What is the expert's option on antiplatelet therapy in moyamoya disease? Results of a worldwide Survey.

BACKGROUND AND PURPOSE: Moyamoya disease (MMD) is a rare idiopathic vasculopathy characterized by an extensive network of fine collaterals in the setting of bilateral progressive stenosis and finally occlusion of the intracranial portion of the internal carotid artery and proximal anterior and medial arteries of Circle of Willis. Owing to the rarity of the disease and ethical concerns, double-blinded, randomized controlled trials about treatment options are completely lacking. The appropriate conservative treatment instead, before or after revascularization surgery, is nearly completely neglected in scientific literature, even in Asia. METHODS: We developed a questionnaire with response options offered in the multiple choice method. The survey was sent with the request to reply within September 2010 to January 2011 by email to experts in the treatment of MMD. As an international expert, authors were selected who had written more than one scientific article or book chapter on the subject. We took special care not to select only Asian scientists, but also considered experts on the disease in Caucasian patients. RESULTS: Among the 77 physicians surveyed, 32 (41.55%) responded. Twenty-one (65.6%) Asian experts and 11 (34.4%) non-Asian experts participated in this survey. The majority of experts thought that long-term antiplatelet therapy is not essential in the treatment of MMD. Ten of 32 (31%) participants agreed to long-term antiplatelet treatment with acetylsalicylic acid 100 mg/day. There was a significant difference in answers between Asian and non-Asian experts (P = 0.0128). CONCLUSION: It is interesting that the majority of Non-Asian respondents recommend antiplatelet drugs, while this is an unusual approach in MMD of the Asian experts. Perhaps, this significant difference is well founded by the different experiences of the experts related to the difference in disease presentation between Asians and Caucasians. The role of conservative and surgical treatment in MMD needs further evaluation with larger cohorts and a focus on long-term clinical outcome.

Short-term lack of cerebral perfusion with good outcome. Advantages of early intracranial pressure monitoring.

A 21-year-old man was injured by a tailboard of a truck. He suffered a severe head injury with bilateral depressed skull fractures necessitating surgical decompression. On admission to the hospital the patient showed bending to pain stimuli (Glasgow Coma Score 5). Anisocoria was noticed from the beginning. Initial intracranial pressure (ICP), measured 3 hours after injury, was 30 mm Hg, and the cerebral perfusion pressure (CPP) was 70 mm Hg. During surgical elevation of the skull fracture on the right side an un-explainable rise of ICP to values of 100 mm Hg occurred, which corresponded to the mean arterial blood pressure (MAP). At the same time both pupils were dilated and fixed indicating a lack of cerebral perfusion. Due to immediate trephination of the opposite side, the ICP was lowered to values below 20 mm Hg, and sufficient cerebral perfusion (above 50 mm Hg) was regained. The patient showed a good recovery and was transferred to a rehabilitation center 5 weeks after injury. This case report emphasizes the importance of early and continuous intracranial pressure monitoring for adequate therapy in neurosurgical emergencies.

Transport of newly synthesized arylsulfatase A to the lysosome via transferrin receptor-positive compartments.

We have studied the convergence of the biosynthetic lysosomal route marked by the newly synthesized lysosomal enzyme arylsulfatase A (ASA) with the endosomal/prelysosomal compartment in ASA overexpressing baby hamster kidney (BHK) cells. A monoclonal antibody against ASA conjugated to transferrin (Tf-alpha ASA) was used to load the endocytic pathway via the transferrin receptor. Subsequent internalization of [125I]labeled ASA and Tf-alpha ASA conjugates at 18 degrees C followed by rewarming to 37 degrees C showed that immunocomplexes were formed within the recycling pathway and released into the medium. Furthermore, in cells labeled with [35S]methionine for 10 min about 54% of newly synthesized ASA passed into Tf-alpha ASA accessible compartments during a 4 hour chase period and accumulated in the medium. These data indicate that in overexpressing BHK cells the majority of newly synthesized ASA is transported to the lysosome via transferrin receptor-containing early endosomes.