A Giant Parathyroid Adenoma Presenting With Parathyroid Crisis.

Giant parathyroid adenomas (GPA) are a benign cause of primary hyperparathyroidism (PHPT) that might present similarly to parathyroid carcinomas (PC). Rarely, PHPT can present with a parathyroid crisis, a life-threatening decompensation with severe hypercalcemia.  A 77-year-old woman presented with lethargy and muscle weakness. Investigation revealed parathyroid hormone-dependent hypercalcemia and an enlarged parathyroid measuring 31x24 mm. The patient was submitted for parathyroidectomy. Histology showed no evidence of malignancy, confirming a GPA.  We report a GPA presenting with a parathyroid crisis. The clinical picture mimicked that of a PC. There are no clinical, analytical, or imagiological features pathognomonic of PC.

Comparison Between Clinical and Pathological Staging After Elective Neck Dissection in Head and Neck Cancer.

INTRODUCTION: Head and neck squamous cell carcinomas (HNSCC) are the most common malignancies in the head and neck. Previous studies have shown discrepancies in clinical and pathological staging, with a significant number of head and neck cancer patients who were not correctly staged. This has important implications regarding the treatment and prognosis of these patients. The aim of this study was to analyze potential disagreements in clinical and pathological staging in patients with head and neck cancer who underwent elective neck dissection. METHODS: A retrospective study of patients with squamous cell carcinoma of the head and neck, who underwent elective neck dissection, between January 2018 and December 2020. RESULTS: We analyzed 87 patients, with an average age of 64 ± 10.05 years, of whom 96.6% were male. The primary tumor location was the glottis (31%), oropharynx (26.4%), hypopharynx (19.5%), supraglottis (11.5%), and oral cavity (11.5%). In 87.3% there was a history of smoking and/or drinking. Pathological N (pN) staging was higher than clinical staging in 34.3% of patients (N1 in 22.9%; N2 in 8%; N3 in 3.4%). There were no significant differences between the number of nodes removed and the pN staging. However, there was a significant survival difference in patients with>15 nodes removed (p=0.05). There was also a significant difference in patients with pN up-staging regarding survival (p=0.005). Pathological T staging was different from clinical T staging (p<0.05), with an up-staging in 18.4% of the patients and a down-staging in 14.9%, without significant differences regarding survival or recurrence (p>0.05). Adjuvant treatment with radiotherapy was performed in 41.4% and with chemo-radiotherapy in 13.8% of the patients. Locoregional recurrence occurred in 17.5%. CONCLUSION: This study revealed that clinical and pathological N staging after elective neck dissection disagreed in a substantial number of patients, with pathological upstaging and significant differences regarding survival. With relation to T staging, there were no significant differences regarding survival. We should be aware of staging disagreements since they can have significant implications on the treatment and prognosis of cancer patients.

Development and validation of the clinical report form for nodular thyroid pathologies

Abstract Objectives: The aim of this study is to develop and validate a novel clinical report form in the format of a structured interview to enable the characterization of the Portuguese population of the Baixo Vouga region with different subtypes of nodular thyroid pathologies (NTyPs). Materials and methods: A structured interview was developed and to the best of our knowledge, this is the first structured interview built and validated for that purpose in Portugal. Results: This structured interview enables the identification of possible correlations between each subtype of nodular lesions and sociodemographic data, consumption habits and lifestyle, endocrine history, and family predisposition. Conclusion: The novel structured interview will simultaneously, enable a detailed characterization of the group of patients with nodular thyroid lesions and will support future metabolomic studies.

Natural history of familial cerebral cavernous malformation syndrome in children: a multicenter cohort study.

PURPOSE: There is limited data concerning neuroimaging findings and longitudinal evaluation of familial cerebral cavernous malformations (FCCM) in children. Our aim was to study the natural history of pediatric FCCM, with an emphasis on symptomatic hemorrhagic events and associated clinical and imaging risk factors. METHODS: We retrospectively reviewed all children diagnosed with FCCM in four tertiary pediatric hospitals between January 2010 and March 2022. Subjects with first available brain MRI and [Formula: see text] 3 months of clinical follow-up were included. Neuroimaging studies were reviewed, and clinical data collected. Annual symptomatic hemorrhage risk rates and cumulative risks were calculated using survival analysis and predictors of symptomatic hemorrhagic identified using regression analysis. RESULTS: Forty-one children (53.7% males) were included, of whom 15 (36.3%) presenting with symptomatic hemorrhage. Seven symptomatic hemorrhages occurred during 140.5 person-years of follow-up, yielding a 5-year annual hemorrhage rate of 5.0% per person-year. The 1-, 2-, and 5-year cumulative risks of symptomatic hemorrhage were 7.3%, 14.6%, and 17.1%, respectively. The latter was higher in children with prior symptomatic hemorrhage (33.3%), CCM2 genotype (33.3%), and positive family history (20.7%). Number of brainstem (adjusted hazard ratio [HR] = 1.37, P = 0.005) and posterior fossa (adjusted HR = 1.64, P = 0.004) CCM at first brain MRI were significant independent predictors of prospective symptomatic hemorrhage. CONCLUSION: The 5-year annual and cumulative symptomatic hemorrhagic risk in our pediatric FCCM cohort equals the overall risk described in children and adults with all types of CCM. Imaging features at first brain MRI may help to predict potential symptomatic hemorrhage at 5-year follow-up.

Common variable immunodeficiency and its inflammatory neurological manifestations: A case report and literature review.

BACKGROUND: Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies characterized by impaired immunoglobulin production and dysregulated immune response. Neurological manifestations have been described in a few patients, and little is known about its clinic and therapeutic approach. Thus, this work aimed to review the literature on it and to help differentiate CVID from its mimics, especially sarcoidosis. METHODS: We described a case report and included a literature review of inflammatory neurological involvement in CVID. RESULTS: A 32-year-old female patient with a medical history of recurrent bacterial infections, temporal focal epilepsy and granulomatous lung disease under study, and cervix squamous cell carcinoma, was initially admitted to the emergency department due to intracranial hypertension. After excluding infectious and neoplastic etiologies, the most likely hypothesis was that granulomatous pulmonary, cerebral, and leptomeningeal inflammatory involvement were associated with sarcoidosis. Two years later, a diagnosis of CVID was made, and the patient was secondarily diagnosed with Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) and related inflammatory brain disease - both complications of CVID. After starting targeted treatment with immunoglobulin replacement and pulse glucocorticoids followed by a chronic taper, the patient became stable. However, three consecutive failures in immunoglobulin intake during the COVID-19 pandemic led to disease recurrence with relapse of neurological manifestations. CONCLUSION: This case illustrates the complex multiple organ manifestations of CVID. When granulomatous conditions arise in these patients, a rare lung disease arising in the context of CVID, the GLILD disease with multisystem involvement, should be taken into consideration. Early treatment with combined steroids and immunotherapy seems to be effective in controlling CVID's neurological manifestations.

Congenital myopathies in adults: A diagnosis not to overlook.

BACKGROUND: Congenital myopathies (CM) were traditionally classified according to the muscle histopathological features, but in recent years, molecular diagnosis has become increasingly important. CM may present a wide phenotype variability, and while adult-onset CM have been increasingly recognized, substantial diagnostic delays are still reported. OBJECTIVES: To describe a cohort of adult CM patients, including clinical, genetic, and histopathological features, and further characterize the subgroup of adult-diagnosed patients. MATERIALS AND METHODS: We performed a retrospective observational cohort study to characterize the CM patients evaluated in our adult Neuromuscular outpatient clinic, including the subgroup of adult-diagnosed patients. RESULTS: We identified 19 CM patients with compatible molecular and/or histological diagnoses, of which 14 were diagnosed in adulthood. Eleven adult-diagnosed patients had symptoms since childhood and 9 had a family history of myopathy. The median age of symptoms' onset was 4 years old and the median age at diagnosis was 37 years old. The most common causative gene was RYR1, followed by TTN and MYH7. Three patients had non-specific features on muscle biopsy, all diagnosed during adulthood. CONCLUSIONS: In our cohort, the majority of CM were diagnosed in adulthood, despite most having pediatric-onset symptoms and positive family history. The diagnostic delay may be associated with mild presentation, slow course, atypical muscle histology, and lack of awareness of adult-onset CM. Studies with larger populations are needed.

Late onset neuromyelitis optica spectrum disorders (LONMOSD) from a nationwide Portuguese study: Anti-AQP4 positive, anti-MOG positive and seronegative subgroups.

INTRODUCTION: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. OBJECTIVE: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). METHODS: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. RESULTS: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). CONCLUSIONS: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.

Spinal involvement in pediatric familial cavernous malformation syndrome.

PURPOSE: The aim of the study was to assess the prevalence and characteristics of spinal cord cavernous malformations (SCCM) and intraosseous spinal vascular malformations (ISVM) in a pediatric familial cerebral cavernous malformation (FCCM) cohort and evaluate clinico-radiological differences between children with (SCCM +) and without (SCCM-) SCCM. METHODS: All patients with a pediatric diagnosis of FCCM evaluated at three tertiary pediatric hospitals between January 2010 and August 2021 with [Formula: see text] 1 whole spine MR available were included. Brain and spine MR studies were retrospectively evaluated, and clinical and genetic data collected. Comparisons between SCCM + and SCCM- groups were performed using student-t/Mann-Whitney or Fisher exact tests, as appropriate. RESULTS: Thirty-one children (55% boys) were included. Baseline spine MR was performed (mean age = 9.7 years) following clinical manifestations in one subject (3%) and as a screening strategy in the remainder. Six SCCM were detected in five patients (16%), in the cervico-medullary junction (n = 1), cervical (n = 3), and high thoracic (n = 2) regions, with one appearing during follow-up. A tendency towards an older age at first spine MR (P = 0.14) and [Formula: see text] 1 posterior fossa lesion (P = 0.13) was observed in SCCM + patients, lacking statistical significance. No subject demonstrated ISVM. CONCLUSION: Although rarely symptomatic, SCCM can be detected in up to 16% of pediatric FCCM patients using diverse spine MR protocols and may appear de novo. ISVM were instead absent in our cohort. Given the relative commonality of asymptomatic SCCM, serial screening spine MR should be considered in FCCM starting in childhood.

Metabolic Profile Characterization of Different Thyroid Nodules Using FTIR Spectroscopy: A Review.

Thyroid cancer's incidence has increased in the last decades, and its diagnosis can be a challenge. Further and complementary testing based in biochemical alterations may be important to correctly identify thyroid cancer and prevent unnecessary surgery. Fourier-transform infrared (FTIR) spectroscopy is a metabolomic technique that has already shown promising results in cancer metabolome analysis of neoplastic thyroid tissue, in the identification and classification of prostate tumor tissues and of breast carcinoma, among others. This work aims to gather and discuss published information on the ability of FTIR spectroscopy to be used in metabolomic studies of the thyroid, including discriminating between benign and malignant thyroid samples and grading and classifying different types of thyroid tumors.

Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study.

INTRODUCTION: Neuromyelitis optica spectrum disorders (NMOSD) are more prevalent in adulthood, with few cases reported in pediatric age (<18 years). In this group, anti-aquaporin 4 (AQP4) antibodies are less frequent, while antibodies against myelin oligodendrocyte glycoprotein (MOG) are more commonly detectable than in adults. OBJECTIVE AND METHODS: Description of pediatric NMOSD cases identified in a national multicentric NMOSD Portuguese registry. RESULTS: Twenty (11.1%) NMOSD cases were diagnosed in pediatric age. Twelve (60%) were female, with a median age of onset of 12.5 (6.8-16.5) years. The presenting feature was transverse myelitis in 10 (50%), 4 of which with simultaneous optic neuritis and 2 with concomitant brainstem syndrome. Nine patients (45%) had pleocytosis in the CSF. Six (30.0%) exhibited anti-AQP4 antibodies, 13 (65.0%) anti-MOG antibodies, and one was seronegative for both. Four anti-AQP4 antibodies-positive patients had ≥1 relapse. Most anti-MOG-positive cases were monophasic (53.8%). In the acute phase, all patients received IV methylprednisolone, nine received IVIg and four plasma exchange. One anti-AQP4-positive patient died. Ten patients (5 anti-AQP4-positive/5 anti-MOG-positive) were on maintenance immunosuppressive therapy at the time of data collection. CONCLUSION: NMOSD may present in pediatric age. It is essential to establish the diagnosis and promptly start therapy to improve the prognosis.

Transient remission of hyperparathyroidism after fine-needle aspiration biopsy.

Primary hyperparathyroidism (PHPT) is the unregulated overproduction of parathyroid hormone (PTH), resulting in abnormal calcium homeostasis. PHPT is most commonly caused by a single adenoma of the parathyroid gland, which can have an intrathyroid location in rare cases. The measurement of intact PTH in the washout fluid obtained by ultrasound (US)-guided fineneedle aspiration (FNA) can be useful in clarifying the aetiology of these lesions. This study presented a 48-year-old man with a background history of symptomatic renal stone disease who was diagnosed with PHPT and referred to our Endocrinology department. A neck US revealed a thyroid nodule with a size of 21 mm in the right lobe. The patient underwent US-guided FNA of the lesion. The measurement of PTH in the washout fluid was significantly elevated. Following the procedure, he reported neck pain and noticed distal paraesthesias in the upper limbs. Blood test results showed significant hypocalcaemia and supplementation with calcium and calcitriol was started. The patient was closely monitored. Recurrence of hypercalcaemia was later observed, and the patient was submitted to surgery. We present a case of FNAinduced transitory remission of PHPT in a patient with an intrathyroid parathyroid adenoma. We conjecture that intra-nodular haemorrhage might have occurred, which temporarily affected the viability of the autonomous parathyroid tissue. A few similar cases of spontaneous or induced remission of PHPT after FNA have been previously described in the literature. This remission can be transitory or permanent, depending on the degree of cellular damage thus follow-up of these patients is recommended.

Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience.

BACKGROUND: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given the absence of randomized clinical trials. AIMS OF THE STUDY: Explore whether tocilizumab, an anti-interleukin-6 monoclonal antibody, is an effective treatment for refractory PCNSV. METHODS: Patients with PCNSV treated with tocilizumab in a single tertiary center were reviewed. RESULTS: Three patients were identified. In two of them, MRI-revealed ischemic lesions. The other presented with a subcortical hemispheric pseudotumoral lesion. Brain biopsy was inconclusive in two patients. Due to a significant number of relapses and clinical deterioration despite other immunosuppressive drugs, tocilizumab was initiated and induced a long remission period up to 44 months. Observed side effects were a fungic infection, neutropenia and thrombocytopenia (both transitory), and a pulmonary embolism in one of the cases. CONCLUSIONS: Tocilizumab might be a therapeutic option for PCNSV (Class IV evidence), given its efficacy and safety. We propose a novel pathway for diagnosis and therapeutics of PCNSV with the purpose of improving the diagnosis, monitoring, and prognosis of this heterogeneous disorder, setting the framework for future use of tocilizumab in this condition.

Multiple Sclerosis in rheumatic patients treated with tumor necrosis factor alpha inhibitors: a single-center retrospective case series and literature review.

Tumor necrosis factor alpha inhibitors (TNFi) are basilar treatments in a number of inflammatory rheumatic conditions and autoimmune phenomena such as de novo neuroinflammatory events were already described in these populations under TNFi. We conducted a single-center retrospective study in a cohort of rheumatic patients treated with TNFi to characterize neurological demyelinating/inflammatory disease in these patients. We report 3 cases (n= 744): all of them had spondyloarthritis, the onset of neurological manifestations occurred between 37 and 58 years old and all of them initially presented with an optic neuritis. The neurological symptoms emerged between 13 and 26 months after starting TNFi. All patients discontinued treatment with TNFi, but one resumed therapy with symptomatic worsening, having to interrupt treatment again. All patients, latter on, fulfilled multiple sclerosis (MS) McDonald criteria 1 and were diagnosed with relapsing-remitting MS. Our study support the prior view of a risk, disease-dependent or agent-dependent, although a causal relationship is yet to be enlightened.

In-vitro mechanical and biological evaluation of novel zirconia reinforced bioglass scaffolds for bone repair.

Bone defects resulting from infections, tumors, or traumas represent a major health care issue. Tissue engineering has been working togehter with medicine to develop techniques to repair bone damage and increase patient's life quality. In that context, scaffolds composed of bioactive ceramics have been explored, although their poor mechanical properties restrain their clinical applications as highly porous structures. As an alternative solution, this study aimed to evaluate the mechanical properties and biological response of novel zirconia reinforced bioactive glass scaffolds (ZRBG) manufactured by the replica method. The microstructure, chemical composition, compressive strength, density, in-vitro bioactivity, and cell viability were analyzed and compared to scaffolds made of monolithic zirconia of similar architecture (45, 60 and 85 ppi). The microstructure of ZRGB scaffolds consisted of a bioactive glass matrix with dispersed zirconia particles (~33% glassy phase) and the compressive strength values (ZRBG scaffolds: 0.33 ± 0.11, 0.41 ± 0.20 and 0.48 ± 0.6 MPa; ZRBG scaffolds with extra BG coating: 0.38 ± 0.13, 0.45 ± 0.11 and 0.50 ± 0.14 MPa for 45, 60 and 80 ppi, respectively) were not statistically different from those of zirconia scaffolds (0.25 ± 0.14 MPa for 45 ppi, 0.32 ± 0.11 MPa for 60 ppi and 0.44 ± 0.07 MPa for 80 ppi). No bioactivity was exhibited by monolithic zirconia scaffolds while significant bioactive response was found for ZRBG scaffolds. The cell viability of ZRBG scaffolds in osteogenic medium was improved up to 171% over zirconia scaffolds. This work provides promosing results for further exploring this technique for implant dentistry.

Response to the letter to the editor: "Score risk scale as a prognostic factor after sudden sensorineural hearing loss".

We appreciate the opportunity to comment the observations on our paper entitled "Score risk scale as a prognostic factor after sudden sensorineural hearing loss", by Capuano et al. This letter highlights several important points, including the role of hyperbaric oxygen therapy and the possible association between patent foramen ovale and sudden sensorineural hearing loss (SSHL). Further research is needed to strengthen the association between cardiovascular risk and SSHL. We thank the authors for their insights into our paper and for adding their experience and observations on the potential role of cardiovascular risk in the etiology of SSHL.

SCORE risk scale as a prognostic factor after sudden sensorineural hearing loss.

INTRODUCTION: Sudden sensorineural hearing loss (SSHL) is an otologic urgency whose treatment is still controversial. Its etiology remains largely unknown in most cases and predicting its prognosis is still a challenge. Cardiovascular risk factors (CVRF) have been implicated in the etiopathogenesis of this entity. OBJECTIVES: Application of the SCORE (Systematic Coronary Risk Evaluation) risk scale in patients with SSHL and evaluation of its potential prognostic value in recovery in patients with CVRF. MATERIALS AND METHODS: Prospective analysis of patients with SSHL admitted for protocol treatment including intravenous corticosteroid therapy associated to weekly intratympanic injection in the event of therapeutic failure or severe hearing loss at admission. Demographic, audiometric, clinical and imaging data were assessed. The SCORE risk scale was applied and the audiometric recovery was compared among different risk groups. RESULTS: Our overall complete and partial recovery rates were 35.9% and 26%, respectively. More than a half of our patients had at least one CVRF. Of these, overweight/obesity, hyperlipidemia and hypertension were the most common. In our sample, patients with CVRF and higher SCORE risk presented higher PTA at admission and also worse hearing outcome, although these results were not statically significant. CONCLUSION: This preliminary study could not confirm the validity for SCORE scale for cardiovascular risk assessment in predicting audiometric recovery in patients with SSHL with multiple comorbidities. Further research with larger samples are needed to elucidate the etiology of SSHL and the exact role of cardiovascular risk factors in the pathophysiology of SSHL. LEVEL OF EVIDENCE: 4.

Digital Versus Conventional Rehabilitation After Total Hip Arthroplasty: A Single-Center, Parallel-Group Pilot Study.

BACKGROUND: The demand for total hip arthroplasty (THA) is rising. In the face of rapidly increasing health care costs, ensuring widespread, cost-effective rehabilitation is a priority. Technologies allowing independent home-based rehabilitation may be the key to facilitate access, improve effectiveness, and lower costs of care. OBJECTIVE: The aim of this study was to assess the feasibility of a novel artificial intelligence-powered digital biofeedback system following THA and compare the clinical outcomes against supervised conventional rehabilitation. METHODS: This was a single-center, parallel-group pilot study, with an 8-week intervention program. Patients were assessed at baseline, during the program (at 4 and 8 weeks), and 3 and 6 months after surgery. The primary outcome was the Timed Up and Go (TUG) score and secondary outcomes were the Hip dysfunction and Osteoarthritis Outcome Scale (HOOS; a patient-reported outcome) and hip range of motion (ROM). RESULTS: A total of 66 patients were included: 35 digital physiotherapy (PT) versus 31 conventional. There were no differences at baseline between groups except for lower HOOS quality of life (QoL) subscale scores in the digital PT group. Clinically relevant improvements were noted in both groups at all time points. The digital PT group showed a retention rate of 86% (30/35). Per-protocol analysis revealed a superiority of the digital PT group for all outcome measures. Intention-to-treat analysis revealed the superiority of the digital PT group at all time points for TUG (change between baseline and 4 and 8 weeks: P<.001; change between baseline and 3 and 6 months: P=.001 and P=.005, respectively), with a difference between median changes of -4.79 seconds (95% CI -7.24 to -1.71) at 6 months post-THA. Between baseline and month 6, results were also superior in the digital PT group for the HOOS sports and QoL subscales and all ROM except for standing flexion. CONCLUSIONS: This study demonstrates this novel solution holds promise in rehabilitation after THA, ensuring better clinical outcomes than conventional rehabilitation while reducing dependence on human resources. TRIAL REGISTRATION: ClinicalTrials.gov NCT03045549; https://clinicaltrials.gov/ct2/show/NCT03045549.

Electrochemical Immunosensor for TNFα-Mediated Inflammatory Disease Screening.

Inflammation associated with cancer, neurodegenerative, ocular, and autoimmune diseases has a considerable impact on public health. Tumor necrosis factor alpha (TNFα) is a key mediator of inflammatory responses, responsible for many of the systemic manifestations during the inflammatory process. Thus, inhibition of TNFα is a commonplace practice in the treatment of these disorders. Successful therapy requires the ability to determine the appropriate dose of anti-TNFα drugs to be administered in a timely manner, based on circulating TNFα levels. In this Letter, we report the development of an immunosensor technology able to quantify TNFα at the picogram level in relevant human body fluids, holding the potential to early detect inflammation  and monitor TNFα levels during treatment, enabling TNFα-targeted treatments to be tailored according to the immune status of an individual patient. This immunosensor technology is significantly more rapid and sensitive than conventional enzyme linked immunosorbent assays, maintaining high specificity and requiring small sample volumes. These features might also be advantageous in the context of personalized medicine, as this analytical platform can deliver advanced diagnostics and reduce clinical burden.

Vanishing Pseudotumoral White Matter Lesions Presenting as Aphasia and Altered Mental Status in a 71-Year-Old Male.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that usually affects the central nervous system (CNS) after an infection and/or vaccination. It is more common in infancy. Here we present a case of late onset ADEM. A 71-year-old male was admitted to the emergency department due to speech difficulty and somnolence. Upon neurological examination he had a mixed aphasia. He performed a brain computed tomography which showed multiple white matter hypodense lesions. After admission to the neurology ward, he performed a lumbar puncture which showed a mildly inflammatory cerebrospinal fluid, negative serological testing and negative oligoclonal bands. Brain magnetic resonance imaging (MRI) confirmed the presence of multiple T2 hyperintense lesions, extensive bilateral frontoparietal lesions with abundant perilesional edema, four with gadolinium enhancement in an open-ring pattern and no mass effect. Anti-aquaporin 4 antibody, virologic and bacteriologic blood testing, screening of autoimmune disorders and occult neoplasm were all unremarkable. He was treated with intravenous methylprednisolone (1 gr) during five days and started to recover, maintaining a slight verbal fluency deficiency. Post-treatment brain MRI showed reduction of previous lesions, corroborating the probable inflammatory/demyelinating etiology. After discharge he maintained follow-up at the neurology outpatient clinic and he is currently asymptomatic with no new lesions and further reduction of the previous ones on follow-up MR scan. Both clinical follow-up of the patient, revealing a monophasic course with complete recovery, and temporal evolution of his brain lesions were essential to establish a diagnosis of ADEM in a septuagenarian patient, in whom other diagnoses have to be considered.

Management of pediatric peritonsillar and deep neck infections- cross- sectional retrospective analysis.

OBJECTIVE: Deep neck infections (DNI) are responsible for significant morbidity in children and healthcare expenditures. Few studies exist specifically addressing the clinical and epidemiologic characterization and management of DNI's in the pediatric population. Our goal was to analyse the demographic characteristics, clinical presentation, diagnostic and therapeutic approaches of peritonsillar and DNI in pediatric patients. METHODS: The medical records of patients, aged up to 18 years, admitted for peritonsillar and DNI at our department, from 2011 to 2016, were retrospectively reviewed and compared with the literature available. Ninety-eight patients were enrolled. RESULTS: The mean age was higher in patients with peritonsillar abscess and lower in patients with retropharyngeal and parapharyngeal infections. Admissions have significantly increased from 2011. There was a seasonal variation for DNI incidence, with a peak incidence in Summer and Spring. All patients included were treated as inpatient and received empirical intravenous antibiotic therapy and steroids regardless of drainage procedures. Incision and drainage was performed in 72 patients. The hospital length of stay was higher among patients with retropharyngeal abscess and in the group with complications. Only 2 patients developed complications during hospital stay. The most common microbiological pattern was monomicrobial and the most commonly isolated pathogens were Streptococcus Pyogenes, Streptococcus Mitis and anaerobic bacteria. CONCLUSIONS: Surgical incision and drainage followed by intravenous antibiotic and steroids proved to be successfull with low morbidity related to surgical approach. However, in selected cases, medical therapy may be an alternative to surgical management in uncomplicated infections.