Erdheim-Chester Disease: A Case Report and Review of the Literature.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. ECD may be asymptomatic or present as a multi-systemic disease with life-threatening manifestations, most commonly involving the skeletal system. Immunohistochemical staining demonstrates cells that are CD68+, CD1a-, and S100- with an absence of Birbeck granules. We report a case of a 69-year old male patient who presented with neurological symptoms - eventually thought to be separate to his diagnosis of ECD. It represents the ability to diagnose ECD based just on radiological findings in an otherwise asymptomatic individual.

Outcome after stroke thrombolysis in patients >80 years treated within 3 hours vs >3-4.5 hours.

OBJECTIVE: To determine outcomes and risks of IV thrombolysis (IVT) in patients with acute ischemic stroke (AIS) >80 years of age within 3 hours compared to >3 to 4.5 hours recorded in the Safe Implementation of Treatment in Stroke (SITS) International Stroke Thrombolysis Registry. METHODS: A total of 14,240 (year 2003-2015) patients >80 years of age with AIS were treated with IVT ≤4.5 hours of stroke onset (3,558 in >3-4.5 hours). Of these, 8,658 (2,157 in >3-4.5 hours) were treated otherwise according to the European Summary of Product Characteristics (EU SmPC) criteria for alteplase. Outcomes were 3-month functional independence (modified Rankin Scale score 0-2), mortality, and symptomatic intracerebral hemorrhage (SICH)/SITS. Results were compared between the groups treated in >3 to 4.5 and ≤3 hours. RESULTS: Median age was 84 years; 61% were female in both groups. Median NIH Stroke Scale score was 12 vs 14 in the >3- to 4.5- and ≤3-hour group, respectively. Three-month functional independence was 34% vs 35% (adjusted odds ratio [aOR] 0.78, 95% confidence interval [CI] 0.69-0.89, p < 0.001); mortality was 31% vs 32% (aOR 1.10, 95% CI 0.97-1.25, p = 0.13); and SICH/SITS was 2.7% vs 1.6% (aOR 1.72, 95% CI 1.25-2.35, p = 0.001). In EU SmPC-compliant patients, 3-month functional independence was 36 vs 37% (aOR 0.79, 95% CI 0.68-0.92, p = 0.002), mortality was 29% vs 29.6% (aOR 1.10, 95% CI 0.95-1.28, p = 0.20), and SICH/SITS was 2.7% vs 1.6% (aOR 1.62, 95% CI 1.12-2.34, p = 0.01). CONCLUSIONS: In this observational study, unselected patients >80 years of age treated with IVT after 3 hours vs earlier had a slightly higher rate of SICH and similar unadjusted functional outcome but poorer adjusted outcome. The absolute difference between the treatment groups is small, and elderly patients should not be denied IVT in the later time window solely because of age without other contraindications.

IV thrombolysis in very severe and severe ischemic stroke: Results from the SITS-ISTR Registry.

OBJECTIVE: To study the safety of off-label IV thrombolysis in patients with very severe stroke (NIH Stroke Scale [NIHSS] scores >25) compared with severe stroke (NIHSS scores 15-25), where treatment is within European regulations. METHODS: Data were analyzed from 57,247 patients with acute ischemic stroke receiving IV tissue plasminogen activator in 793 hospitals participating in the Safe Implementation of Thrombolysis in Stroke (SITS) International Stroke Thrombolysis Registry (2002-2013). Eight hundred sixty-eight patients (1.5%) had NIHSS scores >25 and 19,995 (34.9%) had NIHSS scores 15-25. Outcome measures were parenchymal hemorrhage, symptomatic intracerebral hemorrhage, mortality, and functional outcome. RESULTS: Parenchymal hemorrhage occurred in 10.7% vs 11.0% (p = 0.79), symptomatic intracerebral hemorrhage per SITS-MOST (SITS-Monitoring Study) in 1.4% vs 2.5% (p = 0.052), death at 3 months in 50.4% vs 26.9% (p < 0.001), and functional independence at 3 months in 14.0% vs 29.0% (p < 0.001) of patients with NIHSS scores >25 and NIHSS scores 15-25, respectively. Multivariate adjustment did not change findings from univariate comparisons. Posterior circulation stroke was more common in patients with NIHSS scores >25 (36.2% vs 7.4%, p < 0.001), who were also more often obtunded or comatose on presentation (58.4% vs 7.1%, p < 0.001). Of patients with NIHSS scores >25, 26.2% were treated >3 hours from symptom onset vs 14.5% with NIHSS scores of 15-25. CONCLUSIONS: Our data show no excess risk of cerebral hemorrhage in patients with NIHSS score >25 compared to score 15-25, suggesting that the European contraindication to IV tissue plasminogen activator treatment at NIHSS levels >25 may be unwarranted. Increased mortality and lower rates of functional independence in patients with NIHSS score >25 are explained by higher stroke severity, impaired consciousness on presentation due to posterior circulation ischemia, and longer treatment delays.

Thrombolysis delivery by a regional telestroke network--experience from the U.K. National Health Service.

BACKGROUND: The majority of established telestroke services are based on "hub-and-spoke" models for providing acute clinical assessment and thrombolysis. We report results from the first year of the successful implementation of a locally based telemedicine network, without the need of 1 or more hub hospitals, across a largely rural landscape. METHODS AND RESULTS: Following a successful pilot phase that demonstrated safety and feasibility, the East of England telestroke project was rolled out across 7 regional hospitals, covering an area of 7500 square miles and a population of 5.6 million to enable out-of-hours access to thrombolysis. Between November 2010 and November 2011, 142 telemedicine consultations were recorded out-of-hours. Seventy-four (52.11%) cases received thrombolysis. Median (IQR) onset-to-needle and door-to-needle times were 169 (141.5 to 201.5) minutes and 94 (72 to 113.5) minutes, respectively. Symptomatic hemorrhage rate was 7.3% and stroke mimic rate was 10.6%. CONCLUSIONS: We demonstrate the safety and effectiveness of a horizontal networking approach for stroke telemedicine, which may be applicable to areas where traditional "hub-and-spoke" models may not be geographically feasible.

Emergency department or general practitioner following transient ischaemic attack? A comparison of patient behaviour and speed of assessment in England and Canada.

OBJECTIVE: Many patients present late after a transient ischaemic attack (TIA). This delays intervention and may partly depend on where patients first present--emergency department (ED) or general practitioner (GP). Studying this behaviour could improve stroke prevention through better targeting of public education and allocation of resources. METHODS: Patients with TIA or minor stroke referred to neurovascular clinics in the UK and Canada were studied and the delay from onset to first medical presentation, whether at an ED or GP, was measured. Clinical features, timing and place of presentation were compared. RESULTS: Of 666 patients (469 in the UK and 197 in Canada), only 42% presented on the day of the TIA. The majority (77%) of patients presenting to an ED presented on the same day compared with only 11% of those who presented to a GP. GP delays were longer at weekends. Motor or speech symptoms and prolonged duration were associated with presenting early and to an ED. High-risk patients (ABCD2 score 6-7) in Canada were also more likely to go to an ED. Overall, 65% of Canadian patients and 40% of UK patients went to an ED. CONCLUSIONS: Most patients presenting to an ED go urgently, whereas most going to a GP delay, particularly at weekends. Most Canadian patients, particularly those at high risk, go to an ED whereas most UK patients go to a GP. One way to reduce delay, particularly in the UK, would be to direct all patients with TIA to go to an ED rather than to their GP.