ORCID Profile
0000-0002-7249-210X
Current Organisations
Westmead Hospital
,
University of Sydney
,
Concord Repatriation General Hospital
Does something not look right? The information on this page has been harvested from data sources that may not be up to date. We continue to work with information providers to improve coverage and quality. To report an issue, use the Feedback Form.
Publisher: Wiley
Date: 12-02-2023
DOI: 10.1111/ENE.15693
Abstract: Tremor in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is underrecognized, and the pathophysiology remains incompletely understood. This study evaluated tremor in CIDP and tested the hypothesis, established in other demyelinating neuropathies, that tremor occurs due to mistimed peripheral inputs affecting central motor processing. Additionally, the tremor stability index (TSI) was calculated with the hypothesis that CIDP‐related tremor is more variable than other tremor disorders. Consecutive patients with typical CIDP were prospectively recruited from neuromuscular clinics. Alternative causes of neuropathy and tremor were excluded. Cross‐sectional clinical assessment and extensive tremor study recordings were undertaken. Pearson correlation coefficient was used to compare nerve conduction studies and tremor characteristics, and t ‐test was used for comparisons between groups. Twenty‐four patients with CIDP were included. Upper limb postural and action tremor was present in 66% and was mild according to the Essential Tremor Rating Assessment Scale. Tremor did not significantly impact disability. Surface electromyography (EMG) found high‐frequency spectral peaks in deltoid (13.73 ± 0.66 Hz), biceps brachii (11.82 ± 0.91 Hz), and extensor carpi radialis (11.87 ± 0.91 Hz) muscles, with lower peaks in abductor pollicis brevis EMG (6.07 ± 0.45 Hz) and index finger accelerometry (6.53 ± 0.42 Hz). Tremor was unchanged by weight loading but correlated with ulnar nerve F‐wave latency and median nerve sensory litude. TSI (2.3 ± 0.1) was significantly higher than essential tremor. Postural tremor is a common feature in CIDP. Tremor was unaffected by weight loading, typical of centrally generated tremors, although there was a correlation with peripheral nerve abnormalities. The high beat‐to‐beat variability on TSI and gradation of peak frequencies further suggest a complex pathophysiology. These findings may assist clinicians with the diagnosis of neuropathic tremor.
Publisher: Elsevier BV
Date: 08-2022
DOI: 10.1016/J.CLINPH.2022.04.020
Abstract: To determine the electrodiagnostic characteristics of facial onset sensory and motor neuronopathy (FOSMN). Electrophysiological data from 10 FOSMN patients in Newcastle-upon-Tyne and Sydney were reviewed. Relevant literature was reviewed. Findings on standard electrophysiological assessment were in broad agreement with those published: blink reflexes were abnormal in all but one patient sensory nerve action potentials were reduced but compound muscle action potentials preserved mixed acute and chronic neurogenic change was identified on needle electromyography in bulbar and cervico-thoracic muscles in approximately 50% of patients. Upper limb somatosensory evoked potential (SEP) central conduction times were increased (n = 4) and progressed on repeat testing (n = 3). Upper motor neuron dysfunction was revealed by several measures [ipsilateral motor evoked potentials (MEPs) (n = 1) reduced short interval intra-cortical inhibition on threshold-tracking transcranial magnetic stimulation (n = 2) absent beta-band intermuscular coherence (n = 3)]. Electrodiagnostic investigation of FOSMN should include blink reflex testing, SEPs and tests of upper motor neuron function. The combination of progressive lower motor neuron disease and upper motor neuron disease on neurophysiological investigation provides further support for the contention that FOSMN is a rare variant of motor neurone disease. These findings will aid the neurologist and neurophysiologist in making a confident diagnosis of FOSMN, thus expediting appropriate care.
Publisher: Elsevier BV
Date: 10-2019
DOI: 10.1016/J.MSARD.2019.07.013
Abstract: Tumefactive demyelinating lesions occur as part of the spectrum of multiple sclerosis (MS), but can be difficult to distinguish from other large cerebral lesions such as neoplasm or abscess. To estimate the cost associated with diagnostic investigation of patients with tumefactive demyelination (TD), including associated morbidity, and compare this to more typical relapsing-remitting MS. Retrospective review of medical records of patients seen between 2013 and 2018 in clinics at the Brain and Mind Centre, Sydney, Australia a center with tertiary referral expertise in MS. Thirty-one patients with TD and 31 patients with MS were compared. The cost of investigating TD was more than 7.5 times higher per patient than MS ($18,300 vs $2418, p < 0.01). More patients in the TD group were admitted to hospital (22/31 versus 10/31) and ICU admissions only occurred in the TD group (10/22 versus 0/10). Brain biopsy was performed only in the TD group (7 patients), which contributed to cost differences and also accounted for differences in adverse outcomes. The cost and morbidity related to investigating TD is higher than in typical MS. Improvements in the diagnosis of TD have the potential to improve health and economic outcomes.
Publisher: SAGE Publications
Date: 27-05-2018
Abstract: Immunotherapy is a treatment strategy that has demonstrated survival benefit for metastatic melanoma. Ipilimumab and nivolumab are ex les of immunotherapy, in which monoclonal antibodies antagonize cytotoxic T-lymphocyte-associated protein 4 and programmed death-ligand 1 receptors, respectively, resulting in upregulation of the host immune response to cancer cells. There is increasing recognition of immune-mediated adverse events associated with immune therapies in patients with cancer. We present a case report of a patient who developed Miller Fisher syndrome associated with these therapies for metastatic melanoma along with a discussion of its management.
Publisher: Wiley
Date: 21-06-2023
DOI: 10.1111/JNS.12574
Abstract: Imbalance is a prominent symptom of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Although upper limb tremor in CIDP is described, lower limb tremor has not been assessed. The aim of this study was to determine whether lower limb tremor was present in CIDP and assess potential relationships with imbalance. This was a cross‐sectional observational study of prospectively recruited consecutive patients with typical CIDP ( N = 25). Clinical phenotyping, lower limb nerve conduction and tremor studies, and posturography analyses were performed. The Berg Balance Scale (BBS) ided CIDP patients into those with “good” and “poor” balance. Lower limb tremor was evident in 32% of CIDP patients and associated with poor balance (BBS Tremor 35 [23–46], BBS No Tremor 52 [44–55], p = .035). Tremor frequency was 10.2–12.5 Hz with legs outstretched and on standing, apart from four patients with a lower frequency tremor (3.8–4.6 Hz) while standing. Posturography analysis revealed a high‐frequency spectral peak in the vertical axis in 44% of CIDP patients (16.0 ± 0.4 Hz). This was more likely in those with “good” balance (40% vs. 4%, p = .013). Lower limb tremor is present in one third of CIDP patients and is associated with poor balance. A high‐frequency peak on posturography is associated with better balance in CIDP. Lower limb tremor and posturography assessments could serve as important biomarkers of balance in a clinical setting.
Publisher: Wiley
Date: 24-08-2022
DOI: 10.1002/MUS.27398
Abstract: Imbalance is a common feature of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Intravenous immunoglobulin (IVIg) exerts clinical benefit in CIDP, including improving balance, although objective markers of efficacy are lacking. Posturography is an established objective marker of balance therefore, this study aimed to determine the utility of posturography as an objective marker of treatment efficacy in CIDP. Posturography was performed on 18 CIDP patients, established on IVIg infusions, and results were compared to age‐matched healthy controls. CIDP patients were assessed just prior to IVIg infusion and at the mid‐point of the cycle. Center of pressure (CoP) was measured and the total path traveled by CoP (Sway Path, SP) was calculated for five different conditions: feet placed in parallel 16 cm apart at the medial border with eyes open (16cmEO) and eyes closed (16cmEC) medial borders of the feet touching with eyes open (0cmEO) and eyes closed (0cmEC) and tandem stance. The sway path (SP) was significantly increased in CIDP patients (mean SP 1191 ± 104 mm) when compared to healthy controls (mean SP 724 ± 26 mm, P .001). The increase was most prominent during eyes closed and tandem stance conditions. Treatment with IVIg significantly reduced SP when assessing 0cmEC (1759 ± 324 mm vs. 1081 ± 134 mm, P = .019) and tandem stance (1775 ± 290 mm vs. 1152 ± 113 mm, P = .027). Posturography detected significant improvements in balance following IVIg in CIDP patients established on maintenance therapy. As such, posturography may be considered an objective marker of treatment response in clinical management and therapeutic trials.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 21-02-2020
Publisher: Wiley
Date: 11-2019
DOI: 10.1111/IMJ.14290
Abstract: Management of acute ischaemic stroke is time critical. Reducing time to treatment with thrombolysis is strongly associated with improved outcomes in properly selected patients. However, there are barriers to ensuring timely treatment in the hospital setting. To determine if simple, no-cost protocol changes could improve time to treatment for acute ischaemic stroke at a busy tertiary hospital. Prospectively collected routine clinical data were compared retrospectively before and after a protocol change designed to mirror the successful model from Helsinki University Central Hospital. Consecutive patients who activated a 'code stroke' (presentation consistent with acute stroke, eligible for acute stroke therapy) during working hours were included. Prior to the protocol change, 143 patients activated a code stroke, and 30 patients received thrombolysis. Following the protocol change, 134 patients activated a code stroke, and 14 patients received thrombolysis. The median time to administer thrombolysis was reduced from 76 min (interquartile range 54-91) to 33 min (27-44), P < 0.01. The median time to perform diagnostic computed tomography was unchanged between the two groups, 23 (14-54) min versus 22 (9-49) min, P = 0.12. However, this was reduced on subgroup analysis of patients whose arrival was pre-notified by the ambulance service, 16 (9-22) min versus 8 (4-14) min, P < 0.01. Time to treatment in acute stroke was dramatically improved with a simple intervention. This was achieved without a large stroke team or additional funding, making it highly accessible to other health services also seeking to improve their stroke service.
Publisher: SAGE Publications
Date: 06-08-2021
Abstract: Proximal median nerve injury is an uncommon consequence of anterior shoulder dislocation, especially occurring in isolation of other upper limb peripheral nerve injury. We report the case of an 82-year-old woman with a median nerve injury as detected by clinical and neurophysiological examination following a fall and anterior shoulder dislocation. Magnetic resonance neurography confirmed the diagnosis, but also detected asymptomatic brachial plexus and ulnar nerve involvement. Management was non-operative and there has been some improvement over several months. Our case expands the differential diagnosis for proximal median neuropathy and discusses the utility of neurography in cases of neural injury.
Publisher: Wiley
Date: 16-05-2017
DOI: 10.1111/ENE.13314
Abstract: To determine the clinical utility of the midbrain-to-pons (M/P) ratio as a clinical biomarker of progressive supranuclear palsy (PSP) in patients with non-fluent primary progressive aphasia syndromes. Patients with PSP, progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA) were recruited. Patients were diagnosed clinically, but pathological confirmation was available in a proportion of patients. Midbrain and pons areas were measured using Osirix Lite, a free DICOM viewer. The M/P ratio and Magnetic Resonance Parkinsonism Index were calculated and their diagnostic utility compared. A total of 72 participants were included (16 PSP, 18 PNFA, 16 LPA and 22 controls). Patients with PSP had motor features typical of the syndrome. Both the M/P ratio and Magnetic Resonance Parkinsonism Index differed significantly in PSP compared with controls. The M/P ratio was disproportionately reduced in PSP compared with PNFA and LPA (PSP, 0.182 ± 0.043 PNFA, 0.255 ± 0.034 LPA, 0.258 ± 0.033 controls, 0.292 ± 0.031 P < 0.001). An M/P ratio of ≤0.215 produced a positive predictive value of 77.8% for the diagnosis of PSP syndrome. Pathological examination revealed Alzheimer's disease in three cases (all LPA), pathological PSP in two cases (one clinical PSP and one PNFA) and corticobasal degeneration in one case (PNFA). The M/P ratio was ≤0.215 in both pathological cases of PSP. The M/P ratio was disproportionately reduced in PSP, suggesting its potential as a clinical marker of the PSP syndrome. Larger studies of pathologically confirmed cases are needed to establish the M/P ratio as a biomarker of PSP pathology.
Publisher: BMJ
Date: 06-2021
DOI: 10.1136/BMJNO-2021-000147
Abstract: Headache due to raised intracranial pressure is rarely caused by spinal lesions. We describe a patient with primary histiocytic sarcoma who presented with a new onset headache with features of raised intracranial pressure and subtle signs of cauda equina syndrome due to predominant lower spinal cord infiltration and minimal intracranial involvement. A previously well 54-year-old man presented with a 2-month history of new onset headache with features of raised intracranial pressure. Progression of lower limb weakness was delayed and mild with diagnostic delay resulting from the primary presentation with headache leading to an initial focus on cerebral pathology. Subsequent investigations revealed a previously unreported presentation of primary histiocytic sarcoma infiltrating the cauda equina causing raised intracranial pressure headache. This case highlights the importance of a broad search in the investigation of new onset raised intracranial pressure headache, including imaging of the lower spinal cord. Primary histiocytic sarcoma should be considered in the differential diagnosis of this rare syndrome.
Publisher: BMJ
Date: 30-03-2023
Abstract: Distal sensory polyneuropathy (DSP) is characterised by length-dependent, sensory-predominant symptoms and signs, including potentially disabling symmetric chronic pain, tingling and poor balance. Some patients also have or develop dysautonomia or motor involvement depending on whether large myelinated or small fibres are predominantly affected. Although highly prevalent, diagnosis and management can be challenging. While classic diabetes and toxic causes are well-recognised, there are increasingly erse associations, including with dysimmune, rheumatological and neurodegenerative conditions. Approximately half of cases are initially considered idiopathic despite thorough evaluation, but often, the causes emerge later as new symptoms develop or testing advances, for instance with genetic approaches. Improving and standardising DSP metrics, as already accomplished for motor neuropathies, would permit in-clinic longitudinal tracking of natural history and treatment responses. Standardising phenotyping could advance research and facilitate trials of potential therapies, which lag so far. This review updates on recent advances and summarises current evidence for specific treatments.
No related grants have been discovered for Matthew Silsby.