Pupillary response to sparse multifocal stimuli in multiple sclerosis patients.

OBJECTIVES: The objective of this paper is to investigate the pattern of abnormalities and establish the diagnostic power of multifocal objective pupil perimetry (mfPOP) in multiple sclerosis (MS). METHODS: A prospective study enrolling 35 normal (47.9 ± 16.8 years, 22 females) and 85 MS subjects (49.8 ± 11.3 years, 62 females; 72 relapsing-remitting (RR), and 13 primary or secondary progressives (PorS)). EDSS scores for the RR and PorS groups were 3.53 ± 1.04 (mean ± SD), and 5.9 ± 1.43, respectively. mfPOP responses were obtained from 44 regions/visual field. Each region was analysed according to response time-to-peak and standardised amplitude (AmpStd). Predictive power was measured by percentage area under the receiver operator curve (%AUC). RESULTS: mfPOP responses showed a significant reduction of 0.69 ± 0.04 dB (mean ± SE) in AmpStd and significantly delayed time-to-peak of 25.95 ± 0.89 ms (mean ± SE) in MS subjects compared to control subjects (p<0.001). %AUC was greater for time-to-peak than AmpStd both for RR and PorS patients. Diagnostic power followed the EDSS scores but not a history of optic neuritis. CONCLUSIONS: mfPOP is well tolerated and potentially has a role in the diagnosis and assessment of patients with MS.

Job variation in advanced training in adult neurology in Australia and New Zealand: a follow-up study.

BACKGROUND: Six years ago, a survey of Australian trainees in neurology highlighted several differences in the training offered by the various positions. There has been a subsequent increase in trainee numbers. AIM: This survey aimed to re-examine the workload and exposure provided by individual positions and to compare training in Australia and New Zealand. METHODS: A questionnaire was circulated in 2012 to all advanced trainees in core adult neurology positions in Australia and New Zealand, looking at ward work, outpatient clinics, neurophysiology exposure and on-call commitments. RESULTS: The response rate was 85.7%. There was a 48.7% increase in the number of core training positions in Australia, but an average increase in inpatient workload of 56%. General neurology clinic numbers were unchanged while specialist clinic exposure had risen from 1.0 to 1.8 clinics/week. In some cases, exposure to neurophysiology had fallen. The requirement for out-of-hours on-call had fallen. There were no major differences between positions in Australia and New Zealand. CONCLUSION: There have been significant improvements in advanced training in adult neurology in the 5 years between 2007 and 2012: numbers of trainees have increased, on-call commitments have fallen and exposure to specialist clinics has risen. However, inpatient workload has increased significantly, accompanied by a slight reduction in exposure to training in neurophysiology in some cases. Overall, the changes are encouraging, but more work is still needed to ensure that individual positions meet the training needs of trainees.

Assessment of point-of-care measurement of international normalised ratio using the CoaguChek XS Plus system in the setting of acute ischaemic stroke.

BACKGROUND AND AIMS: Thrombolysis with alteplase (recombinant tissue plasminogen activator) is accepted hyperacute therapy for acute ischaemic stroke. Clotting must be normal before this can be administered safely. Laboratory testing of international normalised ratio (INR) takes 30-60 min, which can significantly delay administration of recombinant tissue plasminogen activator. Previous studies have suggested that point-of-care testing is useful in patients presenting with stroke and improves door-to-needle time. We performed a prospective study of point-of-care testing in patients presenting with acute ischaemic stroke. METHODS: Fifty patients were entered into the study to compare point-of-care testing using the CoaguChek XS system with laboratory testing of INR. RESULTS: Point-of-care testing correlated well with laboratory levels (R = 0.93, P < 0.0001). The standard deviation of difference between the two was 0.115. Overall, point-of-care testing tended to underestimate INR slightly, meaning that an INR value of 1.1 or less was required to be 95% certain that the laboratory value was 1.3 or below. Simultaneous testing using blood from a syringe was more consistent with laboratory results than testing capillary blood through finger prick. CONCLUSION: Point-of-care INR testing correlates well with laboratory values. The results in this study mostly relate to values in the normal range. We suggest that it can be used to try to shorten door-to-needle time.

Response of HIV-infected patients with syphilis to therapy with penicillin or intravenous ceftriaxone.

BACKGROUND: Ceftriaxone is commonly used as an alternative antibiotic drug in treating syphilis but clinical data on its efficacy are limited. OBJECTIVE: To evaluate the response of HIV-infected patients with active syphilis to treatment with penicillin or ceftriaxone. - METHODS: A retrospective study involving 24 consecutive patients with a positive Veneral Disease Research Laboratory test (VDRL) and at least one specific treponemal test. 12 patients were treated with different regimens of high-dose penicillin G for at least 2 weeks. Another 12 patients were treated with ceftriaxone 1-2g per day intravenously for 10-21 days. - RESULTS: After a median follow up of 18,3 months all patients of the penicillin-treated group and 11 of 12 ceftriaxone-treated patients showed a ≥ 4-fold decline in VDRL-titers; 91% of them already within 6 months after therapy. - CONCLUSION: Our serological data demonstrate a comparable efficacy of currently recommened penicillin and ceftriaxone treatment regimens for active syphilis in HIV-infected patients.

[Cancer patients in the intensive care unit]. / Der Krebspatient auf der Intensivstation.

Over the past 20 years, demographic changes and a longer life expectancy of cancer patients has significantly increased the prevalence of this patient group in the intensive care unit (ICU). A fundamental finding is that acute organ dysfunction, rather than the underlying malignancy, determines the prognosis of ICU patients. While hematologic patients often suffer from a more severe disease course, patients with solid tumors do not present an increased hospital mortality compared to the normal population. As with other indications, the decision to transfer a cancer patient to an ICU should be made as soon as possible. While early transfer is associated with reduced hospital mortality, the presence of multiorgan failure on ICU admission is associated with increased mortality. Overall, the intensive care and hospital survival of critically ill hematologic or oncologic patients has improved over the last two decades and is now as high as 50 to 60%. After surviving an intensive care stay, one fifth of all patients have a good long-term prognosis. Thus, the former paradigm of general rejection of cancer patients for ICU care is no longer justified. For optimal care of cancer patients requiring intensive care, close cooperation between hematologists/oncologists and intensive care physicians is essential.

Reversible posterior encephalopathy syndrome associated with bortezomib.

Reversible posterior encephalopathy (RPES) is an uncommon neurological syndrome that is being increasingly reported in association with anti-neoplastic therapies. The first case of reversible posterior encephalopathy associated with the proteosome inhibitor bortezomib is described and the reported experience of the occurrence of RPES with other antineoplastic therapies reviewed. Dysregulation of cerebral vasomotor autoregulation is postulated as the underlying pathophysiology in this case of bortezomib associated RPES.

[Prevalence of cancer patients in German intensive care units]. / Prävalenz von Krebspatienten auf deutschen Intensivstationen.

INTRODUCTION: Cancer is one of the leading causes of death worldwide. Due to increasing comorbidities, age and aggressive chemotherapy, care of cancer patients in intensive care units (ICUs) is more and more necessary. So far, little is known about the care structure of cancer patients in German ICUs. The aim of this work is to collect and evaluate the prevalence and care data of cancer patients on two reference dates. METHODS: German ICUs were invited to participate in a 2-day, prospective, multicenter point prevalence study in ICU cancer patients. Participation in the study was voluntary and the study was not funded. An ethics vote was obtained to conduct the study. The data were anonymously entered into an eCRF (electronic case report form) by the participating centers. Identification of the patients is therefore not possible. RESULTS: About one in four patients on the ICU/IMC ward had hematological-oncological (HO) disease (n = 316/1319, 24%). The proportion depended significantly on the number of beds in each hospital. The most frequent reasons for admission to the ICU/IMC station were postoperative monitoring (n = 83/221, 37.6%), respiratory instability (n = 79/221, 35.7%), circulatory instability (n = 52/221; 23.5%) and the severe infection with sepsis (n = 47/221; 21.3%). In all, 66.5% (n = 147/221) of the patients had a solid tumor and 21.7% (n = 48/221) had hematological cancer, 78.3% (n = 173/221) of the documented cancer patients received "full-code" intensive management, while 42.5% (n = 94/221) of the HO patients were ventilated and 40.7% (n = 90/221) required catecholamines. The median (mean; IQR) SAPS II score was 35 (37.79, IQR = 24-48) and the median (mean, IQR) TISS score was 10 (13.26, IQR = 10-15). Through the analysis and evaluation of the data available in the context of the prevalence study, it was possible for the first time to determine the Germany-wide cross-center prevalence and care situation of hematological cancer patients in intensive care and intermediate care stations. About one in four patients on German ICUs and IMC wards have a major or minor cancer diagnosis (n = 316/1319 = 24%). Care management is complex in this patient population and requires close interdisciplinary collaboration.

A new dominantly inherited pure cerebellar ataxia, SCA 30.

BACKGROUND: The spinocerebellar ataxias (SCAs) are clinically and genetically heterogeneous. Currently, 27 forms are known, with the causative gene identified in 16. Although the majority of dominant pedigrees worldwide have SCAs 1, 2, 3, 6 or 8, new SCAs continue to be delineated. We describe a new disorder: SCA 30. METHODS: An Australian family of Anglo-Celtic ethnicity manifested a relatively pure, slowly evolving ataxia. Six affected and four unaffected members were personally examined in a standardised fashion. MRI and nerve conduction studies were performed in two. An autosomal genome-wide linkage study was undertaken, and an in silico analysis of potential candidate genes in the linkage region was performed. RESULTS: The six affected members had a relatively pure, slowly evolving ataxia developing in mid to late life, with only minor pyramidal signs and no evidence of neuropathy. All had hypermetric saccades with normal vestibulo-ocular reflex gain. Only one displayed (slight) gaze-evoked nystagmus. MRI showed cerebellar atrophy with preservation of nodulus/uvula and brainstem. Linkage analysis excluded currently known SCAs and identified a logarithm (base 10) of odds score of 3.0 at chromosome 4q34.3-q35.1, distinct from all previously reported loci. In silico prioritisation identified the gene ODZ3 as the most likely contender. CONCLUSIONS: SCA 30 is a previously undescribed cause of (relatively) pure adult-onset autosomal dominant cerebellar ataxia. The responsible gene is yet to be determined, but ODZ3 is a plausible candidate.

Atrial fibrillation and anticoagulation in a stroke unit population.

BACKGROUND: Atrial fibrillation (AF) is an important predisposing factor for ischaemic stroke. There is evidence to suggest that even in appropriate candidates warfarin therapy is underutilized. We assessed the prevalence of AF in an Australian stroke unit to determine the degree of undertreatment at presentation. METHODS: A retrospective analysis of all patients admitted to our Stroke Unit between October 2004 and September 2006 was carried out. All patients with a diagnosis of AF, either new or old, were then selected from this group to determine the overall prevalence and anticoagulation status. Data regarding prior stroke, stroke severity and discharge anticoagulation status were also determined. RESULTS: Data from a total of 500 patients were analysed. Our results showed that AF-related strokes accounted for a large proportion (28%) of all admissions and were associated with a larger neurological deficit. Most patients (68%) with a prior diagnosis of AF without having obvious contraindications were either not anticoagulated or under-anticoagulated when presenting with an ischaemic stroke or transient ischaemic attack. CONCLUSION: Our results stress the importance of initiating and maintaining anticoagulation in patients with AF and without obvious contraindications to minimize the risk of subsequent stroke.

Job variation in Australian advanced training in neurology.

BACKGROUND: Like training posts in other medical specialties, many of the 38 Australian core training posts in neurology have been criticized over a variety of issues relating to the quality of training provided. These issues include excessive hours of work (often related to understaffing), high inpatient workload and inadequate exposure to outpatients and/or specialist procedures. To examine these issues, we conducted an audit of Australian advanced training posts in neurology to obtain baseline data. METHODS: Two questionnaires were sent out, one to each head of department and another to the advanced trainees currently in post, requesting information about each of the training posts. The posts were compared with each other on an individual basis and by grouping them into three geographically related groups. RESULTS: There was complete ascertainment and a wide variation in most of the measures examined, including inpatient and ward consult numbers, staffing levels, general neurological and specialist outpatient clinic exposure and overtime requirements. Exposure to neurophysiology clinics and training in electroencephalogram was more uniform. CONCLUSION: Core advanced training jobs in neurology vary considerably across Australia, largely for historical reasons. This situation is suboptimal for many reasons. Training jobs ideally need to be modified to take into account the changing needs of trainees rather than just the service requirements of the various departments, but there are many resource issues involved in achieving this.

Interventions for idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension occurs throughout the world with an estimated incidence of one to three per 100,000 population per year. It occurs most commonly in obese young women but the cause is unknown. It presents a significant threat to sight and is associated with severe morbidity, in the form of headaches in most cases. Several different treatments have been proposed ranging from relatively conservative measures such as diuretic therapy to more invasive treatments such as optic nerve sheath fenestration, stenting of cerebral venous sinuses, or lumbo-peritoneal shunting. OBJECTIVES: The objective of this review was to assess the effects of various forms of treatment for idiopathic intracranial hypertension with a view to producing an evidence-based treatment strategy. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), which contains the Cochrane Eyes and Vision Group Trials Register, on The Cochrane Library (Issue 4, 2004), MEDLINE (1966 to January 2005) and EMBASE (1980 to January 2005). SELECTION CRITERIA: We included only randomised controlled trials in which any intervention used to treat idiopathic intracranial hypertension had been compared to placebo or to another form of treatment in people with a clinical diagnosis of idiopathic intracranial hypertension. DATA COLLECTION AND ANALYSIS: Both authors independently assessed the search results for trials to be included in the review. Discrepancies were resolved by discussion. Since no trials met our inclusion criteria, no assessment of quality or meta-analysis was undertaken. MAIN RESULTS: No randomised controlled trials were found that met the inclusion criteria. AUTHORS' CONCLUSIONS: There is insufficient information to generate an evidence-based management strategy for idiopathic intracranial hypertension. There is inadequate information regarding which treatments are truly beneficial and which are potentially harmful. Properly designed and executed trials are needed.

Saccadic eye movements in essential blepharospasm.

To provide evidence of an organic pathology for essential (idiopathic) blepharospasm, reflex saccadic eye movements in response to randomly stepped visual targets were assessed in seven affected patients and seven age-matched controls using the magnetic scleral search coil technique. The results indicate a significant prolongation in latency and a reduction in gain of horizontal saccades, and an increase in latency and reduction of peak velocity of large downward saccades. These findings suggest an organic component to the aetiology of blepharospasm, the pathology of which also appears to involve the oculomotor system. The pattern of the oculomotor disorder does not allow specific localisation but is consistent with the underlying pathology being localised in the basal ganglia.

Increase in saccadic peak velocity with increased frequency of saccades in man.

Twelve normal subjects (aged 22-80 yr, mean 47 yr) performed three blocks of 20 saccades made to LED targets stepped back and forth. The first and last blocks were performed at a (slow) rate of 0.18 Hz, while the middle block was performed at the faster rate of 1.15 Hz. Mean saccadic amplitude was unaffected by saccade rate, but latency and duration became shorter at the higher frequency. Most interestingly, the peak velocity increased by approx. 6% when saccades were performed at the higher rate. This increase was statistically significant, even after normalization for saccade amplitude. That saccadic frequency may affect saccadic peak velocity must be considered as a potential variable when analysing saccades.

Interventions for idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) occurs throughout the world with an estimated incidence of one to three per 100,000 population per year. It occurs most commonly in obese young women, but the cause is unknown. It presents a significant threat to sight and is associated with severe morbidity in the form of headaches in the majority of cases. Several different treatments have been proposed, ranging from relatively conservative measures such as diuretic therapy to more invasive treatments such as optic nerve sheath fenestration or lumbo-peritoneal shunting. OBJECTIVES: The aim of this review is to assess the evidence from controlled trials looking at the various treatments used for idiopathic intracranial hypertension with a view to producing an evidence-based treatment strategy. SEARCH STRATEGY: We searched the Cochrane Controlled Trials Register - CENTRAL/CCTR (which contains the Cochrane Eyes and Vision Group specialised register) on the Cochrane Library Issue 2, 2002, MEDLINE (1966 to March 2002) and EMBASE (1980 to February 2002). SELECTION CRITERIA: We included only randomised controlled trials in which any intervention used to treat idiopathic intracranial hypertension had been compared to placebo or to another form of treatment in people with a clinical diagnosis of idiopathic intracranial hypertension. DATA COLLECTION AND ANALYSIS: Two reviewers independently assessed the search results for trials to be included in the review. Discrepancies were resolved by discussion. Since no trials met our inclusion criteria, no assessment of quality or meta-analysis was undertaken. MAIN RESULTS: No randomised controlled trials were found that met the inclusion criteria. REVIEWER'S CONCLUSIONS: There is insufficient information to generate an evidence-based management strategy for idiopathic intracranial hypertension. Of the various treatments available, there is inadequate information regarding which are truly beneficial and which are potentially harmful. Properly designed and executed trials are needed.

The ability of pulse oximetry to screen for hypoxemia and hypercapnia in patients breathing room air.

This study was designed to determine whether high room-air pulse oximetry can rule out hypoxemia or moderate hypercapnia. Based on retrospective analysis of 513 arterial blood gas results, oxygen saturation cutpoints were derived. Coincidentally, a room-air oxygen saturation (RAO2 sat) value of 96% was selected as a cutpoint to screen for both hypoxemia (PaO2 < 70 mm Hg) and moderate hypercapnia (PaCO2 > 50 mm Hg). These tests were validated prospectively by using a convenience sample of 213 Emergency Department patients in whom room-air arterial blood gas sampling was ordered. To detect hypoxemia, the sensitivity of RAO(2) sat < or = 96% was 1.0 [0.95-1.0, 95% confidence interval (CI)] and specificity was 0.54 (0.45-0.64, 95% CI). To detect hypercapnia, the sensitivity of RAO(2) sat < or = 96% was 1.0 (0.7-1.0) and specificity was 0.31 (0.25-0.38, 95% CI). We concluded that RAO(2) sat > or = 97% rules out hypoxemia and may also rule out moderate hypercapnia.

Bench/step training with and without extremity loading. Effects on muscular fitness, body composition profile, and psychological affect.

BACKGROUND: To study the effect of bench/step group exercise with and without extremity loading on muscular fitness, body composition, and psychological affect. EXPERIMENTAL DESIGN: a prospective training study. SETTING: general community fitness center. PARTICIPANTS: 44 healthy adult females (age: 21-51 yrs). INTERVENTIONS: 12 weeks of bench/ step exercise (3 sessions/week, 50 min/session, 60-90% HRmax). Subjects were randomly assigned to groups that trained with (WT, n=16) and without (NWT, n=16) 0.68 kg/ankle and 1.36 kg/hand weights while 12 subjects served as non-training controls (NTC). MEASURES: Pre- and postintervention muscular strength and endurance for knee and elbow flexion and extension, and for shoulder abduction and adduction were examined by isokinetic dynamometry. Body composition was assessed with hydrostatic weighing and psychological affect by questionnaire. RESULTS: Thirty-two subjects completed the study. ANOVA revealed that pre- to postintervention changes for body fat (2.6%), fat-free weight (+0.7 kg), fat weight (-1.9 kg), and knee flexion peak torque were significantly different in the bench/step exercise trained (WT+NWT) compared to the NTC study group. Specific comparisons of muscle strength and endurance change scores of WT+NWT relative to NTC, and of WT relative to NWT revealed no other significant differences between groups. Positive and negative affective states were similar among study groups before and after the intervention. CONCLUSIONS: Participation in bench/step group exercise improved body composition but was of limited or no value as a modality to change muscular fitness and psychological affect in healthy adult females. The use of ankle and hand weights failed to enhance training adaptations.

Oculomotor abnormalities in diseases of the basal ganglia.

Disorders of eye movements have been described in diseases of the basal ganglia for over a century. Recent neurophysiological and clinical work has greatly clarified the oculomotor role of these structures: their major involvement appears to be in the generation of "voluntary" saccades, and in the suppression of "reflex" saccades. The observed abnormalities of saccadic eye movements in Huntington's (HD) and Parkinson's (PD) diseases conform very well to predictions based on a combination of known disease pathology and recent neurophysiological work. This is not quite the case for other types of eye movement, such as smooth pursuit, largely because the exact role of the basal ganglia in their generation has not been defined neurophysiologically. Several diseases other than HD and PD both involve the basal ganglia and have effects on eye movements; such diseases include progressive supranuclear palsy and Wilson's disease. Unfortunately, the pathological processes in these conditions are too widespread to allow comment on how well oculomotor abnormalities fit in with predictions.

Unilateral reversal of smooth pursuit and optokinetic nystagmus.

A patient is presented who has had three six-month episodes of oscillopsia over the last eight years. During the episodes, abnormal smooth pursuit and optokinetic nystagmus were noted, but there were no other abnormalities. Oculography during the most recent episode demonstrated that when tracking from right to left, the patient's smooth pursuit was reversed. There are several features of the case which suggest that the aetiology may be a form of congenital nystagmus. A possible alternative mechanism whereby such an abnormality might arise as an acquired lesion is discussed.

A simple guide to nystagmus.

Nystagmus is a potentially mind-boggling subject, as over forty different types are recognized. However, by classifying the various types into a small number of categories, the clinician can make sense of them, and so make use of a very important clinical sign.