ObjectiveTo describe the development of progressive multifocal leukoencephalopathy (PML) in patients with rheumatoid arthritis (RA) treated with rituximab.DesignCase study.SettingClinical care for patients with rheumatologic diseases. Most were referred to academic centers for care after diagnosis (Washington University, St Louis, Missouri; Karolinska Insitute, Stockholm, Sweden; and Royal Melbourne Hospital, Melbourne, Australia) while one was cared for in a neurology practice in Dallas, Texas, with consultation by an academic neurovirologist from the University of Colorado in Denver.PatientsFour patients developing PML in the setting of rituximab therapy for RA.InterventionRituximab therapy.Main Outcome MeasuresClinical and pathological observations.ResultsFour patients from an estimated population of 129 000 exposed to rituximab therapy for RA are reported in whom PML developed after administration of this drug. All were women older than 50 years, commonly with Sjögren syndrome and a history of treatment for joint disease ranging from 3 to 14 years. One case had no prior biologic and minimal immunosuppressive therapy. Progressive multifocal leukoencephalopathy presented as a progressive neurological disorder, with diagnosis confirmed by detection of JC virus DNA in the cerebrospinal fluid or brain biopsy specimen. Two patients died in less than 1 year from PML diagnosis, while 2 remain alive after treatment withdrawal. Magnetic resonance scans and tissue evaluation confirmed the frequent development of inflammatory PML during the course of the disease.ConclusionThese cases suggest an increased risk, about 1 case per 25 000 individuals, of PML in patients with RA being treated with rituximab. Inflammatory PML may occur in this setting even while CD20 counts remain low.

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Background: Fluid-attenuated inversion recovery (FLAIR) hyperintensity within an acute cerebral infarct may reflect delayed onset time and increased risk of hemorrhage after thrombolysis. Given the important implications for clinical practice, we examined the prevalence of FLAIR hyperintensity in patients 3–6 h from stroke onset and its relationship to parenchymal hematoma (PH). Methods: Baseline DWI and FLAIR imaging with subsequent hemorrhage detection (ECASS criteria) were prospectively obtained in patients 3–6 h after stroke onset from the pooled EPITHET and DEFUSE trials. FLAIR hyperintensity within the region of the acute DWI lesion was rated qualitatively (dichotomized as visually obvious or subtle (i.e. only visible after careful windowing)) and quantitatively (using relative signal intensity (RSI)). The association of FLAIR hyperintensity with hemorrhage was then tested alongside established predictors (very low cerebral blood volume (VLCBV) and diffusion (DWI) lesion volume) in logistic regression analysis. Results: There were 49 patients with pre-treatment FLAIR imaging (38 received tissue plasminogen activator (tPA), 5 developed PH). FLAIR hyperintensity within the region of acute DWI lesion occurred in 48/49 (98%) patients, was obvious in 18/49 (37%) and subtle in 30/49 (61%). Inter-rater agreement was 92% (ĸ = 0.82). The prevalence of obvious FLAIR hyperintensity did not differ between studies obtained in the 3–4.5 h and 4.5–6 h time periods (40% vs. 33%, p = 0.77). PH was poorly predicted by obvious FLAIR hyperintensity (sensitivity 40%, specificity 64%, positive predictive value 11%). In univariate logistic regression, VLCBV (p = 0.02) and DWI lesion volume (p = 0.03) predicted PH but FLAIR lesion volume (p = 0.87) and RSI (p = 0.11) did not. In ordinal logistic regression for hemorrhage grade adjusted for age and baseline stroke severity (NIHSS), increased VLCBV (p = 0.002) and DWI lesion volume (p = 0.003) were associated with hemorrhage but FLAIR lesion volume (p = 0.66) and RSI (p = 0.35) were not. Conclusions: Visible FLAIR hyperintensity is almost universal 3–6 h after stroke onset and did not predict subsequent hemorrhage in this dataset. Our findings question the value of excluding patients with FLAIR hyperintensity from reperfusion therapies. Larger studies are required to clarify what implications FLAIR-positive lesions have for patient selection.

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IntroductionAcquired demyelinating neuropathies comprise a diverse spectrum of individual diseases and pathophysiological processes. Differential diagnoses can be distinguished through assessment of region of involvement, time course, neurophysiology and ancillary testing. Where an atypical presentation of chronic inflammatory demyelinating polyradiculoneuropathy arises, further investigation and changes to management are required. We present a single case report from the Townsville Hospital.CaseA 45 year old man presented with two months of altered sensation in the distal lower extremities. Lower limb weakness developed, and three months after symptoms onset the patient had bilateral foot drop, and developed sensory disturbance in the upper limbs. Electrophysiological testing revealed severely reduced lower limb CMAPs with demyelinating range conduction velocity without conduction block; upper limb SNAPs were normal in amplitude with conduction velocity slowing. A lumbar puncture revealed elevated CSF protein 870 mg/L without raised white cells. A trace lambda IgG band of uncertain significance was detected. IVIG was commenced and symptoms initially stabilised. After four months of monthly IVIG, symptoms worsened and neurophysiology revealed further neurogenic changes. Skeletal survey and whole Spine MR STIR sequences did not reveal any bony lesions, and bone marrow biopsy revealed 5% plasmacytosis. The patient’s functional status deteriorated to full-time wheelchair use despite escalation of therapy. Sural nerve biopsy revealed axonal loss and demyelination without inflammation. A final diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes) syndrome was confirmed with vascular endothelial growth factor elevation, and the patient underwent an autologous stem cell transplant with significant improvement in symptoms and functional status by day 100.ConclusionPOEMS syndrome is a rare disorder and should be suspected in atypical cases of CIDP particularly when treatment resistance is present. Extensive investigation is often required to meet diagnostic criteria for POEMS.

ObjectivesTo compare patient satisfaction and perception of tele-epilepsy versus face-to-face consultations in North Queensland.MethodsA questionnaire was completed by consecutive epilepsy patients following consultant neurologist assessment in Townsville, Queensland between October 2013 and October 2014. Telehealth consults were conducted by videoconference between the neurologist and the patient with their local GP/hospital. Questions were answered on a scale of 1 (strongly disagree) to 5 (strongly agree).ResultsSixty-eight surveys were completed; 49 face-to-face and 19 telehealth, with three patients completing both. While the face-to-face group scored significantly greater satisfaction (mean total score 48.91/50, SD 2.54) compared with the telehealth group (mean 46/50, SD 4.95), the absolute difference was low, likely reflective of different numbers in each group. Cumulative scoring of favourable outcomes (strongly agreed, agreed, neutral) demonstrated that all tele-epilepsy patients indicated satisfaction with care provided by the consultant with the help of local staff (84.2%, 10.5% and 5.3%, respectively) and none of the tele-epilepsy patients reported difficulty seeing the doctor through video (89.5%, 10.5%). All telehealth consulted patients reported that the telehealth model saved them time and money. Nearly all (94.7%: 68.4%, 10.5% and 15.8%) of tele-epilepsy patients indicated preference for further video consultation rather than travel for in person attendance.ConclusionsDespite significantly higher face-to-face patient satisfaction, tele-epilepsy patients reported high levels of satisfaction with the tele-epilepsy model of care. As such, tele-epilepsy is a viable model for the delivery of epileptic care within regional settings. Our study was not focused on outcomes or efficacy therefore further research is needed on whether tele-epilepsy provides equal outcomes in care.

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