Moyamoya: Indiana University Medical Center experience.

BACKGROUND: Moyamoya usually presents with cerebral ischemia in children and intracranial hemorrhage in adults. Treatment remains controversial. DESIGN AND OBJECTIVE: We reviewed our experience from June 1995 to August 1999 of 20 adult and pediatric angiographically diagnosed patients with moyamoya to report their clinical presentation, radiological findings, management, and clinical outcomes. RESULTS: The mean age of patients at symptom onset was 17 years (range, 2-54 years). Patients were divided into 2 age groups (group 1, <18 years; group 2, > or =18 years). There were 13 patients in group 1 and 7 patients in group 2. Ischemic strokes or transient ischemic attacks were the predominant initial presentations in both groups. One patient in group 2 had an intraparenchymal brain hemorrhage. Five patients received medical treatment, and 15 had surgical revascularization. The mean time from symptom onset to surgical procedure was significantly longer for patients in group 1 than for those in group 2 (P =.03). The mean follow-up time was 36 months. One patient in group 1 had an ischemic stroke. There was no difference in stroke recurrence, mortality, or modified Rankin scale score among medically or surgically treated patients. CONCLUSIONS: Moyamoya disease may have a different presentation and more benign natural history in our population than in Asian populations. Our findings emphasize the need to better understand the natural history of patients with moyamoya as well as the clinical benefit of different treatment modalities. Structured multicenter randomized clinical trials are needed to further assess the best treatment modalities for patients with moyamoya in the United States.

Protocol violations in community-based rTPA stroke treatment are associated with symptomatic intracerebral hemorrhage.

BACKGROUND: Recombinant tissue plasminogen activator (rTPA) is an established treatment for acute ischemic stroke. The rate and type of protocol violations in rTPA use and their effect on patient outcomes in this setting are not well understood. OBJECTIVE: The objective of this study was to examine associations between protocol violations and outcomes in community-based rTPA use. METHODS: We reviewed medical records of stroke patients treated with rTPA in 10 acute-care hospitals in Indianapolis from July 1996 to February 1998 and assessed complications and outcome. Retrospective National Institute of Health Stroke Scale (on admission and discharge), Canadian Neurological Scale, and length of hospital stay were calculated. Appropriate use of rTPA was determined by the National Institute of Neurological Disorders and Stroke (NINDS) protocol. RESULTS: Fifty patients (mean age, 66 years; 76% white; 56% men) were treated by general neurologists (70%), stroke neurologists (24%), or emergency physicians (6%). Mean times to hospital arrival, brain CT, and start of rTPA infusion were 44, 86, and 141 minutes, respectively. In-hospital mortality rate was 10% (4 intracerebral hemorrhage [ICH], 1 cardiogenic shock). Complications were more frequent among patients with protocol violations (n=8) compared with those without all hemorrhages (75% versus 10%, P:<0.001), symptomatic ICH (38% versus 5%, P:<0.02), and ICH attributable to rTPA, occurring within 36 hours (38% versus 2.4%, P:<0.01), respectively. CONCLUSIONS: NINDS protocol violations are relatively common and are associated with symptomatic cerebral and systemic hemorrhages. When the NINDS protocol is strictly followed, hemorrhage rates in community-based rTPA use are similar to those in the NINDS trial.

Paradoxical embolism to the basilar apex associated with May-Thurner syndrome.

BACKGROUND: Embolic occlusion of intracranial vessels can be caused by material arising proximally, most commonly from the heart, the aorta, or the carotid or vertebral arteries, and rarely from systemic veins. May-Thurner syndrome is an uncommon condition in which there is impaired venous return because of compression of the left common iliac vein by the overlying right common iliac artery, resulting in iliofemoral deep venous thrombosis. OBJECTIVE: To describe a young patient with presumed paradoxical embolism to the basilar apex associated with a patent foramen ovale and May-Thurner syndrome. DESIGN: Single case report. RESULTS: A 16-year-old girl with a history of bulimia and oral contraceptive use had a "top of the basilar" syndrome. She was found to have a patent foramen ovale on transthoracic and transesophageal echocardiography. Magnetic resonance venography of the lower extremities revealed May-Thurner syndrome. Antiphospholipid antibodies (antiphosphatidylserine, anticardiolipin, and antiphosphatidyl-ethanolamine), factor V Leiden mutation by polymerase chain reaction, and homocyst(e)ine levels were normal. Anticoagulation with intravenous unfractionated heparin sodium followed by warfarin sodium was used, resulting in resolution of her neurologic deficits. CONCLUSIONS: Deep venous thrombosis is notorious for its variable clinical manifestations and the potential dire consequences of a missed diagnosis. Physicians caring for patients with presumed paradoxical embolism should assess for May-Thurner syndrome.

Retrospective assessment of initial stroke severity with the NIH Stroke Scale.

BACKGROUND AND PURPOSE: It is important to adjust stroke outcomes for differences in initial stroke severity. The NIH Stroke Scale (NIHSS) is a commonly used stroke severity measure but has been validated for retrospective scoring only in a subset of stroke clinical trial participants. The purpose of this research was to assess the validity and reliability of an algorithm for retrospective NIHSS scoring in a setting with usual chart documentation. METHODS: An algorithm for retrospective NIHSS scoring was developed with written history and physical admission notes. Missing physical examination data were scored as normal. One investigator prospectively scored the admission NIHSS in 32 consecutive stroke patients. Two raters retrospectively scored the NIHSS by applying the algorithm to photocopied admission notes. Linear regression was used to assess interrater reliability and agreement between prospective and retrospective NIHSS scores. The Wilcoxon signed rank test was used to assess systematic scoring bias. Weighted kappa statistics were calculated to assess the level of agreement of individual NIHSS items. RESULTS: Only 1 admission note was complete for all NIHSS elements. Interrater reliability was near perfect (r(2)=0.98, P<0. 001). Agreement between prospective and retrospective NIHSS score was also excellent (r(2)=0.94, P<0.001) and there was no systematic bias in retrospective scores. Agreement for individual items was moderate to high for all items except level of consciousness. CONCLUSIONS: Retrospective NIHSS scoring with the algorithm is reliable and unbiased even when physical examination elements are missing from the written record. Stroke research using retrospective review of charts or of administrative databases should adjust for differences in stroke severity using such an algorithm.

Corticostriatal plasticity is restricted by myelin-associated neurite growth inhibitors in the adult rat.

After unilateral cortical lesions in neonatal rats, the spared unablated hemisphere is known to demonstrate remarkable neuroanatomical plasticity in corticofugal connectivity. This same type of structural plasticity is not seen after similar lesions in adult rats. One possibility for the lack of such a plastic response in the adult central nervous system may be the presence of myelin-associated neurite growth inhibitory proteins NI-35/NI-250. These proteins have previously been found to play a crucial role in preventing axotomized fibers from regenerating after adult rat spinal cord lesions. The aim of this study was to determine if blocking these inhibitory proteins by the application of the specific monoclonal antibody IN-1 would enhance corticostriatal plasticity from the spared hemisphere after unilateral cortical lesions in adult rats. Six- to 8-week-old Lewis rats underwent unilateral aspiration lesion of the sensorimotor cortex. Animals were immediately treated with either monoclonal antibody IN-1 or a control antibody released from hybridoma cells in Millipore filter capsules. After a survival period of 12 weeks, the opposite sensorimotor cortex was stereotaxically injected with the anterograde tracer biotinylated dextran amine, and biotinylated dextran amine-positive corticostriatal fibers were analyzed. The monoclonal antibody IN-1-treated animals showed an increase in corticostriatal fibers in the dorsolateral striatum contralateral to the injection site compared with control antibody-treated animals or normal controls, indicating a specific sprouting response in the deafferented zone. These results support the idea that through blockade of myelin-associated neurite inhibitory proteins, lesion-induced corticofugal plasticity is possible even in the adult central nervous system.