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Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Neurosci+Biobehav+Rev 2017 ; 75 (ä): 378-92 Nephropedia Template TP
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The Neuropathological Signature of Bulbar-onset ALS: A Systematic Review #MMPMID28163193
Shellikeri S; Karthikeyan V; Martino R; Black S; Zinman L; Keith J; Yunusova Y
Neurosci Biobehav Rev 2017[Apr]; 75 (ä): 378-92 PMID28163193show ga
ALS is a multisystem disorder affecting cognitive and motor functions. Bulbar-onset ALS (bALS) may be preferentially associated with language/cognitive impairments, compared with spinal-onset ALS (sALS), stemming from a potentially unique neuropathology. The objective of this systematic review was to compare neuropathology reported for bALS and sALS subtypes in studies of cadaveric brains. Using Cochrane guidelines, we reviewed articles in MEDLINE, Embase, and PsycINFO databases using standardized search terms for ALS and neuropathology, from inception until July 16th 2016. 17 studies were accepted. In summary, both subtypes presented with involvement in motor and frontotemporal cortices, deep cortical structures, and cerebellum, characterized by neuronal loss, spongiosis, myelin pallor, and ubiquitin+ and TDP43+ inclusion bodies. Changes in Broca and Wernicke areas, regions associated with speech and language processing, were noted exclusively in bALS. Further, some bALS cases presented with atypical pathology, neurofibrillary tangles and basophilic inclusions, which were not found in any sALS cases. Given the few studies, all with methodological biases, further work is required to better understand neuropathology of ALS subtypes.