A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement

Sjögren’s syndrome is primarily a chronic systemic autoimmune disease that affects exocrine organs. Neurologic symptoms frequently present as peripheral neuropathy due to small vessel vasculitis. Type and prevalence of central nervous system involvement are still controversial. In this report, we present a 35-year-old woman with primary Sjögren’s syndrome with central nervous system vasculitic involvement.     

Epicardial mass causing cardiac compression: an unusual involvement in lymphomatoid granulomatosis

Lymphomatoid granulomatosis is a rare, diffuse, large B-cell lymphoma that is positive for Epstein-Barr virus. A multiorgan process, it manifests itself chiefly in the lungs but can also affect the skin, nervous system, and kidneys. Cardiac involvement and pericardial effusion are very unusual. We report the case of a 62-year-old man with lymphomatoid granulomatosis involving the heart and lungs. Diagnosis was confirmed with wedge biopsy at pericardiotomy, and the patient was treated with cyclophosphamide, prednisolone, and vincristine. Although the patient was still symptomatic at 6-month follow-up, he was in partial remission with improved functional capacity.     

Comparison of microscopic (pT3a) and gross extravesical extension (pT3b) in pathological staging of bladder cancer: analysis of patient outcomes

Objective

To determine the prognostic value of pT3 bladder urothelial carcinoma substaging in patients without lymphatic involvement.

Patients and methods

Pathologic and clinical data were reviewed on patients who underwent radical cystectomy for urothelial carcinoma between 1991 and 2010. Of the 460 reviewed patients, 74 patients were diagnosed with pathologic T3No urothelial carcinoma of the bladder. The impact of pathologic substaging (pT3a vs. pT3b) was examined to determine the effect on overall and disease-specific survival.

Results

Five years disease-specific and overall survival rates were 46.9 % and 39.6 % for patients with pT3aNo tumor, whereas these ratios were 34.4 and 30.3 %, respectively, for patients with pT3bNo tumor (p > 0.05). Mean disease-specific survival time was 43.94 ± 6.50 months for pT3aNo, while it was 39.01 ± 7.19 months for pT3bNo (p = 0.539). In multivariate cox regression analysis, age (p = 0.459), gender (p = 0.710), urinary diversion type (p = 0.088), and pT3 substaging (p = 0.554) were not noticed as an independent predictive factor for survival.

Conclusion

Macroscopic extravesical extension (pT3b) is not associated with a worse outcome than pT3a disease in lymph node-negative cases of bladder urothelial carcinoma.     

Extradural Giant Multiloculated Arachnoid Cyst Causing Spinal Cord Compression in a Child

Abstract

Background: Spinal extradural arachnoid cysts are rare expanding lesions in the spinal canal. Enlargement may cause progressive signs and symptoms caused by spinal cord compression. They are associated with trauma, surgery, arachnoiditis, and neural tube defects. Most nontraumatic spinal extradural arachnoid cysts are thought to be congenital.

Design: Case report and literature review.

Findings: A 9-year-old boy with mild paraparesis was found to have an extradural multiloculated arachnoid cyst with fibrous septa at T4-L3 levels and anterior compression and displacement of the spinal cord.

Conclusions: Definitive treatment of arachnoid cyst entails radical cyst removal and dura cleft repair. Formation of a postoperative cerebrospinal fluid fistula may require external lumbar drainage.     

Iperplasia bilaterale del processo coronoideo in pazienti pediatrici

ObjectivesTo present an international literature review on the coronoid hyperplasia in pediatric patients, describing the etiology, the diagnosis and the therapeutic options.Materials and methodsA systematic revision of the international literature has been done on Medline database (www.ncbi.nlm.nih.gov/pubmed). Keywords chosen were: “bilateral coronoid hyperplasia”, “coronoid process”, “temporomandibular joint ankylosis”, “coronoid hyperplasia and treatment”, “pediatric patients”.ResultsAmong the pathogenetic factors, there are: temporalis muscle hyperactivity, genetic inheritance, hormonal stimulus, temporomandibular joint dysfunctions, traumas and persistent coronoid cartilage growth centre. The main symptoms are: mandibular hypomobility, alterations in protrusion and in lateral mandibular movements and muscular dystonia. Orthopantomography, magnetic resonance imaging and computed tomography are the diagnostic gold standards. The most widely surgical treatment used is coronoidectomy.ConclusionsOn the scientific evidences present in literature it's possible to conclude that, being coronoid hyperplasia a frequent pediatric condition, an early diagnosis is important to restore stomatognathic multifunctions as soon as possible.     

Oxidative stress in schizophrenia patients treated with long-acting haloperidol decanoate

In this study the role of oxidative stress in schizophrenia was investigated by evaluating the relationship of oxidative stress markers with neurochemistry, psychopathology, and extrapyramidal symptoms. Antioxidant activity of superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase, and concentrations of malondialdehyde, protein carbonyls, nitrite, nitrate, glutathione, dopamine, noradrenaline, adrenaline, and serotonin were measured in 52 outpatients with DSM-IV diagnosis of schizophrenia treated with haloperidol decanoate. Psychopathology and extrapyramidal symptoms were assessed by positive and negative syndrome scale, global assessment of functioning, abnormal involuntary movement scale, Simpson Angus scale, and Barnes akathisia rating scale. Haloperidol dose was positively correlated with plasma protein carbonyls. Longer duration of illness was associated with decreased levels of glutathione peroxidase. Increased activity of superoxide dismutase was associated with increased levels of catalase, glutathione peroxidase, glutathione reductase and reduced glutathione, and decreased concentration of malondialdehyde, indicating joint action of various antioxidative systems. Increased levels of nitrite and noradrenaline were associated with decreased level of malondialdehyde. Akathisia was greater in patients with decreased catalase activity, indicating involvement of impaired antioxidant defense in developing extrapyramidal symptoms. These results confirm the hypothesis that oxidative stress is involved in pathophysiology of schizophrenia and severity of extrapyramidal symptoms.