Michela Conte

Parkinson's disease: Autoimmunity and neuroinflammation

Parkinsons disease is a neurodegenerative disease that causes the death of dopaminergic neurons in the substantia nigra. The resulting dopamine deficiency in the basal ganglia leads to a movement disorder that is characterized by classical parkinsonian motor symptoms. Parkinsons disease is recognized as the most common neurodegenerative disorder after Alzheimers disease. PD ethiopathogenesis remains to be elucidated and has been connected to genetic, environmental and immunologic conditions. The past decade has provided evidence for a significant role of the immune system in PD pathogenesis, either through inflammation or an autoimmune response. Several autoantibodies directed at antigens associated with PD pathogenesis have been identified in PD patients. This immune activation may be the cause of, rather than a response to, the observed neuronal loss. Parkinsonian motor symptoms include bradykinesia, muscular rigidity and resting tremor. The non-motor features include olfactory dysfunction, cognitive impairment, psychiatric symptoms and autonomic dysfunction. Microscopically, the specific degeneration of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies, which are brain deposits containing a substantial amount of α-synuclein, have been recognized. The progression of Parkinsons disease is characterized by a worsening of motor features; however, as the disease progresses, there is an emergence of complications related to long-term symptomatic treatment. The available therapies for Parkinsons disease only treat the symptoms of the disease. A major goal of Parkinsons disease research is the development of disease-modifying drugs that slow or stop the neurodegenerative process. Drugs that enhance the intracerebral dopamine concentrations or stimulate dopamine receptors remain the mainstay treatment for motor symptoms. Immunomodulatory therapeutic strategies aiming to attenuate PD neurodegeneration have become an attractive option and warrant further investigation.

The oncologic radicality of supracricoid partial laryngectomy with cricohyoidopexy in the treatment of advanced N0-N1 laryngeal squamous cell carcinoma†

The aim of the study was to evaluate the oncologic outcome of our series of advanced laryngeal squamous cell carcinomas, primarily treated by supracricoid laryngectomy with cricohyoidopexy or total laryngectomy, to compare the two therapeutic modalities.

Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease.

Hypertrophic pachymeningitis (HP) is a rare disorder that causes thickening of the dura mater. Inflammatory lesions may be located in the cerebral or spinal dura mater or, less frequently, in both locations simultaneously. Numerous clinico-pathological entities cause thickening of the pachymeninges. Indeed, HP is a potential manifestation of many different diseases, but the diagnosis often remains uncertain. Cases in which the pachymeningitis has no known aetiology are termed “idiopathic” HP (IHP). Recently, it has been suggested that IgG4-related disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis. Little is known regarding the pathogenic events of IHP. In a general theory, the inflammatory infiltrate, mainly consisting of B and T lymphocytes, activates fibroblasts and induces collagen deposition, leading to tissue hypertrophy and increased dural thickness. Clinical manifestations of IHP depend upon the location of the inflammatory lesions and compression of the adjacent nervous structures. Three central pathological features are lymphoplasmacytic infiltration, obliterative phlebitis, and storiform fibrosis. MRI is the examination of choice for the preliminary diagnosis of IHP. Histopathological examination of a biopsy specimen of the dura mater would finally confirm the diagnosis. The differential diagnosis for HP is broad and includes infections, autoimmune disorders, and neoplasia. Currently, there is no consensus about treatment for patients with IHP. There is a preference for glucocorticoid treatment on diagnosis followed by the addition of other immunosuppressive agents in the event of a recurrence. Rituximab is used in patients who did not respond to glucocorticoids or to conventional steroid-sparing agents.

Chemical ear peeling: a simple technique for the treatment of chronic external otitis: how we do it.

Dear Editor, Few conditions encountered by otolaryngologists are as frustrating as chronic otitis externa and recurrent exacerbation presents a special challenge for the attending physician. The disease may be extremely therapy-resistant: a great many patients report chronic suffering from otitis externa with inadequate and inefficacious treatment attempts. Current medical treatment is based on topical application of steroids, antibiotics and external auditory canal cleansing. Moreover, there are no long-term outcome data on medical management. In this study we present, showing the shortand longterm clinical outcomes, a new, safe and simple therapeutic technique for the management of chronic otitis externa: chemical ear peeling (CEP).

Is biofilm the cause of chronic otitis externa

This study was undertaken in two phases. In the first phase, we considered patients affected by chronic external otitis treated either by chemical ear peeling (CEP) or by antibiotic/steroid treatment to compare the clinical and microbiological outcomes. In the second phase, we compared the microscopic findings observed in the CEP samples of patients affected by chronic otitis externas acute exacerbation or by acute otitis externa to demonstrate the role of biofilm in the pathogenesis of chronic otitis externa.

Fibrous dysplasia of the maxilla: diagnostic reliability of the study image. Literature review.

Objective Fibrous dysplasia (FD) is a benign bone disorder in facial bones. This study evaluates the possibility of diagnosing fibrous dysplasia on imaging alone, without biopsy of the lesion, which is often burdensome for the patient. Materials and Methods The authors bring their experience of four cases of bone lesions of the maxillofacial region and present a review of published studies. The imaging techniques evaluated are computed tomography (CT) and magnetic resonance imaging (MRI) with and without contrast. Results The literature review demonstrates that it is impossible to make diagnosis of fibrous dysplasia exclusively by imaging. Radiographic images often show a ground-glass appearance, which is characteristic but not pathognomonic of fibrous dysplasia. Conclusion Although CT and MRI images may in many cases suggest a diagnosis of fibrous dysplasia, histological examination or follow-up imaging should follow.

The Importance of the Number of Reflux Episodes in the Diagnosis of Laryngopharyngeal Reflux Disease

Objective The aim of this study is to evaluate the sensitivity parameters of the percentage of time the pH is <4 and >7, as well as the total number of laryngopharyngeal reflux episodes in 24 hours, in patients with suspected laryngopharyngeal reflux disease. Study Design Retrospective controlled study. Setting University hospital. Subjects and Methods The study was conducted on 46 patients with laryngopharyngeal reflux disease and 58 healthy controls. Patients and controls underwent 24 hours of dual-probe pH monitoring of the distal and cervical esophagus. Patients completed a Reflux Symptom Index questionnaire and underwent esophageal manometry. Data concerning the percentage of time the pH was <4 and >7 and the number of reflux episodes registered at the cervical esophagus were collected and evaluated. Results The percentage of time the pH is <4 and the number of laryngopharyngeal reflux episodes seem to be a reliable diagnostic laryngopharyngeal reflux criterion reaching satisfactory sensitivity (81% and 83%, respectively). Although the pH >7 parameter appeared statistically different between the 2 groups (P < .001), the sensitivity of the test appeared to be poor (55%). Conclusion Our study demonstrates the importance of the absolute number of laryngopharyngeal reflux episodes in 24 hours in the diagnosis of patients with suspected laryngopharyngeal reflux, proposing it as a new diagnostic criterion.

Salvage total laryngectomy after conservation laryngeal surgery for recurrent laryngeal squamous cell carcinoma

SUMMARY The aim of the present study was to evaluate the oncological efficacy of salvage total laryngectomy in patients who had previously undergone supracricoid partial laryngectomy or transoral laser microsurgery for treatment of laryngeal squamous cell carcinoma. We retrospectively reviewed the medical, surgical and pathological records of 35 patients who underwent salvage total laryngectomy after recurrence of laryngeal cancer (following supracricoid partial laryngectomy or transoral laser microsurgery). Kaplan-Meier survival curves as well as univariate and multivariate analyses of prognostic factors were performed. No statistically significant differences were seen comparing the supracricoid partial laryngectomy group with the transoral laser microsurgery group for overall survival and disease-specific survival at 3 years (OS = 38% vs. 52%, p = 0.16; DSS = 40% vs. 61%, p = 0.057) or locoregional control at 2 years (LRC = 40% vs. 54%, p = 0.056). A trend indicating worse survival and locoregional control for supracricoid partial laryngectomy patients emerged. Preservation of the osteocartilaginous frame in transoral laser microsurgery could hypothetically result in better salvageability of anterior recurrences with extralaryngeal spread.

Sarcoidosis of the middle ear and chronic suppurative otitis media: coexisting conditions?

Aural polyps are considered a relatively infrequent pathologic condition which represents a complication consisting in an active chronic inflammatory process in 25% to 45% of cases; alternatively, they may be the result of a specific systemic granulomatous disease of a benign tumor or of a malignant tumor. Ear involvement is extremely rare in sarcoidosis: 49 cases (1,2) have been reported in English literature, and only 5 of them were confined exclusively to the external and/or the middle ear (3,4). In this report, we describe a case of chronic suppurative otitis media (CSOM) with an aural polyp of the right ear. After histologic examination, diagnoses of CSOM and coexistent sarcoidosis were formulated.

B Lymphocyte Subsets in Patients with Rhinoscleroma

Objective. To identify the presence of B lymphocytes in the rhinoscleroma granulomas as a possible precursor of plasma cells, whose presence has always been described but whose role is still unclear. Study Design. Case series with chart review. Setting. Sapienza University of Rome. Methods. The study was carried out on 6 patients (3 women, 3 men). The following parameters were examined for each patient: clinical manifestations, number of leukocytes, lymphocytes and lymphocyte subsets (CD3+, CD4+, CD8+, and CD19+) in blood samples, time from the onset of symptoms, biopsies, and expression of CD3, CD4, CD8, and CD20 antigens in tissue samples with immunohistochemical techniques. Results. In this study, the values of CD3+, CD4+, and CD8+ T lymphocyte subsets in venous peripheral blood are in line with previously reported data, whereas CD19+ lymphocyte cells tended to show an ambiguous behavioral pattern. In tissue samples, approximately one-third of the T lymphocyte population showed a CD3+/CD8+ immunophenotype (cytotoxic/suppressor), and two-thirds of the T lymphocytes expressed a CD3+/CD4+ immunophenotype (helper/inducer). The authors also identified an unexpected large amount of CD20+ non-plasmacellular B cells in addition to the plasma cells usually detected in rhinoscleroma biopsies. Conclusions. This study demonstrates the presence of B lymphocytes in rhinoscleroma tissue. It is presumable that the mature B cells activated by antigenic stimulation of Klebsiella rhinoscleromatis are the precursors of the plasma cells typically found in the granuloma of rhinoscleroma.