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.

[Treatment-induced tumor-like lesions in the course of neurooncological therapy]. / Tumort utánzó barrierzavarok a neuroonkológiai terápia során.

The proper interpretation of imaging changes in the course of multimodal neurooncological therapy (neurosurgery, radiotherapy, chemotherapy, stereotactic radiosurgery) is crucial. The appearance of abnormal or new contrast-enhancing lesions does not indicate obvious tumor progression, in the contrary they are frequently induced by the oncological therapy itself. The differentiation of real tumor progression from therapy-induced lesions is essential, since the diagnosis of progressive disease results in the termination of the current regimen and initiation of second or third line therapy, if possible. The most common frequent therapy-induced tumor-like lesions include the followings: pseudoprogression seen at 1-3 months after the completion of concomittant radiochemotherapy of high-grade gliomas, real radiation necrosis which can develop even years after the completion of fractionated external beam radiotherapy of gliomas, and radiation necrosis seen after stereotactic radiosurgery delivered to metastatic brain tumors. The absorbable hemostatic materials applied to the wall of resection cavity during brain tumor surgery might cause delayed disturbancies in the blood brain barrier, inducing abnormal signal changes and contrast enhancement mimicking residual or recurrent tumor. Cerebrovascular ischemic lesions might cause cortical enhancement in the subacute stage, which may be misinterpreted as leptomeningeal tumor spread. The correct assessment of imaging findings requires special knowledge and multidisciplinary consultation, therefore the treatment and follow-up of brain tumor patients should be linked to brain tumor centers staffed by experts in the field of neurosurgery, neurooncology and brain tumor imaging.

Clinical experiences with Creutzfeldt-Jakob disease: three case studies.

The clinical picture, electroencephalographic, imaging and cerebrospinal fluid parameters as well as the molecular background of Creutzfeldt-Jakob disease have been well explored. The diagnostic criteria, offering clinicians a fair chance to identify these patients in vivo, have recently been updated. However, the diagnosis is still a challenge in everyday neurological routine. We report on three of our Creutzfeldt-Jakob patients for calling attention to the classical and the recently defined features of the disease. We conclude that based on the rapidly progressing neuropsychiatric syndrome Creutzfeldt-Jakob disease may be suspected; follow-up EEG may reveal the typical (pseudo)-periodic pattern with progressive deterioration of the background activity. In addition, diffusion-weighted brain MRI imaging (DWI) has high diagnostic value. Detection of 14-3-3 protein in the cerebrospinal fluid supports the in vivo diagnosis.

Lateral ventricle gliomas and central neurocytomas in adults diagnosis and perspectives.

Neuroimaging data of lateral ventricle gliomas and central neurocytomas diagnosed in one institution were reviewed and compared to the corresponding literature data. CT and MRI imaging characteristics of the two tumour types are rather different, both in reported cases as well as in our material. In our series ventricular ependymomas (eight cases) were mostly hyperdense with pronounced contrast uptake. Thirteen subependymomas were hypodense, mostly without enhancement, but occasionally mild or moderate enhancement was noted. Eight subependymal giant cell astrocytomas also displayed hypodense, rarely hyperdense or mixed imaging characteristics, and always showed significant degree of contrast enhancement. Nineteen central neurocytomas showed hypo- or mixed density, but mostly mild to moderate enhancement. Ependymomas and anaplastic astrocytomas and glioblastomas followed the characteristics of the similar extraventricular ones. In our series low-grade astrocytomas, WHO I-II [Louis DN, Ohgaki H, Wiestler OD, Canevee WK. WHO classification of tumours of the central nervous system. Lyon: International Agency for Research on Cancer; 2007] were hypodense without contrast uptake. Our data support those of previous studies in that MRI has been found to be superior to CT for a more precise imaging of lateral ventricle gliomas. However, it is of note that for the verification of an intraventricular tumour CT is also satisfactory. Survival data were available in 65 cases, which have confirmed a favourable outcome in most of the patients with subependymoma, subependymal giant cell astrocytoma, central neurocytomas or pilocytic astrocytoma. Survival of patients with other types of glial tumour was similar to that of patients with a similar tumour, but in an extraventricular localisation.

[Occipital sinus as a potential differential diagnosis in patients suspect of dural sinus thrombosis--a case report]. / A sinus occipitalis mint differenciál-diagnosztikai lehetóség a sinusthrombosis gyanújával vizsgált betegeknél.

INTRODUCTIONS: The diagnosis of dural sinus thrombosis despite of using modern imaging techniques still remains a difficult problem. For satisfactory interpretation of CT and MR scans it is indispensably important the knowledge of anatomical variations and possible imaging artifacts. One of the relatively rare developmental variations--giving chance for making false positive diagnosis--is the occipital sinus with hypoplasia or agenesis of transverse sinus. CASE REPORTS CONCLUSION: The incidence, the anatomical classification, the importance and the possibility of differentiation from sinus thrombosis are reviewed based on case reports of four patients.

[Hormone disorders caused by intrasellar persistent trigeminal artery]. / Hormontüneteket okozó perzisztáló trigeminalis artéria.

Persistent trigeminal artery is a relatively frequent type of intracranial arterial developmental anomalies. The diagnostic tools for demonstration previously consisted of carotid angiography, later CT and DSA and nowadays MR and MRA. The practical benefit of the diagnosis is to avoid any hazard at the operation of associated hypophysis adenomas and aneurysms and could also give a possible explanation for apparent hormonal abnormalities. The authors support the latter possibility with a case report.