Creutzfeldt-Jakob Disease in the hospital setting: a case report and review.

Rapidly progressive dementia is a neurological condition that results in subacute deterioration in cognitive, behavioral and motor function. The most serious diagnosis for a patient with rapidly progressive dementia is Creutzfeldt-Jakob Disease (CJD), a prion-related illness that typically results in death within one year. However, there are numerous autoimmune, infectious and toxic-metabolic causes of rapidly progressive dementia that are potentially reversible with treatment. Thus, the differential diagnosis for a rapidly progressive dementia is critically important. In this article, the authors discuss a case of CJD diagnosed at a St. Paul hospital to illustrate the differential diagnosis of rapidly progressive dementia and highlight the role of neuroimaging.

Larger ACE 68 aspiration catheter increases first-pass efficacy of ADAPT technique.

PURPOSE: To report the efficacy of A Direct Aspiration first-Pass Thrombectomy (ADAPT) technique with larger-bore ACE aspiration catheters as first-line treatment for anterior circulation emergent large vessel occlusions (ELVOs), and assess for the presence of a first-pass effect with ADAPT. METHODS: We retrospectively reviewed 152 consecutive patients with anterior circulation ELVOs treated with the ADAPT technique as first-line treatment using ACE60, 64, or 68 at our institution. Baseline characteristics, procedural variables, and modified Rankin Scale (mRS) at 90 days were recorded. RESULTS: Fifty-seven patients were treated with ACE60 (37.5%), 35 with ACE64 (23%), and 60 with ACE68 (39.5%). Median groin puncture to reperfusion time was 30 min with ACE60, 26 min with ACE64, and 19.5 min with ACE68. Successful reperfusion after the first ADAPT pass was 33% with ACE60 and 53% with ACE68 (P=0.04). The stent-retriever rescue rate was 26% with ACE60, 3% with ACE64, and 10% with ACE68 (P=0.004). In multivariate logistic regression analysis, use of the ACE68 aspiration catheter was an independent predictor of successful reperfusion after the first ADAPT pass (P=0.016, OR1.67, 95% CI 1.1 to 2.54), and successful reperfusion after the first ADAPT pass was an independent predictor of good clinical outcome at 90 days (P=0.0004, OR6.2, 95% CI 2.27 to 16.8). CONCLUSION: Use of the larger-bore ACE 68 aspiration catheter was associated with shorter groin puncture to reperfusion time, higher rate of successful reperfusion after the first ADAPT pass, and lower rate of stent-retriever rescue. Further, a first-pass effect was demonstrated in our ADAPT patient cohort.

Comparison of clinical outcomes in patients with acute ischemic strokes treated with mechanical thrombectomy using either Solumbra or ADAPT techniques.

PURPOSE: To compare rates of symptomatic intracranial hemorrhage (SICH) and good clinical outcome at 90 days in patients with ischemic strokes from anterior circulation emergent large vessel occlusions (ELVO) treated with mechanical thrombectomy using either Solumbra or A Direct Aspiration first-Pass Thrombectomy (ADAPT) techniques. METHODS: We compared clinical characteristics, procedural variables, and clinical outcomes in patients with anterior circulation ELVOs treated with mechanical thrombectomy using either a Solumbra or ADAPT technique at our institution over a 38-month period. SICH was defined using the SITS-MOST criteria. A good clinical outcome was defined as a modified Rankin Scale score of 0-2 at 90 days. RESULTS: One hundred patients were included, 55 in the Solumbra group and 45 in the ADAPT group. Patients in the ADAPT group had higher National Institutes of Health Stroke Scale (NIHSS) (19.2 vs 16.8, p=0.02) and a higher proportion of internal carotid artery terminus thrombi (42.2% vs 20%, p=0.03) than patients in the Solumbra group. Patients in the ADAPT group had a trend toward a lower rate of SICH than patients in the Solumbra group (2.2% vs 12.7%, p=0.07). Patients in the ADAPT group had a significantly higher rate of good clinical outcome at 90 days than patients in the Solumbra group (55.6% vs 30.9%, p=0.015). Use of the ADAPT technique (OR 6 (95% CI 1.0 to 31.2), p=0.049) was an independent predictor of a good clinical outcome at 90 days in our cohort. CONCLUSIONS: In our cohort, the ADAPT technique was associated with significantly higher good clinical outcomes at 90 days in patients with acute ischemic stroke due to anterior circulation ELVOs treated with mechanical thrombectomy.

Late onset multiple sclerosis: Is it really late onset?

BACKGROUND: Multiple sclerosis (MS) is the most common demyelinating disease, and onset over the age of 50 years is referred to as late onset MS (LOMS). It has been thought that LOMS patients will be more likely to exhibit a primary progressive (PPMS) clinical course. OBJECTIVE: To identify the clinical characteristics of demyelinating disease in patients over the age of 50 years from four different MS centers in the Northern Midwest USA. METHODS: We reviewed medical records of all patients seen at the MS centers and identified those who were 50 years of age or more at the time of first spontaneously reported symptoms. We included those who were diagnosed with MS or clinically isolated syndrome (CIS) and excluded MS mimickers. Demographics, initial clinical course diagnosis, clinical characteristics, and any available five-year follow up data were collected. The clinical course was reevaluated in each patient with careful questioning regarding any prior focal neurological symptoms that had persisted for at least 48h, not otherwise explained. Those with a prior event who were initially diagnosed with PPMS or CIS were reclassified as secondary-progressive MS (SPMS) and relapsing-remitting MS (RRMS) respectively. RESULTS: We identified 124 patients from a total of 3700 patients, making LOMS 3.4% MS in our population. The initial clinical course was RRMS in 50 (40%), PPMS in 44 (36%), SPMS in 15 (12%), and CIS in 15 (12%) patients. After reclassification the clinical course was RRMS in 55 (44%), PPMS in 25 (20%), SPMS in 34 (28%), and CIS in 10 (8%) patients. The clinical syndrome was identified as acute for 77 patients (62%) with transverse myelitis (N=25, 32%) as the most common type. The clinical syndrome was chronic for 47 patients (37%) and again transverse myelitis (N=24, 51%) was the most common type. Five-year follow up data was available for 44% of these patients. DISCUSSION: LOMS is rare and RRMS is the most common clinical course. Reclassification of the clinical course, not done before in any other LOMS study, with careful questioning regarding a prior neurological event reveals that SPMS is the most common type of progressive MS and PPMS may be less common than previously thought. Transverse myelitis is the most common clinical presentation.

Patients with transverse myelitis who developed venous thromboembolism while hospitalized have increased rate for inpatient mortality.

The rate of venous thromboembolism (VTE) in patients with transverse myelitis (TM) is not known. Using the National Inpatient Sample database (NIS), from 2006-2010, the rate of VTE in TM patients was 1.3%, which included patients with both deep vein thrombosis (DVT) and pulmonary embolism (PE). The in-hospital mortality was higher in this group compared to TM patients without DVT and PE (11% and 0.5% respectively), and after adjusting for age, sex, presence of DVT and/or PE (odds ratio [OR], 21; 95% CI (2.2-207.0); p<0.008). The data supports the prompt initiation of VTE prophylaxis in patients hospitalized with TM.