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. 2020 Feb 14;7(3):267-272.
doi: 10.1002/mdc3.12900. eCollection 2020 Apr.

Pathology-Proven Corticobasal Degeneration Presenting as Richardson's Syndrome

Ece Bayram  1 , Dennis W Dickson  2 , Stephen G Reich  3 , Irene Litvan  1
Affiliations

Pathology-Proven Corticobasal Degeneration Presenting as Richardson's Syndrome

Ece Bayram et al. Mov Disord Clin Pract. .

Abstract

Background: Corticobasal degeneration (CBD) can present with various clinical phenotypes including Richardson's syndrome (RS). Although neuropathological examination can differentiate CBD and progressive supranuclear palsy (PSP) pathologies, no clinical or imaging findings can differentiate CBD from other pathologies when a patient presents with a variant type of CBD. As these various phenotypes are associated with non-CBD pathologies, clinical diagnostic accuracy can be low for such patients.

Objectives: To present clinical features of two cases with symptom progression in line with PSP-RS, who were diagnosed with CBD based on neuropathological examination.

Methods: Baseline, follow up examinations, and detailed neuropathological examinations of two CBD cases presenting and progressing in line with probable PSP-RS are demonstrated.

Results: The two cases clinically diagnosed as probable PSP-RS were shown to have CBD upon neuropathological examination, which is the gold standard for diagnosis of both PSP and CBD.

Conclusions: These cases emphasize the importance of neuropathology for the definite diagnosis, and stress the need for distinctive markers to increase the reliability of clinical diagnosis before death.

Keywords: corticobasal degeneration; differential diagnosis; neuropathology; phenotype; progressive supranuclear palsy.

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Figures

Figure 1
Figure 1
Case 1. (A) Gross examination of the brain shows thinning of the corpus callosum and enlarged frontal horn of lateral ventricle (*). Inset, lower left: Diencephalon with normal appearing STN (arrow); Inset, lower right: Midbrain with severe neuromelanin loss in SN, (B) Ballooned neuron (arrow) in superior frontal gyrus (H&E), (C) Astrocytic plaque (*) in superior frontal gyrus (phospho‐tau), (D) Striatal pretangles, pleomorphic tangles and astrocytic plaques (phospho‐tau), (E) Pleomorphic tangles and threads in STN (phospho‐tau), (F) Corticobasal bodies (arrow) in SN (H&E), (G) Corticobasal bodies (arrow) in SN (phospho‐tau). Case 2. (H) Gross examination of the brain shows enlarged frontal horn of lateral ventricle (*). Inset, lower left: Diencephalon with normal appearing STN (arrows); Inset, lower right: Midbrain with severe neuromelanin loss and increased in rust (hemosiderin) coloration of SN, (I) Pyknotic ballooned neuron (arrow) in superior frontal gyrus (H&E), (J) Astrocytic plaque (*) and pretangle in superior frontal gyrus (phospho‐tau), (K) Striatal astrocytic plaques(phospho‐tau), (L) Pleomorphic tangles, threads and coiled bodies in STN (phospho‐tau), (M) Corticobasal bodies (arrow) in SN (H&E), (N) Corticobasal bodies (arrow) in SN (phospho‐tau).
See this image and copyright information in PMC

References

    1. Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 2013;80:496–503. - PMC - PubMed
    1. Lee SE, Rabinovici GD, Mayo MC, et al. Clinicopathological correlations in corticobasal degeneration. Ann Neurol 2011;70:327–340. - PMC - PubMed
    1. Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord 2017;32:853–864. - PMC - PubMed
    1. Dickson DW, Bergeron C, Chin SS, et al. Office of Rare Diseases Neuropathologic Criteria for Corticobasal Degeneration. J Neuropathol Exp Neurol 2002;61:935–946. - PubMed
    1. Komori T, Arai N, Oda M, et al. Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 1998;96:401–408. - PubMed