Pituitary apoplexy: Surgical or conservative management? / Agyalapimirigy-apoplexia: Sebészi vagy konzervatív kezelés?

Összefoglaló. Az agyalapimirigy-apoplexia ritka klinikai kórkép, mely hirtelen kialakult bevérzés vagy infarktus következményeként jelenik meg. A hypophysisadenomás betegek 2-12%-ában fordul elo, a leggyakrabban funkcionálisan inaktív daganatokban, de jelentkezhet gyógyszeresen kezelt adenomákban is. Klinikai képe hirtelen kialakuló heves fejfájás, mely látászavarral vagy kettos látással társulhat, de meningealis izgalmi jel, a tudati szint romlása is elofordulhat. A bevérzés miatt kialakult kortikotropinhiány kezelés nélkül mellékvese-elégtelenséghez vezet. A mágneses rezonancia a komputertomográfhoz képest jobban kimutatja az adenoma bevérzését vagy akár infarktusát. Retrospektív tanulmányok a korábbi, azonnali idegsebészeti beavatkozás helyett a konzervatív kezelés létjogosultságát emelik ki. Orv Hetil. 2021; 162(38): 1520-1525. Summary. Pituitary apoplexy is a rare clinical syndrome secondary to haemorrhage or infarction of pituitary adenoma. The prevalence is 2-12% of pituitary adenoma patients especially in nonfunctioning tumours but may be found in medically treated adenomas as well. Its clinical picture is sudden onset of headache with visual disturbances and/or ocular palsy. Meningeal signs and altered consciousness can occur. Corticotropin deficiency if untreated can lead to adrenal insufficiency. Compared to computed tomography, magnetic resonance imaging better demonstrates the haemorrhage or even infarction of pituitary adenoma. Retrospective studies emphasize the wait-and-see management instead of the formerly considered urgent neurosurgical intervention. Orv Hetil. 2021; 162(38): 1520-1525.

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.

Cerebral blood volume mapping with ferumoxytol in dynamic susceptibility contrast perfusion MRI: Comparison to standard of care.

BACKGROUND: Cerebral blood volume (CBV) mapping with a dynamic susceptibility contrast (DSC) perfusion technique has become a clinical tool in diagnosing and follow-up of brain tumors. Ferumoxytol, a long-circulating iron oxide nanoparticle, has been tested for CBV mapping, but the optimal dose has not been established. PURPOSE: To compare ferumoxytol DSC of two different doses to standard of care gadoteridol by analyzing time-intensity curves and CBV maps in normal-appearing brain regions. STUDY TYPE: Retrospective. SUBJECTS: Fifty-four patients with various brain disorders. FIELD STRENGTH/SEQUENCE: 3T MRI. DSC-MRI was performed with 0.1 mmol/kg gadoteridol and 1 day later with ferumoxytol in doses of 1 or 2 mg/kg. ASSESSMENT: Signal changes during first pass, relative CBV (rCBV) in normal-appearing thalamus, putamen, and globus pallidus, and contrast-to-noise ratio (CNR) of the CBV maps were compared between gadoteridol and various doses of ferumoxytol using an automated method. To subjectively assess the quality of the CBV maps, two blinded readers also assessed visual conspicuity of the putamen. STATISTICAL TESTS: Linear mixed effect model was used for statistical comparison. RESULTS: Compared to gadoteridol, 1 mg/kg ferumoxytol showed no difference in CNR (P = 0.6505), peak ΔR2*, and rCBV in the putamen (P = 0.2669, 0.0871) or in the thalamus (P = 0.517, 0.9787); 2 mg/kg ferumoxytol increased peak ΔR2* as well as the CNR (P < 0.0001), but also mildly increased rCBV in putamen and globus pallidus (P = 0.0005, 0.0012). Signal intensities during first pass remained highly above the noise level, with overlapping of 95% confidence intervals with noise only in 3 out of 162 tested regions. Compared to gadoteridol, the visual image quality showed mild improvement with 1 mg/kg (P = 0.02) and marked improvement with 2 mg/kg ferumoxytol (P < 0.0001). DATA CONCLUSION: 1 mg/kg ferumoxytol provides similar imaging results to standard gadoteridol for DSC-MRI, and 2 mg/kg has a benefit of increased CNR, but may also result in mildly increased rCBV values. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 1 J. MAGN. RESON. IMAGING 2018;48:441-448.

Quantitative comparison of delayed ferumoxytol T1 enhancement with immediate gadoteridol enhancement in high grade gliomas.

PURPOSE: Delayed ferumoxytol enhancement on T1 -weighted images appears visually similar to gadoteridol enhancement. The purpose of this study was to quantitatively compare ferumoxytol T1 enhancement to gadoteridol enhancement with an objective, semi-automated method. METHODS: 206 sets of post-gadoteridol and 24 h post-ferumoxytol T1 -weighted scans from 58 high grade glioma patients were analyzed (9 pre-chemoradiation, 111 < 90 days post-chemoradiation, 21 > 90 days post-chemoradiation, 65 post-bevacizumab scans). Enhancement volumes and signal intensities normalized to normal appearing tissue proximal to enhancement were calculated with a semi-automated method. Enhancement cube root volumes (D) and signal intensities (SI) were compared between the 2 contrast agents, and relative difference of D and SI were compared in different treatment groups with multivariate analysis. Within patient differences in D and SI before and after treatment with bevacizumab or steroid were assessed in 26 patients in each treatment group. RESULTS: When compared to gadoteridol, ferumoxytol D was 13.83% smaller and SI was 7.24% lower (P < 0.0001). The relative differences in D and SI between the 2 contrast agents were not significantly different between treatment groups (P > 0.05). Relative difference in D and SI did not change significantly in response to bevacizumab (P = 0.5234 and P = 0.2442, respectively) or to steroid (P = 0.3774, P = 0.0741) in the within patient comparison. CONCLUSION: The correlation between the 2 contrast agents' enhancement size and signal intensity and their similar behavior in response to therapy suggest that ferumoxytol can be used for revealing enhancement in high grade glioma patients. Magn Reson Med 80:224-230, 2018. © 2017 International Society for Magnetic Resonance in Medicine.

Significance of Primary Tumor Location and Histology for Brain Metastasis Development and Peritumoral Brain Edema in Lung Cancer.

BACKGROUND: Brain metastasis of lung cancer adversely affects overall survival (OS) and quality of life, while peritumoral brain edema is responsible for life-threatening complications. METHODS: We retrospectively analyzed the clinicopathological and cerebral radiological data of 575 consecutive lung cancer patients with brain metastases. RESULTS: In adenocarcinoma and squamous cell carcinoma, peritumoral brain edema was more pronounced than in small-cell lung cancer (p < 0.001 and p < 0.001, respectively). There was a positive correlation between the size of metastasis and the thickness of peritumoral brain edema (p < 0.001). It was thicker in supratentorial tumors (p = 0.019), in younger patients (≤50 years) (p = 0.042), and in females (p = 0.016). The time to development of brain metastasis was shorter in central than in peripheral lung cancer (5.3 vs. 9.0 months, p = 0.035). Early brain metastasis was characteristic for adenocarcinomas. A total of 135 patients had brain only metastases (N0 disease) characterized by peripheral lung cancer predominance (p < 0.001) and a longer time to development of brain metastasis (9.2 vs. 4.4 months, p < 0.001). OS was longer in the brain only subgroup than in patients with N1-3 diseases (p < 0.001). CONCLUSIONS: The clinicopathological characteristics of lung cancer are related to the development and radiographic features of brain metastases. Our results might be helpful in selecting patients who might benefit from prophylactic cranial irradiation.

[Novelties and new possibilities in radiological diagnostics of brain tumors]. / Újdonságok és új lehetoségek az agydaganatok radiológiai diagnosztikájában.

Modern MR imaging with advanced techniques such as diffusion, perfusion and functional imaging as well as spectroscopy have improved the characterization of brain tumors. Unlike conventional imaging providing mainly anatomical or structural information, these advanced applications give insight into the microstructure and physiology of brain tumors. Several biomarkers are available which correlate with tumor cellularity, microstructure, vascularity and metabolism. These techniques not only aid in the imaging diagnosis and treatment planning of brain tumors, but they also play a role in clinical management and monitoring treatment effect.

Anti glutamate-decarboxylase antibodies: a liaison between localisation related epilepsy, stiff-person syndrome and type-1 diabetes mellitus.

We present two patients with partial epilepsy, type-1 diabetes and stiff person syndrome associated with high serum auto-antibody levels to glutamate-decarboxylase (anti-GAD). Both patients were or have suffered from additional autoimmune conditions. The presence of stiff person syndrome and elevated anti-GAD levels have to make clinicians look for additional autoimmune conditions including type-1 diabetes. On the other hand, the co-morbidity of partial epilepsy with autoimmune conditions in patients with elevated serum anti-GAD suggests an autoimmune mechanism of partial epilepsy in these cases.

[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.

Imaging changes and cognitive outcome in primary CNS lymphoma after enhanced chemotherapy delivery.

BACKGROUND AND PURPOSE: In children, MR imaging abnormalities consistent with leukoencephalopathy after treatment for hematologic malignancy do not correlate with neurologic dysfunction and are often overinterpreted with regard to clinical significance. We hypothesized that this would also be true in primary CNS lymphoma (PCNSL) patients who attained a complete response (CR) after treatment with chemotherapy and osmotic blood-brain barrier disruption (BBBD). We hypothesized that cognitive function loss measured after tissue diagnosis but before BBBD-enhanced chemotherapy could be correlated with brain changes visualized by imaging, whereas a correlation would not be present after therapy if the patient attained a complete tumor response, analogous to the findings in children. METHODS: Sixteen primary CNS lymphoma patients were followed after CR (no enhancing tumor) by using a methotrexate-based regimen. Neuropsychological (NP) cognitive testing and MR imaging or CT (when MR imaging was not available) were performed before treatment and at completion of the 12-month treatment for each patient. Thereafter, the same studies were available for nine of these 16 CR patients, who were followed for a median of 55 months. Zone I was defined as enhancing tumor, and zone II as surrounding abnormal MR T2 signal intensity or low-attenuation CT. The cognitive scores were converted to Z scores and the MR T2 signal intensity or CT low-attenuated changes were converted to a summary zone II abnormality score. RESULTS: A significant association between neurocognitive data and zone II abnormality was found at baseline after tissue diagnosis but before chemotherapy (r = -.55; P < .028), but no correlation existed at end of treatment. Imaging studies showed that seven patients developed a new T2 or low-attenuation abnormality by the end of treatment, whereas 15 patients showed a decrease, stable appearance, or complete resolution of their baseline zone II abnormality by end of treatment. Although cognitive loss compared with age-matched control subjects was common before starting therapy, by the end of treatment all patients' cognitive function improved significantly (P < .005). CONCLUSION: The current data suggest that neither enhanced chemotherapy delivery nor changes in MR imaging T2 signal intensity or CT low attenuation, in PCNSL patients who attained a CR, were associated with a decrease in cognitive function.

Comparison of two superparamagnetic viral-sized iron oxide particles ferumoxides and ferumoxtran-10 with a gadolinium chelate in imaging intracranial tumors.

BACKGROUND AND PURPOSE: Ultrasmall superparamagnetic iron oxide particles result in shortening of T1 and T2 relaxation time constants and can be used as MR contrast agents. We tested four hypotheses by evaluating MR images of intracranial tumors after infusion of two iron oxide agents in comparison with a gadolinium chelate: 1) Ferumoxtran in contrast to ferumoxides can be used as an intravenous MR contrast agent in intracranial tumors; 2) ferumoxtran enhancement, albeit delayed, is similar to gadolinium enhancement; 3) ferumoxtran-enhanced MR images in contrast to gadolinium-enhanced MR images may be compared with histologic specimens showing the cellular location of iron oxide particles; 4) ferumoxtran can serve as a model for viral vector delivery. METHODS: In 20 patients, ferumoxides and ferumoxtran were intravenously administered at recommended clinical doses. MR imaging was performed 30 minutes and 4 hours after ferumoxides infusion (n = 3), whereas ferumoxtran-enhanced MR imaging (n = 17) was performed 6 and 24 hours after infusion in the first five patients and 24 hours after infusion in the remaining 12. MR sequences were spin-echo (SE) T1-weighted, fast SE T2- and proton density-weighted, gradient-recalled-echo T2*-weighted, and, in four cases, echo-planar T2-weighted sequences. Representative regions of interest were chosen on pre- and postcontrast images to compare each sequence and signal intensity. RESULTS: Despite some degree of gadolinium enhancement in all tumors, no significant T1 or T2 signal intensity changes were seen after ferumoxides administration at either examination time. Fifteen of 17 patients given ferumoxtrans had T1 and/or T2 shortening consistent with iron penetration into tumor. Histologic examination revealed minimal iron staining of the tumor with strong staining at the periphery of the tumors. CONCLUSION: 1) Ferumoxtran can be used as an intravenous MR contrast agent in intracranial tumors, mostly malignant tumors. 2) Enhancement with ferumoxtran is comparable to but more variable than that with the gadolinium chelate. 3) Histologic examination showed a distribution of ferumoxtran particles similar to that on MR images, but at histology the cellular uptake was primarily by parenchymal cells at the tumor margin. 4) Ferumoxtran may be used as a model for viral vector delivery in malignant brain tumors.