Spinal dural arteriovenous fistulas: a report on outcomes in a single-center retrospective cohort treated surgically and/or endovascularly.

AIM: To report on the outcomes of spinal dural arteriovenous fistulas (sDAVFs) treatment in a single-center retrospective cohort. METHODS: Data were retrieved on sDAVF cases treated surgically and endovascularly between January 2009 and January 2020. Sociodemographic, clinical, imaging data, and outcomes were analyzed. RESULTS: Thirty-four patients were identified: 11 female, mean age 64.1 ± 11.5 years; mean time of symptom duration 12 (range 1-149) months. The sDAVF locations were the following: 18 (62.1%) thoracic, 4 (13.8%) lumbar, 4 (13.8%) sacral, and 3 (10%) with multiple location feeders. All patients had a motor deficit and affected walking, and the majority had a sensory deficit, bowel, and bladder dysfunction. Fifteen (44.1%) patients underwent surgical treatment, 7 (20.6%) underwent endovascular treatment, and 12 (35.3%) underwent both (crossover). Radiological myelopathy showed regression in 19 (55.9%) patients. Overall, clinical improvement (decrease in modified Rankin score) following treatment was observed in 14 patients (41.2%), worsening in 1 (2.9%), while other had unchanged status. The proportion of patients with initial treatment failure markedly differed between the before-2014 and after-2014 period. Patients who failed to improve had more extensive myelopathy. CONCLUSION: Patients who underwent surgery or endovascular treatment had on average significant clinical recovery, while those who underwent treatment crossover had negligible improvement. The extent of myelopathy seems to be associated with clinical improvement.

Diffusion-weighted MRI: impact of the size of the ROI in detecting metastases in subcentimeter lymph nodes in head and neck squamous cell carcinoma.

PURPOSE: Our aim is to determine the impact of the size of ROI in detecting subcentimeter metastatic lymph nodes in patients with head and neck squamous cell carcinoma (HNSCC). Secondary aim is to determine the impact of the histopathologic grade of the primary cancer on the ADC value of the metastatic nodes. METHODS: The prospective study included 51 patients with histopathologically proven HNSCC at the primary site. Primary site includes oral cavity, oropharynx, larynx, and hypopharynx. ADC values of the lymph nodes were measured on ADC maps by placing two ROIs in the lymph nodes (0.2 cm2 in the center of the node and the whole node). Lymph nodes were dissected by levels, marked by the surgeon, and sent to the pathologist. RESULTS: By applying a smaller ROI, ADC values have greater sensitivity, specificity, NPV, PPV, and AUC in detecting metastasis compared to the ADC value of the entire node (88.0%, 80.73%, 90.7%, 75.9%, 0.912% versus 80.0%, 77.98%, 85% ,71.4%, and 0.819%, respectively) p < 0.001. Statistically significant negative correlation was established between the tumor grade and the ADC of lymph node at ROI 0.2 cm2and ROI of the whole lymph node (rho = - 0.425; p = 0.002, and rho = - 0.298; p = 0.038, respectively). CONCLUSION: ROI size affects the ADC value of the nodes. The higher histopathological grade of the primary tumor is inversely correlated with the ADC value of the lymph nodes.

Hypopituitarism after gamma knife radiosurgery for pituitary adenoma.

PURPOSE: The aim of the study was to investigate the incidence of and risk factors for hypopituitarism after gamma knife radiosurgery (GKRS) for pituitary adenoma. MATERIALS AND METHODS: We conducted a retrospective analysis of the pituitary function of 90 patients who underwent GKRS for pituitary adenoma at the University Hospital Centre Zagreb between 2003 and 2014. Twenty seven of them met the inclusion criteria and the others were excluded from the study due to pituitary insufficiency which was present before GKRS. Eighteen patients had non-functioning and 9 patients had secretory adenomas. Median patients' age was 56 years (24-82). GKRS was performed using the Leksell gamma knife Model C. The median prescription radiation dose was 20 Gy (15-25) and the median tumor volume size was 3.4 cm3 (0.06-16.81). New onset hypopituitarism was defined as a new deficit of one of the three hormonal axes (corticotroph, thyreotroph, or gonadotroph) ≥3 months following GKRS. SPSS was used for statistical analysis, with the significance level at P<0.05. RESULTS: During the median follow-up period of 72 months (range 6-144), 30% of patients developed new hypopituitarism after GKRS. This corresponds to incidence of one new case of hypopituitarism per 15 patient-years. Age, gender, tumor function, tumor volume, suprasellar extension, prescription dose of radiation, as well as dose-volume to the pituitary gland, stalk and hypothalamus were not predictive factors for the development of hypopituitarism. CONCLUSIONS: In our cohort of patients with pituitary tumors who underwent GKRS, 30% developed new hypopituitarism during the follow-up period.

A Rapid Biochemical and Radiological Response to the Concomitant Therapy with Temozolomide and Radiotherapy in an Aggressive ACTH Pituitary Adenoma.

Background and Importance. In the last eight years temozolomide (TMZ) has been used as the last-line treatment modality for aggressive pituitary tumors to be applied after the failure of surgery, medical therapy, and radiotherapy. The objective was to achieve a rapid control of tumor growth and hormone normalization with concurrent chemoradiotherapy in a patient with very aggressive ACTH pituitary adenoma. Clinical Presentation. We describe a patient with an aggressive ACTH-producing adenoma treated with concurrent temozolomide and radiotherapy. The patient suffered from an aggressive ACTH adenoma resistant to surgical and medical treatment. After two months of concurrent temozolomide and radiotherapy, cortisol normalization and significant tumor shrinkage were observed. After 22 months of follow-up, there is still no evidence of tumor recurrence. Conclusion. Concurrent treatment with temozolomide and irradiation appears to be highly effective in the achievement of the tumor volume control as well as in the control of ACTH secretion in aggressive ACTH adenoma.

Management of Cushing's disease: a single-center experience.

The purpose of this study was to review therapeutic outcomes and comorbidities of patients with Cushing's disease (CD) in a single center. We conducted a retrospective study of 33 patients with CD undergoing transsphenoidal surgery from January 2007 to February 2014 (27 females and 6 males, median age 38 years, range 18-71 years). The diagnosis of Cushing's syndrome was established on the basis of the patient's history, characteristic clinical features, and laboratory data including an elevated 24-h urinary free cortisol level, lack of serum cortisol suppression after dexamethasone suppression tests and an elevated midnight cortisol level. In 28/33 patients, the tumor was visualized on MR of the sellar region, while in 5 it was diagnosed using an inferior petrosal sinus sampling. Out of the 33 patients, 10 had macroadenoma and the remaining 23 had microadenoma. Twenty-one patients (63.6%) had hypertension, 17 (51.5%) dyslipidemia, and 7 (21.2%) had type 2 diabetes or impaired glucose tolerance. The median follow-up period was 28 months. Remission after transsphenoidal surgery was achieved in 78.8% of patients, while 7 patients failed to achieve disease remission. Those patients were treated with second-line treatment modalities (second operation, radiotherapy, bilateral adrenalectomy, and/or ketoconazole). One patient rejected all the treatment modalities after surgery. Cumulative remission after all the treatment modalities was achieved in 87.9% patients. Patients with Cushing's disease should be managed in centers with much experience due to high patient load. In our Center, the remission of the disease has been achieved in 78.8% of the patients following transsphenoidal surgery. Multimodal treatment which included radiotherapy and medical treatment led to biochemical remission of the disease in 87.9% of patients.

Fatal outcome of posterior "reversible" encephalopathy syndrome in metastatic colorectal carcinoma after irinotecan and fluoropyrimidine chemotherapy regimen.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity characterized by headaches, altered mental status, seizures, and visual disturbances. It can occur in many different clinical entities such as severe hypertension and pre-eclampsia, or due to cytotoxic or immunosuppressive therapies. The pathogenesis of PRES is unclear, with dysregulated cerebral auto-regulation and endothelial dysfunction as important mechanisms proposed. Endothelial dysfunction is important especially in cases associated with cytotoxic therapies. Herein, we describe a patient with PRES with fatal outcome, who presented 5 days after the infusion of cycle 1 of irinotecan hydrochloride, leucovorin calcium, and fluorouracil (FOLFIRI) regimen chemotherapy, without prior hypertension and other comorbidity, suggesting a link between PRES and FOLFIRI regimen. To our knowledge, this case report is the first describing PRES after FOLFIRI regimen, although others have described PRES after FOLFIRI with bevacizumab in colonic cancer patients.

Isolated and persistent cognitive dysfunction in a patient with acute disseminated encephalomyelitis.

We report a case of pathology-proven acute disseminated encephalomyelitis (ADEM) in which the patient's symptoms were solely cognitive. Although cognitive dysfunction is a well-recognized symptom in adults with multiple sclerosis, cognitive assessment of adults with ADEM has rarely been reported. A 35-year-old woman was referred to our center for evaluation of cognitive disturbance and demyelinating lesions seen on brain magnetic resonance imaging (MRI). We performed a neurologic examination, full neuropsychological assessment, brain MRI, blood and cerebrospinal fluid analyses, visual evoked potentials, and brain biopsy. The patient's Mini-Mental State Examination score was 26/30. Cognitive assessment revealed multiple severe dysfunctions, mainly in executive and attention tasks. She scored below the normal range on the Digit Span Forward and Backward Test and the Trail Making Test Part B. The Frontal Assessment Battery showed deficits in mental flexibility, motor programming, and inhibitory control. She also scored in the impaired range on tests of verbal fluency and memory. The brain MRI and biopsy confirmed a diagnosis of ADEM. This case report points to the limitations of relying on clinical presentation, neuroimaging, and current controversial diagnostic criteria in diagnosing ADEM in adults, and highlights the essential role of pathologic evaluation.

An infrequent type of stroke with an unusual cause and successful therapy: basilar artery occlusion caused by a cardiac papillary fibroelastoma recanalized 12 hours after onset.

We herein report the case of a 32-year-old woman with sudden onset ataxia, limb dysmetria and somnolence. Emergency radiological findings showed bilateral cerebellar and thalamic infarctions as a result of a basilar artery occlusion. The patient was treated with intra-arterial (IA) and mechanical thrombolysis 12 hours after symptom onset and showed an excellent recovery. A diagnostic workup revealed a tumor mass on the mitral valve that was surgically removed, while a histological analysis confirmed a diagnosis of cardiac papillary fibroelastoma.

Clinical sensitivity and specificity of multiple T2-hyperintensities on brain magnetic resonance imaging in diagnosis of neurofibromatosis type 1 in children: diagnostic accuracy study.

AIM: To determine the prevalence, number, and location of multiple (≥2) T2-hyperintensities on brain magnetic resonance imaging (MRI) in children with neurofibromatosis type 1 (NF1) and their correlation with age, and to establish their sensitivity, specificity, and accuracy for the diagnosis of NF1 in children, especially in the early age (2-7 years). METHODS: We performed a cross-sectional study of 162 patients with NF1 from Croatian Neurofibromatosis Association Database and 163 control children between the ages of 2 and 18 years who underwent brain MRI between 1989 and 2009. RESULTS: Multiple T2-hyperintensities were present in 74% of NF1 patients and 1.8% of controls. They were mainly located in the basal ganglia, brainstem, and cerebellum and were significantly decreased in prevalence and number in the older age. T2-hyperintensities had excellent diagnostic accuracy with the area under the receiver operating characteristic (ROC) curve of 0.849 and 95% confidence interval (CI) of 0.805-0.886. The diagnostic sensitivity, specificity, and accuracy rate of T2-hyperintensities for NF1 were highest in the youngest age (2-7 years): 81% (95% CI 71%-89.1%), 99% (95% CI 92.3%-100%), and 85.8 (95% CI 83.3-93.8), respectively. CONCLUSION: This study strongly suggests the inclusion of T2-hyperintensities on brain MRI on the list of diagnostic criteria for NF1, especially in children of early age, when the clinical penetration of the NF1 gene has not yet been completely finished.

Coexistance of cerebral sinovenous thrombosis and Dandy Walker malformation in newborn.

Cerebral sinovenous thrombosis in neonatal period may cause neurological impairment, epilepsy, and lead to stroke. It is caused primarily by coagulopathy of numerous reasons, occasionally perinatal asphyxia, traumatic delivery and hyperhomocysteinemia. Dandy-Walker malformation is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. Dandy-Walker malformation, variant, and mega cisterna magna represent a spectrum of developmental anomalies. Insults to developing cerebellar hemispheres and the fourth ventricle are believed to be the cause of malformation. Our patient was born from noncomplicated pregnancy, noncomplicated nontraumatic vaginal delivery at term, excellent Apgar scores, without peculiarities in clinical status. She was brest-fed by the 42nd hour of life when she had rightsided seizures during sleep that repeated for five times in next 24 hours. Brain Ultrasound (US) revealed clot in left lateral ventricle, slight dilatation of left ventricle, both sided periventricular echodensity, ischemia, slight enlargement of forth ventricle and a bit smaller cerebellum. There was no visible flow through left transverse, superior sagittal and straight sinus. Magnetic Resonance (MRI) confirmed the finding and showed thrombosis of left and right transverse venous sinuses and confluence of sinuses. Electroencephalogram (EEG) showed leftsided focal changes. The newborn was treated with phenobarbiton for 8 days and had no convulsions during that period. All coagulation parameters, homocistein, lipoproteins (a) and D-dimers were normal. There were no mutations on FV R506Q, PT 20210A, MTHFR 677C/T. No antiphospholipides were found. Heart US showed no structural anomalies. No other patology or risk factors were present at the time. Before discharge, US showed hydrocephalus. Flow in affected sinuses was visible with color Doppler. MRI showed recanalization of affected sinuses, also hydrocephalus and presentation of Dandy Walker On EEG there was borderline finding. Due to progression of hydrocephalus ventriculo-peritoneal shunt was placed. In age of 1 year EEG was slower for age but without focus. Neurological development was normal for age. The question is whether this child had intrauterine insult and inception of Dandy Walker with further postnatal progress of thrombosis and evolution to full picture of Dandy Walker with hydrocephalus OR thrombosis that led to development of hydrocephalus and Dandy Walker malformation in this child were accidental coexistance.

Primary diffuse meningeal melanomatosis.

Primary diffuse meningeal melanomatosis can clinically mimic a wide variety of other conditions, including lymphoma, leukemia, neurosarcoidosis, metastatic carcinoma, acute disseminated encephalomyelitis, subacute meningitis, viral encephalitis, and idiopathic hypertrophic cranial pachymeningitis. We report on a young patient with primary diffuse meningeal melanomatosis who presented with papilledema, flaccid paraparesis, and cognitive impairment. The importance of imaging of the whole central nervous system, cerebrospinal fluid analysis, and pathohistological examination is emphasized in making the appropriate diagnosis.

Sole stenting of large and giant intracranial aneurysms with self-expanding intracranial stents-limits and complications.

BACKGROUND: Intracranial aneurysms may be difficult for endovascular treatment due to size, fusiform shape, or wide neck. In such patients, intracranial stents are used to support the coils in the aneurysm sac, or they may be used as a sole stenting technique to divert the blood flow without coils. The aim of this paper is to contribute to the existing data by reviewing the risks of sole stenting of large and giant aneurysms. METHODS: We treated seven patients with nine aneurysms by self-expanding intracranial stents, either by a single or multiple stents in a stent-in-stent configuration. The follow-up was performed by digital subtraction angiography with a mean follow-up time of 6 months. RESULTS: A positive response to stenting occurred in five out of seven patients (71%) and six out of nine aneurysms (67%). The aneurysms were occluded in two patients, and incomplete results were noted in three patients. The symptoms due to the compression of cranial nerves resolved in four patients (57%). Procedure-related subarachnoid hemorrhage occurred in two out of seven patients (29%), with death of one patient as a result of hemorrhage (14%). CONCLUSIONS: Sole stenting of large and giant aneurysms with self-expanding intracranial stents may be associated with a higher risk than previously reported. The effect of stenting on intra-aneurysmal flow in such aneurysms, even after the placement of multiple overlapping stents, seems to be unpredictable.

Current concepts in the diagnosis of transverse myelopathies.

The clinical symptoms and MRI characteristics of transverse myelopathy (TM) due to non-compressive causes are reviewed, with special emphasis on the differential diagnosis between inflammatory demyelinating lesions, and metabolic and vascular myelopathies. Inflammatory transverse myelopathies are the commonest and most difficult ones to identify. The differentiation between clinically isolated syndromes, multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis and metabolic causes is based on both clinical symptoms and paraclinical signs including magnetic resonance imaging, cerebrospinal fluid analysis, and immunological and biochemical parameters. The most intriguing form of TM is that where there is clinical evidence of complete spinal cord transection, with normal findings in magnetic resonance imaging in the acute phase, but subsequent cord atrophy.