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Six years ago, a survey of Australian trainees in neurology highlighted a number of differences in the training offered by the various positions. There has been a subsequent increase in trainee numbers. This survey aimed to re-examine the workload and exposure provided by individual positions and to compare training in Australia and New Zealand. 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. 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. 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.

Positive family history has been reported in a high proportion of frontotemporal dementia (FTD) patients, but a genetic cause is documented in a minority of cases. To date, very few studies have examined this apparent discord by systematically assessing the strength of family history in FTD. The present study aimed to characterise the heritability of FTD, compared to Alzheimer’s disease (AD) and controls, using the modified Goldman score. Patients with a diagnosis of FTD or AD were recruited from an FTD research clinic. Detailed clinical and neuropsychological assessments were performed to establish the diagnoses according to accepted diagnostic criteria. FTD patients were further classified into established FTD phenotypes, including behavioural variant (bvFTD) and FTD associated with motor neuron disease (FTD-MND). Comparison was made with age and sex-matched control subjects. Family history of neurodegenerative disease was obtained from patients, their carers, and other family members for collateral history. Strength of family history was graded prospectively using the modified Goldman score. In total, 308 participants (122 FTD, 98 AD, 88 control) were included in the study. Family history of neurodegenerative disease did not differ between groups (FTD 39.3%, AD 42.4%, controls 29.5%, p = 0.167), but the “strength” of family history did differ. Specifically, 17.2% of FTD patients reported a strong family history (modified Goldman score 1–3) compared to 2.3% of controls (p

Lymphoma can affect virtually any organ system including the peripheral nervous system. Neuropathy associated with lymphoma may be secondary to direct invasion of lymphomatous cells (neurolymphomatosis), immune mechanisms, or as an adverse effect of chemotherapy or radiotherapy treatment. Establishing an accurate diagnosis is vital in order to initiate appropriate therapy, particularly in the case of neurolymphomatosis. Often determining the underlying pathological process is challenging as imaging and neurophysiological findings may be nonspecific. A 76-year-old man with diffuse large B-cell lymphoma presented with progressive asymmetrical muscle wasting, sensory loss and mild quadriparesis. Neurophysiology was consistent with a distal demyelinating sensorimotor neuropathy. Only minor enhancement of the right C8 nerve root was identified on MRI. In contrast, a whole body 18 F-fluorodeoxyglucose (FDG) positron emission tomography(PET)-CT scan showed avid linear FDG uptake in the brachial plexus bilaterally as well as multiple other sites, consistent with neurolymphomatosis. There was also evidence of active lymphoma in other organs including the liver and pleura. On subsequent scans the FDG PET-CT scan was the most reliable means to determine treatment response. The neurophysiological features of lymphomatous infiltration of the peripheral nervous system were recently described. Features of demyelination were frequently observed and a third of pathologically proven neurolymphomatosis cases fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic criteria for definite chronic inflammatory demyelinating polyneuropathy. This case highlights the sensitivity of FDG PET-CT scan in establishing a diagnosis of neurolymphomatosis because MRI and cerebrospinal fluid analysis are often unhelpful or non-diagnostic. This case also emphasises that neurolymphomatosis should be considered in demyelinating neuropathies associated with lymphoma even when there are minimal or no MRI changes.

Motor neuron disease (MND) and frontotemporal dementia (FTD) share clinical, genetic, and pathological features. Executive and behavioural disturbances are now recognised in MND, but non-executive cognitive impairments such as language dysfunction – a characteristic clinical feature of FTD – have not been explored to our knowledge. The present study investigated language in MND using tools specifically designed for use in different FTD phenotypes, including semantic dementia (SD) and progressive non-fluent aphasia (PNFA). Specifically, semantic deficits were assessed and compared to SD, whereas ability to interpret grammar was compared to PNFA. The study was performed in two parts. In part A, the Sydney language battery was used to assess aspects of semantic knowledge such as object naming, and results were compared to SD. In part B, interpretation of grammar was assessed using the test of reception of grammar (TROG), and results were compared to PNFA. Voxel-based morphometry was used to correlate patterns of cortical atrophy with performance on language measures. In total, 84 participants were included in part A (17 MND, 19 FTD-MND, 22 SD, and 26 controls) and 71 participants in part B (18 MND, 16 FTD-MND, 28 PNFA, and 9 controls). Part A demonstrated semantic deficits in MND and FTD-MND. For example, MND and FTD-MND were impaired on object naming (MND 25 ± 2.9, FTD-MND 17.1 ± 6.6, SD 5.4 ± 3.9, and controls 27 ± 1.9; p

A 42 year old man developed peri-orbital headache and pain on eye movements followed by progressive left monocular blindness. Five days later he developed various visual hallucinations which were both unformed and formed. The patient retained insight and had no symptoms suggestive of psychiatric illness. The hallucinations settled after several days as his visual deterioration reached its nadir. The patient had recently received four weekly injections of grass and olive tree extracts for the treatment of allergic rhinitis as part of an immune desensitisation protocol. Right visual acuity was normal with reduced colour saturation, though acuity was perception of hand movements only on the left. Fundoscopy revealed bilateral optic disc swelling but the remainder of the neurological examination was normal. Magnetic imaging revealed marked oedema and enhancement of the left optic nerve, in association with punctate T2 and FLAIR hyperintensities throughout the subcortical white matter bilaterally. Investigations for systemic vasculitis, antiphospholipid antibody syndrome, sarcoidosis and neuromyelitis optica (aquaporin-4) were negative. Given the clinical and radiological features a diagnosis of acute disseminated encephalomyelitis (ADEM) was made. To our knowledge this is the first reported case of Charles Bonnet syndrome in ADEM. The potential relationship between the illness and recent immune desensitisation therapy is discussed with reference to the literature.