Alzheimer and vascular brain diseases: Focal and diffuse subforms

History Note Dement Neuropsychol 2015 September;9(3):306-310 Alzheimer and vascular brain diseases Focal and diffuse subforms Eliasz Engelhardt1, Lea T. Grinberg2,3 ABSTRACT. Alois Alzheimer is best known for his description of the pre-senile neurodegenerative disease named after him. However, his previous interest in vascular brain diseases, underlying cognitive and behavioral changes, was very strong. Besides describing the Arteriosclerotic atrophy of the brain and the arteriosclerotic subtype of Senile dementia which he viewed as main forms of vascular brain diseases, he also identified and described a series of conditions he considered subforms. These may be divided, as suggested by the authors of the present paper, into 3 groups: gliosis and sclerosis, subcortical atrophies, and apoplectic. The subforms of the three groups present characteristic neuropathological features and clinical, cognitive and behavioral manifestations. These provide the basis, together with part of the main forms, for the contemporary condition known as Vascular Cognitive Impairment. Key words: Alzheimer, brain vascular disease, arteriosclerosis, vascular subtypes, Vascular Cognitive Impairment. ALZHEIMER E DOENÇA VASCULAR CEREBRAL: SUBFORMAS FOCAIS E DIFUSAS RESUMO. Alois Alzheimer é conhecido principalmente pela descrição de uma doença neurodegenerative pré-senil, que recebeu seu nome. Entretanto, previamente, seu interesse em doenças vasculares cerebrais, subjacentes a desordens cognitivas e comportamentais, foi muito forte. Além de descrever a Atrofia arteriosclerótica do cérebro e o subtipo arteriosclerótico da Demência senil, vistas por ele como formas principais de doenças vasculares cerebrais, ele identificou e descreveu uma série de condições que considerou como subformas. Estas podem ser divididas, como sugestão dos autores do presente artigo, em tres grupos: gliose e esclerose, atrofias subcorticais e apoplética. As subformas dos tres grupos apresentam aspectos neuropatológicas e manifestações clínicas, cognitivas e comportamentais, características. Estas forneceram a fonte, juntamente com parte das formas principais, à condição contemporânea conhecida como Comprometimento Cognitivo Vascular. Palavras-chave: Alzheimer, doença vascular cerebral, arteriosclerose, subtipos vasculares, Comprometimento Cognitivo Vascular. INTRODUCTION A loysius [Alois] Alzheimer (1864-1915), psychiatrist and neuropathologist, became renowned for his description of a new disease that carries his name.1 However, his previous remarkable studies on brain vascular disorders underlying cognitive, behavioral and neurological manifestations, became forgotten. He intensively studied the subject, resulting in important conferences and lectures as well as in a few papers on the theme, published between 1894 and 1902. These studies contributed to establish key knowledge on what has become incorporated into the present status on the subject – the Vascular Cognitive Impairment spectrum.2,3 Besides the two main dementia forms, Arteriosclerotic atrophy of the brain (arteriosklerotische Atrophie des Gehirns) and Senile dementia (senile Demenz), both examined by the authors in previous papers,2,3 Alzheimer identified and described a series of conditions (diseases) related to atheromatous vascular degeneration of the brain arteries which he designated “subforms”. He was Full Professor (retired), Cognitive and Behavioral Neurology Unit - Institute of Neurology / Institute of Psychiatry - Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro RJ, Brazil. 2Assistant Professor, Department of Neurology and Pathology, University of California, San Francisco, San Francisco, USA. 33Professor, PhD, Department of Pathology, School of Medicine, University of Sao Paulo, Sao Paulo SP, Brazil. 1 Eliasz Engelhardt. Avenida N.S. de Copacabana 749/708 – 22050-002 Rio de Janeiro RJ – Brasil. E-mail: Disclosure: The authors report no conflicts of interest. Received June 07, 2015. Accepted in final form August 10, 2015. 306 Alzheimer: vascular brain diseases subforms     Engelhardt E, Grinberg LT Dement Neuropsychol 2015 September;9(3):306-310 able to distinguish these processes according to their location and spread of the brain changes, manifested by cortical and/or subcortical clinical symptoms.4-9 Besides the cases considered as typical subforms, he also mentioned the presence of mixed cases, mainly of vascular nature or more rarely, combined with syphilitic pathology, sometimes hampering the interpretation of a given case.9 The vascular subforms described and named by Alzheimer may be divided, here as proposed by the authors of the present paper, into 3 groups: gliosis and sclerosis, subcortical atrophies, and apoplectic. These subforms may also be regarded as focal and diffuse conditions (Table). Table. Alzheimer and vascular brain diseases (Alzheimer [1894-1902]).4-9 Main forms Arteriosclerotic atrophy of the brain (arteriosklerotische Atrophie des Gehirns) (Alzheimer, 1894, 1902)4,9 Senile dementia (senile Demenz) (Alzheimer, 1898, 1902)7,9 Subforms gliosis and sclerosis group • Perivascular gliosis (perivasculäre Gliose) (Alzheimer, 1896, 1897, 1899, 1902)5,6,8,9 • Perivascular sclerosis (perivasculäre Sklerose) (Alzheimer, 1899)8 • Perivascular gliosis of the cerebral cortex (perivasculäre Gliose der Hirnrinde) (Alzheimer, 1898)7 • Perivascular sclerosis of the cerebral cortex (perivasculäre Sklerose der Hirnrinde) (Alzheimer, 1898)7 • Senile sclerosis of the cerebral cortex (senile Sklerose der Hirnrinde) (Alzheimer, 1899)8 • Senile cortical sclerosis [atrophy] (senile Rindenverödung) (Alzheimer, 1902)9 subcortical atrophy group • Arteriosclerotic brain degeneration (arteriosklerotische Hirndegeneration) (Alzheimer, 1898, 1899)7,8 (described by Alzheimer and Binswanger) • Chronic progressive subcortical encephalitis (Encephalitis subcorticalis chronica progressiva) • (Binswanger, 1894)17 (Alzheimer, 1898, 1902)7,9 • Arteriosclerotic atrophy of the hemispheric white matter (arteriosklerotische Atrophie des Hemisphärenmarks) (Alzheimer, 1899)8 apoplectic group • Apoplectic dementia (Dementia apoplectica) (Alzheimer, 1898)7 [or] • Post-apoplectic dementia (Dementia post apoplexiam) (Alzheimer, 1902)9 Hereunder, the characteristics of these subforms will be considered as condensed excerpts of Alzheimer’s descriptions, relevant for the present approach, extracted and brought together from his several writings, and excluding conditions extraneous to the present scope (Box 1, Box 2, and Box 3). Box 1. The gliosis and sclerosis group, comprising subforms described and named by Alzheimer (condensed excerpts). Perivascular gliosis (perivasculäre Gliose). Characterized by severe arteriosclerosis of brain vessels and marked glial proliferation, with disseminated focal lesions in the cortex and white matter, restricted to one or more gyri, or single brain lobes, and expressed clinically as cortical focal disease (hemia (...truncated)