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Superficial siderosis (SS) of the central nervous system is a rare disorder that is caused by chronic or recurrent hemorrhage in the subarachnoid space via a dural defect at the spinal level. The most common clinical features of SS include slow-progressive sensorineural deafness, cerebellar symptoms, and pyramidal tract signs. Considering that SS can present with broad clinical manifestations, for precise diagnosis, this disease must be understood. Anti-Ro/SSA antibodies are commonly detected in patients with Sjögren's syndrome and are utilized as markers for autoimmune diseases. In this report, we present a unique pathological condition in which SS coincided with a positive anti-Ro/SSA antibody test result. During the diagnosis of gait disturbance, an elevation in anti-Ro/SSA antibody was detected, and steroid pulse therapy was initiated as the initial treatment for autoimmune diseases. Head magnetic resonance imaging (MRI) revealed extensive hypointensity as a dark band that surrounded the intracranial basal structures and cerebellar hemispheres. Spinal MRI indicated ventral longitudinal intraspinal fluid collection extending from C7 to T5 as well as a defect in the ventral T2-3 dura mater. Intraoperative visualization revealed that the intradural venous plexus was the source of bleeding that caused the SS. To our knowledge, this report is the first to discuss the presence of anti-Ro/SSA antibodies in patients with SS. The role of anti-Ro/SSA antibodies in the pathophysiology of SS remains unclear; therefore, to confirm a possible association, further research and accumulation of cases are required.
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OBJECTIVE: Surgery is a good treatment option for drug-resistant temporal lobe epilepsy (TLE). 2-deoxy-2-(18F) fluoro-D-glucose (FDG) positron emission tomography (PET) is used to detect epileptic foci as hypometabolic lesions in presurgical evaluation. Visual field defects (VFDs) in the contralateral homonymous upper quadrant are common postoperative complications in TLE. This study aimed to quantify VFDs using pattern deviation probability plots (PDPPs) and examine the effect of hypometabolism in FDG-PET on VFDs. METHODS: This study included 40 patients. Both visual fields were assessed using the Humphrey field analyzer preoperatively and 3 months and 2 years postoperatively. PDPPs with <0.5% confidence level counted in the contralateral homonymous upper quadrant. FDG-PET results were compared between groups with (15 patients) and without (24 patients) hypometabolism in the optic radiation. RESULTS: All 40 patients were evaluated by Humphrey field analyzer at 3 months postoperatively and 39 at 2 years postoperatively. The incidence of VFDs 3 months postoperatively was 35/40 (87.5%), and 17/40 (42.5%) patients had severe VFDs. In cases of surgery on the left temporal lobe, ipsilateral eyes appeared to be more significantly affected than contralateral eyes. VFDs were more severe in patients with FDG hypometabolism than in those without hypometabolism in posteromedial temporal and medial occipital cortex (P < 0.01); however, 85% of patients with FDG hypometabolism had a reduced VFD 2 years postoperatively. CONCLUSIONS: PDPP counting is useful for quantifying VFDs. Preoperative dysfunction indicated by preoperative FDG-PET in the posteromedial temporal and medial occipital cortex could enhance VFDs early after TLE surgery.
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Epilepsia del Lóbulo Temporal , Fluorodesoxiglucosa F18 , Lóbulo Occipital , Tomografía de Emisión de Positrones , Complicaciones Posoperatorias , Lóbulo Temporal , Humanos , Epilepsia del Lóbulo Temporal/cirugía , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/metabolismo , Femenino , Masculino , Adulto , Tomografía de Emisión de Positrones/métodos , Estudios Retrospectivos , Lóbulo Occipital/diagnóstico por imagen , Lóbulo Occipital/metabolismo , Lóbulo Occipital/cirugía , Lóbulo Temporal/metabolismo , Lóbulo Temporal/diagnóstico por imagen , Lóbulo Temporal/cirugía , Estudios de Seguimiento , Persona de Mediana Edad , Adulto Joven , Complicaciones Posoperatorias/metabolismo , Complicaciones Posoperatorias/diagnóstico por imagen , Campos Visuales/fisiología , Radiofármacos , Adolescente , Trastornos de la Visión/etiología , Trastornos de la Visión/diagnóstico por imagen , Trastornos de la Visión/metabolismo , Epilepsia Refractaria/cirugía , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/metabolismoRESUMEN
OBJECTIVE: Spondylotic changes in the cervical spine cause degeneration, leading to cervical spinal canal stenosis. This stenotic change can affect cerebrospinal fluid (CSF) dynamics by compressing the dural sac and reducing space in the subarachnoid space. We examined CSF dynamics at the craniovertebral junction (CVJ) using time-spatial labeling inversion pulse magnetic resonance imaging (Time-SLIP MRI) in patients with cervical spinal canal stenosis. METHODS: The maximum longitudinal movement of the CSF at the CVJ was measured as length of motion (LOM) in the Time-SLIP MRI of 56 patients. The sum of ventral and dorsal LOM was defined as the total LOM. Patients were classified into 3 groups depending on their spinal sagittal magnetic resonance imaging findings: control (n = 27, Kang classification grades 0 and 1), stenosis (n = 14, Kang classification grade 2), and severe stenosis (n = 15, Kang classification grade 3). RESULTS: Time-SLIP MRI revealed pulsatile movement of the CSF at the CVJ. The mean total, ventral, and dorsal LOM was 14.2 ± 9, 8.1 ± 5.7, and 3.8 ± 2.9 mm, respectively. The ventral LOM was significantly larger than the dorsal LOM. The total LOM was significantly smaller in the severe stenosis group (6.1 ± 3.4 mm) than in the control (16.0 ± 8.4 mm) or stenosis (11 ± 5.4 mm) groups (P < 0.001, Kruskal-Wallis H-test). In 5 patients, postoperative total LOM was improved after adequate decompression surgery. CONCLUSIONS: This study demonstrates that CSF dynamics at the CVJ are influenced by cervical spinal canal stenosis. Time-SLIP MRI is useful for evaluating CSF dynamics at the CVJ in patients with spinal canal stenosis.
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Imagen por Resonancia Magnética , Estenosis Espinal , Humanos , Constricción Patológica/patología , Imagen por Resonancia Magnética/métodos , Estenosis Espinal/diagnóstico por imagen , Estenosis Espinal/cirugía , Estenosis Espinal/patología , Radiografía , Canal Medular/diagnóstico por imagen , Canal Medular/patología , Vértebras Cervicales/cirugía , Líquido Cefalorraquídeo/diagnóstico por imagenRESUMEN
To assess the use of indocyanine green (ICG) fluorescence endoscopy to evaluate pituitary blood flow in craniopharyngioma resection and its possible impact on intraoperative decisions regarding pituitary stalk processing. Patients with craniopharyngiomas who had undergone transsphenoidal surgery since March 2021, when an ICG endoscope was introduced at the Kagoshima University Hospital, were included in the study. When targeted tumor removal was approaching completion, 10 mg of ICG was administered intravenously to evaluate blood flow in the pituitary stalk and gland. ICG signals and endocrinological status before and after surgery were evaluated retrospectively. Pituitary stalk and gland blood flow were evaluated as positive (++), weakly positive (+), and no signal (-).Ten patients with craniopharyngiomas underwent transsphenoidal surgery using an ICG endoscope (mean age 56.6 ± 14.2 years; 40% male). Among the eight patients in whom the pituitary stalk was preserved, pituitary function with positive signal on the stalk was intact in two. Two other patients with weakly positive stalk and positive pituitary gland signals showed intact function or minimal pituitary dysfunction. Four patients had impairments in more than three axes with poor ICG signals in the stalk or pituitary gland. Two patients underwent pituitary amputation because of high tumor invasion and lack of ICG signal in the stalk after tumor removal, resulting in panhypopituitarism. A negative ICG signal in the pituitary stalk is likely to indicate postoperative pituitary function loss. Craniopharyngioma surgery using ICG endoscopy may be useful for predicting endocrine prognosis and improving tumor outcomes.
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Craneofaringioma , Neoplasias Hipofisarias , Humanos , Masculino , Adulto , Persona de Mediana Edad , Anciano , Femenino , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Verde de Indocianina , Estudios Retrospectivos , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Hipófisis/diagnóstico por imagen , Hipófisis/cirugía , Endoscopía/métodos , Resultado del TratamientoRESUMEN
Background: Glioblastoma (GBM) is a malignant brain tumor, with radiological and genetic heterogeneity. We examined the association between radiological characteristics and driver gene alterations. Methods: We analyzed the driver genes of 124 patients with IDH wild-type GBM with contrast enhancement using magnetic resonance imaging. We used a next-generation sequencing panel to identify mutations in driver genes and matched them with radiological information. Contrast-enhancing lesion localization of GBMs was classified into 4 groups based on their relationship with the subventricular zone (SVZ) and cortex (Ctx). Results: The cohort included 69 men (55.6%) and 55 women (44.4%) with a mean age of 66.4â ±â 13.3 years. EGFR and PDGFRA alterations were detected in 28.2% and 22.6% of the patients, respectively. Contrast-enhancing lesion touching both the SVZ and Ctx was excluded because it was difficult to determine whether it originated from the SVZ or Ctx. Contrast-enhancing lesions touching the SVZ but not the Ctx had significantly worse overall survival than non-SVZ lesions (441 days vs. 897 days, Pâ =â .002). GBM touching only the Ctx had a better prognosis (901 days vs. 473 days, Pâ <â .001) than non-Ctx lesions and was associated with EGFR alteration (39.4% vs. 13.2%, Pâ =â .015). Multiple contrast lesions were predominant in PDGFRA alteration and RB1-wild type (Pâ =â .036 and Pâ =â .031, respectively). Conclusions: EGFR alteration was associated with cortical lesions. And PDGFRA alteration correlated with multiple lesions. Our results suggest that clarifying the association between driver genes and tumor localization may be useful in clinical practice, including prognosis prediction.
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Background: Telomerase reverse transcriptase promoter (TERTp) mutations are a biological marker of glioblastoma; however, the prognostic significance of TERTp mutational status is controversial. We evaluated this impact by retrospectively analyzing the outcomes of patients with isocitrate dehydrogenase (IDH)- and TERTp-wild-type glioblastomas. Methods: Using custom next-generation sequencing, we analyzed 208 glioblastoma samples harboring wild-type IDH. Results: TERTp mutations were detected in 143 samples (68.8%). The remaining 65 (31.2%) were TERTp-wild-type. Among the TERTp-wild-type glioblastoma samples, we observed a significant difference in median progression-free survival (18.6 and 11.4 months, respectively) and overall survival (not reached and 15.7 months, respectively) in patients with and without phosphatase and tensin homolog (PTEN) loss and/or mutation. Patients with TERTp-wild-type glioblastomas with PTEN loss and/or mutation were younger and had higher Karnofsky Performance Status scores than those without PTEN loss and/or mutation. We divided the patients with TERTp-wild-type into 3 clusters using unsupervised hierarchical clustering: Good (PTEN and TP53 alterations; lack of CDKN2A/B homozygous deletion and platelet-derived growth factor receptor alpha (PDGFRA) alterations), intermediate (PTEN alterations, CDKN2A/B homozygous deletion, lack of PDGFRA, and TP53 alterations), and poor (PDGFRA and TP53 alterations, CDKN2A/B homozygous deletion, and lack of PTEN alterations) outcomes. Kaplan-Meier survival analysis indicated that these clusters significantly correlated with the overall survival of TERTp-wild-type glioblastoma patients. Conclusions: Here, we report that PTEN loss and/or mutation is the most useful marker for predicting favorable outcomes in patients with IDH- and TERTp-wild-type glioblastomas. The combination of 4 genes, PTEN, TP53, CDKN2A/B, and PDGFRA, is important for the molecular classification and individual prognosis of patients with IDH- and TERTp-wild-type glioblastomas.
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Internal carotid artery aplasia or hypoplasia above the cervical bifurcation is rare, occurring in less than 0.01% of the general population. Unilateral neurocristopathy complicated by unilateral internal carotid artery agenesis or hypogenesis has been reported, but bilateral internal carotid artery hypoplasia is rare and scarcely reported. Herein, we report a novel case of Treacher Collins syndrome complicated by bilateral internal carotid artery hypoplasia. A 94-year-old woman presented with complaints of headache and vomiting. Computed tomography revealed a subarachnoid hemorrhage and dysplasia of the bilateral zygoma, mandible, and external auditory meatus. The patient had severe hearing loss and visual impairment. Computed tomography angiography revealed bilateral internal carotid artery hypoplasia and multiple aneurysmal changes in the intracranial arteries. We diagnosed the patient with a ruptured anterior inferior cerebellar artery aneurysm and performed coil embolization. The patient's unique facial features were consistent with neurocristopathy, especially Treacher Collins syndrome. Developmental anomalies of neural crest cells can present as vascular abnormalities and craniofacial malformations. Special care is required for endovascular treatment and airway management in cases of neurocristopathy because of the specific craniofacial anomalies.
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Fusión Vertebral , Insuficiencia Vertebrobasilar , Humanos , Arteria Vertebral/diagnóstico por imagen , Arteria Vertebral/cirugía , Insuficiencia Vertebrobasilar/diagnóstico por imagen , Insuficiencia Vertebrobasilar/etiología , Insuficiencia Vertebrobasilar/cirugía , Fusión Vertebral/efectos adversos , Vértebras Cervicales/diagnóstico por imagenRESUMEN
Background: Solitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are rare mesenchymal tumors of nonmeningothelial origin that comprises <1% of all central nervous system tumors. Case Description: A 45-year-old male presented with sleep apnea (apnea-hypopnea index was 17.1 events/hour) and dysesthesias of the right upper and lower extremities. The magnetic resonance demonstrated a heterogeneous intradural extra-axial C1 mass with syringobulbia and syringomyelia. The right vertebral angiography revealed a hypervascular mass (i.e., intense tumor staining). With the preoperative diagnosis of a spinal hemangioblastoma, the patient underwent tumor removal. However, intraoperative findings demonstrated that the ventral component of the tumor was intramedullary without a dural attachment. Further, the histological diagnosis was consistent with SFT/HPC (HPC phenotype). The postoperative course was uneventful, and the patient's symptoms and the syrinxes spontaneously regressed. Conclusion: A 45-year-old male presented a rare spinal intradural lesion at C1 appeared to be a spinal hemangioblastoma, but proved to be SFT/HPC (HPC phenotype) with intramedullary invasion.
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OBJECTIVE: Pin-type head frame systems have become a worldwide standard procedure, but they can cause some complications on rare occasions. This study aimed to examine the incidence and associated risk factors of depressed skull fracture and related intracranial hematoma (DSFH) due to the use of head frames in our institute over the past 10 years. METHODS: This study included 1749 patients who underwent neurosurgical surgeries using pin-type head frames, including the Mayfield (Integra NeuroSciences, Plainsboro, NJ) skull clamp (721 cases) and the Sugita (Mizuho Ikakogyo Co., Ltd., Tokyo, Japan) head frame (1028 cases). We retrospectively reviewed hospital records of our institute to identify cases of DSFH, and documented the type of head frame used, as well as patient characteristics. RESULTS: The incidence of DSFH was 0.29% (5 of 1749 cases). All 5 cases had an associated epidural hematoma, with a single case having an additional dural laceration (without subdural damage). All perforation sites, located at the parietal bone near the pterion, occurred by the unilateral horizontal screw of the Sugita head frame. None of the patients experienced postoperative neurological decline. CONCLUSIONS: Even in the adult population, the DSFH by the pin-type head frame can occur infrequently. Based on our results, we recommend that the following factors should be considered when the pin-type head frame is used for neurosurgical procedures: location of pin application, thickness and fragility of the skull, and adequate control of compressive forces exerted by the head frame.
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Centros Médicos Académicos , Procedimientos Neuroquirúrgicos/efectos adversos , Complicaciones Posoperatorias/epidemiología , Fractura Craneal Deprimida/epidemiología , Técnicas Estereotáxicas/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Hematoma Epidural Craneal/diagnóstico por imagen , Hematoma Epidural Craneal/epidemiología , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos/instrumentación , Hueso Parietal/diagnóstico por imagen , Hueso Parietal/cirugía , Complicaciones Posoperatorias/diagnóstico por imagen , Estudios Retrospectivos , Fractura Craneal Deprimida/diagnóstico por imagen , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: Spontaneous intracranial hypotension (SIH) is secondary to a cerebrospinal fluid leak at the spinal level without obvious causative events. Several signs on brain and cervical spine magnetic resonance (MR) imaging (MRI) have been associated with SIH but can be equivocal or negative. This retrospective study sought to identify characteristic SIH signs on thoracic spinal MRI. METHODS: Cranial and spinal MR images of 27 consecutive patients with classic SIH symptoms, who eventually received epidural autologous blood patches (EBPs), were analyzed. RESULTS: The most prevalent findings on T2-weighted MRI at the thoracic level were anterior shift of the spinal cord (96.3%) and dorsal dura mater (81.5%), probably caused by dural sac shrinkage. These dural sac shrinkage signs (DSSS) were frequently accompanied by cerebrospinal fluid collection in the posterior epidural space (77.8%) and a prominent epidural venous plexus (77.8%). These findings disappeared in all six patients who underwent post-EBP spinal MRI. Dural enhancement and brain sagging were minimum or absent on the cranial MR images of seven patients, although DSSS were obvious in these seven patients. For 23 patients with SIH and 28 healthy volunteers, a diagnostic test using thoracic MRI was performed by 13 experts to validate the usefulness of DSSS. The median sensitivity, specificity, positive-predictive value, negative-predictive value, and accuracy of the DSSS were high (range, 0.913-0.931). CONCLUSIONS: Detection of DSSS on thoracic MRI facilitates an SIH diagnosis without the use of invasive imaging modalities. The DSSS were positive even in patients in whom classic cranial MRI signs for SIH were equivocal or minimal.
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Hipotensión Intracraneal , Pérdida de Líquido Cefalorraquídeo , Espacio Epidural/diagnóstico por imagen , Humanos , Hipotensión Intracraneal/diagnóstico por imagen , Hipotensión Intracraneal/terapia , Imagen por Resonancia Magnética , Estudios RetrospectivosRESUMEN
BACKGROUND: Narrowness of the spinal canal is associated with the development of cervical myelopathy. While studies have addressed the sagittal diameter of the cervical spinal canal, few evaluated the correlation between the size of the spinal canal and the vertebral level. We addressed this issue. METHODS: Our retrospective study included 102 patients with cranial or spinal disorders. We examined the correlation between the cervical spinal canal diameter (SCD) at C1 to C7 and the inner anteroposterior diameter (IAPD) of the atlas on CT images. RESULTS: At C1, the SCD was largest, at C4 it was smallest. While there was a strong correlation between the IAPD and the SCD at C1 (r = 0.8), the correlation between the size of the atlas and the SCD at C4 to C7 was weak (r = 0.2-0.3). We divided our patients into a normal group (n = 34, SCD ≥ 12 mm at any levels) and a stenosis group (n = 68, SCD < 12 mm at all levels). The mean SCD at C2 to C7 was significantly larger in the normal group. There was no significant difference between the two groups with respect to the IAPD and the SCD at C1. CONCLUSIONS: The size of the subaxial spine does not necessarily affect the size of the atlas. The pathophysiology of spinal canal stenosis should be considered separately at the C1- and the subaxial level.
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Canal Medular , Estenosis Espinal , Vértebras Cervicales/diagnóstico por imagen , Humanos , Estudios Retrospectivos , Canal Medular/diagnóstico por imagen , Estenosis Espinal/diagnóstico por imagen , Columna Vertebral , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: The purpose of the present study was to compare the treatment success rates of primary neurosurgical and endovascular treatments in patients with spinal dural arteriovenous fistulas (dAVFs). METHODS: Data from 199 consecutive patients with thoracic and lumbosacral spinal dAVFs were collected from 18 centers. Angiographic and clinical findings, the rate of initial treatment failure or recurrence by procedures, risk factors for treatment failure, complications, and neurological outcomes were statistically analyzed. RESULTS: Spinal dAVFs were frequently detected in the thoracic region (81%), fed by a single feeder (86%), and shunted into an intradural vein via the dura mater. The fistulous connection between the feeder(s) and intradural vein was located at a single spinal level in 195 patients (98%) and at 2 independent levels in 4 patients (2%). Among the neurosurgical (n = 145), and endovascular (n = 50) treatment groups of single dAVFs (n = 195), the rate of initial treatment failure or recurrence was significantly higher in the index endovascular treatment group (0.68% and 36%). A multivariate analysis identified endovascular treatment as an independent risk factor with significantly higher odds of initial treatment failure or recurrence (OR 69; 95% CI 8.7-546). The rate of complications did not significantly differ between the two treatment groups (4.1% for neurosurgical vs 4.0% for endovascular treatment). With a median follow-up of 26 months, improvements of ≥ 1 point in the modified Rankin Scale (mRS) score and Aminoff-Logue gait and Aminoff-Logue micturition grades were observed in 111 (56%), 121 (61%), and 79 (40%) patients, respectively. Independent risk factors for lack of improvement in the Aminoff-Logue gait grades were multiple treatments due to initial treatment failure or recurrence (OR 3.1) and symptom duration (OR 1.02). CONCLUSIONS: Based on data obtained from the largest and most recently assessed multicenter cohort, the present study shows that primary neurosurgery is superior to endovascular treatment for the complete obliteration of spinal dAVFs by a single procedure.
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BACKGROUND: The endoscopic endonasal approach (EEA) has been accepted as an alternative option for diseases at the craniovertebral junction. However, the inferior destination through the endoscopic endonasal approach is anatomically higher than that of the transoral approach. Therefore, preoperative assessment of accessibility is mandatory for appropriate selection of indication. Using a navigation system, we examined the inferior limit through the endonasal route and evaluated the relationships between surrounding anatomicl structures and the lowest point. METHODS: This study included patients who underwent endoscopic transsphenoidal surgery for intrasellar lesions at our hospital (N = 23). At the start of surgery, the lowest point (target point [TP]) was marked with a straight probe under guidance of the navigation system. We measured 4 parameters on preoperative computed tomography: nasal length, hard palate length, anterior-posterior diameter of the nasopharynx, and nasopalatine angle. Patients were classified into groups depending on whether the TP was at or above (group A) or below (group B) the hard palatine line. RESULTS: TPs were above the hard palatine line in 15 patients (group A) and below the hard palatine line in 8 patients (group B). No TPs reached the nasopalatine line. Nasal length (P = 0.03) and nasopalatine angle (P = 0.01) were larger in group B than in group A. There were no significant differences in anterior-posterior diameter of the nasopharynx or hard palate length. CONCLUSIONS: The hard palatine line is a reliable parameter for assessing the inferior limit of the endoscopic endonasal approach. Nostril size affects accessibility with surgical instruments.
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Nasofaringe/anatomía & histología , Cirugía Endoscópica por Orificios Naturales , Neuroendoscopía , Paladar Duro/anatomía & histología , Enfermedades de la Hipófisis/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neuronavegación , Base del Cráneo/cirugíaRESUMEN
OBJECTIVE: Spinal arteriovenous shunts are rare vascular lesions and are classified into 4 types (types I-IV). Due to rapid advances in neuroimaging, spinal epidural AVFs (edAVFs), which are similar to type I spinal dural AVFs (dAVFs), have recently been increasingly reported. These 2 entities have several important differences that influence the treatment strategy selected. The purposes of the present study were to compare angiographic and clinical differences between edAVFs and dAVFs and to provide treatment strategies for edAVFs based on a multicenter cohort. METHODS: A total of 280 consecutive patients with thoracic and lumbosacral spinal dural arteriovenous fistulas (dAVFs) and edAVFs with intradural venous drainage were collected from 19 centers. After angiographic and clinical comparisons, the treatment failure rate by procedure, risk factors for treatment failure, and neurological outcomes were statistically analyzed in edAVF cases. RESULTS: Final diagnoses after an angiographic review included 199 dAVFs and 81 edAVFs. At individual centers, 29 patients (36%) with edAVFs were misdiagnosed with dAVFs. Spinal edAVFs were commonly fed by multiple feeding arteries (54%) shunted into a single or multiple intradural vein(s) (91% and 9%) through a dilated epidural venous plexus. Preoperative modified Rankin Scale (mRS) and Aminoff-Logue gait and micturition grades were worse in patients with edAVFs than in those with dAVFs. Among the microsurgical (n = 42), endovascular (n = 36), and combined (n = 3) treatment groups of edAVFs, the treatment failure rate was significantly higher in the index endovascular treatment group (7.5%, 31%, and 0%, respectively). Endovascular treatment was found to be associated with significantly higher odds of initial treatment failure (OR 5.72, 95% CI 1.45-22.6). In edAVFs, the independent risk factor for treatment failure after microsurgery was the number of intradural draining veins (OR 17.9, 95% CI 1.56-207), while that for treatment failure after the endovascular treatment was the number of feeders (OR 4.11, 95% CI 1.23-13.8). Postoperatively, mRS score and Aminoff-Logue gait and micturition grades significantly improved in edAVFs with a median follow-up of 31 months. CONCLUSIONS: Spinal epidural AVFs with intradural venous drainage are a distinct entity and may be classified as type V spinal vascular malformations. Based on the largest multicenter cohort, this study showed that primary microsurgery was superior to endovascular treatment for initial treatment success in patients with spinal edAVFs.
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BACKGROUND: Intracranial schwannomas are rarely confined to the skull. We here report a large schwannoma localized in the petrous apex that presented with intratumoral hemorrhage. CASE DESCRIPTION: A 35-year-old woman with mild hearing disturbance and ear fullness underwent computed tomography scan and magnetic resonance imaging, which demonstrated a tumor accompanied with intratumoral hematoma in the right petrous apex. Bone marrow was totally destroyed, but the bone cortex was relatively preserved. Pathologic specimen showed that the tumor was composed of proliferation of elongated neoplastic cells and positive for S-100 protein. It showed a nuclear palisading pattern, compatible with schwannoma. The lack of any cranial nerve signs and relative preservation of canals through which cranial nerves pass suggested the neurilemma cells surrounded vessels or mismigrated fetal neurilemma cells in the petrous apex as origin of the tumor. Benign nature of the tumor and total disappearance of the symptoms, supposedly due to the spontaneous absorption of the hematoma, made the patient choose a wait-and-watch approach. Magnetic resonance imaging studied 7 years after the diagnosis showed significant decrease of the tumor volume and disappearance of the hematoma. CONCLUSIONS: Although it is a large intraosseous schwannoma in the petrous apex, it has a benign nature, its size is reduced due to the hematoma absorption, and the patient is asymptomatic. We observed the patients for 7 years after the diagnosis.
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Hemorragias Intracraneales/diagnóstico por imagen , Neurilemoma/diagnóstico por imagen , Hueso Petroso/diagnóstico por imagen , Adulto , Biopsia , Femenino , Pérdida Auditiva Conductiva/etiología , Humanos , Hemorragias Intracraneales/complicaciones , Imagen por Resonancia Magnética , Neurilemoma/irrigación sanguínea , Neurilemoma/complicaciones , Neurilemoma/patología , Carga TumoralRESUMEN
OBJECTIVE: Spinal dural arteriovenous fistulas (SDAVFs) commonly present with symptoms of myelopathy due to venous congestion in the spinal cord; asymptomatic SDAVFs are rarely encountered. To elucidate the clinical characteristics of asymptomatic SDAVFs, the authors present 5 new cases of asymptomatic SDAVF and report the results of their systematical review of the associated literature. METHODS: Five databases were systematically searched for all relevant English-language articles on SDAVFs published from 1990 to 2018. The clinical features and imaging findings of asymptomatic SDAVFs were collected and compared with those of symptomatic SDAVFs. RESULTS: Twenty cases, including the 5 cases from the authors' experience, were found. Asymptomatic SDAVFs were more prevalent in the cervical region (35.0%); cervical lesions account for only 2% of all symptomatic SDAVFs. The affected perimedullary veins tended to drain more cranially (50.0%) than caudally (10.0%). Four cases of asymptomatic SDAVF became symptomatic, 1 case spontaneously disappeared, and the remaining 15 cases were unchanged or surgically treated. CONCLUSIONS: The higher prevalence of asymptomatic SDAVFs in the cervical spine might be a distinct feature of asymptomatic SDAVFs. Given that venous congestion is the pathophysiology of a symptomatic SDAVF, abundant collateral venous pathways and unique flow dynamics of the CSF in the cervical spine might prevent asymptomatic cervical SDAVFs from becoming symptomatic. In cases in which venous congestion is avoidable, not all asymptomatic SDAVFs will become symptomatic.
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BACKGROUND: Because spinal dural arteriovenous fistulae (SDAVF) are rare and their clinical presentation is nonspecific, they are often overlooked during diagnostic evaluations. Typical magnetic resonance imaging (MRI) findings are intramedullary T2-weighted signal hyperintensity and perimedullary flow voids. There are few reports on the characteristic signs of the cauda equina. We assessed the significance of a new imaging parameter, the cauda equina occupation ratio (CEOR), for the evaluation of SDAVF. METHODS: We retrospectively analyzed the clinical charts and radiological findings of 20 SDAVF patients treated at our institutions. We evaluated sagittal T2-weighted MRI scans and assessed the CEOR, the occupation ratio of the cauda equina compared to the sagittal diameter of the corresponding lumbar spinal canal. The controls were 21 age- and sex-matched subjects. RESULTS: Of the 20 SDAVF, 10 were at the thoracic and 10 at the lumbar spine. There was no significant difference between the preoperative CEOR and the spinal level of the fistulae or the neurological signs. On preoperative MRI scans, the mean CEOR was 56.0 ± 7.8; postoperatively, it was 37.1 ± 7.4 (P = 0.000). The preoperative CEOR was significantly larger in SDAVF patients than in the controls (P = 0.000); postoperatively, it was smaller than in the controls (P = 0.14). CONCLUSIONS: The preoperative CEOR was larger in patients with SDAVF than in the controls. It normalized after successful occlusion of the fistula. Our findings indicate that the CEOR is a useful parameter for the pre- and postoperative evaluation of SDAVF.
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Cauda Equina/diagnóstico por imagen , Malformaciones Vasculares del Sistema Nervioso Central/diagnóstico por imagen , Vértebras Lumbares/diagnóstico por imagen , Médula Espinal/diagnóstico por imagen , Vértebras Torácicas/diagnóstico por imagen , Anciano , Anciano de 80 o más Años , Cauda Equina/cirugía , Malformaciones Vasculares del Sistema Nervioso Central/cirugía , Femenino , Humanos , Vértebras Lumbares/cirugía , Masculino , Persona de Mediana Edad , Cuidados Preoperatorios/métodos , Estudios Retrospectivos , Médula Espinal/cirugía , Vértebras Torácicas/cirugíaAsunto(s)
Malformaciones Vasculares del Sistema Nervioso Central/complicaciones , Cerebelo/irrigación sanguínea , Vértebras Cervicales/patología , Neurilemoma/complicaciones , Neoplasias de la Columna Vertebral/complicaciones , Insuficiencia Vertebrobasilar/etiología , Adulto , Vértebras Cervicales/cirugía , Femenino , Humanos , Neurilemoma/cirugía , Parestesia , Neoplasias de la Columna Vertebral/cirugía , VértigoRESUMEN
OBJECTIVE: The Cobb angle between the lower endplate of C2 and C7 (C2L-C7L angle) is a traditional parameter used for the assessment of the cervical alignment. However, when the lower cervical column is masked by the shoulder, measurements are difficult. In the present study, we inspected 191 X-ray films, measured the Cobb angle between C2L and the endplates at the several levels of the lower cervical column, and assessed their usefulness of such measurements for the determination of cervical sagittal alignment. METHODS: We obtained X-ray films on 191 patients ranging in age from 20 to 93 years. The Cobb angle between C2L and the C7 upper (C7U), the C6 lower (C6L), the C6 upper (C6U), and the C5 lower endplate (C5L) was measured and compared with the C2L-C7L angle. RESULTS: C7L was identified in 116 of 191 patients (60.7%). Except for C2L-C7U angle (P = 0.55), the difference in the mean between C2L-C7L angle and the angle between C2L and the other endplates was statistically significant (P < 0.05). There was a very strong correlation between C2L-C7L angle and C2L-C7U angle (r = 0.99), C2L-C6L angle (r = 0.96), C2L-C6U angle (r = 0.94), and C2L-C5L angle (r = 0.86). CONCLUSIONS: To measure the C2L-C7L angle on unclear X-ray films, C7U can be substituted for C7L. Our measurement data for the C6 and C5 endplates were statistically different; however, the correlation between the C2L-C7L angle and C2L-C6U angle, C2L-C6L angle or C2L-C5L angle was very strong. In patients with unclear lower vertebral bodies, cervical sagittal alignment can be predicted by using adjacent endplates.