Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature.

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol.

CLIPPERS.

We present the clinical case of a woman suffering from CLIPPERS syndrome (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). The images obtained from the brain magnetic resonance show the lesions typical of the disease.

Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: A case report.

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids syndrome (CLIPPERS) is a newly described, underestimated CNS inflammatory disorder involving predominantly the midbrain and the cerebellum. CLIPPERS pathogenesis is largely unknown, and its clinical manifestations are polymorphic and sometimes confounding. Recently clinical, radiological and pathological diagnostic criteria have been proposed to discriminate CLIPPERS from potential mimickers, but the diagnosis still remains challenging. Here we present the case of a patient with radiological findings consistent with CLIPPERS but with atypical clinical presentation, highlighting the importance of a proper diagnostic assessment.

Radiation-induced brain cavernomas in elderly: review of the literature and a rare case report.

Radiation-induced brain cavernomas have been mainly reported in children who underwent radiotherapy for medulloblastoma, leukemia, or low-grade glioma. Otherwise, the "de novo" appearance of a cavernoma in an elderly long-survivor patient after resection and radiotherapy of a glioblastoma is a rare event. We report the case of a 62-year-old female patient who underwent surgical resection of a right temporal glioblastoma, followed by radiation therapy of the operative field and surrounding brain and concomitant adjuvant temozolomide. Four years after the operation, a follow-up Magnetic Resonance revealed a good tumor control and a small round lesion at the superior surface of the right cerebellar hemisphere, close to the margins of the previous irradiation field. The radiological items were consistent with a cavernous angioma. Because of the small size of the malformation and the absence of related symptoms, no treatment was performed. The patient died for tumor progression 86 months after the initial operation, with unchanged cerebellar cavernoma. The occurrence of a cavernous angioma in an elderly patient after radiotherapy for brain glioblastoma is an exceptional event; the distribution of radiotherapy-induced cavernous malformations reported in current literature is presented and the mechanism of their formation is discussed.

Cognitive and functional connectivity alterations in Friedreich's ataxia.

OBJECTIVE: The aim of this study was to perform the first resting-state functional MRI (RS-fMRI) analysis in Friedreich's ataxia (FRDA) patients to assess possible brain functional connectivity (FC) differences in these patients, and test their correlations with neuropsychological performances. METHODS: In total, 24 FRDA patients (M/F: 15/9, mean age 31.3 ± 15.0) and 24 healthy controls (HC; M/F: 15/9, mean age 30.7 ± 15.5) were enrolled in this cross-sectional study. All patients underwent a thorough neuropsychological battery, investigating different cognitive domains. RS-fMRI data were analyzed using a seed-based approach, probing the FC of cortical areas potentially referable to specific executive and cognitive functions compromised in FRDA. RESULTS: Compared to HC, FRDA patients showed overall worse neuropsychological scores in several domains, including global cognitive assessment, spatial memory, visuoperception and visuospatial functions, and executive functions. Analysis of RS-fMRI data showed a higher FC in FRDA patients compared to HC between paracingulate gyri and the medial frontal gryrus, between the superior frontal gyrus and bilateral angular gyri, and between the middle temporal gyrus and the cingulate gyrus, with a reduced FC between the medial frontal gryrus and the cerebellum. INTERPRETATION: We found a reduction in FC between frontal areas and the contralateral cerebellar cortex in FRDA, in line with the known alteration in cerebello-cortical pathway in this condition. On the other hand, a higher FC between different cortical areas was shown, possibly reflecting a compensatory phenomenon. These results, in conjunction with clinical findings, may shed new light on the pattern of supratentorial and infratentorial involvement, and on dynamics of brain plasticity in this disease.

Humongous right atrial lipoma: a correlative CT and MR case report.

A 66-year-old man was referred to our department for further investigation of a right atrial mass incidentally discovered on ultrasound examination (US). US showed an oval mass arising from the free wall of the right atrium and projecting into the atrial cavity. The mass was hypoechoic and had a broad base of attachment on the free wall of the right atrium. This case was referred to our department in order to perform an MR cardiac examination. Our diagnosis was supported by a CT scan study that confirmed the diagnosis of a cardiac lipoma subsequently confirmed at surgery.

Xanthogranulomatous pyelonephritis mimicking a renal cell carcinoma: a unique and challenging case.

We describe an unusual case of xanthogranulomatous pyelonephritis (XGPN) in a 73-year-old woman diagnosed after a blunt abdominal trauma. This case is unique because of the atypical presentation, with absence of symptoms, normal laboratory exams, and unusual computed tomography and magnetic resonance imaging findings. The patient underwent radical nephrectomy because a renal cystic tumor was suspected. Only the histopathological findings suggested the final diagnosis of XGPN.

A spontaneous strangulated transomental hernia: Prospective and retrospective multi-detector computed tomography findings.

Transomental hernias are among the rarest type of all internal hernias which overall account for less than 6% of small bowel obstructions. Most transomental hernias occurring in adults are either iatrogenic or post-traumatic. More rarely, a spontaneous herniation of small bowel loops may result from senile atrophy of the omentum. We report a case of an 86-year-old male who presented with signs and symptoms of small bowel obstruction but had no past surgical or traumatic abdominal history. At contrast-enhanced multi-detector row computed tomography (CT), a cluster of fluid-filled dilated small bowel loops could be appreciated in the left flank, with associated signs of bowel wall ischemia. Swirling of the mesenteric vessels could also be appreciated and CT findings were prospectively considered consistent with a strangulated small bowel volvulus. At laparotomy, no derotation had to be performed but up to 100 cm of gangrenous small bowel loops had to be resected because of a transomental hernia through a small defect in the left part of the greater omentum. Retrospective reading of CT images was performed and findings suggestive of transomental herniation could then be appreciated.

Primary signet ring cell carcinoma of the appendix mimicking acute appendicitis.

Primary signet ring cell carcinoma of the appendix is a very rare neoplasm that usually presents with signs and symptoms of acute appendicitis and in particular with a right lower abdominal pain. Preoperative imaging detection of appendiceal adenocarcinoma has an important value because it may result in an appropriate surgical procedure. We report a rare case of primary signet ring cell carcinoma of the vermiform appendix in an 80-year-old man who was misdiagnosed on computed tomography (CT) scan as acute appendicitis.

Modifications of brain tissue volumes in facioscapulohumeral dystrophy.

Facioscapulohumeral muscular dystrophy (FSHD), a pathology primarily characterized by involvement of the muscles in the face, shoulder and upper arm, can be associated to several CNS disorders, including sensorineural hearing deficits, schizophrenia, epilepsy and mental retardation. Aim of our study was to verify if brain tissue volumes, as measured by segmentation of MRI studies, are altered in FSHD. Volumes of gray matter (GM), white matter (WM), and cerebrospinal fluid (CSF) were compared, taking into account head size age and sex, both globally (by multiple regression analysis) and regionally (by optimized voxel-based morphometry-VBM) in thirty patients with FSHD and 39 normal subjects (NS). FSHD patients had significantly lower GM volumes and higher CSF volumes (P < 10(-4)). GM loss showed a borderline correlation with clinical severity (P < 0.05). Brain tissue volumes did not correlate with disease duration, size of the genetic deletion, age at onset and the presence at MRI of WM hyperintensities (detected in 4/22 patients). At VBM three clusters of GM loss were detected, in the left precentral cortex (Brodmann areas 6, 2 and 44, P < 10(-14) corrected for multiple comparisons at cluster level), in the anterior cingulate (Brodmann areas 33, 24 and 11, P < 10(-4)) and in the right fronto-polar region (Brodmann area 10, P < 5.10(-3)). To the best of our knowledge, this is the first study to demonstrate a reduction in GM volume in FSHD. We hypothesize that localized GM loss in FSHD is the consequence of a selective involvement of specific CNS structures.

Giant infiltrating lipoma of the face: CT and MR imaging findings.

Infiltrating lipoma is an uncommon mesenchymal neoplasm that characteristically infiltrates adjacent tissues and tends to recur after excision. This type of lipoma is extremely rare in the head and neck region. We report a case of a giant infiltrating lipoma of the face, studied with CT and MR imaging.

Novel methodology for the archiving and interactive reading of clinical magnetic resonance spectroscopic imaging.

Archiving clinical magnetic resonance spectroscopic imaging (MRSI) data and presenting the data to specialists (e.g., neuroradiologists, neurosurgeons, neurologists, neuro-oncologists, and MR scientists) who work in different physical locations is a practical problem of significance. This communication describes a novel solution. The study hypothesis was that it is possible to use widely available distributed computing techniques to create a clinical MRSI user interface addressable from any personal computer with a suitable network connection. A worldwide web MRSI archive and interface system was created that permits the user to interactively view individual MRSI voxel spectra with correlation to MR images and to parametric spectroscopic images. Web browser software (i.e., Netscape and Internet Explorer) permits users in various physical locations to access centrally archived MRSI data using a variety of operating systems and client workstations. The system was used for archiving and displaying more than 1000 clinical MRSI studies performed at the authors' institution. The system also permits MRSI data to be viewed via the Internet from distant locations worldwide. The study illustrates that widely available software operating within highly distributed electronic networks can be used for archiving and interactive reading of large amounts of clinical MRSI data.

Small bowel metastases of lung cancer as unique metastatic lesions: CT, MRI and small bowel enema findings. a case report.

Small bowel metastases of lung cancer as unique secondary lesions are a very rare occurrence and may be clinically missed due to the aspecificity of the symptoms. Diagnosis is usually made at acute abdominal symptomatology that requires emergency surgical treatment. We report a case of 69-year-old woman, previously treated for epidermoid lung carcinoma, complaining only of aspecific asthenia; blood cell count and chemistry showed a moderate but progressive anemia; no signs of small bowel occlusion were present. The follow-up CT scan showed two large masses at the small bowel level, without any evidence of hepatic, lung, adrenal or brain metastases. MRI and small bowel enema confirmed the presence of the masses, and the diagnosis of small bowel metastases was hypothesized. Surgical specimens of the masses confirmed the radiological suspicion.

Recrudescence of pulmonary tuberculosis: radiological and CT features in an asymptomatic Southern Italian young population.

AIM: The authors report the results observed in apparently healthy young adults undergoing chest radiography for pre-employment screening. MATERIALS AND METHODS: Between July 1996 and December 1997, 2292 young adults underwent conventional chest radiography for pre-employment screening purposes. Of these, 378 subjects (16.4%) in whom the chest X-ray showed alterations indicative for pulmonary parenchymal lesions were selected for the study. The lesions were divided into four groups: 1) single nodular opacity (n=98/378=25.9%); 2) multiple nodular opacities (n=25/378=6.6%); 3) single or multiple parenchymal consolidation(s) (n=203/378= 53.7%); 4) interstitial disease ( %). All subjects received a tuberculin skin test. The subjects in groups 1, 2 and 3 underwent the diagnostic protocol, which consisted of tuberculin skin test (TST) and CT scan for groups 1 and 2, and a repeat chest radiograph at 30 days for group 3. The subjects in group 4 were studied by high-resolution CT (HRCT). RESULTS: In group 1, CT showed nodular opacities with psammomatous calcifications in 13 cases, vascular dilatations in 3, calcified nodule in 7 subjects with positive TST, and uncalcified nodular lesions in 75 subjects (53/75 with positive TST). In group 2, FNAB confirmed the preliminary diagnosis of metastases in 2 cases; CT demonstrated the pleural origin of opacities in 1 patient, and multiple nodular lesions in 12 subjects; it invalidated the radiographic findings in 3 subjects, and demonstrated multiple nodules associated to increased interstitial thickening in 3 subjects. In group 3, the lung alterations had completely disappeared in 106 subjects, whereas in 97 subjects there was reduced extension of the previously observed lesions, but appearance of new sites of parenchymal consolidation. In group 4 CT confirmed the radiographic suspicion in 14 cases. CONCLUSIONS: Tubercular infection is currently increasing in industrialised countries. In our study it showed a relatively high prevalence of about 4.5% in a young asymptomatic population.