David R Williams

Summary

Country: Australia

Publications

  1. ncbi Psychogenic palatal tremor
    David R Williams
    Neurology Registrar, St Vincent s Hospital, Melbourne, Australia
    Mov Disord 19:333-5. 2004
  2. ncbi Genetic variation at the tau locus and clinical syndromes associated with progressive supranuclear palsy
    David R Williams
    The Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, Queen Square, London, United Kingdom
    Mov Disord 22:895-7. 2007
  3. ncbi Tauopathies: classification and clinical update on neurodegenerative diseases associated with microtubule-associated protein tau
    D R Williams
    Reta Lila Weston Institute of Neurological Studies, University College London, London, UK
    Intern Med J 36:652-60. 2006
  4. ncbi A new dominant distal myopathy affecting posterior leg and anterior upper limb muscles
    D R Williams
    St Vincent s Hospital, Melbourne, Australia
    Neurology 64:1245-54. 2005
  5. ncbi Gaze-evoked brainstem myoclonus
    David R Williams
    Neurology Registrar, St Vincent s Hospital, Melbourne, Australia
    Mov Disord 19:346-9. 2004
  6. doi Concomitant progressive supranuclear palsy and multiple system atrophy: more than a simple twist of fate?
    Laura Silveira-Moriyama
    Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK
    Neurosci Lett 467:208-11. 2009
  7. ncbi Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome
    David R Williams
    Queen Square Brain Bank for Neurological Disorders, London, UK
    Brain 130:1566-76. 2007
  8. doi The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy
    Luke A Massey
    Sara Koe PSP Research Centre, Rita Lila Weston Institute for Neurology Studies and Queen Square Brain Bank, Department of Molecular Neurosciences, UCL Institute of Neurology, London, UK
    Neurology 80:1856-61. 2013
  9. doi Lewy- and Alzheimer-type pathologies in Parkinson's disease dementia: which is more important?
    Yaroslau Compta
    Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Brain 134:1493-505. 2011
  10. doi Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy
    Luke A Massey
    Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 27:1754-62. 2012

Collaborators

Detail Information

Publications36

  1. ncbi Psychogenic palatal tremor
    David R Williams
    Neurology Registrar, St Vincent s Hospital, Melbourne, Australia
    Mov Disord 19:333-5. 2004
    ..The heterogenous nature of essential palatal tremor and its relationship with PPT, voluntary palatal movements, and tics is discussed...
  2. ncbi Genetic variation at the tau locus and clinical syndromes associated with progressive supranuclear palsy
    David R Williams
    The Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, Queen Square, London, United Kingdom
    Mov Disord 22:895-7. 2007
    ..No mutations were found in 75 patients (21 PSP-P), and H1c was associated with both Richardson's syndrome and PSP-P compared with controls. Routine screening for MAPT mutations in atypical PSP is not recommended...
  3. ncbi Tauopathies: classification and clinical update on neurodegenerative diseases associated with microtubule-associated protein tau
    D R Williams
    Reta Lila Weston Institute of Neurological Studies, University College London, London, UK
    Intern Med J 36:652-60. 2006
    ..This review examines the important clinical features of the most prevalent tauopathies and the molecular and pathological features that underpin the classification system...
  4. ncbi A new dominant distal myopathy affecting posterior leg and anterior upper limb muscles
    D R Williams
    St Vincent s Hospital, Melbourne, Australia
    Neurology 64:1245-54. 2005
    ..To report a dominant, slowly progressive early onset distal myopathy with sparing of the tibialis anterior...
  5. ncbi Gaze-evoked brainstem myoclonus
    David R Williams
    Neurology Registrar, St Vincent s Hospital, Melbourne, Australia
    Mov Disord 19:346-9. 2004
    ..It had the unusual characteristic of being evoked by sustained up gaze and left gaze...
  6. doi Concomitant progressive supranuclear palsy and multiple system atrophy: more than a simple twist of fate?
    Laura Silveira-Moriyama
    Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK
    Neurosci Lett 467:208-11. 2009
    ..The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance...
  7. ncbi Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome
    David R Williams
    Queen Square Brain Bank for Neurological Disorders, London, UK
    Brain 130:1566-76. 2007
    ..The grading system we have developed provides an easy-to-use and sensitive tool for the morphological assessment of PSP-tau pathology and allows for consideration of the clinical diversity that is known to occur in PSP...
  8. doi The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy
    Luke A Massey
    Sara Koe PSP Research Centre, Rita Lila Weston Institute for Neurology Studies and Queen Square Brain Bank, Department of Molecular Neurosciences, UCL Institute of Neurology, London, UK
    Neurology 80:1856-61. 2013
    ..We aimed to develop in histologically proven disease a simple measure of the midbrain and pons on sagittal MRI to identify PSP...
  9. doi Lewy- and Alzheimer-type pathologies in Parkinson's disease dementia: which is more important?
    Yaroslau Compta
    Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Brain 134:1493-505. 2011
    ..Cortical amyloid-β and age at disease onset seem to determine the rate to dementia...
  10. doi Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy
    Luke A Massey
    Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 27:1754-62. 2012
    ..However, the low sensitivity of these and macroscopic findings at autopsy suggest a need for imaging techniques sensitive to microstructural abnormalities without regional atrophy...
  11. doi Does corticobasal degeneration exist? A clinicopathological re-evaluation
    Helen Ling
    Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, 1 Wakefield Street, London WC1N 1PJ, UK
    Brain 133:2045-57. 2010
    ..Despite these diagnostic difficulties we conclude that corticobasal degeneration is a discrete clinicopathological entity but with a broader clinical spectrum than was originally proposed...
  12. doi Testing an aetiological model of visual hallucinations in Parkinson's disease
    David A Gallagher
    Institute of Neurology, University College London, UK
    Brain 134:3299-309. 2011
    ..These clinical data are supported by the pathological study, in which higher overall cortical Lewy body counts, and in particular areas implicated in visuoperception and executive function, were associated with visual hallucinations...
  13. pmc Phenotype, genotype, and worldwide genetic penetrance of LRRK2-associated Parkinson's disease: a case-control study
    Daniel G Healy
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Lancet Neurol 7:583-90. 2008
    ..LRRK2-associated PD be distinguished from idiopathic PD; which mutations in LRRK2 are pathogenic; and what is the age-specific cumulative risk of PD for individuals who inherit or are at risk of inheriting a deleterious mutation in LRRK2?..
  14. doi The effect of drug treatment on neurogenesis in Parkinson's disease
    Sean S O'Sullivan
    Reta Lila Weston Institute of Neurological Studies, University College London, London, United Kingdom
    Mov Disord 26:45-50. 2011
    ..Our findings suggest a positive impact of chronic L-dopa use on the number of NSC in the SVZ of PD patients, which may have relevance for future studies on neuroprotection in neurodegenerative diseases...
  15. doi Hyposmia in progressive supranuclear palsy
    Laura Silveira-Moriyama
    Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, United Kingdom
    Mov Disord 25:570-7. 2010
    ..Further prospective studies including patients with early PSP and PSP-P with postmortem confirmation might help clarify if smell tests could be useful when the differential diagnosis lies between PD and PSP...
  16. doi MM2 subtype of sporadic Creutzfeldt-Jakob disease may underlie the clinical presentation of progressive supranuclear palsy
    Igor N Petrovic
    Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, 1 Wakefield St, London, WC1N 1PJ, UK
    J Neurol 260:1031-6. 2013
    ..This case and the literature support the notion that biochemical properties of the prion protein can influence the clinical presentation of sCJD...
  17. pmc Visual hallucinations in the differential diagnosis of parkinsonism
    Kelly Bertram
    Neurology Department, Alfred Hospital, Melbourne, Victoria, Australia
    J Neurol Neurosurg Psychiatry 83:448-52. 2012
    ..In this review, the published frequencies of VH in these different conditions are compared to put into context the notion of VH as a clinical clue to underlying Lewy body pathology...
  18. ncbi Kufor Rakeb disease: autosomal recessive, levodopa-responsive parkinsonism with pyramidal degeneration, supranuclear gaze palsy, and dementia
    David R Williams
    The Queen Square Brain Bank for Neurological Disorders Reta Lila Weston Institute for Neurological Studies, University College London, London, United Kingdom
    Mov Disord 20:1264-71. 2005
    ..Several new features were identified, including facial-faucial-finger mini-myoclonus, visual hallucinations, and oculogyric dystonic spasms...
  19. doi J. Clifford Richardson and 50 years of progressive supranuclear palsy
    David R Williams
    Faculty of Medicine, Monash University, Alfred Hospital Campus, Melbourne, Australia
    Neurology 70:566-73. 2008
    ..To trace the historical events leading to Richardson's clinical description of progressive supranuclear palsy (PSP) in the context of subsequent observations of its clinical heterogeneity and pathologic overlap with other tauopathies...
  20. ncbi Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism
    David R Williams
    The Queen Square Brain Bank for Neurological Disorders, University College London, UK
    Brain 128:1247-58. 2005
    ..The different tau isoform deposition in the basal pons suggests that this may ultimately prove to be a discrete nosological entity...
  21. ncbi Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy
    David R Williams
    Faculty of Medicine Neurosciences, Monash University Alfred Hospital Campus, Melbourne, Australia
    Mov Disord 22:2235-41. 2007
    ..This relatively uncommon presentation of PSP-tau pathology has less severe tau accumulation than in the more common, "classic" PSP clinical phenotype: Richardson's disease...
  22. doi Characterising the uncommon corticobasal syndrome presentation of sporadic Creutzfeldt-Jakob disease
    Will Lee
    Department of Neuroscience, The Alfred Hospital, Commercial Road, Melbourne, Victoria 3004, Australia
    Parkinsonism Relat Disord 19:81-5. 2013
    ..It may occur in other neurodegenerative disorders including sporadic Creutzfeldt-Jakob disease (sCJD). Current CBD diagnostic criteria outline features of CBS but fail to distinguish CBD from other causative pathologies...
  23. doi Cognitive testing in the diagnosis of parkinsonian disorders: a critical appraisal of the literature
    Will Lee
    Department of Neuroscience, The Alfred Hospital, Melbourne, Victoria, Australia
    Mov Disord 27:1243-54. 2012
    ..Even these features must be interpreted in conjunction with other clinical characteristics to be helpful diagnostically. © 2012 Movement Disorder Society...
  24. doi Development and assessment of sensitive immuno-PCR assays for the quantification of cerebrospinal fluid three- and four-repeat tau isoforms in tauopathies
    Connie Luk
    Reta Lila Weston Institute, UCL Institute of Neurology, London, UK
    J Neurochem 123:396-405. 2012
    ....
  25. doi Serotonergic involvement in levodopa-induced dyskinesias in Parkinson's disease
    Perdita A Cheshire
    Van Cleef Roet Centre for Nervous Diseases, 4th Floor, Monash University, Alfred Hospital, 89 Commercial Road, Melbourne, Victoria 3004, Australia
    J Clin Neurosci 19:343-8. 2012
    ....
  26. doi The use of a color coded probability scale to interpret smell tests in suspected parkinsonism
    Laura Silveira-Moriyama
    Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, United Kingdom
    Mov Disord 24:1144-53. 2009
    ..We suggest smell tests could be used routinely in challenging cases where there is diagnostic uncertainty and help inform decision making relating to the need for neuro imaging...
  27. doi What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)?
    David R Williams
    Van Cleef Roet Centre for Nervous Diseases, Monash University, Melbourne, Victoria, Australia
    Mov Disord 25:357-62. 2010
    ..PSP-P shares many clinical features with PD and DLB, MSA and VP, but visual hallucinations, drug induced dyskinesias and autonomic dysfunction are very uncommon and may be helpful exclusion criteria...
  28. ncbi Visual hallucinations in the diagnosis of idiopathic Parkinson's disease: a retrospective autopsy study
    David R Williams
    Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, University College London, London, UK
    Lancet Neurol 4:605-10. 2005
    ..The cause of VH is now thought to be nerve-cell loss and Lewy-body pathology in the ventral-temporal regions of the brain. However, the use of VH as a clinical sign of PD has not been investigated...
  29. doi Biological fluid biomarkers in neurodegenerative parkinsonism
    Michael Eller
    Department of Neurosciences, Alfred Hospital, Melbourne, Victoria, Australia
    Nat Rev Neurol 5:561-70. 2009
    ..Despite such difficulties, improved technology, in conjunction with advances in nosology and pathology, means that biomarkers are poised to enter routine clinical practice to aid the differentiation of parkinsonian disorders...
  30. doi α-Synuclein in Parkinson disease and other neurodegenerative disorders
    Michael Eller
    Department of Neurosciences, Alfred Hospital, Melbourne, Australia
    Clin Chem Lab Med 49:403-8. 2011
    ..Future studies using standardized techniques and larger patient numbers are awaited to realise the full potential of αS as a more definitive diagnostic biomarker...
  31. ncbi Nonmotor symptoms as presenting complaints in Parkinson's disease: a clinicopathological study
    Sean S O'Sullivan
    Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, United Kingdom
    Mov Disord 23:101-6. 2008
    ..Presenting with NMS does not affect the motor response to medication, but is associated with shorter disease duration (chi(2) P = 0.016)...
  32. doi Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges
    David R Williams
    Faculty of Medicine Neurosciences, Monash University, Melbourne, Australia
    Lancet Neurol 8:270-9. 2009
    ..In this way, the development of the clinical features can be informative in assigning less common nosological categories that give clues to the underlying pathology and an understanding of the expected clinical course...
  33. doi Psychogenic propriospinal myoclonus
    David R Williams
    Faculty of Medicine Neurosciences, Monash University, Alfred Hospital Campus, Melbourne, Australia
    Mov Disord 23:1312-3. 2008
    ..Spontaneous remissions and disappearance of the jerks, sustained for 2 years, following removal of superficial surgical screws support the diagnosis of a psychogenic movement disorder...
  34. doi Clinical, laboratory and electrophysiological features of Morvan's Fibrillary Chorea
    Will Lee
    Department of Neuroscience, Alfred Hospital, Commercial Road, Melbourne, Victoria 3004, Australia Van Cleef Roet Centre for Nervous Diseases, Monash University, Melbourne, Victoria, Australia
    J Clin Neurosci 20:1246-9. 2013
    ..Early initiation of immunotherapies and malignancy screening are important to prevent adverse outcomes in a condition that generally responds favourably to treatment. ..
  35. doi Diagnosis of dystonic syndromes--a new eight-question approach
    Kelly L Bertram
    Department of Neurology, Alfred Hospital, Commercial Road, Melbourne, VIC 3004, Australia
    Nat Rev Neurol 8:275-83. 2012
    ..The aim of this approach is to inform both specialists and general neurologists on the appropriate diagnostic test for each patient who presents with a possible dystonic syndrome...
  36. pmc Retinal crystals in type 2 idiopathic macular telangiectasia
    Ferenc B Sallo
    Department of Research and Development, Moorfields Eye Hospital, NHS Foundation Trust, London, United Kingdom
    Ophthalmology 118:2461-7. 2011
    ..To characterize the phenotype and investigate the associations of intraretinal crystalline deposits in a large cohort with type 2 idiopathic macular telangiectasia (MacTel)...