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

Elastofibroma and the external auditory canal.

An elastofibroma is a rare fibro-elastic pseudotumor, first reported in 19611. It is usually located at the lower pole of the scapula and rarely at other sites. The etiology is unclear, but may be related to abnormal elastogenesis caused by repetitive trauma or friction between the scapula and chest wall2,3. The choice of treatment is controversial: some have suggested a marginal excision because it carries a low recurrence risk whereas others adopt a wait-and-see approach in asymptomatic patients because malignant transformation of the lesion has not been reported4. We present the case of a man with an elastofibroma of the external auditory canal (EAC). To our knowledge, this is the first case of elastofibroma localized in the EAC to be reported in the English language literature

Repositioning maneuver for the treatment of the apogeotropic variant of horizontal canal benign paroxysmal positional vertigo

Objective: The purpose of this study was to determine the effectiveness of a new physical maneuver in the treatment of the apogeotropic variant of horizontal canal benign paroxysmal positional vertigo. Study Design: Case review. Setting: Outpatient clinic. Patients: The diagnosis of apogeotropic horizontal canal benign paroxysmal positional vertigo was based on the history of recurrent sudden crisis of vertigo associated with bursts of horizontal apogeotropic paroxysmal nystagmus provoked by turning the head from the supine to either lateral position. The patients were three men and five women ranging in age from 31 to 73 years (average, 49.2 yr). Interventions: All patients were treated with a repositioning maneuver based on the hypothesis that the syndrome is caused by the presence of free-floating dense particles inside the endolymph of the anterior arm of the horizontal canal. The maneuver favors their shifting into the posterior arm of the canal. Patients were reexamined immediately after the treatment and underwent Gufoni's liberatory maneuver for the geotropic variant of horizontal canal benign paroxysmal positional vertigo. Main Outcome Measure: The treatment outcome was considered as responsive when, after one repositioning maneuver, nystagmus shifted from apogeotropic to geotropic. Results: The repositioning maneuver resulted in a transformation from the apogeotropic variant into a geotropic variant of horizontal canal benign paroxysmal positional vertigo in all patients. Conclusion: This maneuver represents a simple and effective approach to the treatment of the apogeotropic variant of horizontal canal benign paroxysmal positional vertigo. It favors the shifting of the canaliths from the anterior into the posterior arm of the horizontal canal from where they can migrate into the utricle with Gufoni's maneuver

Unilateral vestibular schwannoma associated with a Jacobson's schwannoma

Coexistence of unilateral vestibular schwannoma and Jacobson's schwannoma growing in the same intracranial site is rarely observed. We present the case of 36-year-old woman with primary diagnosis of vestibular schwannoma and subsequent appearance of schwannoma to the Jacobson's nerve. Initial wait and see strategy was performed offered us the opportunity to describe Jacobson's lesion features at computed tomography over a period of 4 years. Subtotal petrosectomy with infralabyrinthine approach was subsequently executed to remove the growing mass of the temporal bone. The Jacobson's schwannoma increased its size from 0.4 cm for years whereas vestibular schwannoma size was unchanged after 7 years observation. The concomitant removal of both schwannomas is still associated with the size of the CPA lesion and to patient's symptoms