Diffuse lewy body disease presenting as corticobasal syndrome and progressive supranuclear palsy syndrome

2013 ◽  
Vol 28 (8) ◽  
pp. 1153-1155 ◽  
Author(s):  
Aaron Haug ◽  
Philip Boyer ◽  
Benzi Kluger
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Lewy Body ◽  
Lewy Body Disease ◽  
Corticobasal Syndrome ◽  
Diffuse Lewy Body Disease

scholarly journals Diffuse Lewy body disease manifesting as corticobasal syndrome

Neurology ◽  
2018 ◽  
Vol 91 (3) ◽  
pp. e268-e279 ◽  
Author(s):  
Koji Kasanuki ◽  
Keith A. Josephs ◽  
Tanis J. Ferman ◽  
Melissa E. Murray ◽  
Shunsuke Koga ◽  
...  
Keyword(s):  
Motor Cortex ◽  
Alzheimer Disease ◽  
Progressive Supranuclear Palsy ◽  
Lewy Body ◽  
Disease Onset ◽  
Lewy Bodies ◽  
Lewy Body Disease ◽  
Corticobasal Syndrome ◽  
Imaging Biomarkers ◽  
Diffuse Lewy Body Disease

ObjectiveTo describe clinical and pathologic characteristics of diffuse Lewy body disease (DLBD) manifesting as corticobasal syndrome (CBS).MethodsIn 523 autopsy-confirmed cases of DLBD, we identified 11 patients diagnosed with CBS. For comparison, we studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). Given previous studies suggesting the importance of pathology in peri-Rolandic cortices in CBS, we used digital pathology to count Lewy bodies and to quantify intracytoplasmic and neuritic α-synuclein and phospho-tau burden in the motor cortex.ResultsDLBD patients with antemortem features of CBS were significantly younger at disease onset and less likely to have REM sleep behavior disorder than DLBD cases who met clinical criteria for DLB during life. Patients with DLBD manifesting as CBS had more Lewy bodies in the motor cortex than DLBD manifesting as clinically probable DLB. Three cases had concomitant progressive supranuclear palsy and 4 cases had concomitant Alzheimer disease as probable correlates of CBS.ConclusionThe neuropathology underlying CBS is heterogeneous, including corticobasal degeneration, Alzheimer disease, and progressive supranuclear palsy. This study suggests that atypical variants of Lewy body disease with severe peri-Rolandic Lewy-related pathology can present clinically as CBS. Patients with DLBD who present as CBS tend to have an earlier age at onset and are less likely to have clinical features of DLB, such as dream enactment behavior during sleep, visual hallucinations, and levodopa-responsive parkinsonism. Future studies with biofluid or molecular imaging biomarkers for α-synuclein will permit better recognition of this uncommon pathologic substrate of CBS.

scholarly journals Differential Diagnosis of Parkinsonism

1999 ◽  
Vol 26 (S2) ◽  
pp. S1-S4 ◽  
Author(s):  
A. Jon Stoessl ◽  
Jean Rivest
Keyword(s):  
Differential Diagnosis ◽  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Essential Tremor ◽  
Clinical Features ◽  
Lewy Body ◽  
Lewy Body Disease ◽  
Corticobasal Degeneration ◽  
Diffuse Lewy Body Disease ◽  
Degenerative Disorders

The diagnosis of Parkinson's disease is predominantly clinical, based on a combination of the cardinal features of tremor, bradykinesia and rigidity. The differential essentially lies between other conditions resulting in tremor, of which essential tremor is the commonest, and other akinetic-rigid syndromes. These include progressive supranuclear palsy, multiple system atrophy, toxins and other degenerative disorders, including diffuse Lewy body disease and corticobasal degeneration. The key clinical features of these disorders and a practical diagnostic approach are briefly reviewed in this article.

An autopsy case of corticobasal syndrome with pure diffuse Lewy Body Disease

Neurocase ◽  
2021 ◽  
pp. 1-7
Author(s):  
Keiko Ichinose ◽  
Mutsufusa Watanabe ◽  
Saneyuki Mizutani ◽  
Toru Tanizawa ◽  
Toshiki Uchihara ◽  
...  
Keyword(s):  
Lewy Body ◽  
Lewy Body Disease ◽  
Corticobasal Syndrome ◽  
Autopsy Case ◽  
Diffuse Lewy Body Disease

Reader response: Diffuse Lewy body disease manifesting as corticobasal syndrome: A rare form of Lewy body disease

Neurology ◽  
2019 ◽  
Vol 93 (9) ◽  
pp. 411.2-412
Author(s):  
Rossella Infante ◽  
Alex Incensi ◽  
Giovanni Rizzo ◽  
Vincenzo Donadio ◽  
Rocco Liguori
Keyword(s):  
Reader Response ◽  
Lewy Body ◽  
Lewy Body Disease ◽  
Corticobasal Syndrome ◽  
Rare Form ◽  
Diffuse Lewy Body Disease

I716F APP mutation associated with Alzheimer's and diffuse Lewy body disease

2013 ◽  
Vol 333 ◽  
pp. e335
Author(s):  
E. Sieczkowski ◽  
I. Milenkovic ◽  
R. Giera ◽  
R. Höftberger ◽  
T. Ströbel ◽  
...  
Keyword(s):  
Lewy Body ◽  
Lewy Body Disease ◽  
Diffuse Lewy Body Disease

Clinical features of diffuse Lewy body disease in the elderly: analysis of 12 cases

1996 ◽  
pp. 153-160 ◽  
Author(s):  
S. Kuzuhara ◽  
M. Yoshimura ◽  
T. Mizutani ◽  
H. Yamanouchi ◽  
Y. Ihara
Keyword(s):  
Clinical Features ◽  
Lewy Body ◽  
Lewy Body Disease ◽  
The Elderly ◽  
Diffuse Lewy Body Disease
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