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Professor Julie Snowden

Professor Julie Snowden is a Consultant Neuropsychologist and Honorary Professor of Neuropsychology. Professor Snowden was instrumental in establishing the Cerebral Function Unit as a multi-disciplinary diagnostic clinic in the early 1980s. Her special interest is in young-onset degenerative brain disorders and their associated cognitive and behavioural changes. She has played an important role in the characterisation of focal degenerative disorders including frontotemporal dementia and progressive aphasic syndromes. Her experimental studies of semantic dementia have increased understanding of the interrelationship between language and memory. She helped to establish a multidisciplinary research clinic into Huntington’s disease and remains involved in the Neuropsychological Working Group for the European Huntington’s disease network.  

She has published more than 140 scientific papers and book chapters and is first author of a clinical monograph on frontotemporal lobe degeneration.

Professor Snowden is a practitioner member of the British Psychological Society Division of Neuropsychology. She is a member of a number of International research organisations including the World Federation of Neurology research group into Aphasia and Cognitive Disorders and the Memory Disorders Research Society.

Selected publications

Vardy E, Holt R, Gerhard A, Richardson A, Snowden JS, Neary D.   History of a suspected delirium is more common in Dementia with Lewy bodies than Alzheimer’s disease: a retrospective study.   Int J Geriatric Psychiatry 2014; 29(2): 178-81.

Lant S, Robinson A, Thompson J, Rollinson S, Pickering-Brown S, Snowden J, Yvonne Davidson, Gerhard A.  Patterns of Microglial Cell Activation in Frontotemporal Lobar Degeneration. Neuropathol Appl Neurobiol 2014 in press

Allen N, Robinson AC, Snowden J, Davidson YS, Mann DM.  Patterns of cerebral amyloid angiopathy define histopathological phenotypes in Alzheimer’s disease.  Neuropathol Appl Neurobiol 2013 in press

Harris JM, Gall C, Thompson JC, Richardson AM, Neary D, DuPlessis F, Pal P, Mann DM, Snowden JS, Jones M.  Classification and pathology of primary progressive aphasia.  Neurology 2013; 81(21): 1832-9.

Kobylecki C, Thompson JC, Jones M, Mills SJ, Shaunak S, Ironside JW, Snowden JS, Richardson AM.  Sporadic Creutzfeldt-Jakob disease presenting as progressive nonfluent aphasia with speech apraxia.  Alzheimer Dis Assoc Disord 2013: 27(4): 384-6.  (epubJun 22, 2012)

Mann DMA, Rollinson S, Robinson A, Bennion Callister J, Thompson JC, Snowden JS, Gendron T, Petrucelli L, Masuda-Suzukake M, Hasegawa M, Davidson Y, Pickering-Brown S.  Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. Acta Neuropathol Communications  2013; 1(1): 68.

Davidson YS, Robinson AC, Snowden JS, Mann DM.  Pathological assessment for the presence of  hexanucleotide repeat expansions in C9ORF72 in Alzheimer’s disease.  Acta Neuropathol Commun  2013; 1(1): 50.

Harris J, Gall C, Thompson JC, Richardson AM, Neary D, Mann D, Snowden JS, Jones M.  Sensitivity and specificity of FTDC criteria for behavioral variant frontotemporal dementia. Neurology 2013; 80: 1881-7. (epub 19/04/2013)

Neary D, Snowden JS.  Frontotemporal dementia, motor neuron  disease and clinicopathological criteria. Commentary.  J Neurol Neurosurg Psychiatry 2013; 84: 713-4 (epub 12/03/2013)  DOI 10.1136/jnnp-2012-304549

Snowden JS, Harris J, Richardson A, Rollinson S, Thompson JC, Neary D, Mann DMA, Pickering-Brown S.  Frontotemporal dementia with amyotrophic lateral sclerosis: a clinical comparison of patients with and without repeat expansions in C9ORF72. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2013; 14(3): 172-6.  (epub 21/02/2013) DOI 10.3109/21678421.2013.765485

Ghosh A, Dutt A, Bhargava P, Snowden J.  Environmental dependency behaviours in frontotemporal dementia: have we been underrating them?  J Neurol 2013; 260: 861-8.  (epub 31/10/2012) DOI 10.1007/s00415-012-6722-0

Davidson YS, Robinson AC, Hu Q, Mishra M, Baborie A, Jaros E, Perry RH, Cairns NJ, Richardson A, Gerhard A, Neary D, Snowden JS, Bigio EH, Mann DM.  Nuclear Carrier and RNA binding Proteins in Frontotemporal Lobar Degeneration associated with Fused in Sarcoma (FUS) pathological changes. Neuropathol Appl Neurobiol 2013; 39: 157-65.

Nalls MA, Duran, RD, Lopez, G, Kurzawa-Akanbi, M, McKeith IG, Chinnery PF, Morris CM, Theuns J,  Crosiers D, Cras P, Engelborghs S, Paul De Deyn P, Van Broeckhoven C, Mann DMA, Snowden J, Pickering-Brown S, Halliwel N, Davidson Y, Gibbon L, Harris J, Sheerin U-M, Bras J, Hardy J, Clark L, Marder K, Honig LS, Berg D, Maetzler W, Brockmann K, Gasser T, Novellino F, Quattrone A, Annesi G, De Marco EV, Rogaeva E, Masellis M, Black SE, Bilbao JM, Foroud T, Ghetti B, Nichols WC, Pankratz N, Halliday G, Lesage S, Klebe S, Durr A, Duyckaerts C, Brice A, Giasson BI, Trojanowski JQ, Hurtig HI, Tayebi N, Landazabal C, Knight MA, Keller M, Singleton AB, Wolfsberg TG, Sidransky E. A Multicenter Study of Glucocerebrosidase Mutations in Dementia with Lewy Bodies. . JAMA Neurol 2013; 70: 727-35 (epub 15/04/2013)

Snowden JS, Rollinson S, Lafon C, Harris J, Thompson J, Richardson AM, Jones M, Gerhard A, Neary D, Mann DM, Pickering-Brown S.  Psychosis, C9ORF72 and dementia with Lewy bodies.  J Neurol Neurosurg Psychiatry 2012; 83(10):1031-2. (epubJul 27)

Lee, J-M; Ramos, E M; Lee, J-H; Gillis, T; Mysore, J S; Hayden, M R; et al.  CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion. Neurology. (2012) 78:10 690-5. (JS collaborator only)

Snowden JS, Kindell J, Thompson JC, Richardson AMT, Neary D.  Progressive aphasia presenting with deep dyslexia and dysgraphia.  Cortex 2012; 48(9): 1234-9 (Mar 7)

Majounie E, Renton AE, Mok K, Dopper EGP, Waite A, Rollinson S, Chio A et al.  Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross sectional study. Lancet Neurology 2012 in press (International collaborative study. JS part of the Chromosome 9-ALS/FTD Consortium).

Rollinson S, Halliwell N, Young K, Bennion Callister J, Toulson G, Gibbons L, Davidson Y, Robinson A, Gerhard A, Richardson A, Neary D, Snowden J, Mann D, Pickering-Brown SM.  Analysis of the hexanucleotide repeat in C9ORF72 in Alzheimer's disease. Neurobiology of Aging 2012 ; 33(8): 1846 e5-6.

Luzzi S, Cafazzo V, Silvestrini M, Provinciali L, Pickering-Brown S, Mann D, Snowden JS.  Semantic dementia associated with corticobasal syndrome: a further variant of frontotemporal lobe degeneration?  Journal of Neurology 2012 (Jan 10) 259 (7), 1478-80.

Vardy ER, Brown K, Stopford CL, Thompson JC, Richardson AM, Neary D, Kasheker N, Morgan K, Mann DM, Snowden JS.  Cognitive phenotypes in Alzheimer’s disease and genetic variants in ACE and IDE.  Neurobiology of Aging 2012 ; 33: 1486 e1-2

Snowden JS, Rollinson S, Thompson JC, Harris J, Stopford CL, Richardson A, Jones M, Gerhard A, Davidson Y, Robinson A, Gibbons L, Hu Q, DuPlessis D, Neary D, Mann DMA, Pickering-Brown S.  Distinct clinical and pathological characteristics in patients with frontotemporal dementia and C9ORF72 mutations. Brain 2012; 135: 693-708.

Rollinson S, Bennion J, Toulson G, Halliwell N, Usher S, Snowden J, Richardson A, Neary D, Mann D, Pickering-Brown SM.  Analysis of optineurin in frontotemporal lobar degeneration.  Neurobiology of Aging 2012; 33: 425.e1- 425.e2. (Feb)

Thompson JC, Pridham J, Sollom AC, Howard E, Snowden JS, Craufurd D.  Longitudinal Evaluation of Neuropsychiatric Symptoms in Huntington’s Disease.  The Journal of Neuropsychiatry and Clinical Neurosciences 2012; 24: 53-60.

Snowden JS, Thompson JC, Neary D. Famous people knowledge and the right and left temporal lobes.  Behavioural Neurology 2012; 25: 35-44.

Stopford CL, Thompson JC, Neary D, Richardson AM, Snowden JS.  Working memory, attention and executive function in Alzheimer’s disease and frontotemporal dementia.  Cortex 2012; 48: 429-46.

Renton AE, Majounie E, Waite A, Simon-Sanchez J, Rollinson S, Gibbs JR, Schymick JC, Laaksovirta H, van Swieten JC, Myllykangas L, Kalimo H, Paetau A, Abramzon Y, Remes AM, Kaganovich A, Scholz SW, Duckworth J, Ding J, Harmer DW, Hernandez DG, Johnson JO, Mok K, Ryten M, Trabzuni D, Guerreiro RJ, Orrell RW, Neal J, Murray A, Pearson J, Jansen IE, Sondervan D, Seelaar H, Blake D, Young K, Halliwell N, Callister JB, Toulson G, Richardson A, Gerhard A, Snowden J, Mann D, Neary D, et al. A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD.  Neuron 2011; 72: 257-68.

Davidson YS, Raby S, Foulds PG, Robinson A, Thompson JC, Sikkink S, Yusuf I, Amin H, Du Plessis D, Troakes C, Al-Sarraj S, Esiri MM, Prasher VP, Allsop D, Neary D, Pickering-Brown SM, Snowden JS, Mann DMA.  TDP-43 pathological changes in early onset familial and sporadic Alzheimer's disease, late onset Alzheimer's disease and Down's Syndrome: association with age, hippocampal sclerosis and clinical phenotype" Acta Neuropathol 2011; 122: 703-13.

Snowden JS, Thompson JC, Stopford CL, Richardson AMT, Gerhard A, Neary D, Mann DMA. Clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships. Brain  2011; 134: 2478-92.

Josephs KA, Hodges JR, Snowden JS, Mackenzie IR, Neumann M, Mann DM, Dickson DW.  Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 2011; 122: 137-53.

Snowden JS, Hu Q, Rollinson S, Halliwell N, Robinson A, Davidson YS, Momeni P, Baborie A, Griffiths T, Jaros E, Perry RH, Richardson A, Pickering-Brown SM, Neary D, Mann D. The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene. Acta Neuropathol 2011; 122: 99-110.

Dakson A, Yakota O, Esiri M, Bigio EH, Horan M, Pendleton N, Richardson A, Neary D, Snowden JS, Robinson A, Davidson YS, Mann DM.  Granular expression of prolyl-peptidyl PIN1 is a constant and specific feature of Alzheimer’s disease pathology and is independent of tau, Abeta and TDP-43 pathology.  Acta Neuropathol 2011; 121: 635-49.

Rollinson S, Mead S, Snowden J, Richardson A, Rohrer J, Halliwell N, Usher S, Neary D, Mann D, Hardy J., Pickering-Brown S. FTLD genomewide association study replication confirms a risk locus shared with ALS. Neurobiology of Aging 2011; 32: 758: e1-7.

Baborie A, Griffiths TD, Jaros E, McKeith IG, Burn DJ, Richardson A, Ferrari R, Moreno J, Momeni P, Deplessis D, Pal P, Rollinson S, Pickering-Brown S, Thompson JC, Neary D, Snowden JS, Perry R, Mann DM.  Pathological correlates of frontotemporal lobar degeneration in the elderly.  Acta Neuropathol 2011; 121: 365-71.

Nishioka K, Ross OA, Vilarino-Guell C, Cobb SA, Kachergus JM, Mann DM, Snowden J, Richardson AM, Neary D, Robinson CA, Rajput A, et al .  Glucocerebrosidase mutations in diffuse Lewy body disease. Parkinsonism and Related Disorders 2011 ; 17: 55-7.

Chen-Plotkin AS, Martinez-Lage M, Sleiman PMA, Hu W, Greene R, McCarty Wood E et al.  Genetic and clinical features of progranulin-associated FTLD.  Archives of Neurology 2011; 68: 488-97.

Julien CL, Neary D, Snowden JS.  Understanding quantity in semantic dementia.  Cognitive Neuropsychology 2010; 27: 3-29.

Julien CL, Neary D, Snowden JS.  Personal experience and arithmetic meaning in semantic dementia.  Neuropsychologia 2010; 48: 278-87. 

Thompson JC, Poliakoff E, Sollom AC, Howard E, Craufurd D, Snowden JS.  Automaticity and attention in Huntington’s disease: when two hands are not better than one.  Neuropsychologia 2010; 48: 171-8.

Rollinson S, Rizzu P, Sikkink S, Baker M, Halliwell N, Snowden J, et al.  Ubiquitin Associated Protein 1 is a risk factor for frontotemporal lobar degeneration.  Neurobiol Aging 2009; 30: 656-65.

Davidson Y, Amin H, Kelley T, et al. (1 of 18 authors). TDP-43 in ubiquitinated inclusions in the inferior olives in frontotemporal lobar degeneration and in other neurodegenerative diseases: a degenerative process distinct from normal ageing.  Acta Neuropathol 2009; 118: 359-69.

Colombo R, Tavian D, Baker MC, Richardson AMT, Snowden JS, Neary D, Mann DMA, Pickering-Brown.  Recent origin and spread of a common Welsh MAPT splice mutation causing frontotemporal lobar degeneration.  Neurogenetics  2009; 10: 313-8.

Luzzi S, Viticchi G, Piccirilli M, Fabi K, Pesallaccia M, Bartolini M, Provinciali L, Snowden J.  Foreign accent syndrome as the initial sign of primary progressive aphasia.  J Neurol Neurosurg Psychiatry 2008; 79: 79-81

Stopford CL, Snowden JS, Thompson JC, Neary D.  Variability in cognitive presentation of Alzheimer’s disease.  Cortex 2008; 44: 185-95.

Gibbons ZC, Richardson A, Neary D, Snowden JS.  Behaviour in amyotrophic lateral sclerosis.  Amyotroph Lat Sclerosis 2008; 9: 67-74.

Bowen D, Procter A, Mann D, Snowden J, Esiri M, Neary D, Francis P.  Imbalance of a serotonergic system in frontotemporal dementia: implication for pharmacotherapy.  Psychopharmacology 2008; 196: 603-10. 

Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AMT, Neary D, Snowden JS, Mann DMA.  Frequency and clinical characteristics of progranulin mutation carriers in the Manchester Frontotemporal Lobar Degeneration cohort: Comparison to patients with MAPT and no known mutations.  Brain 2008; 131: 721-31.

Moreaud O, Belliard S, Snowden J, et al.  Démence sémantique: réflexions d’un groupe de travail pour des critères de diagnostic en français et la constitution d’une cohorte de patients. Revue Neurologique 2008; 164: 343-353.

Snowden JS, Austin NA, Sembi S, Thompson JC, Craufurd D, Neary D.  Emotion recognition in Huntington’s disease and frontotemporal dementia.  Neuropsychologia 2008; 46: 2638-49.

Foulds P, McCauley E, Gibbons L, Davidson Y, Pickering-Brown SM, Neary D, Snowden JS, Allsop D, Mann DMA.  TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer’s disease and frontotemporal lobar degeneration.  Acta Neuropathol 2008; 116: 141-6.

Julien C, Thompson JC, Neary D, Snowden JS.  Arithmetic knowledge in semantic dementia: is it invariably preserved?  Neuropsychologia 2008; 46: 2732-44

Gibbons ZC, Snowden JS, Thompson JC, Happe F, Richardson A, Neary D.  Inferring thought and action in motor neurone disease. Neuropsychologia 2007; 45: 1196-207.

Julien C,  Thompson JC, Snowden JS, Craufurd D.  Psychiatric symptoms in preclinical Huntington’s disease.  J Neurol Neurosurg Psychiatry 2007; 78: 939-43.

McNeill R, Sare GM, Manoharan M, Testa HJ, Mann DMA, Neary D, Snowden JS, Varma A.  Accuracy of SPECT in differentiating frontotemporal dementia from Alzheimer’s disease  J Neurol Neurosurg Psychiatry 2007;78:350-5.

Davidson Y, Kelley T, Mackenzie IRA, Pickering-Brown S, Du Plessis D, Neary D, Snowden JS, Mann DMA.  Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43.  Acta Neuropathol 2007; 113:521-33.

Luzzi S, Snowden JS, Neary D, Coccia M, Provinciali L, Lambon Ralph MA.  Distinct patterns of olfactory impairment in Alzheimer’s disease, semantic dementia, frontotemporal dementia and corticobasal degeneration.  Neuropsychologia 2007;45:1823-31.

Snowden JS, Stopford C, Julien C, Thompson JC, Davidson Y, Gibbons L, et al. Cognitive phenotypes in Alzheimer’s disease and genetic risk.  Cortex 2007; 43: 835-45.

Stopford CL, Snowden JS, Thompson JC, Neary D.  Distinct memory profiles in Alzheimer’s disease.  Cortex 2007; 43: 846-57.

Davidson Y, Gibbons L, Pritchard A, Hardicre J, Wren J, Stopford C, … Snowden JS, Mann DM.  Apolipoprotein E epsilon4 allele frequency and age of onset of Alzheimer’s disease.  Dement Geriatr Cogn Disord 2007; 23: 60-6.

Rollinson S, Snowden JS, Neary D, Morrison KE, Mann DMA, Pickering-Brown SM.  TDP-43 gene analysis in frontotemporal lobar degeneration.  Neuroscience letters 2007: 419: 1-4.

Snowden JS, Neary D, Mann DMA.  Frontotemporal lobar degeneration: clinical and pathological relationships.  Acta Neuropathol 2007; 114: 31-8.

Rademakers R, Baker M, Gass J, et al  (1 of 51 authors)  Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C-T (Arg493X) mutation: an international initiative.  Lancet Neurol 2007; 6: 857-68.

Snowden JS, Pickering-Brown SM, Du Plessis D, Mackenzie IRA, Varma A, Mann DMA, Neary D.  Progressive anomia revisited: focal degeneration associated with progranulin gene mutation.  Neurocase 2007; 13: 366-77.