Parkinson's disease: Autoimmunity and neuroinflammation.

Parkinson's 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. Parkinson's disease is recognized as the most common neurodegenerative disorder after Alzheimer's 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 Parkinson's 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 Parkinson's disease only treat the symptoms of the disease. A major goal of Parkinson's 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.

Use of the pedicled nasoseptal flap in the endoscopic management of cholesterol granulomas of the petrous apex.

BACKGROUND: Nowadays the endoscopic approach represents a useful alternative to traditional surgical approaches in the treatment of cholesterol granulomas (CGs) of the petrous apex (PA). Recently the nasoseptal flap (NSF) has been employed to permit long-term patency of drainage site. The purpose of this study is to report our experience with the NSF in the endoscopic management of CG and to analyze the advantages, limitations, and outcomes of the technique. METHODS: A retrospective analysis was carried out on 10 patients affected by CG of the PA who had been treated endoscopically, using the NSF. RESULTS: An endoscopic transpterygoid approach was used for 6 patients and the remaining 4 were treated using the transclival approach. A NSF was used in all 10 cases. In 6 cases the flap was ipsilateral to the lesion whereas in 4 it was contralateral. In 90% of our patients no evidence of disease was observed after a mean follow-up period of 35.7 months, with resolution of their symptoms. One patient presented a recurrence because of a technical error (inadequate placement of the flap in the cavity), and has been retreated endoscopically. CONCLUSION: The pedicled NSF seems to be helpful in avoiding the concentric growth of the granulomatous cyst epithelium while assuring ventilation and drainage of the cyst. However, bigger studies with longer-term follow-up are needed to confirm these findings. Correct and meticulous placement of the flap inside the cystic cavity is the most critical issue for the success of the procedure.

Subtotal petrosectomy and cerebrospinal fluid leakage in unilateral anacusis.

Objective This study presents a group of patients experiencing recurrent cerebrospinal fluid (CSF) leakage associated with ipsilateral anacusis who underwent subtotal petrosectomies with the goal of stopping the CSF leak and preventing meningitis. Materials and Methods Eight patients with CSF leakage were enrolled: three patients with giant vestibular schwannomas had CSF leakage after gamma knife failure and subsequent removal via a retrosigmoid approach; two patients had malformations at the level of the inner ear with consequent translabyrinthine fistulas; two had posttraumatic CSF leakages; and one had a CSF leakage coexisting with an encephalocele. Two patients developed meningitis that resolved with antibiotic therapy. Each patient had preoperative anacusis and vestibular nerve areflexia on the affected side. Results The patients with congenital or posttraumatic CSF leaks had undergone at least one unsuccessful endaural approach to treat the fistula. All eight patients were treated successfully with a subtotal petrosectomy. The symptoms disappeared within 2 months postoperatively. No meningitis, signs of fistula, or other symptoms occurred during the follow-up. Conclusion A subtotal petrosectomy should be the first choice of treatment in patients with recurrent CSF leakage whenever there is associated unilateral anacusis.

Vestibular neurolabyrinthitis: a follow-up study with cervical and ocular vestibular evoked myogenic potentials and the video head impulse test.

OBJECTIVES: The aim of this study was to evaluate prospectively, in a group of patients affected by vestibular neurolabyrinthitis (VN), a diagnostic protocol including cervical vestibular evoked myogenic potentials (C-VEMPs), ocular vestibular evoked myogenic potentials (O-VEMPs), and the video head impulse test (vHIT). METHODS: The diagnosis of VN was based on the patient's clinical history, an absence of associated auditory or neurologic symptoms, and a neuro-otological examination with an evaluation of lateral semicircular canal function by use of the Fitzgerald-Hallpike caloric vestibular test and the ice test. RESULTS: In our series, 55% of the cases were superior and inferior VN, 40% were superior VN, and 5% were inferior VN. These cases, however, comprised different degrees of vestibular involvement, as the individual vestibular end organs have different prognoses. Four patients had only deficits of the horizontal and superior semicircular canals or their ampullary nerves. CONCLUSIONS: The implementation of C-VEMPs, O-VEMPs, and the vHIT in a vestibular diagnostic protocol has made it possible to observe patients with ampullary VN in a way that has not been feasible with other types of vestibular examinations. The age of the patient seems to have some impact on recovery from VN. When recovery occurs in the utricular and saccular nerves first and in the ampullary nerves subsequently, it may be reasonable to expect a more favorable outcome.

Vascular leiomyoma and geniculate ganglion.

Objectives Discussion of a rare case of angioleiomyoma involving the geniculate ganglion and the intratemporal facial nerve segment and its surgical treatment. Design Case report. Setting Presence of an expansive lesion englobing the geniculate ganglion without any lesion to the cerebellopontine angle. Participants A 45-year-old man with a grade III facial paralysis according to the House-Brackmann scale of evaluation. Main Outcomes Measure Surgical pathology, radiologic appearance, histological features, and postoperative facial function. Results Removal of the entire lesion was achieved, preserving the anatomic integrity of the nerve; no nerve graft was necessary. Postoperative histology and immunohistochemical studies revealed features indicative of solid vascular leiomyoma. Conclusion Angioleiomyoma should be considered in the differential diagnosis of geniculate ganglion lesions. Optimal postoperative facial function is possible only by preserving the anatomical and functional integrity of the facial nerve.

Selective vestibular neurolabyrinthitis of the lateral and superior semicircular canal ampulla and ampullary nerves.

OBJECTIVES: The diagnosis of vestibular neurolabyrinthitis is based on the sudden appearance of vertigo that lasts for hours or days without associated cochlear or central nervous system signs or symptoms. The advent of the video head impulse test, cervical vestibular evoked myogenic potential testing, and ocular vestibular evoked myogenic potential testing has provided interesting clinical evidence for evaluating and monitoring the damage to specific compartments of the vestibular apparatus. These various methods of testing individual end-organ functions may have a clinical impact on the vestibular workup of neurolabyrinthitis. METHODS: This report describes 3 patients with acute vestibular neurolabyrinthitis in whom caloric tests, cervical vestibular evoked myogenic potentials, ocular vestibular evoked myogenic potentials, and the video head impulse test led to a suspicion of peripheral vestibular deficits of the lateral or superior semicircular canal ampulla or ampullary nerves. RESULTS: In our patients, the examination results (normal hearing, absence of responses on caloric testing, and bilateral preservation of cervical and ocular vestibular evoked myogenic potentials) disclosed an acute partial superior vestibular neurolabyrinthitis. CONCLUSIONS: To our knowledge, these are the first reported cases in which selective damage to the lateral and superior semicircular canals and their nerves caused by neurolabyrinthitis was demonstrated clinically. Our clinical results indicate that the damage can be selective for specific vestibular end organs.

Petrous bone fractures violating otic capsule.

OBJECTIVE: This study presents our experience with a series of patients suffering from petrous bone fractures violating the otic capsule who underwent subtotal petrosectomy combined with eustachian tube, middle ear, and mastoid obliteration, with the goal of preventing cerebrospinal fluid (CSF) leak and meningitis. METHODS: This study enrolled 26 patients between 1997 and 2011. The clinical symptoms, otoscopy, and preoperative and postoperative audiometry and facial function, as well as CSF leak or meningitis, were evaluated in each patient. The entire group underwent a subtotal petrosectomy using the technique described in detail by Fisch. In addition, each patient was interviewed using a questionnaire to evaluate the impact on quality of life. RESULTS: Intraoperatively, we found significant CSF leaks in 14 patients (42.5%). No patient reported other episodes of CSF leak or meningitis after the surgery. The patients' responses of facial nerve function were slightly worse than the House-Brackmann evaluation (50% versus 42.3%; p < 0.05). The vast majority (88.5%) of the patients experienced no social impact. CONCLUSION: Our findings suggest the importance of not underestimating the risk for CSF leak in the petrous bone fractures violating the otic capsule. Preoperative counseling regarding the various troublesome complications must adequately motivate candidates to undergo surgery by pointing out the positive impact of the proposed treatment.

Predictors of ear barotrauma in aircrews exposed to simulated high altitude.

INTRODUCTION: Ear barotrauma is an adverse effect related to hypobaric exposure. Ear, nose, and throat (ENT) diseases are risk factors for barotrauma in aircrews trained in a hypobaric chamber, but excluding affected subjects from exposure does not abolish the risk in asymptomatic trainees. We investigated other possible predictors, including history of ENT diseases, ENT clinical abnormalities, altitude, and subject's age. METHODS: After a complete ENT evaluation including otoscopy and tympanometry, 314 aircrews underwent hypobaric chamber training. Two altitude training profiles up to 35,000 ft (10,668 m) and 25,000 ft (7620 m), respectively, were used. Subjects were grouped according to if they were asymptomatic, had acute barotitis, or reported delayed ear pain the day after the exposure. RESULTS: There were 7 men who had acute barotitis (incidence of 2.3%) and 28 men who had delayed ear pain (incidence of 9.2%). A significant association resulted between history of ENT diseases and delayed ear pain and between abnormal ENT findings and acute barotitis in subjects exposed to the higher profile. Altitude was associated with increased risk of delayed ear pain. Delayed ear pain was associated with older age in subjects exposed to the lower altitude and younger age in subjects exposed to the higher altitude. DISCUSSION: Our data suggest that in subjects exposed to 35,000 ft (10,668 m), the history of previous ENT diseases and younger age may be valid predictors of delayed ear pain, while abnormal ENT findings may predict acute barotitis. At 25,000 ft (7620 m), subjects with older age may have increased risk of delayed ear pain.