Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology

ABSTRACT Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

[1]  T. Ferman,et al.  Diffuse Lewy body disease manifesting as corticobasal syndrome , 2018, Neurology.

[2]  T. Uchihara,et al.  Reader response: Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB consortium , 2018, Neurology.

[3]  Alan J. Thomas,et al.  Diagnosis and management of dementia with Lewy bodies , 2017, Neurology.

[4]  D. Bennett,et al.  TDP-43 pathology, cognitive decline, and dementia in old age. , 2013, JAMA neurology.

[5]  Mark Hallett,et al.  Criteria for the diagnosis of corticobasal degeneration , 2013, Neurology.

[6]  H. Arai,et al.  Neuropathological investigation of the hypometabolic regions on positron emission tomography with [18F] fluorodeoxyglucose in patients with dementia with Lewy bodies , 2012, Journal of the Neurological Sciences.

[7]  V. Pankratz,et al.  Inclusion of RBD improves the diagnostic classification of dementia with Lewy bodies , 2011, Neurology.

[8]  J. Trojanowski,et al.  Clinicopathological correlations in corticobasal degeneration , 2011, Annals of neurology.

[9]  T. Ellmore,et al.  Reduced volume of the putamen in REM sleep behavior disorder patients. , 2010, Parkinsonism & related disorders.

[10]  William T. Hu,et al.  Anatomical differences between CBS‐corticobasal degeneration and CBS‐Alzheimer's disease , 2010, Movement disorders : official journal of the Movement Disorder Society.

[11]  E. Bigio,et al.  Phosphorylated TDP-43 pathology and hippocampal sclerosis in progressive supranuclear palsy , 2010, Acta Neuropathologica.

[12]  Carles Falcon,et al.  Frontal and associative visual areas related to visual hallucinations in dementia with Lewy bodies and Parkinson's disease with dementia , 2010, Movement disorders : official journal of the Movement Disorder Society.

[13]  C. Jack,et al.  Mild cognitive impairment associated with limbic and neocortical Lewy body disease: a clinicopathological study. , 2010, Brain : a journal of neurology.

[14]  Y. Nagahama,et al.  Neural correlates of psychotic symptoms in dementia with Lewy bodies. , 2010, Brain : a journal of neurology.

[15]  T Revesz,et al.  A clinico-pathological study of subtypes in Parkinson's disease. , 2009, Brain : a journal of neurology.

[16]  M. Marti,et al.  Parkinsonism, dysautonomia, REM behaviour disorder and visual hallucinations mimicking synucleinopathy in a patient with progressive supranuclear palsy , 2009, Journal of Neurology, Neurosurgery, and Psychiatry.

[17]  K. Josephs,et al.  Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. , 2009, Parkinsonism & related disorders.

[18]  J. Trojanowski,et al.  Concomitant TAR-DNA-Binding Protein 43 Pathology Is Present in Alzheimer Disease and Corticobasal Degeneration but Not in Other Tauopathies , 2008, Journal of neuropathology and experimental neurology.

[19]  A. Drzezga,et al.  Cerebral Metabolic Dysfunction in Patients with Dementia with Lewy Bodies and Visual Hallucinations , 2008, Dementia and Geriatric Cognitive Disorders.

[20]  E. David Williams,et al.  Nicotinic α4β2 receptor binding in dementia with Lewy bodies using 123I-5IA-85380 SPECT demonstrates a link between occipital changes and visual hallucinations , 2008, NeuroImage.

[21]  Deborah C Mash,et al.  Cortical and amygdalar Lewy body burden in Parkinson's disease patients with visual hallucinations. , 2006, Parkinsonism & related disorders.

[22]  S Minoshima,et al.  Diagnosis and management of dementia with Lewy bodies , 2005, Neurology.

[23]  Hansjürgen Bratzke,et al.  Stages in the development of Parkinson’s disease-related pathology , 2004, Cell and Tissue Research.

[24]  Howard J. Rosen,et al.  Distinctive Neuropsychological Patterns in Frontotemporal Dementia, Semantic Dementia, And Alzheimer Disease , 2003, Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology.

[25]  G. E. Smith,et al.  Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism , 2003, Neurology.

[26]  Irene Litvan,et al.  Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia , 2003, Annals of neurology.

[27]  G. Halliday,et al.  Clinical correlates of selective pathology in the amygdala of patients with Parkinson's disease. , 2002, Brain : a journal of neurology.

[28]  G. Halliday,et al.  Visual hallucinations in Lewy body disease relate to Lewy bodies in the temporal lobe. , 2002, Brain : a journal of neurology.

[29]  B. Boeve,et al.  Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy , 2001, Movement disorders : official journal of the Movement Disorder Society.

[30]  M N Rossor,et al.  Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype , 2001, Neurology.

[31]  L. Hakamies,et al.  [Corticobasal degeneration]. , 2001, Duodecim; laaketieteellinen aikakauskirja.

[32]  E. Perry,et al.  Delusions associated with elevated muscarinic binding in dementia with Lewy bodies , 2000, Annals of neurology.

[33]  T. Komori,et al.  Tau‐positive dial Inclusions in Progressive Supranuclear Palsy, Corticobasal Degeneration and Pick's Disease , 1999, Brain pathology.

[34]  K. Ishii,et al.  Regional cerebral glucose metabolism in dementia with Lewy bodies and Alzheimer's disease , 1998, Neurology.

[35]  C. Marsden,et al.  Clinical presentation and pharmacological therapy in corticobasal degeneration. , 1998, Archives of neurology.

[36]  K. Kosaka.  Diffuse Lewy body disease. , 1995, Internal medicine.

[37]  J. Trojanowski,et al.  Editorial on Consensus Recommendations for the Postmortem Diagnosis of Alzheimer Disease from the National Institute on Aging and the Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer Disease , 1997, Journal of neuropathology and experimental neurology.

[38]  M. L. Schmidt,et al.  α-Synuclein in Lewy bodies , 1997, Nature.

[39]  K. Kosaka,et al.  Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB) , 1996, Neurology.

[40]  K. Jellinger,et al.  Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. , 1996, Neurology.

[41]  P. Thompson,et al.  Corticobasal Degeneration , 2020, Definitions.

[42]  E. Perry,et al.  Evidence of a Monoaminergic‐Cholinergic Imbalance Related to Visual Hallucinations in Lewy Body Dementia , 1990, Journal of neurochemistry.

[43]  C. Marsden,et al.  Clinical and pathological features of corticobasal degeneration. , 1990, Advances in neurology.

[44]  K Kosaka,et al.  Diffuse type of Lewy body disease: progressive dementia with abundant cortical Lewy bodies and senile changes of varying degree--a new disease? , 1984, Clinical neuropathology.