Waiting for platelet counts causes unsubstantiated delay of thrombolysis therapy.

BACKGROUND: Platelet counts (PCs) <100,000/µl are considered as a contraindication for intravenous thrombolysis (IVT). While US guidelines recommend IVT initiation before the availability of clotting tests, the guidelines of the European Stroke Organization give no such practical advice. We aimed to assess the incidence of thrombocytopenia in IVT patients, outcome after thrombolysis in affected patients and the time gained by initiating treatment prior to availability of PC results. METHODS: All patients with thrombocytopenia were identified in our prospectively acquired thrombolysis database. Baseline demographic data, intracerebral hemorrhage rates as well as functional outcome were assessed. The median time between initiation of thrombolysis and availability of PCs was calculated. RESULTS: Of 625 IVT patients, 3 (0.5%) had thrombocytopenia at stroke onset. None of them developed intracerebral hemorrhage (ICH) or died during the follow-up. Waiting for PCs would have delayed treatment in 72.4% of the patients, with a median hypothetical delay of 22 min (interquartile range: 11-41 min). CONCLUSIONS: To date, there are no sufficient data to evaluate the ICH risk in thrombocytopenic patients. However, thrombocytopenia is rare in IVT patients. Thus, generally waiting for PC results prior to initiation of IVT is not warranted. Avoiding this significant delay yields shorter door-to-needle times and potentially more effective treatment.

Off-label thrombolysis for acute ischemic stroke: rate, clinical outcome and safety are influenced by the definition of 'minor stroke'.

BACKGROUND: Several contraindications for intravenous thrombolysis are not based on controlled trials. Specialized stroke centers often apply less restrictive criteria. The aim of our study was to analyze how many patients at our institution receive off-label thrombolysis. In addition, clinical outcome and safety data were compared to those from patients treated on-label, and the influence of different definitions of 'minor stroke' were examined. METHODS: Consecutive thrombolysis patients treated between January 2006 and January 2010 were included. Patients treated off-label were compared to patients given on-label therapy according to the European license. Since no specified definition for 'minor neurological deficit' exists in the license, two distinct definitions were considered off-label, i.e. National Institutes of Health Stroke Scale score (NIHSSS) <1 (definition 1) and NIHSSS ≤4 (definition 2). RESULTS: Of a total of 422 patients, 232 (55%) were treated off-label. The most prevalent off-label criteria (OLCs) were the following: age >80 years (n = 113), minor stroke (definition 1, n = 3; definition 2, n = 84), elevated blood pressure necessitating aggressive treatment (n = 75), time window >3 h (n = 71) and major surgery or trauma within the preceding 3 months (n = 20). In group comparisons, off-label patients had an overall worse outcome using definition 1 for minor stroke, while there was no difference when definition 2 was applied. In multivariate analysis, off-label therapy (definition 1) in general and age >80 years were independent predictors of poor outcome. None of the contraindications were associated with an increased bleeding risk. CONCLUSIONS: Off-label therapy is frequently applied at our center and is not associated with higher complication rates. Overall outcome of off-label treatment largely depends on the definition used for minor stroke. Besides age >80 years, a known poor prognostic factor, no other specific OLC was associated with poor outcome. Our data suggest that the criteria in the European license may be too restrictive.

Sonographic monitoring of ventricle enlargement in posthemorrhagic hydrocephalus.

BACKGROUND AND OBJECTIVE: Intraventricular hemorrhage often leads to obstructive hydrocephalus, necessitating placement of extraventricular drainage to prevent increasing intracranial pressure and subsequent herniation. For clamping and removal of the drainage, repeated CT scans are required to rule out recurrent hydrocephalus. We performed a prospective observational study on the use of transcranial duplex sonography to monitor changes in width of the lateral ventricles during clamping as an alternative to CT. METHODS: Patients with hydrocephalus after intracranial or subarachnoid hemorrhage were monitored by transcranial duplex sonography (TDS). Serial examinations were carried out before and directly after placement of extraventricular or lumbar drainage as well as every 12 hours until 48 hours after removal of all drainages. Clinicians were blinded for all ultrasound results. Receiver operating characteristic analysis and calculation of the positive and negative predictive values was used to identify the optimal cutoff point in increased ventricle width that best predicted reopening of the drainage by the clinician. RESULTS: Ninety-two attempts to clamp either lumbar or extraventricular drainage were monitored in 37 patients during a 1-year period. A cutoff value for increase of ventricular width of 5.5 mm yielded high sensitivity (100%) and specificity (83%) in combination with a 100% negative predictive value for reopening of the drainage. CONCLUSIONS: TDS can be used to monitor ventricular width in experienced neurologic intensive care units. Because of its noninvasive character and suitability for bedside use, it offers a valuable alternative to repeated CT scans.

[Myosin storage myopathy: a rare subtype of protein aggregate myopathies]. / Myosinspeichermyopathie: eine seltene Unterform der Proteinaggregationsmyopathien.

Myopathies with pathological protein aggregates comprise a numerically significant group of sporadic and hereditary muscle disorders. A rare disease entity within the group of protein aggregate myopathies is the myosin storage myopathy, which is caused by heterozygous mutations in the MYH7 gene which encodes the slow/beta-myosin heavy chain. We report the clinical, myopathological and MRI findings in the first German patient suffering from a myosin storage myopathy due to a heterozygous R 1845W missense mutation.

Autosomal dominant nemaline myopathy caused by a novel alpha-tropomyosin 3 mutation.

Nemaline myopathy (NM) is a genetically and clinically heterogenous muscle disorder, which is myopathologically characterized by nemaline bodies. Mutations in six genes have been reported to cause NM: Nebulin (NEB Pelin 1999), alpha-skeletal muscle actin (ACTA1 Nowak 1999), alpha-slow tropomyosin (TPM3 Laing 1995), beta-tropomyosin (TPM2 Donner 2002), slow troponin T (TNNT1 Johnston 2000) and cofilin 2 (CFL2 Agrawal 2007). The majority of cases are due to mutation in NEB and ACTA1. We report on the clinical, myopathological and muscle MRI findings in a German family with autosomal dominant NM due to a novel pathogenic TPM3 mutation (p.Ala156Thr).