Corticobasal syndrome: A case of discordance between the clinical findings and the imaging biomarkers

Author(s):  
E. Carrillo-Villamizar ◽  
A. Cardozo-Saavedra ◽  
C. Lorenzo-Bosquet ◽  
S. Lucas del Pozo ◽  
E. Martínez-Sáez ◽  
...  
Neurology ◽  
2018 ◽  
Vol 91 (3) ◽  
pp. e268-e279 ◽  
Author(s):  
Koji Kasanuki ◽  
Keith A. Josephs ◽  
Tanis J. Ferman ◽  
Melissa E. Murray ◽  
Shunsuke Koga ◽  
...  

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.


2021 ◽  
Vol 8 (3) ◽  
pp. 491-492
Author(s):  
Jacy Bezerra Parmera ◽  
Sonia Maria Dozzi Brucki ◽  
Artur Martins Coutinho ◽  
Ricardo Nitrini

2021 ◽  
Author(s):  
Jacy Parmera ◽  
Artur Coutinho ◽  
Isabel Almeida ◽  
Camila Carneiro ◽  
Carla Ono ◽  
...  

Background: Corticobasal syndrome (CBS) is neurodegenerative disorder related to multiple underlying pathologies. Objective: To investigated if dividual FDG-PET patterns could distinguish CBS due to Alzheimer’s disease (AD) from other pathologies based on [11C]Pittsburgh Compound-B (PIB)-PET. Methods: Forty-five patients with probable CBS were prospectively evaluated. They underwent FDG-PET and were divided into groups: related to AD (CBS FDG-AD) or non-AD (CBS FDG-nonAD). Thirty patients underwent PIB-PET on a PET-MRI to assess their amyloid status. FDG and PIB-PET were classified individually on visual analysis, and PET-MRI quantitative group analyses were performed. Results: CBS FDG-AD group (33.3%) showed worse cognitive performances, displayed more myoclonus and hallucinations. CBS FDG-nonAD group (66.7%) presented more dystonia, ocular motor dysfunction, perseveration, and dysarthria. All CBS FDG-AD patients tested positive at PIB-PET compared to 3 out of 20 in the non-AD group. The individual FDG-PET classification had 76.92% of sensitivity, 100% of specificity and 88.5% of accuracy to detect positive PIB-PET scans. Individuals with positive and negative PIB-PET showed hypometabolism in temporoparietal areas and in thalamus and brainstem, respectively, disclosing metabolic signatures. Conclusion: CBS is mainly distinguished by two variants (AD and non-AD), with different cognitive profiles and possibly motor features. FDG-PET was useful depicting their specific degeneration patterns and brain amyloid deposition.


1965 ◽  
Vol 30 (4) ◽  
pp. 325-335
Author(s):  
George E. Lynn ◽  
Jack A. Willeford
Keyword(s):  

2001 ◽  
Vol 6 (1) ◽  
pp. 1-3
Author(s):  
Robert H. Haralson

Abstract The AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), Fifth Edition, was published in November 2000 and contains major changes from its predecessor. In the Fourth Edition, all musculoskeletal evaluation and rating was described in a single chapter. In the Fifth Edition, this information has been divided into three separate chapters: Upper Extremity (13), Lower Extremity (14), and Spine (15). This article discusses changes in the spine chapter. The Models for rating spinal impairment now are called Methods. The AMA Guides, Fifth Edition, has reverted to standard terminology for spinal regions in the Diagnosis-related estimates (DRE) Method, and both it and the Range of Motion (ROM) Method now reference cervical, thoracic, and lumbar. Also, the language requiring the use of the DRE, rather than the ROM Method has been strengthened. The biggest change in the DRE Method is that evaluation should include the treatment results. Unfortunately, the Fourth Edition's philosophy regarding when and how to rate impairment using the DRE Model led to a number of problems, including the same rating of all patients with radiculopathy despite some true differences in outcomes. The term differentiator was abandoned and replaced with clinical findings. Significant changes were made in evaluation of patients with spinal cord injuries, and evaluators should become familiar with these and other changes in the Fifth Edition.


1974 ◽  
Vol 110 (3) ◽  
pp. 382-388 ◽  
Author(s):  
H. M. Doeglas

2007 ◽  
Vol 177 (4S) ◽  
pp. 486-487
Author(s):  
Paholo G. Barboglio ◽  
Brian Cohen ◽  
Angelo E. Gousse
Keyword(s):  

2011 ◽  
Vol 81 (5) ◽  
pp. 328-334 ◽  
Author(s):  
Oya Halicioglu ◽  
Sezin Asik Akman ◽  
Sumer Sutcuoglu ◽  
Berna Atabay ◽  
Meral Turker ◽  
...  

Aim: Nutritional vitamin B12 deficiency in infants may occur because the maternal diet contains inadequate animal products. Clinical presentations of the infants who had nutritional vitamin B12 deficiency were analyzed in this study. Subjects and Methods: Patients with nutritional vitamin B12 deficiency were enrolled in the study between 2003 and 2010. The diagnosis was based on a nutritional history of mothers and infants, clinical findings, hematological evaluation, and low level of serum vitamin B12. Results: Thirty children aged 1 - 21 months constituted the study group. Poverty was the main cause of inadequate consumption of animal products of the mothers. All infants had predominantly breastfed. The most common symptoms were developmental delay, paleness, apathy, lethargy, anorexia, and failure to thrive. Hematological findings were megaloblastic anemia (83.3 %), thrombocytopenia (30 %), and severe anemia (13.3 %). All of the mothers had low serum B12 levels; eight of them had megaloblastic anemia. Conclusion: The unusual clinical manifestations of vitamin B12 deficiency may also be seen apart from neurological and hematological findings. Nutritional vitamin B12 deficiency due to maternal deficiency might be a serious health problem in infants. Therefore, screening and supplementation of pregnant and lactating women to prevent infantile vitamin B12 deficiency should be considered.


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