Eligibility for intravenous thrombolysis in acute ischemic stroke patients presenting in the 4.5-9 h window.

PURPOSE: Recent randomized-controlled clinical trials have provided preliminary evidence for expanding the time window of intravenous thrombolysis (IVT) in acute ischemic stroke (AIS) patients by applying certain neuroimaging criteria. We prospectively assessed the potential eligibility for IVT in the extended time window (4.5-9 h) among consecutive AIS patients treated in a comprehensive stroke center during a nine-month period. METHODS: Potential eligibility for IVT in the extended time window was evaluated by using inclusion criteria from the EXTEND trial. All patients were underwent baseline emergent neurovascular imaging using either computed tomography angiography/computed tomography perfusion (CTA/CTP) or magnetic resonance angiography/magnetic resonance perfusion (MRA/MRP). Images were post processed by the automated software RAPID. RESULTS: Our study population consisted of 317 AIS patients, and, among them, 31 (9.8 %) patients were presented in the time window of 4.5-9 h. Seven patients (2.2 %) fulfilled the EXTEND neuroimaging criteria. Four patients (1.3 %) were treated with IVT because they fulfilled both clinical and neuroimaging EXTEND criteria. Patients eligible for EXTEND neuroimaging criteria had no ischemic core lesion, whereas the mean volume of critical hypoperfusion was relatively small (17.0 ± 11.8 ml). There was no hemorrhagic complication in any of the patients treated with IVT. The median mRS score at three months was 0 (range: 0-3) among patients who were eligible for EXTEND neuroimaging criteria. CONCLUSION: Our everyday clinical practice experience suggests 9.8 % of consecutive AIS patients present in the 4.5-9 h window and 2.2 % adhere to EXTEND neuroimaging eligibility criteria for IVT. Only 1.3% of AIS is eligible for IVT according to EXTEND neuroimaging and clinical eligibility criteria.

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult.

Anti-NMDA receptor (NMDA-r) encephalitis is a relatively rare cause of autoimmune encephalitis with divergent clinical presentations. We report a case of an adult patient with anti-NMDA-r encephalitis presenting with isolated, abrupt-onset aphasia. Her condition remained unaltered over a period of 6 months. The patients' electroencephalogram findings were typical for NMDA-r encephalitis; however, her magnetic resonance imaging and cerebrospinal fluid analysis were normal. She responded well to immunotherapy, and aphasia eventually resolved. The natural course of the present case contradicts the rapidly progressive nature of typical NMDA-r encephalitis. Furthermore, it broadens the clinical spectrum of anti-NMDA-r encephalitis, to incorporate isolated, nonprogressive aphasia.

Simple linear brainstem MRI measurements in the differential diagnosis of progressive supranuclear palsy from the parkinsonian variant of multiple system atrophy.

Differential diagnosis of progressive supranuclear palsy (PSP) and the parkinsonian variant of multiple system atrophy (MSA-P) from Parkinson's disease (PD) can be difficult, particularly in atypical cases or early in the disease course. The Magnetic Resonance Parkinsonism Index (MRPI) utilizes linear and surface (planimetry) measurements and has been proposed as a dual MRI biomarker, with high values indicative of PSP and low values of MSA. The aim of this study was to examine the utility of simple linear MRI brainstem measurements, without the use of MRI planimetry, in the diagnosis of patients with Parkinsonism and compare them to the MRPI. A total of 51 patients (PSP: 24, MSA-P: 9, PD: 18) and 15 healthy controls were included. Simple linear MRI distances of brainstem structures were measured. These included midbrain and pons diameters as well as superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) widths. All relevant indices, including ratios and products, were also calculated. The SCP by midbrain product (SCP × midbrain) provided improved sensitivity (100 vs. 91%) and identical specificity (98%) for the diagnosis of PSP, compared to the MRPI. Neither the MRPI nor any of the linear measurements were able to discriminate MSA-P from PD. The SCP by midbrain product is a novel, potent MRI biomarker for PSP.

Neurocognitive impairment in Whipple disease with central nervous system involvement.

Young-onset dementias pose a major challenge to both clinicians and researchers. Cognitive decline may be accompanied by systemic features, leading to a diagnosis of "dementia plus" syndromes. Whipple disease is a rare systemic illness characterized by arthralgias, chronic diarrhea, weight loss, fever, and abdominal pain. Central nervous system involvement, including severe cognitive deterioration, may precede systemic manifestations, appear during the course of the disease, or even be the only symptom. We report a previously highly functional 48-year-old man whom we first suspected of having early-onset neurodegenerative dementia but then diagnosed with Whipple disease based on a detailed clinical and laboratory evaluation. Initial neuropsychological evaluation revealed marked impairment in the patient's fluid intelligence and severe cognitive deficits in his information processing speed, complex attention, memory, visuomotor and construction dexterities, problem solving, and executive functions. At neuropsychological follow-up 21 months later, his information processing speed had improved only slightly and deficits persisted in his other cognitive functions. Repeat brain magnetic resonance imaging at that time showed that he had responded to antibiotic treatment. Because Whipple disease can cause young-onset "dementia plus" syndromes that may leave patients with neurocognitive deficits even after apparently successful treatment, we recommend comprehensive neuropsychological assessment for early detection of residual and reversible cognitive processes and evaluation of treatment response.

Evidence of an association between the scavenger receptor class B member 2 gene and Parkinson's disease.

Lysosomal protein 2 (LIMP2), the product of the scavenger receptor class B member 2 (SCARB2) gene, is a ubiquitously expressed transmembrane protein that is the mannose-6-phosphate-independent receptor for glucocerebrosidase (ß-GCase); a deficiency in this protein causes Gaucher disease. Several studies have shown a link between mutations in the ß-GCase gene and diseases characterized clinically by Parkinsonism and by the presence of Lewy body-related pathology. We hypothesized that genetic variants in the SCARB2 gene could be risk factors for Parkinson's disease (PD). A candidate-gene study of 347 Greek patients with sporadic PD and 329 healthy controls was conducted to investigate the association between 5 polymorphisms in the SCARB2 gene (rs6824953, rs6825004, rs4241591, rs9991821, and rs17234715) and the development of PD. The single-locus analysis for the 5 polymorphisms revealed an association only for the rs6825004 polymorphism: the generalized odds ratio (OR(G) ) was 0.68 (95% confidence interval [CI], 0.51-0.90), and the OR for the allelic test was OR = 0.71 (95% CI, 0.56-0.90). Haplotype analysis showed an association for the GCGGT haplotype (P < .01). Our study supports a genetic contribution of the SCARB2 locus to PD; future studies in larger cohorts are necessary to verify this finding.

Peripheral neuropathies in Sjogren syndrome: a new reappraisal.

BACKGROUND: The prevalence of peripheral neuropathy in patients with Sjögren syndrome remains unclear owing to conflicting results in the published series, with numbers ranging from 2% to over 60% of Sjögren syndrome patients. Whether peripheral neuropathy is a feature of the systemic or glandular disease or whether it is related to a circulating antineuronal antibody remains also uncertain. METHODS: The authors reviewed the records of patients with primary Sjögren syndrome (pSS), fulfilling the Revised European-American Classification Criteria, seen in their department from 1992 to 2009. The patients with previously recorded neuropathic features were re-examined clinically and electrophysiologically. Other causes of polyneuropathy were excluded. The authors also searched for circulating antineural antibodies using immunofluorescence and western blot and for antibodies against muscarinic and nicotinic acetylcholine receptors as potential biomarkers. RESULTS: 509 cases met the diagnostic criteria for pSS. Among these, 44 patients were recorded as having neuropathic symptoms. After completing the evaluation, however, only nine (1.8%) had polyneuropathy with objective clinical signs and abnormal electrophysiological findings. The neuropathy was axonal in all, in five pure sensory and in four sensorimotor. The patients with peripheral neuropathy had extraglandular manifestations such as palpable purpura and vasculitis. No evidence of antineural autoimmunity was found, and no candidate biomarkers were identified. CONCLUSION: Polyneuropathy is a rare manifestation of pSS occurring in 1.8% of patients. In the majority of patients, it is a late event and frequently associated with systemic disease or risk factors for lymphoma development.

Association between asymptomatic median mononeuropathy and diabetic polyneuropathy severity in patients with diabetes mellitus.

BACKGROUND: Asymptomatic median mononeuropathy (AMM) and diabetic polyneuropathy (DPN) often coexist and can be difficult to distinguish electrophysiologically. Moreover, the potential association between AMM and DPN has not been extensively evaluated. OBJECTIVE: We investigated the relation between AMM and DPN severity in consecutive diabetic patients. METHODS: The non-dominant limb was studied electrophysiologically in 100 consecutive diabetic patients with no symptoms of carpal tunnel syndrome on the non-dominant side. AMM was diagnosed based on previously validated electrophysiological criteria. DPN severity was graded according to the Michigan diabetic neuropathy score. RESULTS: AMM was discovered in 28% of the study population (Adjusted Wald 95% CI: 20%-37%). It was more common in women, displayed a tendency of being more common in patients over 50 years old and correlated with the severity of DPN and the number of abnormal nerves on nerve conduction studies. It was present in 18.1% of patients without evidence of DPN. No correlation was found with the duration and type of diabetes. In multivariate logistic regression models increasing severity of DPN was independently associated with the presence AMM (Wald test=10.557, df=3, p=0.014). Patients with DPN stage III and IV had a five-fold (OR=5.06, 95% CI=1.49-17.19) and a four-fold (OR=4.50, 95% CI=1.15-17.65) respectively increased likelihood to present with AMM in comparison to DPN stage I (reference group). CONCLUSIONS: Our results confirmed the high incidence of AMM in diabetic patients. AMM was present in a significant number of patients in the absence of DPN and the likelihood of AMM detection increased with increasing severity of DPN.

Tenecteplase Averting Mechanical Thrombectomy in Emergent Large Vessel Occlusion.

INTRODUCTION: Tenecteplase has recently been studied as an alternative thrombolytic agent in acute stroke, with a possible superior effect in achieving reperfusion of large intracranial vessels. CASE REPORT: A 90-year-old female patient was admitted to our stroke unit because of acute onset of dysarthria, left-sided neglect, and hemiparesis. Brain computed tomography (CT) coupled with CT angiography and CT perfusion (postprocessed with the use of RAPID software) demonstrated right proximal middle cerebral artery occlusion with a large penumbra/small ischemic core pattern. The patient was subsequently treated with bolus tenecteplase infusion (0.25 mg/kg). Mechanical thrombectomy was abandoned because the patient has rapidly improved. The patient was discharged to her own home 4 days later with no neurological deficit and functionally independent (modified Rankin scale of 0). CONCLUSION: This case exemplifies the potential of tenecteplase in achieving swift reperfusion in patients with large vessel occlusion associated with a substantial mismatch penumbral pattern.