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Background: Alveolar rhabdomyosarcoma (ARMS) shows a predilection for the peripheral extremities and is very rarely identified as a primary in the brain. Here, we report a case of ARMS with multiple lesions exclusively within the central nervous system (CNS). Case Description: A 20-year-old man presented to our hospital with a gradually increasing headache and disturbance of consciousness. Neuroimaging showed hydrocephalus and multiple tumor lesions, including in the brainstem and cerebellum, with uniform gadolinium enhancement on T1-weighted magnetic resonance imaging, as well as spinal cord seeding. Cerebrospinal fluid (CSF) analysis showed a slightly elevated cell count (6/µL; normal, <5/µL) and highly elevated protein (153 mg/dL). In addition, atypical cells were cytologically identified in the CSF. No other laboratory findings were abnormal. Emergency ventricular drainage was performed to control cerebral pressure, followed by a biopsy to confirm the diagnosis. Histological examination revealed a fascicular arrangement of oval cells with eosinophilic cytoplasm and tumor cells with pleomorphic nuclei and prominent nucleoli. Immunohistochemical studies showed negative results for glial fibrillary acidic protein and positive results for desmin and myogenin. In addition, molecular analysis revealed that this tumor had the H3F3A p.Lys28Met mutation and no paired box (PAX)3-forkhead box O1 (FOXO1) or PAX7-FOXO1 fusion genes. ARMS was, therefore, diagnosed. Chemotherapy and radiotherapy were subsequently initiated, but tumor growth could not be controlled, and the patient died 6 months after surgery. Conclusion: This report describes an extremely rare case of ARMS arising exclusively within the CNS.
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We revisited the anatomo-functional characteristics of the basal temporal language area (BTLA), first described by Lüders et al. (1986), using electrical cortical stimulation (ECS) in the context of Japanese language and semantic networks. We recruited 11 patients with focal epilepsy who underwent chronic subdural electrode implantation and ECS mapping with multiple language tasks for presurgical evaluation. A semiquantitative language function density map delineated the anatomo-functional characteristics of the BTLA (66 electrodes, mean 3.8 cm from the temporal tip). The ECS-induced impairment probability was higher in the following tasks, listed in a descending order: spoken-word picture matching, picture naming, Kanji word reading, paragraph reading, spoken-verbal command, and Kana word reading. The anterior fusiform gyrus (FG), adjacent anterior inferior temporal gyrus (ITG), and the anterior end where FG and ITG fuse, were characterized by stimulation-induced impairment during visual and auditory tasks requiring verbal output or not, whereas the middle FG was characterized mainly by visual input. The parahippocampal gyrus was the least impaired of the three gyri in the basal temporal area. We propose that the BTLA has a functional gradient, with the anterior part involved in amodal semantic processing and the posterior part, especially the middle FG in unimodal semantic processing.
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Mapeamento Encefálico , Idioma , Lobo Temporal , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , População do Leste Asiático , Estimulação Elétrica , Epilepsias Parciais/fisiopatologia , Epilepsias Parciais/cirurgia , Japão , Imageamento por Ressonância Magnética , Lobo Temporal/fisiologiaRESUMO
PURPOSE: While spikes and sharp waves are considered as markers of epilepsy in conventional electroencephalography, ictal direct current (DC) shifts and high-frequency oscillations (HFOs) appear to be useful biomarkers for epileptogenicity. We analyzed how ictal DC shifts and HFOs were affected by focal status epilepticus and antiseizure medications (ASMs). METHODS: A 20-year-old female patient who underwent long-term intracranial electrode implantation for epilepsy surgery presented with 72 habitual seizures and a focal status epilepticus episode lasting for 4 h. Ten, 3, and 10 consecutive habitual seizures were analyzed before the status, after the status, and after ASM (valproate) loading, respectively. RESULTS: Before and immediately after the status, ictal DC shifts remained the same in terms of the amplitude, duration, and slope of DC shifts. High-frequency oscillations also remained the same in terms of the duration, frequency, and power except for the power of the lower frequency band. After ASM loading, the duration, amplitude, and slope of the ictal DC shift were significantly attenuated. The duration, frequency, and power of the HFOs were significantly attenuated. Furthermore, the interval between the DC onset and HFO onset was significantly longer and the interval between the HFO onset and ictal DC shift peak was significantly shorter. CONCLUSIONS: The attenuation of ictal DC shifts and HFOs after ASM loading implies that astrocyte and neuronal activity may be both attenuated by ASMs. This finding may help with our understanding of the pathophysiology of epilepsy and can aid with the discovery of new approaches for epilepsy management.
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OBJECTIVE: The prognosis of glioblastoma (GBM) correlates with residual tumor volume after surgery. In fluorescence-guided surgery, 5-aminolevulinic acid (ALA) has been used to maximize resection while avoiding neurological morbidity. However, not all tumor cells, particularly glioma stem cells (GSCs), display 5-ALA-mediated protoporphyrin IX (PpIX) fluorescence (5-ALA fluorescence). The authors searched for repositioned drugs that affect mitochondrial functions and energy metabolism, identifying berberine (BBR) as a potential enhancer of 5-ALA fluorescence. In this study, they investigated whether BBR can enhance 5-ALA fluorescence in GSCs and whether BBR can be applied to clinical practice as a 5-ALA fluorescence enhancer. METHODS: The effects of BBR on 5-ALA fluorescence in glioma and GSCs were evaluated by flow cytometry (fluorescence-activated cell sorting [FACS]) analysis. As 5-ALA is metabolized for heme synthesis, the effects of BBR on mRNA expressions of 7 enzymes in the heme-synthesis pathway were analyzed. Enzymes showing significantly higher expression than control in all cells were identified and protein analysis was performed. To examine clinical availability, the detectability and cytotoxicity of BBR in tumor-transplanted mice were analyzed. RESULTS: Fluorescence microscopy revealed much more intense 5-ALA fluorescence in both GSCs and non-stem cells with 5-ALA and BBR than with 5-ALA alone. FACS showed that BBR greatly enhanced 5-ALA fluorescence compared with 5-ALA alone, and enhancement was much higher for GSCs than for glioma cells. Among the 7 enzymes examined, BBR upregulated mRNA expressions of ALA synthetase 1 (ALAS1) more highly in all cells, and activated ALAS1 through deregulating ALAS1 activity inhibited by the negative feedback of heme. An in vivo study showed that 5-ALA fluorescence with 5-ALA and BBR was significantly stronger than with 5-ALA alone, and the sensitivity and specificity of BBR-enhanced fluorescence were both 100%. In addition, BBR did not show any cytotoxicity for normal brain tissue surrounding the tumor mass. CONCLUSIONS: BBR enhanced 5-ALA-mediated PpIX fluorescence by upregulating and activating ALAS1 through deregulation of negative feedback inhibition by heme. BBR is a clinically used drug with no side effects. BBR is expected to significantly augment fluorescence-guided surgery and photodynamic therapy.
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Background: Primary intramedullary spinal cord lymphoma (PISCL) is an extremely rare condition. Early diagnosis is very difficult due to the nonspecific clinical and imaging findings. A biopsy is essential for a definitive diagnosis, but courage is required to perform the surgery. Here, we present a case of PISCL and suggest useful indicators for accurate diagnosis of this pathological entity. Case Description: A 70-year-old woman presented with subacute bilateral lower-limb paralysis, disturbance of warm and pain sensations, and vesicorectal disturbance. Magnetic resonance imaging showed a contrast-enhanced mass from C7 to Th2 and large, edematous lesions from the upper cervical to lower thoracic spinal cord. Elevated uptake of 18F-fluoro-2-deoxy-D-glucose (FDG) was identified in the enhanced regions on FDG-positron emission tomography (PET). Cerebrospinal fluid (CSF) analysis revealed highly elevated levels of ß2-microglobulin (ß2-MG). Steroid pulse therapy and therapeutic plasma exchange were performed for suspected myelitis, but symptoms did not improve. Spinal cord biopsy was, therefore, performed for treatment-resistant myelopathy. Histopathological examination revealed diffuse large B-cell lymphoma, which was diagnosed as PISCL because systemic examination showed no other findings suggestive of malignant lymphoma. Conclusion: In cases with poor response to treatment and a progressive course, PISCL should be considered, and spinal cord biopsy should be performed if PET shows increased 18F-FDG uptake and ß2-MG is elevated in CSF.
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This study aimed to evaluate the clinical usefulness of zero-echo time(ZTE)-based magnetic resonance imaging(MRI)in planning an optimal surgical approach and applying ZTE for anatomical guidance during transcranial surgery. P atients who underwent transcranial surgery and carotid endarterectomy and for whom ZTE-based MRI and magnetic resonance angiography(MRA)data were obtained, were analyzed by creating ZTE/MRA fusion images and 3D-ZTE-based MRI models. We examined whether these images and models could be substituted for computed tomography imaging during neurosurgical procedures. Furthermore, the clinical usability of the 3D-ZTE-based MRI model was evaluated by comparing it with actual surgical views. ZTE/MRA fusion images and 3D-ZTE-based MRI models clearly illustrated the cranial and intracranial morphology without radiation exposure or the use of an iodinated contrast medium. The models allowed the determination of the optimum surgical approach for cerebral aneurysms, brain tumors near the brain surface, and cervical internal carotid artery stenosis by visualizing the relationship between the lesions and adjacent bone structures. However, ZTE-based MRI did not provide useful information for surgery for skull base lesions, such as vestibular schwannoma, because bone structures of the skull base often include air components, which cause signal disturbances in MRI. ZTE sequences on MRI allowed distinct visualization of not only the bone but also the vital structures around the lesion. This technology is minimally invasive and useful for preoperative planning and guidance of the optimum approach during surgery in a subset of neurosurgical diseases.
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Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Humanos , Imageamento por Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Angiografia por Ressonância Magnética , Crânio , Encéfalo/diagnóstico por imagem , Encéfalo/cirurgiaRESUMO
BACKGROUND: Amide proton transfer (APT) imaging has been proposed as a technique to assess tumor metabolism. However, the relationship between APT imaging and other quantitative modalities including positron emission tomography (PET) has not been investigated in detail. This study aimed to evaluate the clinical usefulness of APT imaging in determining the metabolic status of malignant glioma and to compare findings with those from 11C-methionine (Met)-PET. METHODS: This research analyzed APT imaging data from 20 consecutive patients with malignant glioma treated between January 2022 and July 2023. Patients underwent tumor resection and correlations between tumor activity and intensity of APT signal were investigated. We also compared 11C-Met-PET and APT imaging for the same regions of the perifocal tumor invasion area. RESULTS: Clear, diagnostic APT images were obtained from all 20 cases. Mean APT intensity (APTmean) was significantly higher in the glioblastoma (GBM), IDH wild type group (27.2 ± 12.8%) than in other gliomas (6.0 ± 4.7%; p < 0.001). The cut-off APTmean to optimally distinguish between GBM and other malignant gliomas was 12.8%, offering 100% sensitivity and 83.3% specificity. These values for APTmean broadly matched the tumor-to-contralateral normal brain tissue ratio from 11C-Met-PET analysis (r = 0.66). The APT signal was also observed in the gadolinium non-contrast region on T1-weighted imaging, appearing to reflect the surrounding tumor-infiltrated area. CONCLUSIONS: APT imaging can be used to evaluate the area of tumor invasion, similar to 11C-Met-PET. APT imaging revealed low invasiveness in patients and was useful in preoperative planning for tumor resection, facilitating maximum tumor resection including the tumor invasive area.
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Neoplasias Encefálicas , Glioblastoma , Glioma , Humanos , Prótons , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/metabolismo , Metionina , Amidas/metabolismo , Imageamento por Ressonância Magnética/métodos , Glioma/diagnóstico por imagem , Glioma/cirurgia , Glioma/metabolismo , Tomografia por Emissão de Pósitrons/métodos , RacemetioninaRESUMO
Background: Dandy-Walker syndrome (DWS) is a well-known developmental anomaly. An occipital meningocele (OMC) is recognized as a malformation that is relatively often associated with DWS, but the association of DWS with OMC has been reported in approximately 40 cases. We present herein a rare clinical course of DWS with OMC, in which the sac was small at birth and became progressively larger. Case Description: A 5-day-old baby boy was referred to our hospital due to OMC. He was born at 33 gestational weeks due to premature rupture of the membranes. He was diagnosed as having DWS associated with OMC. The OMC was covered with skin and its maximum diameter at birth was 3 cm. Magnetic resonance imaging showed an occipital bone defect and continuity of the fourth ventricle, posterior fossa cyst, and OMC sac. The aqueduct was patent, and no hydrocephalus was found. The OMC sac increased progressively with moderate hydrocephalus and reached 7 cm at the age of 54 days when his weight was 2508 g. A cystoperitoneal shunt and repair were performed after sinus venography by contrast computed tomography (CT). At the age of 1 year and 8 months, he had moderate developmental disabilities. Conclusion: In most cases reported, the OMC was relatively small, and large and giant sizes were reported in only six cases. Almost all cases remained the same size as at birth and underwent surgical intervention as early as possible. It was possible to understand the relationship between the occipital bone defect and abnormal running of sinuses such as the superior sagittal sinus, torcular Herophili, and transverse sinus preoperatively from the CT venography (CTV) image. CTV may be an effective and important method for safely performing repair and shunt.
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High invasiveness is a characteristic of glioblastoma (GBM), making radical resection almost impossible, and thus, resulting in a tumor with inevitable recurrence. GBM recurrence may be caused by glioma stem-like cells (GSCs) that survive many kinds of therapy. GSCs with high expression levels of CD44 are highly invasive and resistant to radio-chemotherapy. CD44 is a multifunctional molecule that promotes the invasion and proliferation of tumor cells via various signaling pathways. Among these, paired pathways reciprocally activate invasion and proliferation under different hypoxic conditions. Severe hypoxia (0.5-2.5% O2) upregulates hypoxia-inducible factor (HIF)-1α, which then activates target genes, including CD44, TGF-ß, and cMET, all of which are related to tumor migration and invasion. In contrast, moderate hypoxia (2.5-5% O2) upregulates HIF-2α, which activates target genes, such as vascular endothelial growth factor (VEGF)/VEGFR2, cMYC, and cyclin D1. All these genes are related to tumor proliferation. Oxygen environments around GBM can change before and after tumor resection. Before resection, the oxygen concentration at the tumor periphery is severely hypoxic. In the reparative stage after resection, the resection cavity shows moderate hypoxia. These observations suggest that upregulated CD44 under severe hypoxia may promote the migration and invasion of tumor cells. Conversely, when tumor resection leads to moderate hypoxia, upregulated HIF-2α activates HIF-2α target genes. The phenotypic transition regulated by CD44, leading to a dichotomy between invasion and proliferation according to hypoxic conditions, may play a crucial role in GBM recurrence.
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Recurrent glioblastoma multiforme (GBM) is largely attributed to peritumoral infiltration of tumor cells. As higher CD44 expression in the tumor periphery correlates with higher risk of GBM invasion, the present study analyzed the relationship between CD44 expression and magnetic resonance imaging (MRI)-based invasiveness of GBM on a large scale. We also quantitatively evaluated GBM invasion using 5-aminolevulinic acid (5-ALA) spectroscopy to investigate the relationship between CD44 expression and tumor invasiveness as evaluated by intraoperative 5-ALA intensity. Based on MRI, GBM was classified as high-invasive type in 28 patients and low-invasive type in 22 patients. High-invasive type expressed CD44 at a significantly higher level than low-invasive type and was associated with worse survival. To quantitatively analyze GBM invasiveness, the relationship between tumor density in the peritumoral area and the spectroscopic intensity of 5-ALA was investigated. Spectroscopy showed that the 5-ALA intensity of infiltrating tumor cells correlated with tumor density as represented by the Ki-67 staining index. No significant correlation between CD44 and degree of 5-ALA-based invasiveness of GBM was found, but invasiveness of GBM as evaluated by 5-ALA matched the classification from MRI in all except one case, indicating that CD44 expression at the GBM periphery could provide a reliable biomarker for invasiveness in GBM.
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Background: The efficacy of perioperative prophylactic antiepileptic drug therapy in "seizure-naïve" patients with brain tumor, including glioblastoma (GBM), remains controversial. This study investigated whether perampanel (PER) is effective and safe for preventing perioperative onset of epileptic seizures, so-called early seizure, in patients with brain tumors. Methods: Forty-five patients underwent tumor resection through craniotomy for a primary supratentorial brain tumor at Ehime University Hospital between April 2021 and July 2022. PER was administered from the 1st to the 6th day after surgery for seizure prophylaxis. Occurrence of early seizure, hematological toxicities, and various side effects were recorded on postoperative days 7 and 14. In addition, the clinical course of these patients was compared with 42 brain tumor patients under the same treatment protocol who received levetiracetam (LEV) for seizure prophylaxis between April 2017 and October 2018. Results: In 45 patients with brain tumor, including GBM, who received PER administration, no early seizures were identified within 7 days postoperatively. No adverse drug reactions such as hematological toxicity, liver or kidney dysfunction, or exanthematous drug eruption were observed in any cases. As side effects, somnolence was reported in 14 patients (31.1%), vertigo in 3 patients (6.7%), and headache in 3 patients (6.7%). Although somnolence and vertigo were difficult to assess in the case of intraparenchymal tumors, particularly GBM, these side effects were not identified in patients with extraparenchymal tumors such as meningiomas, epidermoid cysts, and pituitary adenomas. In addition, no significant differences were identified compared to patients who received LEV. Conclusion: The efficacy and safety of PER in preventing early seizures among patients with brain tumors were retrospectively evaluated. Perioperative administration of PER to patients with brain tumors may reduce the risk of early seizures without incurring serious side effects, showing no significant differences compared to patients who received LEV.
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INTRODUCTION: Meningiomas are the most common primary central nervous system tumors. Predicting the grade and proliferative activity of meningiomas would influence therapeutic strategies. We aimed to apply the multiple parameters from preoperative diffusion tensor images for predicting meningioma grade and proliferative activity. METHODS: Nineteen patients with low-grade meningiomas and eight with high-grade meningiomas were included. For the prediction of proliferative activity, the patients were divided into two groups: Ki-67 monoclonal antibody labeling index (MIB-1 LI) < 5% (lower MIB-1 LI group; n = 18) and MIB-1 LI ≥ 5% (higher MIB-1 LI group; n = 9). Six features, diffusion-weighted imaging, fractional anisotropy, mean, axial, and radial diffusivities, and raw T2 signal with no diffusion weighting, were extracted as multiple parameters from diffusion tensor imaging. The two-level clustering approach for a self-organizing map followed by the K-means algorithm was applied to cluster a large number of input vectors with the six features. We also validated whether the diffusion tensor-based clustered image (DTcI) was helpful for predicting preoperative meningioma grade or proliferative activity. RESULTS: The sensitivity, specificity, accuracy, and area under the curve of receiver operating characteristic curves from the 16-class DTcIs for differentiating high- and low-grade meningiomas were 0.870, 0.901, 0.891, and 0.959, and those from the 10-class DTcIs for differentiating higher and lower MIB-1 LIs were 0.508, 0.770, 0.683, and 0.694, respectively. The log-ratio values of class numbers 13, 14, 15, and 16 were significantly higher in high-grade meningiomas than in low-grade meningiomas (p < .001). With regard to MIB-1 LIs, the log-ratio values of class numbers 8, 9, and 10 were higher in meningiomas with higher MIB-1 groups (p < .05). CONCLUSION: The multiple diffusion tensor imaging-based parameters from the voxel-based DTcIs can help differentiate between low- and high-grade meningiomas and between lower and higher proliferative activities.
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INTRODUCTION AND IMPORTANCE: Spindle cell oncocytoma (SCO) of the pituitary gland is very difficult to differentiate from other pituitary neoplasms and is often misdiagnosed based on imaging procedure features. We report a rare case of SCO arising from the neurohypophysis and suggest a useful diagnostic criterion for accurate diagnosis and surgical pitfalls. CASE PRESENTATION: A 53-year-old man was admitted to our hospital with slight headache and diplopia. Neuroimaging revealed pituitary tumour in the suprasellar and sellar regions with speckled gadolinium enhancement on T1-weighted magnetic resonance imaging, as a so-called blooming artefact. The enhanced anterior pituitary gland was located anteriorly. Computed tomography (CT)-scan demonstrated an isodense mass without calcification showing strong contrast enhancement with iodine contrast medium. Laboratory findings showed no abnormalities. Subtotal resection of the tumour was achieved by an endoscopic endonasal transsphenoidal approach. Histological examinations showed spindle-shaped to epithelioid tumour cells featuring eosinophilic and granular cytoplasm staining strongly for anti-mitochondrial antibody and thyroid transcription factor 1. The tumour was therefore diagnosed as SCO, belonging to tumours of the posterior pituitary. Headache and diplopia were disappeared immediately postoperatively, and follow-up at 12 months demonstrated no signs of recurrence. CLINICAL DISCUSSION: SCO of the pituitary gland is a rare tumour that originates from the neurohypophysis and is difficult to diagnose on routine neuroimaging procedure. CONCLUSION: Accurate diagnosis requires careful identification of clinical signs, neuroimaging features including contrast-enhanced CT, and analysis of combined results from morphological and immunohistochemical evaluation of tumour tissue.
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Background: The ventriculus terminalis (VT) is a cystic embryological remnant of the conus medullaris that usually regresses after birth. This structure rarely persists into adulthood and may produce neurological symptoms. We recently encountered three cases of symptomatic enlarging VT. Case Description: The three female patients were 78, 64, and 67 years old. Symptoms included pain, numbness, motor weakness, and frequent urination that gradually worsened. Magnetic resonance imaging revealed cystic dilatations of slow growing VT. These patients showed marked improvement after cyst-subarachnoid shunt using a syringo-subarachnoid shunt tube. Conclusion: Symptomatic enlarging VT is an extremely rare cause of conus medullaris syndrome and the optimal treatment strategy remains unclear. Surgical management may thus be appropriate for patients with symptomatic enlarging VT.
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PURPOSE: The purpose of this study was to evaluate the clinical usefulness of zero-echo-time (ZTE)-based magnetic resonance imaging (MRI) in planning the optimum surgical approach and applying ZTE for anatomical guidance during transcranial surgery. METHODS: Eleven of 26 patients who underwent transcranial surgery and carotid endarterectomy and in whom ZTE-based MRI and magnetic resonance angiography (MRA) data were obtained were analyzed by creating ZTE/MRA fusion images and 3D ZTE-based MRI models. We examined whether these images and models can be substituted for computed tomography imaging for neurosurgical procedures. Furthermore, the clinical usability of the 3D ZTE-based MRI models was evaluated by comparing them with actual surgical views. RESULTS: Zero-echo-time/MRA fusion images and 3D ZTE-based MRI models clearly illustrated the cranial and intracranial morphology without radiation exposure or the use of iodinated contrast medium. The models allowed determination of the optimum surgical approach to cerebral aneurysms, brain tumors near the brain surface, and cervical internal carotid artery stenosis by visualizing the relationship of lesions with adjacent bone structures. However, ZTE-based MRI did not provide useful information for surgery for skull base lesions such as vestibular schwannoma because bone structures of the skull base often include air components, which cause signal disturbance in MRI. CONCLUSIONS: Zero-echo-time sequences on MRI allowed distinct visualization of not only bone but also vital structures around the lesion. This technology has low invasiveness for patients and was useful for preoperative planning and guidance of the optimum approach during surgery in a subset of neurosurgical diseases.
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Neurocirurgia , Humanos , Tomografia Computadorizada por Raios X/métodos , Imageamento por Ressonância Magnética/métodos , Angiografia por Ressonância Magnética , Procedimentos NeurocirúrgicosRESUMO
The measurement of somatosensory evoked potentials(SEPs)is a basic clinical technique used for functional mapping and monitoring of brain and spinal cord responses during surgery. Since the potential evoked by a single stimulus is smaller compared to the surrounding electrical activity(background brain activity and/or electromagnetic artifacts), the average measurement of responses to multiple controlled stimuli across time-locked trials must be taken to determine the resultant waveform. SEPs can be analyzed in terms of polarity, latency from stimulus onset, or the amplitude from the baseline for each waveform component. The amplitude is used for monitoring purposes, while the polarity is used for mapping purposes. For example, an amplitude 50% lower than that of the control waveform may suggest the presence of significant influence on the sensory pathway, whereas a phase reversal in polarity, established by a cortical SEP distribution, usually indicates localization to the central sulcus.
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Potenciais Somatossensoriais Evocados , Medula Espinal , Humanos , Potenciais Somatossensoriais Evocados/fisiologia , Encéfalo/cirurgia , Córtex CerebralRESUMO
Background: Intravascular large B-cell lymphoma (IVLBCL) is a rare entity among large B-cell non-Hodgkin lymphomas and is often difficult to diagnose. We report the case of a patient with IVLBCL who presented with central nervous system (CNS) symptoms alone, in which positron emission tomography (PET) enabled a rapid and accurate diagnosis. Case Description: An 81-year-old woman was admitted to our hospital with a 3-month history of gradually progressive dementia and declining spontaneity. Magnetic resonance imaging revealed multiple hyperintense lesions bilaterally on diffusion-weighted imaging without enhancement on gadolinium-enhanced T1-weighted imaging. Laboratory findings showed elevated serum lactate dehydrogenase (626 U/L) and soluble interleukin-2 receptor (sIL-2R) (4692 U/mL). Cerebrospinal fluid (CSF) analysis showed slightly elevated levels of protein (166 mg/dL) and lymphocytic cells (29/µL), and ß2-microglobulin (ß2-MG) (4.6 mg/L) was highly elevated. Whole-body computed tomography revealed faint ground-glass opacities in the upper and middle lung fields and diffuse enlargement of both kidneys without lymph node swelling. 18F-fluorodeoxyglucose (FDG)-PET showed diffuse and remarkably high FDG uptake in both upper lungs and kidneys without uptake by lymph nodes, suggesting a malignant hematological disease. IVLBCL was confirmed histologically by incisional random skin biopsy from the abdomen. Chemotherapy using R-CHOP regimen in combination with intrathecal methotrexate injection was started on day 5 after admission and follow-up neuroimaging showed no signs of recurrence. Conclusion: IVLBCL presenting with CNS symptoms alone is rare and often has a poor prognosis associated with delayed diagnosis, and various evaluations (including systemic analysis) are therefore necessary for early diagnosis. FDG-PET, in addition to identification of clinical symptoms and evaluation of serum sIL-2R and CSF ß2-MG, enables rapid therapeutic intervention in IVLBCL presenting with CNS symptoms.
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INTRODUCTION AND IMPORTANCE: When treating adrenocorticotropic hormone (ACTH)-producing adenoma, accurate tumor localization is critical. We report a case of Cushing's disease in which MRI with a spoiled-gradient echo 3D T1-weighted sequence was useful in precise localization of an ACTH-producing adenoma and deciding appropriate treatment strategy. CASE PRESENTATION: A 47-year-old woman was admitted to our hospital with signs and symptoms of Cushing's disease. Laboratory findings showed hypercortisolemia and suggested Cushing's disease. However, neuroimaging on conventional pituitary MRI using a spin-echo (SE) protocol did not confirm pituitary adenoma in the sella turcica. Inferior petrosal sinus sampling suggested a higher central/peripheral ratio of ACTH after corticotropin-releasing hormone (CRH) administration on the right side. Reviewing the dynamic MRI using an SE protocol from that perspective, we vaguely identified a 5.0 mm area of gradual contrast on the right side of the pituitary gland. In addition, pituitary MRI with a spoiled-gradient echo 3D T1-weighted sequence, a 2.0 mm hypo-enhancing region was identified on the right side within the anterior pituitary gland. The tumor was resected completely removing the right pituitary gland including the tumor. The histological diagnosis was ACTH-producing pituitary adenoma. Symptoms of Cushing's disease gradually improved and endocrinological function normalized. Follow-up neuroimaging after 1 year showed no signs of recurrence. CLINICAL DISCUSSION: In the treatment of Cushing's disease, accurate detection of ACTH-producing pituitary adenoma is crucial to maximizing curative rates. However, exact confirmation of the tumor location is very difficult. CONCLUSION: MRI with a spoiled-gradient echo 3D T1-weighted sequence may facilitate accurate tumor localization and appropriate treatment strategy.
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BACKGROUND: The role of surgery in primary central nervous system lymphoma (PCNSL) is to allow pathological diagnosis from tumor biopsy. However, PCNSL is often difficult to distinguish from other tumors, particularly glioblastoma multiforme (GBM). Quantitative evaluations to facilitate differentiation between PCNSL and GBM would be useful. Here, we investigated the best examinations for exact differentiation of PCNSL from GBM among preoperative examinations, including imaging studies and tumor markers. METHODS: Various examinations were performed for 68 patients with PCNSL , including serum soluble interleukin 2 receptor, ß2-microglobulin (MG) in cerebrospinal fluid (CSF), diffusion-weighted imaging, 11C-methionine-positron emission tomography (PET), and 18F-fluorodeoxyglucose (FDG)-PET. These results were compared with findings from 28 patients with consecutive GBM who underwent the same examinations to evaluate the utility and accuracy of different investigations. RESULTS: CSF ß2-MG ≥2.0 mg/L was relatively specific for PCNSL, offering 95.0% sensitivity and 85.7% specificity. Tumor-to-contralateral normal brain tissue ratio ≥2.4 on 18F-FDG-PET was also quite specific for PCNSL, offering 83.8% sensitivity and 95.2% specificity. No other examinations displayed any significant differences in quantitative differential markers between PCNSL and GBM. CONCLUSIONS: Both ß2-MG ≥2.0 mg/dL in CSF and tumor-to-contralateral normal brain tissue ratio ≥2.4 from 18F-FDG-PET allow quantitative differentiation of PCNSL from GBM, potentially representing clinically useful indicators. These findings could lead to innovative methods for differentiating PCNSL from GBM as well as new treatment strategies for other brain tumors.
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Neoplasias Encefálicas , Neoplasias do Sistema Nervoso Central , Glioblastoma , Linfoma , Humanos , Glioblastoma/diagnóstico por imagem , Glioblastoma/cirurgia , Fluordesoxiglucose F18 , Linfoma/diagnóstico por imagem , Linfoma/cirurgia , Diagnóstico Diferencial , Tomografia Computadorizada por Raios X , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Sistema Nervoso Central , Neoplasias do Sistema Nervoso Central/diagnóstico por imagem , Neoplasias do Sistema Nervoso Central/cirurgiaRESUMO
It has been reported that the incidence of epilepsy in individuals over the age of 75 years is similar to or higher than one year, suggesting that epilepsy is an important clinical condition in older people. The aging rate in Japan, which reached 27.3% in 2017, makes this an issue of increasing concern. Diagnosis begins with the collection of medical history and knowledge of seizure semiology. In the elderly, most seizures are of extratemporal origin with various seizure semiologies. Difficulties in diagnosis lie in the influence of the autonomic nervous system and nonconvulsive status epilepticus(NCSE). It is necessary to suspect "epilepsy" for any sudden-onset episodes of atypical symptoms and unusual behaviors. NCSE can be diagnosed by recording EEG for more than 6 hours. The narrow therapeutic window and pharmacokinetics complicated by aging make treatment much more difficult with anti-seizure medication(ASM). Comorbidities and drug interactions should also be considered. Stroke is the most common etiology of geriatric epilepsy. Despite several clinical reports on the risk factors of post-stroke epilepsy(PSE)on the preventive administration of enzyme-inducing ASM, a treatment protocol has not yet been established. Prophylactic administration is not recommended; however, ASM is often administered in the acute phase. Therefore, some guidelines for non-enzyme-inducing ASM are required for efficient control of PSE.