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  • Online publication date: December 2016

Chapter 17 - Early clinical features of the parkinsonian-related dementias

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1.Nath, U, Ben-Shlomo, Y, Thomson, R, Lees, A, Burn, D. Clinical features and natural history of progressive supranuclear palsy A clinical cohort study. Neurology. 2003;60(6):910–16.
2.Ratnavalli, E, Brayne, C, Dawson, K, Hodges, JR. The prevalence of frontotemporal dementia. Neurology. 2002;58(11):1615–21.
3.Savica, R, Grossardt, BR, Bower, JH, Ahlskog, JE, Rocca, WA. Incidence and pathology of synucleinopathies and tauopathies related to parkinsonism. JAMA Neurology. 2013;70(7):859–66.
4.Savica, R, Grossardt, BR, Bower, JH, Boeve, BF, Ahlskog, JE, Rocca, WA. Incidence of dementia with Lewy bodies and Parkinson disease dementia. JAMA Neurology. 2013;70(11):1396–402.
5.Tran, HT, Chung, CH-Y, Iba, M, Zhang, B, Trojanowski, JQ, Luk, KC, et al. α-Synuclein immunotherapy blocks uptake and templated propagation of misfolded α-synuclein and neurodegeneration. Cell Reports. 2014;7(6):2054–65.
6.Schneider, SA, Hardy, J, Bhatia, KP. Syndromes of neurodegeneration with brain iron accumulation (NBIA): an update on clinical presentations, histological and genetic underpinnings, and treatment considerations. Movement Disorders. 2012;27(1):4253.
7.Mak, CM, Lam, C-W. Diagnosis of Wilson’s disease: a comprehensive review. Critical Reviews in Clinical Laboratory Sciences. 2008;45(3):263–90.
8.Steele, JC. Parkinsonism– dementia complex of Guam. Movement Disorders. 2005;20(S12):S99–107.
9.Huang, Y, Halliday, G. Can we clinically diagnose dementia with Lewy bodies yet? Translational Neurodegeneration. 2013;2(1):4.
10.McKeith, I. Dementia with Lewy bodies and Parkinson’s disease with dementia: where two worlds collide. Practical Neurology. 2007;7(6):374–82.
11.McKeith, I, Dickson, D, Lowe, J, Emre, M, O’Brien, J, Feldman, H, et al. Diagnosis and management of dementia with Lewy bodies. Third report of the DLB consortium. Neurology. 2005;65(12):1863–72.
12.Aarsland, D, Andersen, K, Larsen, JP, Lolk, A. Prevalence and characteristics of dementia in Parkinson disease: an 8-year prospective study. Archives of Neurology. 2003;60(3):387–92.
13.Aarsland, D, Kvaløy, J, Andersen, K, Larsen, J, Tang, M, Lolk, A, et al. The effect of age of onset of PD on risk of dementia. Journal of Neurology. 2007;254(1):3845.
14.Emre, M, Aarsland, D, Brown, R, Burn, DJ, Duyckaerts, C, Mizuno, Y, et al. Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Movement Disorders. 2007;22(12):1689–707.
15.Hughes, T, Ross, H, Musa, S, Bhattacherjee, S, Nathan, R, Mindham, R, et al. A 10-year study of the incidence of and factors predicting dementia in Parkinson’s disease. Neurology. 2000;54(8):1596–603.
16.Brüggemann, N, Klein, C. Parkin type of early-onset Parkinson disease. Seattle (WA): University of Washington; 2013.
17.Elbaz, A, Bower, JH, Peterson, BJ, Maraganore, DM, McDonnell, SK, Ahlskog, JE, et al. Survival study of Parkinson disease in Olmsted county, Minnesota. Archives of Neurology. 2003;60(1):91–6.
18.Morgan, JC, Currie, LJ, Harrison, MB, Bennett, JP, Trugman, JM, Wooten, GF. Mortality in levodopa-treated Parkinson’s disease. Parkinson’s Disease. Article ID 426976, 8 pages, 2014. doi:10.1155/2014/426976.
19.Puschmann, A. Monogenic Parkinson’s disease and parkinsonism: clinical phenotypes and frequencies of known mutations. Parkinsonism & Related Disorders. 2013;19(4):407–15.
20.Wickremaratchi, M, Perera, D, O’loghlen, C, Sastry, D, Morgan, E, Jones, A, et al. Prevalence and age of onset of Parkinson’s disease in Cardiff: a community based cross sectional study and meta-analysis. Journal of Neurology, Neurosurgery & Psychiatry. 2009;80(7):805–7.
21.Geser, F, Wenning, GK, Poewe, W, McKeith, I. How to diagnose dementia with Lewy bodies: state of the art. Movement Disorders. 2005;20(S12):S11S20.
22.Han, D, Wang, Q, Gao, Z, Chen, T, Wang, Z. Clinical features of dementia with Lewy bodies in 35 Chinese patients. Translational Neurodegeneration. 2014;3(1):1.
23.Jellinger, KA, Wenning, GK, Seppi, K. Predictors of survival in dementia with Lewy bodies and Parkinson dementia. Neuro-degenerative Diseases. 2006;4(6):428–30.
24.Olichney, JM, Galasko, D, Salmon, D, Hofstetter, C, Hansen, L, Katzman, R, et al. Cognitive decline is faster in Lewy body variant than in Alzheimer’s disease. Neurology. 1998;51(2):351–7.
25.Tanner, CM, Ottman, R, Goldman, SM, Ellenberg, J, Chan, P, Mayeux, R, et al. Parkinson disease in twins: an etiologic study. Journal of the American Medical Association. 1999;281(4):341–6.
26.Klatka, LA, Louis, ED, Schiffer, RB. Psychiatric features in diffuse Lewy body disease A clinicopathologic study using Alzheimer’s disease and Parkinson’s disease comparison groups. Neurology. 1996;47(5):1148–52.
27.Williams, MM, Xiong, C, Morris, JC, Galvin, JE. Survival and mortality differences between dementia with Lewy bodies vs Alzheimer disease. Neurology. 2006;67(11):1935–41.
28.Apaydin, H, Ahlskog, JE, Parisi, JE, Boeve, BF, Dickson, DW. Parkinson disease neuropathology: later-developing dementia and loss of the levodopa response. Archives of Neurology. 2002;59(1):102–12.
29.Dickson, DW. Dementia with Lewy bodies: neuropathology. Journal of Geriatric Psychiatry and Neurology. 2002;15(4):210–16.
30.Tsuboi, Y, Dickson, DW. Dementia with Lewy bodies and Parkinson’s disease with dementia: are they different? Parkinsonism & Related Disorders. 2005;11:S47–51.
31.McKeith, I, Dickson, D, Lowe, J, Emre, M, O’Brien, J, Feldman, H. Dementia with Lewy bodies: diagnosis and management: third report of the DLB Consortium. Neurology. 2005;65(12):1863–72.
32.Boeve, B, Silber, M, Saper, C, Ferman, T, Dickson, D, Parisi, J, et al. Pathophysiology of REM sleep behaviour disorder and relevance to neurodegenerative disease. Brain. 2007;130(11):2770–88.
33.Ferman, T, Boeve, B, Smith, G, Lin, S-C, Silber, M, Pedraza, O, et al. Inclusion of RBD improves the diagnostic classification of dementia with Lewy bodies. Neurology. 2011;77(9):875–82.
34.Ferman, TJ, Smith, GE, Boeve, BF, Graff-Radford, NR, Lucas, JA, Knopman, DS, et al. Neuropsychological differentiation of dementia with Lewy bodies from normal aging and Alzheimer’s disease. The Clinical Neuropsychologist. 2006;20(4):623–36.
35.Simard, M, van Reekum, R, Cohen, T. A review of the cognitive and behavioral symptoms in dementia with Lewy bodies. The Journal of Neuropsychiatry and Clinical Neurosciences. 2000;12:425–50.
36.Petersen, RC. Mild cognitive impairment as a diagnostic entity. Journal of Internal Medicine. 2004;256(3):183–94.
37.Ferman, TJ, Smith, GE, Kantarci, K, Boeve, BF, Pankratz, VS, Dickson, DW, et al. Nonamnestic mild cognitive impairment progresses to dementia with Lewy bodies. Neurology. 2013;81:2032–8.
38.Donaghy, PC, McKeith, IG. The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis. Alzheimer’s Research & Therapy. 2014;6(4):46.
39.Ballard, C, Aarsland, D, Francis, P, Corbett, A. Neuropsychiatric symptoms in patients with dementias associated with cortical Lewy bodies: pathophysiology, clinical features, and pharmacological management. Drugs & Aging. 2013;30(8):603–11.
40.Stinton, C, McKeith, I, Taylor, J-P, Lafortune, L, Mioshi, E, Mak, E, et al. Pharmacological management of Lewy body dementia: a systematic review and meta-analysis. American Journal of Psychiatry. 2015;172(8):731–42.
41.McKeith, I, Del Ser, T, Spano, P, Emre, M, Wesnes, K, Anand, R, et al. Efficacy of rivastigmine in dementia with Lewy bodies: a randomised, double-blind, placebo-controlled international study. The Lancet. 2000;356(9247):2031–6.
42.Weintraub, D, Hurtig, HI. Presentation and management of psychosis in Parkinson’s disease and dementia with Lewy bodies. The American Journal of Psychiatry. 2007;164(10):1491–8.
43.Aarsland, D, Ballard, C, Larsen, JP, McKeith, I. A comparative study of psychiatric symptoms in dementia with Lewy bodies and Parkinson’s disease with and without dementia. International Journal of Geriatric Psychiatry. 2001;16(5):528–36.
44.Marantz, AG, Verghese, J. Capgras' syndrome in dementia with Lewy bodies. Journal of Geriatric Psychiatry and Neurology. 2002;15(4):239–41.
45.Chiba, Y, Fujishiro, H, Ota, K, Kasanuki, K, Arai, H, Hirayasu, Y, et al. Clinical profiles of dementia with Lewy bodies with and without Alzheimer’s disease‐like hypometabolism. International Journal of Geriatric Psychiatry. 2014.
46.Arnulf, I, Bonnet, A-M, Damier, P, Bejjani, B-P, Seilhean, D, Derenne, J-P, et al. Hallucinations, REM sleep, and Parkinson’s disease. A medical hypothesis. Neurology. 2000;55(2):281–8.
47.Boeve, B, Silber, M, Parisi, J, Dickson, D, Ferman, T, Petersen, R. Neuropathologic findings in patients with REM sleep behavior disorder and a neurodegenerative disorder. Neurology. 2001;56:A299.
48.Boeve, BF, Silber, MH, Ferman, TJ, Parisi, JE, Dickson, DW, Smith, GE, et al. REM sleep behavior disorder in Parkinson’s disease, dementia with Lewy bodies, and multiple system atrophy. In Mental and Behavioral Dysfunction in Movement Disorders, ed. Bédard, M-A, Agid, Y, Chouinard, S, Fahn, S, Korczyn, AD, Lespérance, P. New York: Springer; 2003. pp. 383–97.
49.Perry, E, Kerwin, J, Perry, R, Blessed, G, Fairbairn, A. Visual hallucinations and the cholinergic system in dementia. Journal of Neurology, Neurosurgery, and Psychiatry. 1990;53(1):88.
50.Auning, E, Rongve, A, Fladby, T, Booij, J, Hortobágyi, T, Siepel, FJ, et al. Early and presenting symptoms of dementia with lewy bodies. Dementia and Geriatric Cognitive Disorders. 2010;32(3):202–8.
51.Tiraboschi, P, Salmon, DP, Hansen, LA, Hofstetter, RC, Thal, LJ, Corey-Bloom, J. What best differentiates Lewy body from Alzheimer’s disease in early-stage dementia? Brain. 2006;129(3):729–35.
52.Yamamoto, R, Iseki, E, Marui, W, Togo, T, Katsuse, O, Kato, M, et al. Non‐uniformity in the regional pattern of Lewy pathology in brains of dementia with Lewy bodies. Neuropathology. 2005;25(3):188–94.
53.Del Ser, T, McKeith, I, Anand, R, Cicin‐Sain, A, Ferrara, R, Spiegel, R. Dementia with Lewy bodies: findings from an international multicentre study. International Journal of Geriatric Psychiatry. 2000;15(11):1034–45.
54.Tiraboschi, P, Guerra, UP. How to distinguish dementia with Lewy bodies from Alzheimer disease? Drugs. 2010;2:3.
55.Tschampa, H, Neumann, M, Zerr, I, Henkel, K, Schröter, A, Schulz-Schaeffer, W, et al. Patients with Alzheimer’s disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. Journal of Neurology, Neurosurgery & Psychiatry. 2001;71(1):33–9.
56.Molloy, S, McKeith, I, O’Brien, J, Burn, D. The role of levodopa in the management of dementia with Lewy bodies. Journal of Neurology, Neurosurgery & Psychiatry. 2005;76(9):1200–3.
57.Boeve, BF, Silber, MH, Ferman, TJ, Lucas, JA, Parisi, JE. Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy. Movement Disorders. 2001;16(4):622–30.
58.Boeve, BF, Silber, MH, Ferman, TJ. Current management of sleep disturbances in dementia. Current Neurology and Neuroscience Reports. 2002;2(2):169–77.
59.Deschenes, CL, McCurry, SM. Current treatments for sleep disturbances in individuals with dementia. Current Psychiatry Reports. 2009;11(1):20–6.
60.Iranzo, A, Tolosa, E, Gelpi, E, Molinuevo, JL, Valldeoriola, F, Serradell, M, et al. Neurodegenerative disease status and post-mortem pathology in idiopathic rapid-eye-movement sleep behaviour disorder: an observational cohort study. The Lancet Neurology. 2013;12(5):443–53.
61.Terzaghi, M, Sinforiani, E, Zucchella, C, Zambrelli, E, Pasotti, C, Rustioni, V, et al. Cognitive performance in REM sleep behaviour disorder: a possible early marker of neurodegenerative disease? Sleep Medicine. 2008;9(4):343–51.
62.Lessig, S, Ubhi, K, Galasko, D, Adame, A, Pham, E, Remidios, K, et al. Reduced hypocretin (orexin) levels in dementia with Lewy bodies. Neuroreport. 2010;21(11):756.
63.Akaogi, Y, Asahina, M, Yamanaka, Y, Koyama, Y, Hattori, T. Sudomotor, skin vasomotor, and cardiovascular reflexes in 3 clinical forms of Lewy body disease. Neurology. 2009;73(1):5965.
64.Ballard, C, Shaw, F, McKeith, I, Kenny, R. High prevalence of neurovascular instability in neurodegenerative dementias. Neurology. 1998;51(6):1760–2.
65.Hishikawa, N, Hashizume, Y, Yoshida, M, Sobue, G. Clinical and neuropathological correlates of Lewy body disease. Acta Neuropathologica. 2003;105(4):341–50.
66.Thaisetthawatkul, P, Boeve, B, Benarroch, E, Sandroni, P, Ferman, T, Petersen, R, et al. Autonomic dysfunction in dementia with Lewy bodies. Neurology. 2004;62(10):1804–9.
67.Pakiam, A, Bergeron, C, Lang, AE. Diffuse Lewy body disease presenting as multiple system atrophy. The Canadian Journal of Neurological Sciences. 1999;26(2):127–31.
68.Hawkes, C, Del Tredici, K, Braak, H. Parkinson’s disease: a dual‐hit hypothesis. Neuropathology and Applied Neurobiology. 2007;33(6):599614.
69.Jain, S, Goldstein, DS. Cardiovascular dysautonomia in Parkinson disease: from pathophysiology to pathogenesis. Neurobiology of Disease. 2012;46(3):572–80.
70.Oka, H, Morita, M, Onouchi, K, Yoshioka, M, Mochio, S, Inoue, K. Cardiovascular autonomic dysfunction in dementia with Lewy bodies and Parkinson’s disease. Journal of the Neurological Sciences. 2007;254(1):72–7.
71.Walker, Z, Stevens, T. Dementia with Lewy bodies: clinical characteristics and diagnostic criteria. Journal of Geriatric Psychiatry and Neurology. 2002;15(4):188–94.
72.Wilson, RS, Yu, L, Schneider, JA, Arnold, SE, Buchman, AS, Bennett, DA. Lewy bodies and olfactory dysfunction in old age. Chemical Senses. 2011:bjq139.
73.Maurage, CA, Ruchoux, MM, De Vos, R, Surguchov, A, Destee, A. Retinal involvement in dementia with Lewy bodies: a clue to hallucinations? Annals of Neurology. 2003;54(4):542–7.
74.Armstrong, RA. Visual signs and symptoms of dementia with Lewy bodies. Clinical and Experimental Optometry. 2012;95(6):621–30.
75.Postuma, RB, Gagnon, JF, Vendette, M, Desjardins, C, Montplaisir, JY. Olfaction and color vision identify impending neurodegeneration in rapid eye movement sleep behavior disorder. Annals of Neurology. 2011;69(5):811–18.
76.Brooks, DJ. Diagnosis and management of atypical Parkinsonian syndromes. Journal of Neurology, Neurosurgery & Psychiatry. 2002;72(suppl 1):i10–6.
77.Mosimann, UP, Müri, RM, Burn, DJ, Felblinger, J, O'Brien, JT, McKeith, IG. Saccadic eye movement changes in Parkinson’s disease dementia and dementia with Lewy bodies. Brain. 2005;128(6):1267–76.
78.Ferman, T, Smith, G, Boeve, B, Ivnik, R, Petersen, R, Knopman, D, et al. DLB fluctuations: specific features that reliably differentiate DLB from AD and normal aging. Neurology. 2004;62(2):181–7.
79.Walker, M, Ayre, G, Cummings, J, Wesnes, K, McKeith, I, O’Brien, J, et al. Quantifying fluctuation in dementia with Lewy bodies, Alzheimer’s disease, and vascular dementia. Neurology. 2000;54(8):1616–25.
80.Walker, M, Ayre, G, Perry, E, Wesnes, K, McKeith, I, Tovee, M, et al. Quantification and characterization of fluctuating cognition in dementia with Lewy bodies and Alzheimer’s disease. Dementia and Geriatric Cognitive Disorders. 1999;11(6):327–35.
81.Walker, M, Ballard, C, Ayre, G, Wesnes, K, Cummings, J, McKeith, I, et al. The clinician assessment of fluctuation and the One-Day Fluctuation Assessment Scale. Two methods to assess fluctuating confusion in dementia. The British Journal of Psychiatry. 2000;177(3):252–6.
82.Hughes, AJ, Daniel, SE, Kilford, L, Lees, AJ. Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. Journal of Neurology, Neurosurgery & Psychiatry. 1992;55(3):181–4.
83.Cools, R. Dopaminergic modulation of cognitive function-implications for L-DOPA treatment in Parkinson’s disease. Neuroscience & Biobehavioral Reviews. 2006;30(1):123.
84.Cools, R, Barker, RA, Sahakian, BJ, Robbins, TW. L-Dopa medication remediates cognitive inflexibility, but increases impulsivity in patients with Parkinson’s disease. Neuropsychologia. 2003;41(11):1431–41.
85.Caviness, JN, Driver‐Dunckley, E, Connor, DJ, Sabbagh, MN, Hentz, JG, Noble, B, et al. Defining mild cognitive impairment in Parkinson’s disease. Movement Disorders. 2007;22(9):1272–7.
86.Litvan, I, Aarsland, D, Adler, CH, Goldman, JG, Kulisevsky, J, Mollenhauer, B, et al. MDS task force on mild cognitive impairment in Parkinson’s disease: Critical review of PD‐MCI. Movement Disorders. 2011;26(10):1814–24.
87.Litvan, I, Goldman, JG, Tröster, AI, Schmand, BA, Weintraub, D, Petersen, RC, et al. Diagnostic criteria for mild cognitive impairment in Parkinson’s disease: Movement Disorder Society Task Force guidelines. Movement Disorders. 2012;27(3):349–56.
88.Marras, C, Armstrong, MJ, Meaney, CA, Fox, S, Rothberg, B, Reginold, W, et al. Measuring mild cognitive impairment in patients with Parkinson’s disease. Movement Disorders. 2013;28(5):626–33.
89.Broeders, M, De Bie, R, Velseboer, D, Speelman, J, Muslimovic, D, Schmand, B. Evolution of mild cognitive impairment in Parkinson disease. Neurology. 2013;81(4):346–52.
90.Pfeiffer, HCV, Løkkegaard, A, Zoetmulder, M, Friberg, L, Werdelin, L. Cognitive impairment in early‐stage non‐demented Parkinson’s disease patients. Acta Neurologica Scandinavica. 2014;129(5):307–18.
91.Reijnders, JS, Ehrt, U, Weber, WE, Aarsland, D, Leentjens, AF. A systematic review of prevalence studies of depression in Parkinson’s disease. Movement Disorders. 2008;23(2):183–9.
92.Kostić, VS, Pekmezović, T, Tomić, A, Ječmenica-Lukić, M, Stojković, T, Špica, V, et al. Suicide and suicidal ideation in Parkinson’s disease. Journal of the Neurological Sciences. 2010;289(1):40–3.
93.Strowd, RE, Cartwright, MS, Okun, MS, Haq, I, Siddiqui, MS. Pseudobulbar affect: prevalence and quality of life impact in movement disorders. Journal of Neurology. 2010;257(8):1382–7.
94.Santamaria, J, Tolosa, E, Valles, A. Parkinson’s disease with depression. A possible subgroup of idiopathic parkinsonism. Neurology. 1986;36(8):1130. la Riva, P, Smith, K, Xie, SX, Weintraub, D. Course of psychiatric symptoms and global cognition in early Parkinson disease. Neurology. 2014;83(12):1096–103.
96.Fénelon, G, Mahieux, F, Huon, R, Ziégler, M. Hallucinations in Parkinson’s disease. Prevalence, phenomenology and risk factors. Brain: A Journal of Neurology. 2000;123(4):733–45.
97.Sternberg, EJ, Alcalay, RN, Levy, OA, Louis, ED. Postural and intention tremors: a detailed clinical study of essential tremor vs. Parkinson’s disease. Frontiers in Neurology. 2013;4.
98.Jankovic, J, Schwartz, KS, Ondo, W. Re-emergent tremor of Parkinson’s disease. Journal of Neurology, Neurosurgery & Psychiatry. 1999;67(5):646–50.
99.Gelb, DJ, Oliver, E, Gilman, S. Diagnostic criteria for Parkinson disease. Archives of Neurology. 1999;56(1):33–9.
100.Galna, B, Lord, S, Burn, DJ, Rochester, L. Progression of gait dysfunction in incident Parkinson’s disease: Impact of medication and phenotype. Movement Disorders. 2015;30(3):359–67.
101.Morishita, T, Rahman, M, Foote, KD, Fargen, KM, IV Jacobson, CE, Fernandez, HH, et al. DBS candidates that fall short on a levodopa challenge test: alternative and important indications. The Neurologist. 2011;17(5):263.
102.Jankovic, J. Motor fluctuations and dyskinesias in Parkinson’s disease: clinical manifestations. Movement Disorders. 2005;20(S11):S11–S6.
103.Doherty, KM, van de Warrenburg, BP, Peralta, MC, Silveira-Moriyama, L, Azulay, J-P, Gershanik, OS, et al. Postural deformities in Parkinson’s disease. The Lancet Neurology. 2011;10(6):538–49.
104.Riley, D, Lang, A, Blair, R, Birnbaum, A, Reid, B. Frozen shoulder and other shoulder disturbances in Parkinson’s disease. Journal of Neurology, Neurosurgery & Psychiatry. 1989;52(1):63–6.
105.Barbeau, A. Diagnostic problems in Parkinson’s disease. Clinical Neurology and Neurosurgery. 1984;86(3):164–71.
106.Gjerstad, M, Boeve, B, Wentzel-Larsen, T, Aarsland, D, Larsen, J. Occurrence and clinical correlates of REM sleep behaviour disorder in patients with Parkinson’s disease over time. Journal of Neurology, Neurosurgery & Psychiatry. 2008;79(4):387–91.
107.Jahan, I, Hauser, RA, Sullivan, KL, Miller, A, Zesiewicz, TA. Sleep disorders in Parkinson’s disease. Neuropsychiatric Disease and Treatment. 2009;5:535.
108.Fronczek, R, Overeem, S, Lee, SY, Hegeman, IM, Van Pelt, J, Van Duinen, SG, et al. Hypocretin (orexin) loss in Parkinson’s disease. Brain: A Journal of Neurology. 2007;130(6):1577–85.
109.Thannickal, TC, Lai, Y-Y, Siegel, JM. Hypocretin (orexin) cell loss in Parkinson’s disease. Brain. 2007;130(6):1586–95.
110.Boeve, BF, Silber, MH, Ferman, TJ. REM sleep behavior disorder in Parkinson’s disease and dementia with Lewy bodies. Journal of Geriatric Psychiatry and Neurology. 2004;17(3):146–57.
111.Claassen, DO, Kutscher, SJ. Sleep disturbances in Parkinson’s disease patients and management options. Nature and Science of Sleep. 2011;3:125.
112.Peeraully, T, Tan, E-K. Linking restless legs syndrome with Parkinson’s disease: clinical, imaging and genetic evidence. Translational Neurodegeneration. 2012;1(6).
113.Boeve, B, Dickson, D, Olson, E, Shepard, J, Silber, M, Ferman, T, et al. Insights into REM sleep behavior disorder pathophysiology in brainstem-predominant Lewy body disease. Sleep Medicine. 2007;8(1):60–4.
114.Boeve, BF, Saper, CB. REM sleep behavior disorder A possible early marker for synucleinopathies. Neurology. 2006;66(6):796–7.
115.Livia Fantini, M, Gagnon, JF, Petit, D, Rompré, S, Décary, A, Carrier, J, et al. Slowing of electroencephalogram in rapid eye movement sleep behavior disorder. Annals of Neurology. 2003;53(6):774–80.
116.Massicotte‐Marquez, J, Carrier, J, Décary, A, Mathieu, A, Vendette, M, Petit, D, et al. Slow‐wave sleep and delta power in rapid eye movement sleep behavior disorder. Annals of Neurology. 2005;57(2):277–82.
117.Eisensehr, I, Linke, R, Noachtar, S, Schwarz, J, Gildehaus, F, Tatsch, K. Reduced striatal dopamine transporters in idiopathic rapid eye movement sleep behaviour disorder. Comparison with Parkinson’s disease and controls. Brain: A Journal of Neurology. 2000;123(6):1155–60.
118.Johannes Schwarz, M, Noachtar, S. Increased muscle activity during rapid eye movement sleep correlates with decrease of striatal presynaptic dopamine transporters. IPT and IBZM SPECT imaging in subclinical and clinically manifest idiopathic REM sleep behavior disorder, Parkinson’s disease, and controls. Sleep. 2003;26(5):507.
119.Mazza, S, Soucy, J, Gravel, P, Michaud, M, Postuma, R, Massicotte-Marquez, J, et al. Assessing whole brain perfusion changes in patients with REM sleep behavior disorder. Neurology. 2006;67(9):1618–22.
120.Caselli, RJ, Chen, K, Bandy, D, Smilovici, O, Boeve, BF, Osborne, D, et al. A preliminary fluorodeoxyglucose positron emission tomography study in healthy adults reporting dream-enactment behavior. 2006;29(7):927.
121.Ferini–Strambi, L, Di Gioia, M, Castronovo, V, Oldani, A, Zucconi, M, Cappa, S. Neuropsychological assessment in idiopathic REM sleep behavior disorder (RBD) Does the idiopathic form of RBD really exist? Neurology. 2004;62(1):41–5.
122.Stiasny-Kolster, K, Doerr, Y, Möller, J, Höffken, H, Behr, T, Oertel, W, et al. Combination of ‘idiopathic’ REM sleep behaviour disorder and olfactory dysfunction as possible indicator for α-synucleinopathy demonstrated by dopamine transporter FP-CIT-SPECT. Brain. 2005;128(1):126–37.
123.Ferini-Strambi, L, Oldani, A, Zucconi, M, Smirne, S. Cardiac autonomic activity during wakefulness and sleep in REM sleep behavior disorder. Sleep. 1996;19(5):367–9.
124.Postuma, RB, Lang, AE, Massicotte-Marquez, J, Montplaisir, J. Potential early markers of Parkinson disease in idiopathic REM sleep behavior disorder. Neurology. 2006;66(6):845–51.
125.Abbott, R, Ross, G, White, L, Tanner, C, Masaki, K, Nelson, J, et al. Excessive daytime sleepiness and subsequent development of Parkinson disease. Neurology. 2005;65(9):1442–6.
126.Marrinan, S, Emmanuel, AV, Burn, DJ. Delayed gastric emptying in Parkinson’s disease. Movement Disorders. 2014;29(1):2332.
127.Pellicano, C, Benincasa, D, Pisani, V, Buttarelli, FR, Giovannelli, M, Pontieri, FE. Prodromal non-motor symptoms of Parkinson’s disease. Neuropsychiatric Disease and Treatment. 2007;3(1):145.
128.Bayulkem, K, Lopez, G. Nonmotor fluctuations in Parkinson’s disease: clinical spectrum and classification. Journal of the Neurological Sciences. 2010;289(1):8992.
129.Braak, H, Del Tredici, K, Rüb, U, de Vos, RA, Steur, ENJ, Braak, E. Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiology of Aging. 2003;24(2):197211.
130.Braak, H, Ghebremedhin, E, Rüb, U, Bratzke, H, Del Tredici, K. Stages in the development of Parkinson’s disease-related pathology. Cell and Tissue Research. 2004;318(1):121–34.
131.Donadio, V, Incensi, A, Leta, V, Giannoccaro, MP, Scaglione, C, Martinelli, P, et al. Skin nerve α-synuclein deposits: A biomarker for idiopathic Parkinson’s disease. Neurology. 2014;82(15):1362–9.
132.Espay, AJ, LeWitt, PA, Kaufmann, H. Norepinephrine deficiency in Parkinson’s disease: The case for noradrenergic enhancement. Movement Disorders. 2014;29(14):1710–19.
133.Wang, N, Gibbons, CH, Lafo, J, Freeman, R. α-Synuclein in cutaneous autonomic nerves. Neurology. 2013;81(18):1604–10.
134.Xiao, Q, Chen, S, Le, W. Hyposmia: a possible biomarker of Parkinson’s disease. Neuroscience Bulletin. 2014;30(1):134–40.
135.Goldstein, DS, Holmes, C, Bentho, O, Sato, T, Moak, J, Sharabi, Y, et al. Biomarkers to detect central dopamine deficiency and distinguish Parkinson disease from multiple system atrophy. Parkinsonism & Related Disorders. 2008;14(8):600–7.
136.Cecchini, MP, Osculati, F, Ottaviani, S, Boschi, F, Fasano, A, Tinazzi, M. Taste performance in Parkinson’s disease. Journal of Neural Transmission. 2014;121(2):119–22.
137.Shah, M, Deeb, J, Fernando, M, Noyce, A, Visentin, E, Findley, LJ, et al. Abnormality of taste and smell in Parkinson’s disease. Parkinsonism & Related Disorders. 2009;15(3):232–7.
138.Ford, B. Pain in Parkinson’s disease. Movement Disorders. 2010;25(S1):S98–103.
139.Nolano, M, Provitera, V, Lanzillo, B, Santoro, L. Neuropathy in idiopathic Parkinson disease: an iatrogenic problem? Annals of Neurology. 2011;69(2):427–8.
140.Armstrong, RA. Visual symptoms in Parkinson’s disease. Parkinson’s Disease. Epub: vol. 2011, Article ID 908306, 9 pages, 2011. doi:10.4061/2011/908306.
141.Lepore, FE. Parkinson’s disease and diplopia. Neuro-Ophthalmology. 2006;30(2–3):3740.
142.Nowacka, B, Lubiński, W, Honczarenko, K, Potemkowski, A, Safranow, K. Ophthalmological features of Parkinson’s disease. Medical Science Monitor: International Medical Journal of Experimental and Clinical Research. 2014;20:2243.
143.Pieri, V, Diederich, N, Raman, R, Goetz, C. Decreased color discrimination and contrast sensitivity in Parkinson’s disease. Journal of the Neurological Sciences. 2000;172(1):711.
144.Pinkhardt, EH, Kassubek, J. Ocular motor abnormalities in Parkinsonian syndromes. Parkinsonism & Related Disorders. 2011;17(4):223–30.
145.Almer, Z, Klein, KS, Marsh, L, Gerstenhaber, M, Repka, MX. Ocular motor and sensory function in Parkinson’s disease. Ophthalmology. 2012;119(1):178–82.
146.Abe, N, Fujii, T, Hirayama, K, Takeda, A, Hosokai, Y, Ishioka, T, et al. Do Parkinsonian patients have trouble telling lies? The neurobiological basis of deceptive behaviour. Brain: A Journal of Neurology. 2009;132(5):1386–95.
147.Santangelo, G, Vitale, C, Trojano, L, Errico, D, Amboni, M, Barbarulo, AM, et al. Neuropsychological correlates of theory of mind in patients with early Parkinson’s disease. Movement Disorders. 2012;27(1):98105.
148.Thaler, A, Posen, J, Giladi, N, Manor, Y, Mayanz, C, Mirelman, A, et al. Appreciation of humor is decreased among patients with Parkinson’s disease. Parkinsonism & Related Disorders. 2012;18(2):144–8.
149.Hillier, A, Beversdorf, D, Raymer, A, Williamson, D, Heilman, K. Abnormal emotional word ratings in Parkinson’s disease. Neurocase. 2007;13(2):81–5.
150.Wieser, MJ, Mühlberger, A, Alpers, GW, Macht, M, Ellgring, H, Pauli, P. Emotion processing in Parkinson’s disease: dissociation between early neuronal processing and explicit ratings. Clinical Neurophysiology. 2006;117(1):94102.
151.Gray, HM, Tickle-Degnen, L. A meta-analysis of performance on emotion recognition tasks in Parkinson’s disease. Neuropsychology. 2010;24(2):176.
152.Steele, JC, Richardson, JC, Olszewski, J. Progressive supranuclear palsy: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Archives of Neurology. 1964;10(4):333–59.
153.De Bruin, V, Lees, A. Subcortical neurofibrillary degeneration presenting as Steele‐Richardson‐Olszewski and other related syndromes: A review of 90 pathologically verified cases. Movement Disorders. 1994;9(4):381–9.
154.Schrag, A, Ben-Shlomo, Y, Quinn, N. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. The Lancet. 1999;354(9192):1771–5.
155.Williams, DR, de Silva, R, Paviour, DC, Pittman, A, Watt, HC, Kilford, L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain: A Journal of Neurology. 2005;128(6):1247–58.
156.Chiu, WZ, Kaat, LD, Seelaar, H, Rosso, SM, Boon, AJ, Kamphorst, W, et al. Survival in progressive supranuclear palsy and frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry. 2010;81(4):441–5.
157.Diroma, C, Dell’Aquila, C, Fraddosio, A, Lamberti, S, Mastronardi, R, Russo, I, et al. Natural history and clinical features of progressive supranuclear palsy: a clinical study. Neurological Sciences. 2003;24(3):176–7.
158.Litvan, I, Agid, Y, Calne, D, Campbell, G, Dubois, B, Duvoisin, R, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Report of the NINDS-SPSP International Workshop. Neurology. 1996;47(1):19.
159.O’Sullivan, S, Massey, L, Williams, D, Silveira-Moriyama, L, Kempster, P, Holton, J, et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain: A Journal of Neurology. 2008;131(5):1362–72.
160.Williams, DR, Lees, AJ. What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy‐parkinsonism (PSP‐P)? Movement Disorders. 2010;25(3):357–62.
161.Litvan, I, Mangone, C, McKee, A, Verny, M, Parsa, A, Jellinger, K, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. Journal of Neurology, Neurosurgery & Psychiatry. 1996;60(6):615–20.
162.Respondek, G, Roeber, S, Kretzschmar, H, Troakes, C, Al‐Sarraj, S, Gelpi, E, et al. Accuracy of the National Institute for Neurological Disorders and Stroke/Society for Progressive Supranuclear Palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy. Movement Disorders. 2013;28(4):504–9.
163.Wenning, GK, Litvan, I, Tolosa, E. Milestones in atypical and secondary parkinsonisms. Movement Disorders. 2011;26(6):1083–95.
164.Williams, DR, Lees, AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. The Lancet Neurology. 2009;8(3):270–9.
165.Josephs, K, Boeve, B, Duffy, J, Smith, G, Knopman, D, Parisi, J, et al. Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. Neurocase. 2005;11(4):283–96.
166.Josephs, KA, Duffy, JR, Strand, EA, Whitwell, JL, Layton, KF, Parisi, JE, et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain: A Journal of Neurology. 2006;129(6):1385–98.
167.Bak, T, Crawford, L, Hearn, V, Mathuranath, P, Hodges, JR. Subcortical dementia revisited: similarities and differences in cognitive function between progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA). Neurocase. 2005;11(4):268–73.
168.Dubois, B, Pillon, B, Legault, F, Agid, Y, Lhermitte, F. Slowing of cognitive processing in progressive supranuclear palsy: a comparison with Parkinson’s disease. Archives of Neurology. 1988;45(11):1194–9.
169.Pillon, B, Blin, J, Vidailhet, M, Deweer, B, Sirigu, A, Dubois, B, et al. The neuropsychological pattern of corticobasal degeneration: Comparison with progressive supranuclear palsy and Alzheimer’s disease. Neurology. 1995;45(8):1477–83.
170.Pillon, B, Deweer, B, Michon, A, Malapani, C, Agid, Y, Dubois, B. Are explicit memory disorders of progressive supranuclear palsy related to damage to striatofrontal circuits? Comparison with Alzheimer’s, Parkinson’s, and Huntington’s diseases. Neurology. 1994;44(7):1264.
171.Grafman, J, Litvan, I, Gomez, C, Chase, TN. Frontal lobe function in progressive supranuclear palsy. Archives of Neurology. 1990;47(5):553–8.
172.Dubois, B, Slachevsky, A, Pillon, B, Beato, R, Villalponda, J, Litvan, I. “Applause sign” helps to discriminate PSP from FTD and PD. Neurology. 2005;64(12):2132–3.
173.Vidailhet, M, Rivaud, S, Gouider‐Khouja, N, Pillon, B, Bonnet, AM, Gaymard, B, et al. Eye movements in Parkinsonian syndromes. Annals of Neurology. 1994;35(4):420–6.
174.Ghosh, BC, Calder, AJ, Peers, PV, Lawrence, AD, Acosta-Cabronero, J, Pereira, JM, et al. Social cognitive deficits and their neural correlates in progressive supranuclear palsy. Brain: A Journal of Neurology. 2012:aws128.
175.Rippon, G, Boeve, B, Parisi, J, Dickson, D, Ivnik, R, Jack, C, et al. Late-onset frontotemporal dementia associated with progressive supranuclear palsy/argyrophilic grain disease/Alzheimer’s disease pathology. Neurocase. 2005;11(3):204–11.
176.Karnik, NS, D'Apuzzo, M, Greicius, M. Non-fluent progressive aphasia, depression, and OCD in a woman with progressive supranuclear palsy: neuroanatomical and neuropathological correlations. Neurocase. 2006;12(6):332–8.
177.Arnulf, I, Merino-Andreu, M, Bloch, F, Konofal, E, Vidailhet, M, Cochen, V, et al. REM sleep behavior disorder and REM sleep without flatonia in patients with progressive supranuclear palsy. Sleep. 2005;28(3):349–54.
178.De Cock, VC, Vidailhet, M, Arnulf, I. Sleep disturbances in patients with parkinsonism. Nature Clinical Practice Neurology. 2008;4(5):254–66.
179.Montplaisir, J, Petit, D, Decary, A, Masson, H, Bédard, M, Panisset, M, et al. Sleep and quantitative EEG in patients with progressive supranuclear palsy. Neurology. 1997;49(4):9991003.
180.Batla, A, Nehru, R, Vijay, T. Vertical wrinkling of the forehead or Procerus sign in progressive supranuclear palsy. Journal of the Neurological Sciences. 2010;298(1):148–9.
181.Cordato, NJ, Halliday, GM, Caine, D, Morris, JG. Comparison of motor, cognitive, and behavioral features in progressive supranuclear palsy and Parkinson’s disease. Movement Disorders. 2006;21(5):632–8.
182.Romano, S, Colosimo, C. Procerus sign in progressive supranuclear palsy. Neurology. 2001;57(10):1928.
183.Wenning, GK, Geser, F, Krismer, F, Seppi, K, Duerr, S, Boesch, S, et al. The natural history of multiple system atrophy: a prospective European cohort study. The Lancet Neurology. 2013;12(3):264–74.
184.Wenning, G, Shlomo, YB, Magalhaes, M, Danie, S, Quinn, N. Clinical features and natural history of multiple system atrophy. Brain: A Journal of Neurology. 1994;117(4):835–45.
185.Watanabe, H, Saito, Y, Terao, S, Ando, T, Kachi, T, Mukai, E, et al. Progression and prognosis in multiple system atrophy. Brain: A Journal of Neurology. 2002;125(5):1070–83.
186.Schrag, A, Wenning, GK, Quinn, N, Ben‐Shlomo, Y. Survival in multiple system atrophy. Movement Disorders. 2008;23(2):294–6.
187.Gilman, S, Wenning, G, Low, Pa, Brooks, D, Mathias, C, Trojanowski, J, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71(9):670–6.
188.Chang, C, Chang, Y, Chang, W, Lee, Y, Wang, Y, Lui, C, et al. Cognitive deficits in multiple system atrophy correlate with frontal atrophy and disease duration. European Journal of Neurology. 2009;16(10):1144–50.
189.Stankovic, I, Krismer, F, Jesic, A, Antonini, A, Benke, T, Brown, RG, et al. Cognitive impairment in multiple system atrophy: A position statement by the neuropsychology task force of the MDS Multiple System Atrophy (MODIMSA) study group. Movement Disorders. 2014;29(7):857–67.
190.Kim, H-J, Jeon, BS, Kim, YE, Kim, J-Y, Kim, YK, Sohn, C-H, et al. Clinical and imaging characteristics of dementia in multiple system atrophy. Parkinsonism & Related Disorders. 2013;19(6):617–21.
191.Benrud‐Larson, LM, Sandroni, P, Schrag, A, Low, PA. Depressive symptoms and life satisfaction in patients with multiple system atrophy. Movement Disorders. 2005;20(8):951–7.
192.Schrag, A, Sheikh, S, Quinn, NP, Lees, AJ, Selai, C, Mathias, C, et al. A comparison of depression, anxiety, and health status in patients with progressive supranuclear palsy and multiple system atrophy. Movement Disorders. 2010;25(8):1077–81.
193.Balas, M, Balash, Y, Giladi, N, Gurevich, T. Cognition in multiple system atrophy: neuropsychological profile and interaction with mood. Journal of Neural Transmission. 2010;117(3):369–75.
194.Fetoni, V, Soliveri, P, Monza, D, Testa, D, Girotti, F. Affective symptoms in multiple system atrophy and Parkinson’s disease: response to levodopa therapy. Journal of Neurology, Neurosurgery & Psychiatry. 1999;66(4):541–4.
195.Kojovic, M, Cordivari, C, Bhatia, K. Myoclonic disorders: a practical approach for diagnosis and treatment. Therapeutic Advances in Neurological Disorders. 2011;4(1):4762.
196.Wenning, GK, Stefanova, N. Recent developments in multiple system atrophy. Journal of Neurology. 2009;256(11):1791–808.
197.Chou, KL, Forman, MS, Trojanowski, JQ, Hurtig, HI, Baltuch, GH. Subthalamic nucleus deep brain stimulation in a patient with levodopa-responsive multiple system atrophy: case report. Journal of Neurosurgery. 2004;100(3):553–6.
198.Shih, LC, Tarsy, D. Deep brain stimulation for the treatment of atypical parkinsonism. Movement Disorders. 2007;22(15):2149–55.
199.Ullman, M, Vedam-Mai, V, Resnick, AS, Yachnis, AT, McFarland, NR, Merritt, S, et al. Deep brain stimulation response in pathologically confirmed cases of multiple system atrophy. Parkinsonism & Related Disorders. 2012;18(1):86–8.
200.Aerts, MB, Esselink, RA, Post, B, van de Warrenburg, BP, Bloem, BR. Improving the diagnostic accuracy in parkinsonism: a three-pronged approach. Practical Neurology. 2012;12(2):7787.
201.Köllensperger, M, Geser, F, Ndayisaba, JP, Boesch, S, Seppi, K, Ostergaard, K, et al. Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Movement Disorders. 2010;25(15):2604–12.
202.Ghorayeb, I, Bioulac, B, Tison, F. Sleep disorders in multiple system atrophy. Journal of Neural Transmission. 2005;112(12):1669–75.
203.Iranzo, A. Sleep and breathing in multiple-system atrophy. Current Treatment Options in Neurology. 2007;9(5):347–53.
204.Tison, F, Wenning, G, Volonte, M, Poewe, W, Henry, P, Quinn, N. Pain in multiple system atrophy. Journal of Neurology. 1996;243(2):153–6.
205.Boeve, BF, Lang, AE, Litvan, I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Annals of Neurology. 2003;54(S5):S15–9.
206.Mathew, R, Bak, TH, Hodges, JR. Diagnostic criteria for corticobasal syndrome: a comparative study. Journal of Neurology, Neurosurgery & Psychiatry. 2011:jnnp-2011–300875.
207.Wenning, G, Litvan, I, Jankovic, J, Granata, R, Mangone, C, McKee, A, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. Journal of Neurology, Neurosurgery & Psychiatry. 1998;64(2):184–9.
208.Armstrong, MJ, Litvan, I, Lang, AE, Bak, TH, Bhatia, KP, Borroni, B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496503.
209.Lee, SE, Rabinovici, GD, Mayo, MC, Wilson, SM, Seeley, WW, DeArmond, SJ, et al. Clinicopathological correlations in corticobasal degeneration. Annals of Neurology. 2011;70(2):327–40.
210.Boeve, B, Maraganore, D, Parisi, J, Ahlskog, J, Graff-Radford, N, Caselli, R, et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology. 1999;53(4):795.
211.Schneider, J, Watts, R, Gearing, M, Brewer, R, Mirra, S. Corticobasal degeneration. Neuropathologic and clinical heterogeneity. Neurology. 1997;48(4):959–68.
212.Alexander, S, Rittman, T, Xuereb, J, Bak, T, Hodges, J, Rowe, J. Validation of the new consensus criteria for the diagnosis of corticobasal degeneration. Journal of Neurology, Neurosurgery & Psychiatry. 2014:jnnp-2013–307035.
213.Massman, PJ, Kreiter, KT, Jankovic, J, Doody, RS. Neuropsychological functioning in cortical-basal ganglionic degeneration. Differentiation from Alzheimer’s disease. Neurology. 1996;46(3):720–6.
214.Blake, ML, Duffy, J, Boeve, B, Ahlskog, J, Maraganore, D. Speech and language disorders associated with corticobasal degeneration. Journal of Medical Speech–Language Pathology. 2003;11(3):131–46.
215.Rinne, J, Lee, M, Thompson, P, Marsden, C. Corticobasal degeneration: a clinical study of 36 cases. Brain: A Journal of Neurology. 1994;117(5):1183–96.
216.Cummings, J, Litvan, I. Neuropsychiatric aspects of corticobasal degeneration. In Litvan, I, Goetz, C, Lang, A, editors. Corticobasal Degeneration and Related Disorders. London: Lippincott, Williams & Wilkins; 2000. pp. 147–52.
217.Geda, YE, Boeve, BF, Negash, S, Graff-Radford, NR, Knopman, DS, Parisi, JE, et al. Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. The Journal of Neuropsychiatry and Clinical Neurosciences. 2006;19(1):7780.
218.Bruns, MB, Josephs, KA. Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy. International Review of Psychiatry. 2013;25(2):197209.
219.Roche, S, Jacquesson, J-M, Destee, A, Defebvre, L, Derambure, P, Monaca, C. Sleep and vigilance in corticobasal degeneration: a descriptive study. Neurophysiologie Clinique/Clinical Neurophysiology. 2007;37(4):261–4.
220.Kompoliti, K, Goetz, C, Boeve, B, Maraganore, D, Ahlskog, J, Marsden, C, et al. Clinical presentation and pharmacological therapy in corticobasal degeneration. Archives of Neurology. 1998;55(7):957–61.
221.Tang-Wai, D, Josephs, K, Boeve, B, Dickson, D, Parisi, J, Petersen, R. Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology. 2003;61(8):1134–5.
222.Leyton, CE, Hodges, JR. Frontotemporal dementias: Recent advances and current controversies. Annals of Indian Academy of Neurology. 2010;13(Supp l2):S74.
223.Rohrer, JD, Warren, JD. Phenotypic signatures of genetic frontotemporal dementia. Current Opinion in Neurology. 2011;24(6):542–9.
224.Siuda, J, Fujioka, S, Wszolek, ZK. Parkinsonian syndrome in familial frontotemporal dementia. Parkinsonism & Related Disorders. 2014;20(9):957–64.
225.Baker, M, Mackenzie, IR, Pickering-Brown, SM, Gass, J, Rademakers, R, Lindholm, C, et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature. 2006;442(7105):916–19.
226.Gass, J, Cannon, A, Mackenzie, IR, Boeve, B, Baker, M, Adamson, J, et al. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Human Molecular Genetics. 2006;15(20):29883001.
227.Boeve, BF, Hutton, M. Refining frontotemporal dementia with parkinsonism linked to chromosome 17: introducing FTDP-17 (MAPT) and FTDP-17 (PGRN). Archives of Neurology. 2008;65(4):460–4.
228.Sharon, JS, Takada, LT, Rankin, KP, Yokoyama, JS, Rutherford, NJ, Fong, JC, et al. Frontotemporal dementia due to C9ORF72 mutations. Clinical and imaging features. Neurology. 2012;79(10):1002–11.
229.Rascovsky, K, Hodges, JR, Knopman, D, Mendez, MF, Kramer, JH, Neuhaus, J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain: A Journal of Neurology. 2011;134(9):2456–77.
230.Lindquist, S, Duno, M, Batbayli, M, Puschmann, A, Braendgaard, H, Mardosiene, S, et al. Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease. Clinical Genetics. 2013;83(3):279–83.
231.Ferman, T, McRae, C, Arvanitakis, Z, Tsuboi, Y, Vo, A, Wszolek, Z. Early and pre-symptomatic neuropsychological dysfunction in the PPND family with the N279K tau mutation. Parkinsonism & Related Disorders. 2003;9(5):265–70.
232.Frank, AR, Wszolek, ZK, Jack, CR, Boeve, BF. Distinctive MRI findings in pallidopontonigral degeneration (PPND). Neurology. 2007;68(8):620–1.
233.Geschwind, DH, Robidoux, J, Alarcón, M, Miller, BL, Wilhelmsen, KC, Cummings, JL, et al. Dementia and neurodevelopmental predisposition: cognitive dysfunction in presymptomatic subjects precedes dementia by decades in frontotemporal dementia. Annals of Neurology. 2001;50(6):741–6.
234.Gregory, C, Lough, S, Stone, V, Erzinclioglu, S, Martin, L, Baron‐Cohen, S, et al. Theory of mind in patients with frontal variant frontotemporal dementia and Alzheimer’s disease: theoretical and practical implications. Brain: A Journal of Neurology. 2002;125(4):752–64.
235.Seelaar, H, Rohrer, JD, Pijnenburg, YA, Fox, NC, Van Swieten, JC. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. Journal of Neurology, Neurosurgery & Psychiatry. 2010:jnnp. 2010.212225.
236.Torralva, T, Kipps, CM, Hodges, JR, Clark, L, Bekinschtein, T, Roca, M, et al. The relationship between affective decision-making and theory of mind in the frontal variant of fronto-temporal dementia. Neuropsychologia. 2007;45(2):342–9.
237.Hodges, JR. Frontotemporal dementia (Pick’s disease): clinical features and assessment. Neurology. 2001;56(Suppl 4):S6–10.
238.Miller, BL, Boone, K, Cummings, JL, Read, SL, Mishkin, F. Functional correlates of musical and visual ability in frontotemporal dementia. The British Journal of Psychiatry. 2000;176(5):458–63.
239.Miller, BL, Diehl, J, Freedman, M, Kertesz, A, Mendez, M, Rascovsky, K. International approaches to frontotemporal dementia diagnosis: from social cognition to neuropsychology. Annals of Neurology. 2003;54(S5):S7–10.
240.Miller, BL, Seeley, WW, Mychack, P, Rosen, HJ, Mena, I, Boone, K. Neuroanatomy of the self. Evidence from patients with frontotemporal dementia. Neurology. 2001;57(5):817–21.
241.Rankin, KP, Kramer, JH, Mychack, P, Miller, BL. Double dissociation of social functioning in frontotemporal dementia. Neurology. 2003;60(2):266–71.
242.Woolley, J, Gorno-Tempini, M-L, Seeley, W, Rankin, K, Lee, S, Matthews, B, et al. Binge eating is associated with right orbitofrontal-insular-striatal atrophy in frontotemporal dementia. Neurology. 2007;69(14):1424–33.
243.Benussi, L, Ghidoni, R, Pegoiani, E, Moretti, DV, Zanetti, O, Binetti, G. Progranulin Leu271LeufsX10 is one of the most common FTLD and CBS associated mutations worldwide. Neurobiology of Disease. 2009;33(3):379–85.
244.Ranasinghe, K, Pressman, P, Perry, D, Rankin, K, Miller, B. Clinical Characteristics of bvFTD across CDR stages (P3. 201). Neurology. 2014;82(Suppl 10):P3.201–P3.
245.Arvanitakis, Z, Witte, RJ, Dickson, DW, Tsuboi, Y, Uitti, RJ, Slowinski, J, et al. Clinical-pathologic study of biomarkers in FTDP-17 (PPND family with N279K tau mutation). Parkinsonism & Related Disorders. 2007;13(4):230–9.
246.Boeve, BF, Tremont-Lukats, IW, Waclawik, AJ, Murrell, JR, Hermann, B, Jack, CR, et al. Longitudinal characterization of two siblings with frontotemporal dementia and parkinsonism linked to chromosome 17 associated with the S305N tau mutation. Brain: A Journal of Neurology. 2005;128(4):752–72.
247.Anderson, K, Hatfield, C, Kipps, C, Hastings, M, Hodges, J. Disrupted sleep and circadian patterns in frontotemporal dementia. European Journal of Neurology. 2009;16(3):317–23.
248.Cipriani, G, Lucetti, C, Danti, S, Nuti, A. Sleep disturbances and dementia. Psychogeriatrics. 2015;15(1):6574.
249.Coco, DL, Cupidi, C, Mattaliano, A, Baiamonte, V, Realmuto, S, Cannizzaro, E. REM sleep behavior disorder in a patient with frontotemporal dementia. Neurological Sciences. 2012;33(2):371–3.
250.Boeve, BF, Lin, S-C, Strongosky, A, Dickson, DW, Wszolek, ZK. Absence of rapid eye movement sleep behavior disorder in 11 members of the pallidopontonigral degeneration kindred. Archives of Neurology. 2006;63(2):268–72.
251.Ahmed, R, Iodice, V, Daveson, N, Kiernan, M, Piguet, O, Hodges, J. Autonomic dysregulation in frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry. 2014:jnnp-2014–309424.
252.Cheshire, WP, Tsuboi, Y, Wszolek, ZK. Physiologic assessment of autonomic dysfunction in pallidopontonigral degeneration with N279K mutation in the tau gene on chromosome 17. Autonomic Neuroscience. 2002;102(1):71–7.
253.Wszolek, Z, Lagerlund, T, Steg, R, McManis, P. Clinical neurophysiologic findings in patients with rapidly progressive familial parkinsonism and dementia with pallido-ponto-nigral degeneration. Electroencephalography and Clinical Neurophysiology. 1998;107(3):213–22.
254.Marek, K, Jennings, D, Lasch, S, Siderowf, A, Tanner, C, Simuni, T, et al. The Parkinson progression marker initiative (PPMI). Progress in Neurobiology. 2011;95(4):629–35.