Iodine-123 Ioflupane Scintigraphy for the Diagnosis of Lewy Body Disease Presenting as a Posterior Cortical Atrophy

This chapter provides a brief overview of the different forms of dementia syndromes and provides a simple algorithm for initial differential diagnosis. Rapidly progressive dementias have to be excluded which require specific investigations to detect Creutzfeldt–Jakob as well as inflammatory and autoimmune diseases. A lead symptom-based approach in patients with slowly progressive cognitive and behavioural impairments without neurological symptoms is applied: progressive and primary impairments in recent memory are characteristic of typical Alzheimer’s dementia, primary behavioural changes point to the behavioural variant of frontotemporal dementia, primary impairments of language or speech are distinctive for progressive aphasias, fluctuating impairments of attention are a hallmark of Lewy body dementia, whereas primary visuospatial impairments suggest a posterior cortical atrophy. The chapter further discusses updated vascular dementia guidelines and DSM-5 revisions of defining dementia. Current diagnostic criteria for the different dementias are referenced and the role of neuroimaging is illustrated.

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Nightmares without atonia as an early symptom of diffuse Lewy bodies disease

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2003000600009 ◽

2003 ◽

Vol 61 (4) ◽

pp. 936-941 ◽

Cited By ~ 10

Author(s):

Paulo Roberto de Brito-Marques ◽

Roberto Vieira de Mello ◽

Luciano Montenegro

Keyword(s):

Lewy Body ◽

Neuropsychiatric Symptoms ◽

Senile Plaques ◽

Lewy Bodies ◽

Neuronal Loss ◽

Lewy Body Disease ◽

Behavioral Effect ◽

Cortical Atrophy ◽

Limbic Cortex ◽

Resonance Spectroscopy

A male 70 years old patient with diffuse or ''pure'' Lewy body disease is described. The diagnosis was made based on clinical features of nightmares with no atonia, attention deficits with fluctuation in cognitive function, incapacity to find his way around the neighbourhood and other formerly familiar environments and mild neuropsychiatric symptoms. Neuropsychological assessment showed memory deficits, visuospatial and visuo-constructive disturbances. He had neither parkinsonism nor recurrent visual hallucinations typically well formed and detailled. Neuroimaging (computed tomography and magnetic resonance spectroscopy) showed mild diffuse cortical atrophy, mostly on the left temporal lobe and a decrease of N-acetil-aspartate levels. A cholinesterase inhibitor was prescribed to this patient during 6 months with clinically relevant behavioral effect. Diagnosis confirmation was made by post-mortem neuropathological findings. Macroscopical features were mild atrophy on the frontal, parietal and temporal lobes, notedly on the frontal lobes. Microscopically, there was neuronal loss and diffuse classic Lewy bodies. Brainstem (substantia nigra, raphe nucleus, locus coeruleus, pedunculopontine nucleus), limbic cortex, and neocortex (frontal, parietal and temporal) were the areas of predilection for Lewy bodies. Hematoxylin-eosin and Bielschowsky staining did not show neuronal swelling (balooned cell), argyrophilic inclusion (Pick's bodies), neurofibrillary tangles nor senile plaques. Immunohistochemical staining for anti-tau, anti-beta-amyloid, and anti-prion protein were negative. Antiubiquitine reaction was positive for Lewy body in the cerebral cortex and brainstem.

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Association of β-Amyloid and Basal Forebrain With Cortical Thickness and Cognition in Alzheimer and Lewy Body Disease Spectra

Neurology ◽

10.1212/wnl.0000000000013277 ◽

2021 ◽

pp. 10.1212/WNL.0000000000013277

Author(s):

Han Soo Yoo ◽

Seun Jeon ◽

Enrica Cavedo ◽

MinJin Ko ◽

Mijin Yun ◽

...

Keyword(s):

Cognitive Dysfunction ◽

Cortical Thickness ◽

Basal Forebrain ◽

Lewy Body ◽

Brain Magnetic Resonance Imaging ◽

Lewy Body Disease ◽

Cortical Atrophy ◽

Periventricular White Matter ◽

Intracranial Volume ◽

Β Amyloid

Objective:Cholinergic degeneration and β-amyloid contribute to brain atrophy and cognitive dysfunction in Alzheimer’s disease (AD) and Lewy body disease (LBD), but their relationship has not been comparatively evaluated.Methods:In this cross-sectional study, we recruited 28 normal controls (NC), 55 patients with AD mild cognitive impairment (MCI), 34 patients with AD dementia, 28 patients with LBD MCI, and 51 patients with LBD dementia. The subjects underwent cognitive evaluation, brain magnetic resonance imaging to measure the basal forebrain (BF) volume and global cortical thickness (CTh), and 18F-Florbetaben (FBB) positron emission tomography to measure the standardized uptake value ratio (SUVR). Using general linear models and path analyses, the association of FBB-SUVR and BF volume with the CTh and/or cognitive dysfunction were evaluated in AD spectrum (AD and NC) and LBD spectrum (LBD and NC), respectively. Covariates included age, sex, education, deep and periventricular white matter hyperintensities, intracranial volume, hypertension, diabetes mellitus, and hyperlipidemia.Results:BF volume mediated the association between FBB-SUVR and CTh both in AD and LBD spectra, while FBB-SUVR was associated with CTh independently of BF volume only in LBD spectrum. Significant correlation between voxel-wise FBB-SUVR and CTh was observed only in LBD group. FBB-SUVR was independently associated with widespread cognitive dysfunction both in AD and LBD spectra, especially in the memory domain [standardized beta (B) for AD spectrum = -0.60, B for LBD spectrum = -0.33]. In AD spectrum, BF volume was associated with memory dysfunction (B = 0.18), and CTh was associated with language (B = 0.21) and executive (B = 0.23) dysfunction. In LBD spectrum, however, BF volume and CTh were independently associated with widespread cognitive dysfunction.Conclusions:There is a common β-amyloid-related degenerative mechanism with or without the mediation of BF in AD and LBD spectra, while the association of BF atrophy with cognitive dysfunction is more profound and there is localized β-amyloid-cortical atrophy interaction in LBD spectrum.

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