Tension Pneumocephalus With Acute Cerebellar Symptoms Due to an Intradiploic Epidermoid Cyst Eroding the Mastoid Bone.

This case report presents a unique presentation of an intradiploic epidermoid cyst (IDEC) in a 55-year-old female. She presented with acute cerebellar symptoms triggered by a Valsalva maneuver. IDECs are a rare type of intracranial epidermoid cysts. They are benign and have a slow growth pattern that translates into progressively developing symptoms instead of acute symptoms. Symptoms include local deformities, focal neurologic deficits, and pain. This patient developed acute cerebellar symptoms due to erosion of the mastoid bone that created a pathway between the eustachian tube and the intracranial space via the mastoid air cells. Consequently, tension pneumocephalus emerged via a ball-valve effect that caused a significant mass effect in the posterior fossa. Surgical resection of the IDEC and closing of the mastoid air cells resulted in symptom relief by restoring the integrity of the intracranial-extracranial barrier. This case highlights that a higher level of vigilance is warranted for an IDEC in the proximity of aerated bone structures, such as the mastoid air cells and the paranasal sinuses, and that a more proactive approach is advocated.

Longitudinal MRI in the context of in utero surgery for open spina bifida: A descriptive study.

INTRODUCTION: Fetal surgery for open spina bifida (OSB) requires comprehensive preoperative assessment using imaging for appropriate patient selection and to evaluate postoperative efficacy and complications. We explored patient access and conduct of fetal magnetic resonance imaging (MRI) for prenatal assessment of OSB patients eligible for fetal surgery. We compared imaging acquisition and reporting to the International Society of Ultrasound in Obstetrics and Gynecology MRI performance guidelines. MATERIAL AND METHODS: We surveyed access to fetal MRI for OSB in referring fetal medicine units (FMUs) in the UK and Ireland, and two NHS England specialist commissioned fetal surgery centers (FSCs) at University College London Hospital, and University Hospitals KU Leuven Belgium. To study MRI acquisition protocols, we retrospectively analyzed fetal MRI images before and after fetal surgery for OSB. RESULTS: MRI for fetal OSB was accessible with appropriate specialists available to supervise, perform, and report scans. The average time to arrange a fetal MRI appointment from request was 4 ± 3 days (range, 0-10), the average scan time available was 37 ± 16 min (range, 20-80 min), with 15 ± 11 min (range, 0-30 min) extra time to repeat sequences as required. Specific MRI acquisition protocols, and MRI reporting templates were available in only 32% and 18% of units, respectively. Satisfactory T2-weighted (T2W) brain imaging acquired in three orthogonal planes was achieved preoperatively in all centers, and 6 weeks postoperatively in 96% of FSCs and 78% of referring FMUs. However, for T2W spine image acquisition referring FMUs were less able to provide three orthogonal planes presurgery (98% FSC vs. 50% FMU, p < 0.001), and 6 weeks post-surgery (100% FSC vs. 48% FMU, p < 0.001). Other standard imaging recommendations such as T1-weighted (T1W), gradient echo (GE) or echoplanar fetal brain and spine imaging in one or two orthogonal planes were more likely available in FSCs compared to FMUs pre- and post-surgery (p < 0.001). CONCLUSIONS: There was timely access to supervised MRI for OSB fetal surgery assessment. However, the provision of images of the fetal brain and spine in sufficient orthogonal planes, which are required for determining eligibility and to determine the reversal of hindbrain herniation after fetal surgery, were less frequently acquired. Our evidence suggests the need for specific guidance in relation to fetal MRI for OSB. We propose an example guidance for MRI acquisition and reporting.

Neurocysticercosis: An Uncommon Cause of Acute Supratentorial Hydrocephalus.

We report a 29-year-old woman with acute supratentorial hydrocephalus due to intraventricular neurocysticercosis (NC). Aqueductal stenosis due to web formation and a free floating intraventricular cyst with scolex were pathognomonic and led to the diagnosis of NC. Worldwide, NC is the most important parasitic infection of the central nervous system but is very uncommon in non-endemic regions. Intraventricular abnormalities occur in approximately 30% of the patients. Magnetic resonance imaging (MRI) plays a crucial role in the diagnostic work-up and in guiding intervention. Teaching Point: Brain magnetic resonance imaging in intraventricular neurocysticercosis is pathognomonic and essential in guiding treatment.

Optic Neuropathy Revealing Severe Superficial Siderosis in the Setting of Long-standing Low-grade Intracranial Neoplasm.

Two cases of optic neuropathy due to superficial siderosis (SS) are reported in two patients, aged 29 and 38 years, operated for intracranial neoplasms, the first one with a desmoplasic infantile ganglioglioma excised in 1991, and the other one with a pilocytic astrocytoma, operated on in 1997, 1998 and 2016. Both patients presented with progressive loss of visual acuity, as a result of bilateral optic nerve atrophy, as well as unsteadiness, ataxic gait and hearing loss. Magnetic resonance imaging (MRI) of the brain and spine, including gradient echo (GRE) T2-weighted acquisitions, revealed thin optic nerves and strong hypointensity with susceptibility artefacts corresponding to haemosiderin deposits within the meningeal layers of the spine, the infra- and supratentorial spaces of the brain and the peri-optic sheaths in both patients. The cerebrospinal fluid (CSF) was macroscopically haemorrhagic in one patient, who underwent a dynamic myelography, which failed to reveal any trans-dural CSF leakage. Neuro-ophthalmological symptoms due to SS, such as visual acuity loss, have been scarcely reported. MRI using GRE T2-weighted sequences highlighting the presence of haemosiderin deposits plays a key role in the diagnosis of this condition. Treatment should aim at preventing haemosiderin deposition by treating the cause of the subarachnoid bleeding.

Two Cases Presenting With Unilateral Adduction Deficit Associated With Human Adenosine Deaminase 2 Deficiency.

Deficiency of human adenosine deaminase 2 (DADA2) is an auto-inflammatory inborn error of immunity caused by biallelic deleterious mutations in the gene encoding ADA2. The purpose of this article is to raise awareness among ophthalmologists and pediatricians to consider DADA2 as a possible diagnosis for patients with acute onset of diplopia. The authors describe two pediatric patients who presented with double vision due to uni-lateral adduction deficit, and discuss the importance of recognizing this clinically as an ophthalmologist. If a child presents with a sudden eye movement abnormality, ophthalmologists must be aware of the possibility of an ischemic insult due to an underlying genetic disorder (eg, DADA2), especially in patients with a positive familial history or associated clinical signs such as a personal history of characteristic skin lesions or paresis of other cranial nerves. Given the multi-organ involvement in this disorder, a multi-disciplinary approach is crucial to have a timely diagnosis and to treat this rare disorder appropriately. [J Pediatr Ophthalmol Strabismus. 2021;58(4):e22-e26.].

An update on congenital melanocytic nevus syndrome: A case report and literature review.

Congenital melanocytic nevus syndrome (CMNS) is a rare condition characterized by pigmented skin lesions that are usually present at birth and are associated with an increased risk of neurological abnormalities and malignant melanoma. It mostly results from a post-zygotic NRAS mutation of neural-derived crest cells, leading to uncontrolled cell growth. Because of the increased knowledge of the genetics underlying CMNS, targeted therapy becomes a promising treatment option. We present a case of CMNS in a newborn. Physical examination at birth showed a giant congenital melanocytic nevus, extending from the occipital to the lower lumbar region. A magnetic resonance imaging scan revealed multiple cerebral and cerebellar parenchymal lesions. Genetic analysis of the cutaneous lesions showed the presence of an NRAS Q61R mutation. The patient was treated with dermabrasion to reduce the color intensity of the nevus. However, this was complicated by recurrent wound infections and laborious wound healing. At the age of 1 year, the patient had an age-appropriate psychomotor development, without neurological deficits.

Cortical spectral matching and shape and volume analysis of the fetal brain pre- and post-fetal surgery for spina bifida: a retrospective study.

PURPOSE: A retrospective study was performed to study the effect of fetal surgery on brain development measured by MRI in fetuses with myelomeningocele (MMC). METHODS: MRI scans of 12 MMC fetuses before and after surgery were compared to 24 age-matched controls without central nervous system abnormalities. An automated super-resolution reconstruction technique generated isotropic brain volumes to mitigate 2D MRI fetal motion artefact. Unmyelinated white matter, cerebellum and ventricles were automatically segmented, and cerebral volume, shape and cortical folding were thereafter quantified. Biometric measures were calculated for cerebellar herniation level (CHL), clivus-supraocciput angle (CSO), transverse cerebellar diameter (TCD) and ventricular width (VW). Shape index (SI), a mathematical marker of gyrification, was derived. We compared cerebral volume, surface area and SI before and after MMC fetal surgery versus controls. We additionally identified any relationship between these outcomes and biometric measurements. RESULTS: MMC ventricular volume/week (mm3/week) increased after fetal surgery (median: 3699, interquartile range (IQR): 1651-5395) compared to controls (median: 648, IQR: 371-896); P = 0.015. The MMC SI is higher pre-operatively in all cerebral lobes in comparison to that in controls. Change in SI/week in MMC fetuses was higher in the left temporal lobe (median: 0.039, IQR: 0.021-0.054), left parietal lobe (median: 0.032, IQR: 0.023-0.039) and right occipital lobe (median: 0.027, IQR: 0.019-0.040) versus controls (P = 0.002 to 0.005). Ventricular volume (mm3) and VW (mm) (r = 0.64), cerebellar volume and TCD (r = 0.56) were moderately correlated. CONCLUSIONS: Following fetal myelomeningocele repair, brain volume, shape and SI were significantly different from normal in most cerebral layers. Morphological brain changes after fetal surgery are not limited to hindbrain herniation reversal. These findings may have neurocognitive outcome implications and require further evaluation.

Posterior reversible encephalopathy syndrome possibly induced by pemetrexed maintenance therapy for lung cancer: a case report and literature review.

Introduction Advances in systemic chemotherapy, molecular targeted therapy and immunotherapy have extended and improved the quality of life of patients with cancer. However, the central nervous system is very susceptible to complications of systemic cancer and its treatment. Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and neuroradiologic entity which has garnered increasing recognition in the past two decades. Cancer patients are generally treated with cytotoxic agents, immunotherapy, molecular targeted therapies or glucosteroids which are more frequently associated with PRES. Case presentation A 59-year old female, known with a relapse of her lung adenocarcinoma, had been treated with 4 cycles of cisplatin (75 mg/m²) and pemetrexed (500 mg/m²). Six weeks after this combination chemotherapy and within 28 h after the administration of pemetrexed maintenance therapy, she developed a generalised epileptic insult. Magnetic resonance imaging (MRI) of the brain showed bilateral areas of increased signal intensity in the subcortical parietal and frontal white matter. She was treated with a broad spectrum antiseizure drug, levetiracetam 750 mg twice daily and strict control of blood pressure. Discussion Diagnosis of PRES should be considered in all patients with neurologic symptoms who are at risk to develop PRES. It is crucial to establish the diagnosis as soon as possible since there is no specific treatment of PRES other than correction of the underlying risk factors and preventing seizure recurrence. Administration of pemetrexed is a possible risk factor for the development of PRES.

Correction to: Secondary B-cell lymphoma associated with the Epstein-Barr virus in chronic lymphocytic leukemia patients.

[This corrects the article DOI: 10.1007/s12308-016-0273-8.].

The dentato-rubro-olivary pathway revisited: New MR imaging observations regarding hypertrophic olivary degeneration.

Hypertrophic olivary degeneration (HOD) following a lesion of the dentato-rubro-olivary pathway (DROP) is a well-known imaging finding and has extensively been described in the recent literature. We reviewed our patients with HOD as a result of a lesion of the DROP in order to analyze the disruption of the DROP and the resulting HOD in comparison with the literature. We observed unusual imaging findings in four patients. In two patients it concerned new observations related to the timing and imaging appearances of HOD. HOD became only visible 6 years after a lesion in the red nucleus in one patient and a cystic degeneration of the olivary nucleus was seen 3 years after the HOD in a second patient. In two patients we found HOD that could only be explained by the existence of an afferent feedback loop between the dentate nucleus and the inferior olivary nucleus and by the knowledge that these fibers run through the ipsilateral olivary nucleus before ending in the contralateral olivary nucleus. In one of these patients the lesion was located in the inferior cerebellar peduncle. In the other patient the lesion was located on the midline in the medulla oblongata. The imaging findings in these patients reveal new observations in the stages of imaging appearances in HOD and shed light on the forgotten dentato-olivary afferent feedback loop of the DROP. Clin. Anat. 30:543-549, 2017. © 2017 Wiley Periodicals, Inc.

10-year follow-up after implantation of the Bryan Cervical Disc Prosthesis.

PURPOSE: Cervical arthroplasty is being used as an alternative for cervical fusion, but long-term follow-up results have rarely been reported. In this paper, we present 10-year follow-up results after implantation of the Bryan Cervical Disc Prosthesis in a single center. METHODS: 89 patients underwent implantation of a single-level Bryan Cervical Disc Prosthesis to treat radiculopathy and/or myelopathy. Clinical (Neurological Success, Neck Disability Index (NDI), Neck- and Arm-Pain, and SF-36) and radiological follow-up was prospectively organized up to 10 years after surgery. Adverse events and second surgeries were recorded and evaluated. RESULTS: Ten-year follow-up data were available for 72 (81%) patients. Maintenance or improvement of the neurological state was seen in 89% of patients after 10-year follow-up. SF-36 PCS scores improved significantly at all follow-up points. SF-36 MCS improvement was significant at 4 and 6 year, but not at 8- and 10-year follow-up. Significant improvement for NDI, and Neck- and Arm-Pain scores was found for the subgroup of patients in whom these data were available. Mean angular motion of the prosthesis at 10-year follow-up was 8.6°. Mobility of the device, defined as >2° of angular motion, was reached in 81% of patients. During the study period, 21 patients (24%) developed new or recurrent radiculopathy or myelopathy, the majority of these being treated conservatively. Seven patients (8%) required 8 additional spine surgeries to treat persistent or recurrent symptoms. Of these, 2 patients (2%) were reoperated at the index level and at 5 (6%) an adjacent level. CONCLUSION: In this study, favorable long-term clinical outcome after implantation of the Bryan Cervical Disc Prosthesis was seen, with the majority of prostheses remaining mobile after 10-year follow-up. However, still 6% of patients required adjacent level surgery.

Secondary B-cell lymphoma associated with the Epstein-Barr virus in chronic lymphocytic leukemia patients.

Up to 10 % of chronic lymphocytic leukemia (CLL) patients present with aggressive secondary B-cell lymphoma (most frequently diffuse large B-cell lymphoma, DLBCL) which may be clonally related to the CLL (i.e., Richter transformation, RT, 80 % of the cases) or de novo (20 % of the cases). Several genetic lesions associated with RT have already been identified, but the potential role of the Epstein-Barr virus (EBV) has been largely overlooked. In this study, we describe six CLL patients who developed a secondary EBV-positive (EBV+) B-cell lymphoma (five DLBCL, one Hodgkin lymphoma) and compare their clinicopathological characteristics to ten CLL patients with EBV-negative (EBV-) secondary B-cell lymphomas (all DLBCL). All 16 patients had a history of iatrogenic immunosuppression or chemotherapy. Eighty percent had received fludarabine as part of the CLL treatment. Most secondary lymphomas were clonally related to the previous CLL (3/4 EBV+, 7/7 EBV- cases tested). Notably EBV+ RT was associated with a trend for older age at onset (median 72 vs. 63 years, p value >0.05), longer interval between CLL and RT diagnosis (median 4.2 vs. 2.9 years, p value >0.05), and shorter overall survival (median 4 vs. 10 months, p value >0.05). These differences were not significant, probably due to small sample size. Immunohistochemical profiling suggested more frequent overexpression of TP53 and MYC in EBV- compared to EBV+ secondary lymphoma. Based on this small retrospective single center series, we hypothesize that EBV+ RT may constitute a separate subgroup of RT. Larger series are required to validate this suggestion.

Giant arachnoid granulations mimicking pathology. A report of three cases.

We describe three cases of incidentally found lesions in the dural venous sinuses on magnetic resonance imaging, that other had erroneously considered pathological entities. We made the diagnosis of giant arachnoid granulations. The differential diagnosis with thrombosis or intrasinusal tumoral lesions was easily made on the basis of three typical radiological features of the granulations: the hyperintensity of the lesions on FLAIR, a blood vessel within the lesion and bone erosion.

Integrating diffusion kurtosis imaging, dynamic susceptibility-weighted contrast-enhanced MRI, and short echo time chemical shift imaging for grading gliomas.

BACKGROUND: We assessed the diagnostic accuracy of diffusion kurtosis imaging (DKI), dynamic susceptibility-weighted contrast-enhanced (DSC) MRI, and short echo time chemical shift imaging (CSI) for grading gliomas. METHODS: In this prospective study, 35 patients with cerebral gliomas underwent DKI, DSC, and CSI on a 3 T MR scanner. Diffusion parameters were mean diffusivity (MD), fractional anisotropy, and mean kurtosis (MK). Perfusion parameters were mean relative regional cerebral blood volume (rrCBV), mean relative regional cerebral blood flow (rrCBF), mean transit time, and relative decrease ratio (rDR). The diffusion and perfusion parameters along with 12 CSI metabolite ratios were compared among 22 high-grade gliomas and 14 low-grade gliomas (Mann-Whitney U-test, P < .05). Classification accuracy was determined with a linear discriminant analysis for each MR modality independently. Furthermore, the performance of a multimodal analysis is reported, using a decision-tree rule combining the statistically significant DKI, DSC-MRI, and CSI parameters with the lowest P-value. The proposed classifiers were validated on a set of subsequently acquired data from 19 clinical patients. RESULTS: Statistically significant differences among tumor grades were shown for MK, MD, mean rrCBV, mean rrCBF, rDR, lipids over total choline, lipids over creatine, sum of myo-inositol, and sum of creatine. DSC-MRI proved to be the modality with the best performance when comparing modalities individually, while the multimodal decision tree proved to be most accurate in predicting tumor grade, with a performance of 86%. CONCLUSIONS: Combining information from DKI, DSC-MRI, and CSI increases diagnostic accuracy to differentiate low- from high-grade gliomas, possibly providing diagnosis for the individual patient.