Surgical therapy in advanced sinonasal carcinomas - retrospective study.

BACKGROUND AND AIM: Sinonasal tumors are a rare and heterogeneous group of malignant tumors with different histopathological characteristics and clinical presentation. These tumors are usually treated through surgery. The aim of this study is to present our results of surgical therapy in patients with an advanced sinonasal tumor. METHODS: This retrospective study included patients with an advanced sinonasal tumor who were surgically treated. The surgical technique combined both a frontal transbasal approach together with an endoscopic endonasal approach. The parameters used for evaluation were the histological type of tumor, the radicality of resection (complete vs. incomplete), the frequency of recurrence, the surgical and postoperative complications, the type of subsequent oncological therapy and the overall survival. RESULTS: The group consisted of ten patients seven were men and three were women. Complete resection (defined as R0) was achieved in 8 (80%) of the cases, subcomplete resection was achieved in 2 (20%) of the cases. The overall survival period was 28.7 months (95% confidence interval 15.9-41.6). CONCLUSION: The combination of the frontal transbasal approach with the endoscopic endonasal approach is a suitable surgical strategy that enables easier achievement of complete tumor resection, reconstruction of the anterior skull base and reduces the need for extensive surgical approaches.

Role of risk factors, scoring systems, and prognostic models in predicting the functional outcome in meningioma surgery: multicentric study of 552 skull base meningiomas.

Despite the importance of functional outcome, only a few scoring systems exist to predict neurologic outcome in meningioma surgery. Therefore, our study aims to identify preoperative risk factors and develop the receiver operating characteristics (ROC) models estimating the risk of a new postoperative neurologic deficit and a decrease in Karnofsky performance status (KPS). A multicentric study was conducted in a cohort of 552 consecutive patients with skull base meningiomas who underwent surgical resection from 2014 to 2019. Data were gathered from clinical, surgical, and pathology records as well as radiological diagnostics. The preoperative predictive factors of functional outcome (neurologic deficit, decrease in KPS) were analyzed in univariate and multivariate stepwise selection analyses. Permanent neurologic deficits were present in 73 (13.2%) patients and a postoperative decrease in KPS in 84 (15.2%). Surgery-related mortality was 1.3%. A ROC model was developed to estimate the probability of a new neurologic deficit (area 0.74; SE 0.0284; 95% Wald confidence limits (0.69; 0.80)) based on meningioma location and diameter. Consequently, a ROC model was developed to predict the probability of a postoperative decrease in KPS (area 0.80; SE 0.0289; 95% Wald confidence limits (0.74; 0.85)) based on the patient's age, meningioma location, diameter, presence of hyperostosis, and dural tail. To ensure an evidence-based therapeutic approach, treatment should be founded on known risk factors, scoring systems, and predictive models. We propose ROC models predicting the functional outcome of skull base meningioma resection based on the age of the patient, meningioma size, and location and the presence of hyperostosis and dural tail.

Periradicular corticosteroid infiltration for radicular pain - comparison of Diprophos and Depomedrone and ozone effects.

OBJECTIVES: To determine the treatment effect of corticosteroids in periradicular therapy (PRT) for radicular pain and to compare different types of corticosteroids and ozone. We also examined the effect in different indication groups for periradicular therapy for each type of treatment agent. BACKGROUND: Various studies have examined the therapeutic value of periradicular infiltration using treatment agents consisting of local anesthetic and corticosteroids or ozone application for radicular pain. This is the first study to compare different types of corticosteroids and ozone. METHODS: Eligible patients with radicular pain who failed conservative management were divided into five indication groups and prospectively followed to assess the PRT effect of corticosteroids or ozone application. PRT was performed under computer tomography (CT) monitoring. A set of three PRT applications in three weeks was applied and the outcome was evaluated using a visual analogue score for back and leg pain. The in-group and between-group treatment effect was tested using the Wilcoxon signed-rank test and the Kruskal-Wallis H-test with Dunn's post-hoc tests, respectively. The dependency between treatment effectiveness and indication for each group was tested using the Kruskal-Wallis H-test and Dunn's post-hoc tests. RESULTS: We prospectively followed 150 patients, randomized into three groups of 50 patients each. The follow-up rate was 100%. All three treatment agents showed a statistically significant treatment effect (P<0.001). The statistically significant effect was higher in betamethasone (Diprophos) versus methylprednisolone (Depomedrone) (P=0.019) and Diprophos versus ozone (P<0.001). Diprophos also showed the highest decrease of VAS after therapy versus VAS prior to therapy (median decrease = 4) compared to Depomedrone and ozone (median decrease = 3 and 2, respectively). The statistically significant outcome was better with the indication of spondylolisthesis and disc herniation (P=0.019) indication for the Diprophos group and between spinal stenosis and spondylolisthesis (P=0.022) and spondylolisthesis and disc herniation (P=0.016) for the ozone group. CONCLUSION: Clinical improvement occurred in all three groups but Diprophos showed the statistically best treatment effect compared to Depomedrone and ozone. Disc herniation resulting in radicular pain had a statistically significant better effect in comparison with spondylolisthesis in the Diprophos and ozone groups, but the ozone group showed heterogeneity depending on treatment effect and indication.

IDH1/2 Mutations in Patients With Diffuse Gliomas: A Single Centre Retrospective Massively Parallel Sequencing Analysis.

Patients below 55 years were genetically studied because the prevalence of isocitrate dehydrogenase 1 (IDH1) decreases in older patients and on grounds of cost-effectiveness, as suggested by the World Health Organization (WHO) in 2016. The aim of our study was to use novel massively parallel sequencing (MPS) approaches to examine rare variants of IDH1/2 in Czech diffuse astrocytic and oligodendroglial tumors (gliomas) patients below 55 years of age who had been immunohistochemically (IHC) diagnosed as IDH1 R132H negative. The IHC IDH1 status (wild type or mutant) of 275 tissue samples was analyzed using antibodies against the IDH1 R132H protein. Sixty-three samples of 55 years old patients with IHC IDH1 WT status were genotyped using two different MPS technologies to detect rare IDH1 and IDH2 variants. The tiered IHC (60 positive) and molecular (10 positive) approach thus revealed that 70 of the 275 samples (25%) bore IDH1/IDH2 mutations. The combined molecular and IHC approach thus revealed that 70 of the 275 samples (25%) considered in the study bore IDH1/IDH2 mutations. IHC detection of the IDH1 R132H variant should be routinely complemented with MPS to detect rare IDH1/2 variants in glioma patients below 55 years of age with negative IHC result of IDH R132H variant.

The Influence of Gene Aberrations on Survival in Resected IDH Wildtype Glioblastoma Patients: A Single-Institution Study.

This prospective population-based study on a group of 132 resected IDH-wildtype (IDH-wt) glioblastoma (GBM) patients assesses the prognostic and predictive value of selected genetic biomarkers and clinical factors for GBM as well as the dependence of these values on the applied therapeutic modalities. The patients were treated in our hospital between June 2006 and June 2015. Clinical data and tumor samples were analyzed to determine the frequencies of TP53, MDM2, EGFR, RB1, BCR, and CCND1 gene aberrations and the duplication/deletion statuses of the 9p21.3, 1p36.3, 19q13.32, and 10p11.1 chromosome regions. Cut-off values distinguishing low (LCN) and high (HCN) copy number status for each marker were defined. Additionally, MGMT promoter methylation and IDH1/2 mutation status were investigated retrospectively. Young age, female gender, Karnofsky scores (KS) above 80, chemoradiotherapy, TP53 HCN, and CCND1 HCN were identified as positive prognostic factors, and smoking was identified as a negative prognostic factor. Cox proportional regression models of the chemoradiotherapy patient group revealed TP53 HCN and CCND1 HCN to be positive prognostic factors for both progression-free survival and overall survival. These results confirmed the influence of key clinical factors (age, KS, adjuvant oncotherapy, and smoking) on survival in GBM IDH-wt patients and demonstrated the prognostic and/or predictive importance of CCND1, MDM2, and 22q12.2 aberrations.

Effect of angiotensin converting enzyme inhibitors on the development of chronic subdural haematoma.

AIMS: Angiotensin converting enzyme inhibitors (ACEI) have been recently discussed in connection with the medical treatment of chronic subdural haematoma (CSDH). They may improve the treatment results. The objective of our study was to evaluate the impact of ACEI on the development of CSDH. The first question was to assess the impact of ACEI on postoperative CSDH healing. The second was to assess the impact of ACEI on the development of CSDH as such. PATIENTS AND METHODS: The study recruited patients treated surgically for CSDH at our department in the 2013-2018 period. Based on medical records, we retrospectively evaluated the clinical condition of the patients, their history (mainly pharmacological - the use of ACEI) and the course of treatment focussing on the reoccurrence of disease necessitating further therapeutic interventions. For the purpose of evaluating the impact of ACEI on postoperative CSDH healing, the patients were divided into two groups: those using ACEI and those without this medication. The results were compared. We also compared the prevalence of ACEI use in patients with CSDH with the prevalence of ACEI in the comparable population. The difference of the rates allowed us to evaluate the impact of ACEI on the development of CSDH itself. RESULTS: Of the 217 patients after surgery for CSDH, 79 continued the use of ACEI; the remaining 138 patients did not use this medication. Patients using ACEI after the surgery experienced a recurrence in 24 (30.4%) cases; patients without ACEI in 37 (26.8%) cases. A negligibly higher number of recurrences was recorded in patients with postoperative use of ACEI, but this difference was not statistically significant (P=0.574). Of a total of 230 patients who underwent surgery for CSDH, 81 were using ACEI chronically (35.2%). In the control group of 100 patients, 38 (38.0%) patients used ACEI. The difference was not statistically significant (P=0.629), so it is not possible to assume that ACEIs influence the development of CSDH as such. CONCLUSION: The initial high hopes for a positive ACEI effect on the healing of CSDH are now waived after the publication of several recent studies. According to our present knowledge, the development of CSDH does not appear to be influenced by ACEI use.

Distal Aneurysms of Cerebellar Arteries-Case Series.

(1) Background: Distal aneurysms of cerebellar arteries are very rare. The authors report their case series of distal aneurysms of the cerebellar arteries solved successfully by microsurgery or by endovascular treatment (Table 1) (2) Materials and Methods: Between January 2010 and March 2020, 346 aneurysms were treated in our institution. Eleven aneurysms in seven patients were located on distal cerebellar arteries and, in three patients, the aneurysms were combined with arteriovenous malformations. There were four women and three men, ranging from 50 to 72 years of age. Five patients presented with different grades of subarachnoid hemorrhage or intraventricular bleeding, and two patients were diagnosed because of headache. Aneurysm location was the posterior inferior cerebellar artery in six cases, the superior cerebellar artery in three cases, and the anterior inferior cerebellar artery in 2 cases. One patient had three aneurysms, and two patients had two aneurysms. (3) Results: Nine aneurysms were treated by microsurgery trapping or clipping and, in two patients, the associated arteriovenous malformation (AVM) was resected. Two aneurysms were treated by endovascular coiling, and one associated AVM was successfully embolized. Clinical follow-up was a mean of 11.5 months (range, 3-45 months). (4) Conclusion: The authors present their experience with the treatment of 11 peripheral aneurysms on distal branches of the cerebellar circulation in seven patients which were excluded from circulation by microsurgery or endovascular treatment. In three patients, the associated AVM was treated (two with microsurgery, one with embolization).

Identification of Meningioma Patients at High Risk of Tumor Recurrence Using MicroRNA Profiling.

BACKGROUND: Meningioma growth rates are highly variable, even within benign subgroups, with some remaining stable, whereas others grow rapidly. OBJECTIVE: To identify molecular-genetic markers for more accurate prediction of meningioma recurrence and better-targeted therapy. METHODS: Microarrays identified microRNA (miRNA) expression in primary and recurrent meningiomas of all World Health Organization (WHO) grades. Those found to be deregulated were further validated by quantitative real-time polymerase chain reaction in a cohort of 172 patients. Statistical analysis of the resulting dataset revealed predictors of meningioma recurrence. RESULTS: Adjusted and nonadjusted models of time to relapse identified the most significant prognosticators to be miR-15a-5p, miR-146a-5p, and miR-331-3p. The final validation phase proved the crucial significance of miR-146a-5p and miR-331-3p, and clinical factors such as type of resection (total or partial) and WHO grade in some selected models. Following stepwise selection in a multivariate model on an expanded cohort, the most predictive model was identified to be that which included lower miR-331-3p expression (hazard ratio [HR] 1.44; P < .001) and partial tumor resection (HR 3.90; P < .001). Moreover, in the subgroup of total resections, both miRNAs remained prognosticators in univariate models adjusted to the clinical factors. CONCLUSION: The proposed models might enable more accurate prediction of time to meningioma recurrence and thus determine optimal postoperative management. Moreover, combining this model with current knowledge of molecular processes underpinning recurrence could permit the identification of distinct meningioma subtypes and enable better-targeted therapies.

Ventriculoperitoneal shunt in treating of idiopathic normal pressure hydrocephalus-single-center study.

OBJECT: Idiopathic normal pressure hydrocephalus (iNPH) is the only variant of dementia disorders possibly treatable by neurosurgical intervention. iNPH is a neurodegenerative condition clinically characterized by gait ataxia, urinary incontinence, and memory disturbance. We present one of the largest single-center studies, which was designed to prove efficacy of our low-pressure setting of gravitational valve at all three symptoms of iNPH and to find statistically significant cut-off time for best clinical improvement according to the duration of symptoms. METHODS: Sixty-one consecutive patients (mean age 74.9 ± 5.3) with iNPH were prospectively observed from the time of surgery with minimal 6 months follow-up. All patients underwent implantation of the same type of gravitational valve with the same setting-pro GAV with low opening pressure at 5 cm H2O-and were operated by the same team of 2 neurosurgeons. We statistically evaluated gait disturbance, psychological changes, and incontinence preoperatively and at 6 months after surgery and timing of the surgery according to the duration of symptoms and to the age. RESULTS: Paired t test showed a statistically significant increase in MMSE, a statistically significant decrease in 10-m walk test and 360 deg. rotation test (p < 0.0001). The correlation among the change of the MMSE, the walk test, and the rotation test, and the age and time of symptoms' duration was verified by Pearson's correlation coefficient. Pearson's correlation coefficient showed a medium strong correlation between the change of MMSE and the time of symptoms (r = - 0.580; p < 0.0001) and between the change of the number of steps and the time of symptoms (r = 0.517, p < 0.0001). There was a statistically significant weak (poor) correlation between the change of the walk test and the time of symptoms (r = 0.351, p = 0.006). All 3 ROC tests confirmed optimal cut-off for the best improvement of symptoms as 9.5 months of the symptom duration. CONCLUSIONS: We proved statistical significant optimal cut-off for the best improvement of the symptoms as 9.5 months of the symptom duration. This study also confirmed successful treatment of iNPH with VP shunting using low pressure setting of gravitational valve with overall improvement in 75% and low over drainage complications in 5% We proved statistically significant increase in MMSE, decrease in 10 m walk test and number of steps test, p < 0.0001.

Mastoid foramen, mastoid emissary vein and clinical implications in neurosurgery.

BACKGROUND: Mastoid emissary vein is especially important from the neurosurgical point of view, because it is located in variable number in the area of the occipitomastoid suture and it can become a source of significant bleeding in surgical approaches through the mastoid process, especially in retrosigmoid craniotomy, which is used for approaches to pathologies localized in the cerebellopontine angle. Ideal imaging method for diagnosis of these neglected structures when planning a surgical approach is high-resolution computed tomography. The aim of this work was to provide detailed information about this issue. METHODS: We studied a group of 295 skulls obtained from collections of five anatomy departments and the National Museum. Both quantitative and qualitative parameters of the mastoid foramen were evaluated depending on side of appearance and gender. Individual distances of the mastoid foramen from clearly defined surface landmarks (asterion, apex of mastoid process, foramen magnum) and other anatomical structures closely related to this issue (width of groove for sigmoid sinus, diameters of internal and external openings of mastoid foramen) were statistically processed. RESULTS: The most frequently represented type of the mastoid foramen is type II by Louis (41.2%). The differences between right and left sides were not statistically significant. In men there was a higher number of openings on the right side and in qualitative parameters the type III and IV predominated, whereas in women the types I and II were more frequent. In men, greater distances from the mastoid foramen were observed when evaluating qualitative parameters for defined surface landmarks. Mean size of the external opening diameter was 1.3 mm; however, several openings measured up to 7 mm. CONCLUSIONS: Despite excellent knowledge of anatomy, however, good pre-operative examination using imaging methods and mastering of microsurgical techniques create the base for successful treatment of pathological structures in these anatomically complex areas.

Longitudinal brain activation changes related to electrophysiological findings in patients with cervical spondylotic myelopathy before and after spinal cord decompression: an fMRI study.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is the most common cause of spinal cord dysfunction, potentially leading to severe disability. Abnormal cervical spine magnetic resonance imaging (MRI) and motor evoked potentials (MEPs) are independent predictors of disease progression. Abnormal MRI is accompanied by various activation changes in functional brain MRI (fMRI), whereas preoperative and postoperative fMRI adaptations associated with abnormal preoperative MEP remain unknown. METHODS: Twenty patients (9 males, average age 56.6) with evidence of spinal cord compression on MRI and clinical signs of mild CSM were included. Participants were classified according to their preoperative MEP and underwent three brain fMRI examinations: before surgery, 6, and 12 months after surgery while performing repeated extension-flexion of each wrist. RESULTS: Functional MRI activation was compared between two subsets of patients, with normal and clearly abnormal MEP (right wrist: 8 vs. 8; left wrist: 7 vs. 9). At baseline, abnormal MEPs were associated with hyperactivation in the cerebellum. At the first follow-up, further hyperactivations emerged in the contralateral sensorimotor cortices and persisted for 1 year. In normal baseline MEP, activation mostly decreased in the ipsilateral sensorimotor cortex postoperatively. The ipsilateral sensorimotor activation after 1-year follow-up correlated with baseline MEP. CONCLUSIONS: Abnormal corticospinal MEP findings in cervical spondylotic myelopathy were associated with differences in brain activation, which further increased after spinal cord decompression and did not resolve within 12-month follow-up. In summary, surgery may come too late for those patients with abnormal MEP to recover completely despite their mild clinical signs and symptoms.

Prospective study of hypothalamo-hypophyseal dysfunction in children and adolescents following traumatic brain injury.

BACKGROUND AND AIMS: Retrospective studies of TBI have found a neuroendocrine dysfunction following traumatic brain injury in 23 to 60% of adults and 15 to 21% of children. Our aims were to determine the prevalence of hypothalamo-hypophyseal dysfunction in children following brain injury, assess its relationship to the type of injury and the course of the acute post-traumatic phase. PATIENTS AND METHODS: Body development (growth, pubertal development, and skeletal maturity) were evaluated in 58 patients (21 girls) after a brain injury rated 3 to 12 on the Glasgow Coma Scale (GCS). The patients underwent standard endocrine tests - TSH, fT4, IGF-1, PRL, morning cortisol, FSH, LH, and testosterone in boys and estradiol in girls - in the early post-traumatic period (2 to 14 days; T0) and at 3, 6, and 12 months after the injury (T3, T6, and T12). Dynamic tests were carried out in patients with abnormalities in their clinical examination and/or laboratory results. An MRI was performed on all patients at T12. RESULTS: The median age at the time of injury was 11.3 (0.5 to 18.7) years. Of the 58 patients, 23 had GCS < 8, corresponding to severe brain injury. At T0, diabetes insipidus (DI) was diagnosed in 12 patients, and the syndrome of inappropriate antidiuretic hormone secretion (SIADH) was found in 4 patients. Frequent hormonal changes simulated central hypothyroidism (in 45% of patients) and hypogonadotropic hypogonadism (in 25% of adolescents who were already pubertal at the time of injury > Tanner II). Examination at T3 (n = 58) confirmed a combined pituitary hormone deficiency in two boys and DI in another one. At T6 (n = 49), hormonal dysfunctions were diagnosed in two boys (precocious puberty and growth hormone deficiency). At T12 (n = 39), a new endocrine dysfunction was diagnosed in five patients (growth hormone deficiency in two, hypogonadotropic hypogonadism in two, and in one patient, already diagnosed with a growth hormone deficiency, central hypothyroidism, as well). Brain MRI revealed an empty sella in two patients with growth hormone deficiency. Patients with GCS < 8 had more symptoms of SIADH or DI in the early post-traumatic period 11/23 vs. patients with GCS of 8 to 13 (4/35), and more frequent hormonal disorder (6/23) than individuals with moderate trauma (3/35), P = 0.0135. The incidence of endocrine dysfunction at T0 significantly correlated with the severity of injury (P = 0.05), but it was not an indicator for the development of a late hormonal disorder. CONCLUSION: Within a year after injury, a hormonal disorder was found in 17.6% of the patients. Neuroendocrine dysfunction as a late consequence of craniocerebral trauma in children and adolescents was less frequent than in adults. Risk factors for its development are the gravity of the injury, brain scan pathology, and possibly the development of DI, SIADH, or CSWS in the acute post-traumatic phase.

Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report.

Ectopic cilia are extremely rare congenital anomalies in which eyelash follicles appear in an abnormal place on the eyelid, most typically on the lateral quadrant of the anterior surface of the upper eyelid. In the majority of cases, simple surgical excision of ectopic cilia is indicated because of its cosmetic aspect. There is usually no associated medical co-morbidity with this anomaly. The authors report an unusual case of ectopic cilia associated with an orbital dermoid cyst and sinus tract. A 3-year-old boy was initially diagnosed with ectopic cilia on the left upper eyelid. There was no history of inflammation or swelling of the eyelid. An ophthalmological examination revealed only 1 mm of ptosis; no proptosis, inferior displacement, or palpable orbital mass was present. During surgical excision of the ectopic cilia, a thin sinus tract was identified, leading posteriorly to the orbit. Magnetic resonance imaging performed after the excision showed a supraorbital extraconal mass just below the roof of the left orbit. A supraorbital 2-piece craniotomy was performed with total extirpation of the dermoid cyst. The cyst was removed en bloc without damage to the extraocular muscles, but the sinus tract could no longer be identified. Follow-up MRI was performed 6 months after surgery and showed no evidence of recurrence. A follow-up ophthalmological examination showed no signs of inferior displacement or proptosis. To the best of the authors' knowledge, this case is the first reported instance of ectopic cilia associated with a dermoid cyst and sinus tract in which no typical clinical signs and symptoms of possible orbital pathology were present. This case highlights the value of radiological examination in all cases of ectopic cilia prior to surgical excision.

Resection versus biopsy of glioblastomas in eloquent brain areas.

AIM: The aim of this study was to compare resection and biopsy of glioblastoma (GBM) in eloquent brain areas (EBA). METHODS: This was a prospective evaluation of 38 patients with GBM in EBA. 22 were treated by surgical resection and 16 by biopsy. Preoperative KPS, neurological status and size of lesion on MRI were assessed. One week and three months postoperatively KPS, neurological status and Performance Status (PS) WHO were evaluated. Extent of resection (EOR) and overall survival (OS) were described. Overall mean age of the patients was 64.3 years, the mean lesion size in the resection group was 47.7 mm and in the biopsy group 51.0 mm. RESULTS: Worsening or development of permanent neurological deficits 3 months after surgery were significantly lower in the resection group (23%), than the biopsy group (94%). In the resection group the median pre and postoperative KPS three months after surgery was 80.0. In the biopsy group the median pre and postoperative KPS was 68.1 one week after the procedure. In the resection group, 3 months after surgery, the median PS was 1, in the biopsy group one week after surgery the median PS was 2. The difference was statistically insignificant. The mean OS after resection was 12.2 months, and after biopsy 3.5 months. The difference was highly statistically significant. The mean EOR was 90%. CONCLUSION: This is the first prospective study, to our knowledge, that compares the results of resection and biopsy of primary GBM in EBA. For patients in good clinical condition with tumors in or near EBA, recommended is as radical resection of GBM as possible.

An adult multifocal medulloblastoma with diffuse acute postoperative cerebellar swelling: immunohistochemical and molecular genetics analysis.

Medulloblastoma (MB), the most common malignant tumor typically affecting children, occurs only exceptionally in adults. Multifocal presentation of this malignancy in adulthood is even much rarer­only four cases with favorable postoperative course have been reported, so far. The study illustrates a very rare rapid postoperative clinical deterioration due to diffuse cerebellar swelling (DCS) in an adult multifocal MB (MMB). To the best of their knowledge, authors for the first time performed genetic analysis of MMB and demonstrated expression patterns of selected markers that put the patient within the sonic hedgehog (SHH) molecular subgroup and at least partially explain her unsatisfactory clinical course. Herein, authors summarized the relevant literature concerning this issue with the aim to determine features that would facilitate diagnosis and therapy of such a scarce clinical entity.

MEG3: a novel long noncoding potentially tumour-suppressing RNA in meningiomas.

Meningiomas represent one of the most common types of primary intracranial tumours. However, the specific molecular mechanisms underlying their pathogenesis remain uncertain. Loss of chromosomes 22q, 1p, and 14q have been implicated in most meningiomas. Inactivation of the NF2 gene at 22q12 has been identified as an early event in their pathogenesis, whereas abnormalities of chromosome 14 have been reported in higher-grade as well as recurrent tumours. It has long been supposed that chromosome 14q32 contains a tumour suppressor gene. However, the identity of the potential 14q32 tumour suppressor remained elusive until the Maternally Expressed Gene 3 (MEG3) was recently suggested as an ideal candidate. MEG3 is an imprinted gene located at 14q32 that encodes a non-coding RNA (ncRNA). In meningiomas, loss of MEG3 expression, its genomic DNA deletion and degree of promoter methylation have been found to be associated with aggressive tumour growth. These findings indicate that MEG3 may have a significant role as a novel long noncoding RNA tumour suppressor in meningiomas.

A case report of rapid spontaneous redistribution of acute supratentorial subdural hematoma to the entire spinal subdural space presenting as a Pourfour du Petit syndrome and review of the literature.

OBJECTIVE: This report illustrates the rare rapid spontaneous redistribution of an acute intracranial supratentorial subdural hematoma (AISSDH) to the entire spinal subdural space (SSS). The study is also unique in that the spinal subdural hematoma (SSH) manifested by the extremely rare Pourfour du Petit Syndrome (PPS). METHODS: A 66-year-old man sustained blunt head trauma. On admission to the regional hospital, he scored 6 on GCS and his pupils were of equal size reacting to light. Initial computed tomography (CT) scan showed a unilateral AISSDH. The patient was referred to our department and arrived 16 h following the accident, at which time a repeat CT scan revealed almost complete resolution of the AISSDH without clinical improvement. On the 9th postinjury day transient anisocoria and tachycardia without spinal symptomatology developed. Since neither neurological examination nor follow-up CT scans showed intracranial pathology explaining the anisocoria, the patient was treated further conservatively. During the next 3 days circulatory instability developed and the patient succumbed to primary traumatic injury. Autopsy revealed a SSH occupying the entire SSS. CONCLUSION: This case calls attention to the unique combination of the displacement of an AISSDH to the SSS and the presentation of this clinical entity by the PPS.

Dysfunction of hypothalamic-hypophysial axis after traumatic brain injury in adults.

OBJECT: Traumatic brain injury (TBI) is a major cause of serious morbidity and mortality. The incidence is 100-500/100,000 inhabitants/year. Chronic pituitary dysfunction is increasingly recognized after TBI. To define the incidence of endocrine dysfunction and risk factors, the authors describe a prospectively assessed group of patients in whom they documented hormonal functions, early diagnosis, and treatment of neuroendocrine dysfunction after TBI. METHODS: Patients aged 18-65 years were prospectively observed from the time of injury to 1 year postinjury; the Glasgow Coma Scale score ranged from 3 to 14. Patients underwent evaluation of hormonal function at the time of injury and at 3, 6, and 12 months postinjury. Magnetic resonance imaging was also conducted at 1 year postinjury. RESULTS: During the study period, 89 patients were observed. The mean age of the patients was 36 years, there were 23 women, and the median Glasgow Coma Scale score was 7. Nineteen patients (21%) had primary hormonal dysfunction. Major deficits included growth hormone dysfunction, hypogonadism, and diabetes insipidus. Patients in whom the deficiency was major had a worse Glasgow Outcome Scale score, and MR imaging demonstrated empty sella syndrome more often than in patients without a deficit. CONCLUSIONS: To the authors' knowledge, this is the third largest study of its kind worldwide. The incidence of chronic hypopituitarism after TBI was higher than the authors expected. After TBI, patients are usually observed on the neurological and rehabilitative wards, and endocrine dysfunction can be overlooked. This dysfunction can be life threatening and other clinical symptoms can worsen the neurological deficit, extend the duration of physiotherapy, and lead to mental illness. The authors recommend routine pituitary hormone testing after moderate or severe TBI within 6 months and 1 year of injury.

Unusual manifestation of multiple myeloma: focal affection of central nervous system in a patient with chronic lymphocytic leukaemia.

INTRODUCTION: Involvement of the central nervous system as the first manifestation of multiple myeloma is very rare. AIM: To present an unusual case of the primomanifestation of a multiple myeloma in the form of a focal affection of the central nervous system in a patient with chronic lymphocytic leukaemia. METHODS AND RESULTS: A female patient diagnosed with chronic B-lymphocytic leukaemia with gradually increasing right-sided cerebellar symptomatology. The CT examination revealed expansion of the cranial vault with significant compression of brain structures. The tumour was extirpated and the histological examination led to a diagnosis of a plasmocytic myeloma. A thorough examination confirmed the diagnosis of multiple myeloma with significant osteolytic involvement of the skeleton. A combined chemo- and radiotherapy resulted in adjustment in the focal neurological finding, and a partial remission of the multiple myeloma was achieved. CONCLUSION: The above presented case describes two very unusual states: the primomanifestation of a multiple myeloma in form of a focal affection of the central nervous system, and the coincidence of a multiple myeloma as the second haematological malignancy in a patient with chronic B-lymphocytic leukaemia.

Can we identify patients with carotid occlusion who would benefit from EC/IC bypass? Review.

Occlusion of the internal carotid artery (CAO) is associated with a high mortality rate and frequent disability in survivors. Even in patients with good clinical recovery there is a high risk of recurrent stroke, mainly in those with impaired cerebral vasomotor reactivity (CVR). Current evidence based therapeutic options for patients with symptomatic CAO include antithrombotic medication and control of vascular risk factors. For stenosis of the contralateral internal or ipsilateral external carotid artery, endarterectomy or percutaneous transluminal angioplasty may be considered. Ongoing symptoms may cease after tapering antihypertensive medications. Extracranial to intracranial (EC/IC) arterial bypass surgery has been used since 1967 in patients with CAO. However, the international randomized EC/IC Bypass Study (1985) failed to confirm the effectiveness of EC/IC bypass for preventing cerebral ischemia in patients with symptomatic CAO when compared to those assigned to the best medical care. Nevertheless, the conclusion of the EC/IC Bypass Study has several objections and downfalls. Since then, there has been a revival of interest in cerebral revascularization procedures owing to the substantial progression of surgical techniques and the use of more advanced diagnostic methods. Thus, it has recently been reported that EC/IC bypass surgery can be useful in preventing stroke in patients with hemodynamic compromise. The main problem is to identify the small subgroup of surgical candidates. Presently, single photon emission computed tomography (SPECT), positron emission tomography (PET), transcranial Doppler sonography (TCD), computed tomography (CT) with administration of (133)Xe, perfusion CT, near infrared spectroscopy (NIRS), and functional magnetic resonance imaging (fMRI) are being used for this purpose.