Acute disseminated encephalomyelitis presenting as fever of unknown origin: case report

BACKGROUND: Fever of unknown origin (FUO) can be defined as a body temperature higher than 38.3°C on several occasions over more than 3 weeks, the diagnosis of which remains uncertain after 1 week of evaluation. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system with a wide range of clinical manifestations. The highest incidence of ADEM is observed during childhood and it usually occurs following a viral or bacterial infection or, more rarely, following a vaccination, or without a preceding cause. CASE PRESENTATION: Here, we describe an atypical case of ADEM that initially manifested as several weeks of FUO in a fifteen years old boy. CONCLUSIONS: This case report suggests a new possible syndromic association between ADEM and FUO, which should be considered in the clinical examination of patients with FUO, especially in the presence of also modest neurologic or neuropsychiatric symptoms

Multiple vertebral hemangiomas of the thoracic spine with atypical radiological features and aggressive behavior causing myelopathy: A case report

Abstract Background Vertebral Haemangiomas (VHs) are frequent and generally asymptomatic benign tumors, involving the spine, usually incidentally found on computed tomography and magnetic resonance. Despite being usually asymptomatic and quiescent lesions, VHs can occasionally manifest aggressive features, leading to clinical manifestations such as back pain and neurological deficits. Case report. We report a case of a 54-year-old man, presented with 5 months history of pain, associated with lower limbs paraesthesia and weakness, gait disturbance and episodes of accidental falls. Radiological evaluation by spine pre- and post-contrast MRI indicated multiple vertebral hypervascular lesions, compatible with haemangiomas, involving from T3 to T11 levels, showing several different features (typical and atypical); aggressive haemangioma radiological pattern may be valuable at T3 and T4 vertebras. A thoracic spine pre- and post-contrast computed tomography confirmed the radiological diagnosis of multiple aggressive haemangiomas. Discussion Aggressive VH consists in a very rare subset of vertebral haemangiomas characterized by a greater tendency in being symptomatic. They may show atypical radiological features, that make their diagnosis very complex. In the recent years, many strategies for treatment of symptomatic or aggressive VHs have been developed, but the optimal treatment strategy is still controversial. Conclusion Although aggressive VHs being extremely rare, recognizing radiological features of these lesions is mandatory to achieve a correct diagnosis and appropriate therapeutic targets

ADC Benchmark Range for Correct Diagnosis of Primary and Recurrent Middle Ear Cholesteatoma

Objectives. Magnetic resonance imaging (MRI) and in particular diffusion-weighted imaging (DWI) have been broadly proven to be the reference imaging method to discriminate between cholesteatoma and noncholesteatomatous middle ear lesions, especially when high tissue specificity is required. The aim of this study is to define a range of apparent diffusion coefficient (ADC) values within which the diagnosis of cholesteatoma is almost certain. Methods. The study was retrospectively conducted on a cohort of 124 patients. All patients underwent first- or second-look surgery because primary or secondary acquired cholesteatoma was clinically suspected; they all had preoperative MRI examination 15 days before surgery, including DWI from which the ADC maps were calculated. Results. Average ADC value for cholesteatomas was 859,4 × 10−6 mm2/s (range 1545 × 10−6 mm2/s; IQR = 362 × 10−6 mm2/s; σ = 276,3 × 10−6 mm2/s), while for noncholesteatomatous inflammatory lesions, it was 2216,3 × 10−6 mm2/s (range 1015 × 10−6 mm2/s; IQR = 372,75 × 10−6 mm2/s; σ = 225,6 × 10−6 mm2/s). Interobserver agreement with Fleiss’ Kappa statistics was 0,96. No overlap between two groups’ range of values was found and the difference was statistically significant for p<0.0001. Conclusions. We propose an interval of ADC values that should represent an appropriate benchmark range for a correct differentiation between cholesteatoma and granulation tissue or fibrosis of noncholesteatomatous inflammatory lesions

Diagnostic accuracy of short-time inversion recovery sequence in Graves' ophthalmopathy before and after prednisone treatment

Introduction: In Graves' Ophthalmopathy, it is important to distinguish active inflammatory phase, responsive to immunosuppressive treatment, from fibrotic unresponsive inactive one. The purpose of this study is, first, to identify the relevant orbital magnetic resonance imaging signal intensities before treatment, so to classify patients according to their clinical activity score (CAS), discriminating inactive (CAS3) subjects and, second, to follow post-steroid treatment disease. Methods: An observational study was executed on 32 GO consecutive patients in different phases of disease, based on clinical and orbital Magnetic Resonance Imaging parameters, compared to 32 healthy volunteers. Orbital Magnetic Resonance Imaging was performed on a 1.5 tesla Magnetic Resonance Unit by an experienced neuroradiologist blinded to the clinical examinations. Results: In pre-therapy patients, compared to controls, a medial rectus muscle statistically significant signal intensity ratio (SIR) in short-time inversion recovery (STIR) (long TR/TE) sequence was found, as well as when comparing patients before and after treatment, both medial and inferior rectus muscle SIR resulted significantly statistically different in STIR. These increased outcomes explain the inflammation oedematous phase of disease, moreover after steroid administration, compared to controls; patients presented lack of that statistically significant difference, thus suggesting treatment effectiveness. Conclusion: In our study, we proved STIR signal intensities increase in inflammation oedematous phase, confirming STIR sequence to define active phase of disease with more sensibility and reproducibility than CAS alone and to evaluate post-therapy involvement. © 2014 Springer-Verlag

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

The “chameleon” sellar lesions: a case report of unexpected sellar lesions

IntroductionThe sellar region and its boundaries represent a challenging area, harboring a variety of tissues of different linings. Therefore, a variety of diseases can arise or involve in this area (i.e., neoplastic or not). A total of three challenging cases of “chameleon” sellar lesions treated via EEA were described, and the lesions mimicked radiological features of common sellar masses such as craniopharyngiomas and/or pituitary adenomas, and we also report a literature review of similar cases.MethodsA retrospective analysis of three primary cases was conducted at the Università degli Studi di Napoli Federico II, Naples, Italy. Clinical information, radiological examinations, and pathology reports were illustrated.ResultsA total of three cases of so-called “chameleon” sellar lesions comprising two men and one woman were reported. Based on the intraoperative finding and pathological examination, we noticed that case 1 had suprasellar glioblastoma, case 2 had a primary neuroendocrine tumor, and case 3 had cavernous malformation.ConclusionNeurosurgeons should consider “unexpected” lesions of the sellar/suprasellar region in the preoperative differential diagnosis. A multidisciplinary approach with the collaboration of neurosurgeons, neuroradiologists, and pathologists plays a fundamental role. The recognition of unusual sellar lesions can help surgeons with better preoperative planning; so an endoscopic endonasal approach may represent a valid surgical technique to obtain decompression of the optic apparatus and vascular structures and finally a pathological diagnosis

Internal jugular vein fenestration: a rare but possible event. A case report and review of the literature

While fenestration and duplication are relatively common in the arteries, they are extremely rare in the venous compartment: internal jugular vein fenestration has been reported occurring in 0.4% of unilateral neck dissections. Familiarity with these morphological anomalies is important for the radiologist and for the surgeon to prevent neurovascular injury, especially in neck surgery and interventional catheterization. We present the case of a patient harboring a fenestration of the left internal jugular vein, diagnosed by magnetic resonance angiography, and a systematic review of the literature. To our knowledge, from 1985 until 2016 only 36 patients (including the present) were diagnosed as having an internal jugular vein morphological anomaly. Out of 36 patients, only 11 (30,5%) were diagnosed using radiological imaging; the high rate of intra-operative diagnoses (22/36, 62,5%) is likely related to the limited use of diagnostic imaging or to misdiagnosis/misinterpretation of a relatively unknown and rare morphological anomaly. A contrast enhanced computed tomography or magnetic resonance angiography should be considered in case of vascular procedures in a patient with known internal jugular vein anomaly

Similarities and differences between younger and older disease onset patients with newly diagnosed systemic lupus erythematosus

Objectives: Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. Methods: We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18-45 years) and late onset (&gt;45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. Results: Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and &gt;45 years), except for the higher frequency of discoid rash in the group with age at diagnosis &gt;45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis &gt;45 years (20.4% vs. 5.9%, p=0.009) compared to their counterparts, while renal involvement was more frequent in the group with age at diagnosis 18-45 years (21.4% vs. 6.1%, p=0.03).A sub analysis at 36 months on patients without hypertension and osteoporosis at enrollment showed that patients with older age at onset had a higher frequency of these comorbidities, compared to their counterparts. Conclusions: In our cohort, younger disease SLE onset seems to correlate with a more active immunological profile, while late onset with a higher incidence of comorbidities