multiforme 17.43 months. CONCLUSIONS: Temozolomide chemotherapy in patients with recurrent malignant astrocytoma and glioblastoma proved to be efficacious and similar good results were achieved as with a nitrosourea based combined chemotherapy. Even in those patients who received previous chemotherapy temozolomide is well tolerated and a relatively long time to progression was achieved in cases of recurrent malignant gliomas. In a few number of patients where BCNU had been previously failed with temozolomide stable disease was achieved. Temozolomide seems to be a promising drug in the chemotherapy of malignant gliomas and can be applied as a second line chemotherapy, as well.

Role of early radiotherapy in the treatment of supratentorial WHO Grade II astrocytomas: long-term results of 97 patients.

OBJECT: To determine principal prognostic factors and the effect of timing of radiotherapy (RT) on disease-specific survival (DSS) and progression-free survival (PFS) in WHO Grade II astrocytomas. METHODS: Histologic slides of 166 consecutive patients with the original tissue diagnosis of low-grade, non-pilocytic astrocytoma were reviewed. One-hundred and six were selected where two additional certified neuropathologist agreed on the grading of WHO Grade II astrocytoma. In 97 out of 106 cases follow-up informations were available. Early postoperative RT was given to 36 out of 97 patients (37%). The two groups of patients (early vs. delayed RT) were well balanced in respect to extent of surgery and other main clinical prognostic factors. Median follow-up of surviving patients was 79 months. The 5- and 10-year PFS was 52.2% and 30.7% with early RT and 39.5% and 12.4% with delayed RT (p = 0.0388). In respect to DSS, there was no significant difference in the 5- and 10-year actuarial survival rate according to the timing of RT (60.5% and 26.5% vs. 66.6% and 23.7%; p = 0.7545). Age (p = 0.0145) and extent of surgery (p = 0.0473) were significant prognostic variables in respect to DSS. Subdividing the irradiated group based on the extent of surgery, early RT in the subtotal group significantly improved 5-year PFS (60.0% vs. 12.4%; p = 0.0036) and DSS (66.7% vs. 49.8%; p = 0.0389). However, postoperative RT had no influence on PFS (p = 0.6812) and DSS (p = 0.3987) in the group with extensive resection. CONCLUSION: Early postoperative RT in subtotally resected, Grade II astrocytomas significantly improves both progression-free and disease-specific survival. Early RT does not benefit patients with extensive resection, RT should be withheld in these patients until progression.

[Chemotherapy of recurrent supratentorial malignant gliomas (phase II study)]. / A recidív supratentorialis malignus gliomák kemoterápiás kezelése (II. fázisú tanulmány).

At the Hungarian National Institute of Neurosurgery 73 recurrent supratentorial malignant tumours were treated by chemotherapy during the last ten years. Chemotherapy was applied after postoperative radiotherapy but in some cases following reoperation only. All cases were clinically and by CT or MRI verified recurrences. Forty-three patients received BCNU-DBD (dibromodulcitol) treatment (23 anaplastic astrocytoma--AA, and 20 glioblastoma multiforme--GM): day 1. BCNU 150 mg/sq.m. in i.v. infusion, day 2. dibromdulcitol 1000 mg/sq orally was given. This course was repeated every six weeks, altogether 2-8 times. Sixteen patients with AA responded with complete or partial regression but only 6 did with GM. Median survival was 14 and 7 months, the difference proved to be significant, p = 0.0091. PCV combination (procarbazine, CCNU, vincristine) was applied to 16 patients with AA and 14 cases with recurrent oligodendroglioma (O). Treatment started with vincristine 1.5 mg/sq.m. i.v. (2.0 mg maximum), the next day CCNU 100 mg/sq.m. was given, followed by procarbazine 60 mg/sq.m. on days 8-22. and finished by the same dose of vincristine on day 30. The course was repeated after one month, mostly six times. Six patients with AA did not respond; in cases of oligodendroglioma all but one responded with complete or partial improvement. It is remarkable that no significant difference was found between the survivals of BCNU-DBD or PCV treated AA patients. Chemotherapy of supratentorial malignant glioma recurrences with nitroso-ureas and their combination proved to be efficacious. It also seems, that in recurrent cases lower grade gliomas show better response rate than glioblastomas.

[Treatment of recidive malignant gliomas with temozolomide]. / Recidív malignus gliomák kezelése temozolomide-dal.

INTRODUCTION: The prognosis of malignant gliomas despite of the recent advances of diagnostical and therapeutical techniques remains poor. The majority of gliomas following total removal and postoperative radiotherapy recurs. In case of recurrencies reoperation is rarely possible and chemotherapy is the last treatment modality. METHODS: Forty patients with recurrent malignant gliomas had been treated with temozolomide (Temodal). The treatment had to be stopped in four cases. RESULTS: Complete remission was observed in 3, partial in 11, progressive disease in 4 and stable disease in 50% of the cases with CT and/or MR images. The mean progress free interval was 6.25 and the mean survival time 9 months. According to the primary histology the mean survival time for glioblastoma patients was 6.8 and for anaplastic astrocytoma or mixed oligoastrocytoma patients 12.2 months. CONCLUSIONS: Due to its low toxicity and relatively long survival time after recurrency temozolomide seems to be a promising drug in the treatment of recurrent malignant gliomas.

Effect of a single high-dose gamma irradiation on cultured cells in human cerebral arteriovenous malformation.

OBJECT: The purpose of this study was to analyze the effect of single high-dose gamma irradiation at a cellular biological level on tissue cultures obtained in patients who underwent surgery for cerebral arteriovenous malformation (AVM). METHODS: The cell proliferation indices and changes in activation of p53, p21Waf-1, and mdm-2 were determined. Additionally, immunohistochemical investigations for vimentin, desmin, alpha-smooth muscle actin (alpha-SMA), glial fibrillary acidic protein, Factor VIII-related antigen (F-VIII), cytokeratin, S100, and transforming growth factor-beta (TGFbeta) were performed on cultured AVM cells after a single high-dose irradiation. Normal human brain microvessel endothelial (HBE) cells and aortic smooth muscle cells served as controls. The proliferation index decreased on the 5th day after irradiation and remained depressed over the observation period in the irradiated AVM cultures. The p53, p21Waf-1, and mdm-2 messenger RNA measurements showed considerable elevation both in AVM cultures and HBE cells after 15-Gy irradiation, which indicated apoptosis. Immunohistochemistry revealed strong vimentin positivity in the nonirradiated cultures, which gradually decreased in the irradiated cultures. Transforming growth factor-beta positivity was demonstrated in the irradiated specimens, indicating transformation of fibroblastic cells into activated myofibroblastic elements. This transformation was confirmed by demonstrating elevated SMA expression as well in the radiation-treated fibroblasts. CONCLUSIONS: The presence of TGFbeta and alpha-SMA activity in the irradiated AVM cells suggests that along with the genetically confirmed apoptotic activity, fibroblast transformation into myofibroblasts might be one of the mechanisms leading to shrinkage and obliteration of AVMs after single high-dose gamma irradiation.