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.

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.

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.

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.

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.

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.

CSF opening pressure: reference interval and the effect of body mass index.

We prospectively recorded CSF opening pressure in 242 adults who had a lumbar puncture with concomitant measurement of weight and height. The 95% reference interval for lumbar CSF opening pressure was 10 to 25 cm CSF. Body mass index had a small but clinically insignificant influence on CSF opening pressure.

Spontaneous intracranial hypotension causing confusion and coma: a headache for the neurologist and the neurosurgeon.

Spontaneous intracranial hypotension presenting with confusion and coma has rarely been reported. A case is presented and the clinical features of spontaneous intracranial hypotension are 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.

Creutzfeldt-Jakob disease and the eye. I. Background and patient management.

This article attempts to summarise our current understanding of TSEs as they affect man. Specific aspects relevant to ophthalmological practice, in particular the management of patients in day-to-day clinical practice and with respect to corneal transplantation, have been discussed. In the companion article we discuss the specific ophthalmic and neuro-ophthalmic features of these diseases.

Myasthenia gravis presenting with stridor.

The case is described of a 72 year old woman who presented with a two year history of exertional stridor in whom the diagnosis of myasthenia gravis was delayed. Although an uncommon cause, myasthenia gravis should be included in the differential diagnosis of stridor.

Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases.

Two further cases of Creutzfeldt-Jakob disease (CJD) in association with cadaveric dura mater grafts are described. The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. This observation may reflect the route of inoculation of the agent.

Ocular and neurological Behçet's disease without orogenital ulceration?

A 28 year old West Indian patient is described who had a relapsing and remitting steroid-sensitive illness for 3 years. The clinical features included uveitis and widespread CNS involvement. The patient was treated as though he had neurosarcoidosis. Post mortem examination revealed histological changes compatible with a diagnosis of Behçet's disease, but at no time did he suffer from oral or genital ulceration or arthritis. The authors suggest a new term to encompass such an entity: the "Behçet's MINUS" syndrome (multifocal intermittent neurological and uveitic syndrome).

Saccadic eye movements in Parkinson's disease: I. Delayed saccades.

The saccadic eye movements of nine patients with Parkinson's disease were compared to those of nine age-matched controls in two paradigms generating volitional saccades. In both paradigms, subjects had to make delayed saccades to peripheral LED targets: a peripheral target appeared 700 msec before a buzzer sounded, the buzzer being the signal to make a saccade to the target. In the first paradigm ("centre-off"), the fixation target was extinguished simultaneously with buzzer onset. In the second ("centre-remain") it was not extinguished until 1000 msec later. The results showed that for outward saccades in both paradigms, there was no difference between Parkinsonian patients and controls, but saccadic latencies were significantly shorter in the "centre-remain" paradigm. The initial outward saccades were indistinguishable from the normal, reflex saccades of the same subjects. However, saccades returning to the centre (a type of remembered target saccade) were hypometric and showed multistepping. Both effects were more pronounced in patients with Parkinson's disease. The significance of these findings in terms of current hypotheses about the nature of the Parkinsonian saccadic deficit is discussed.

Saccadic eye movements in Parkinson's disease: II. Remembered saccades--towards a unified hypothesis?

Ten patients with mild to moderate Parkinson's disease were compared with ten age-matched normal controls in a series of saccadic paradigms in order to test various hypotheses relating to the origin of the Parkinsonian saccadic defect. The paradigms comprised a reflex saccade paradigm, a standard remembered saccade paradigm, a remembered saccade paradigm with delayed centre-offset, and a remembered saccade paradigm with a second target flash immediately prior to saccade execution. Finally, subjects executed both reflex and remembered saccades in a standard remembered paradigm (the "two-saccade" paradigm). As has been reported previously, Parkinsonian subjects demonstrated hypometria on all remembered saccade paradigms, particularly the "two-saccade" paradigm. There was, however, no significant difference between the first three remembered saccade paradigms. These studies serve to refute a simple attentional capture hypothesis, and a hypothesis that suggests that the abnormality of remembered saccades is due to concurrent reflex saccade suppression. On the basis of the results, further hypotheses are advanced in an attempt to explain all published work on Parkinsonian saccades.