Alzheimer and vascular brain disease: Senile dementia / Alzheimer e doença vascular cerebral: Demência senil

Alois Alzheimer is best known for his description of a novel disease, subsequently named after him. However, his wide range of interests also included vascular brain diseases. He described Senile dementia, a highly heterogeneous condition, and was able not only to distinguish it from syphilitic brain disease, but also to discriminate two clinicopathological subtypes, that may be labeled a "arteriosclerotic subtype", comparable to the present clinicopathological continuum of "Vascular cognitive impairment", and another as a "neurodegenerative subtype", characterized by primary [cortical] ganglion cell [nerve cells] degeneration, possibly foreshadowing a peculiar presenile disease that he was to describe some years later and would carry his name. He also considered the possibility of a senile presentation of this disease subtype, which was described by Oskar Fischer a short time later. Considering the clinicopathological overlapping features of the "arteriosclerotic subtype" of Senile dementia with Arteriosclerotic atrophy of the brain, it might be possible to consider that both represent a single condition.

Alois Alzheimer é conhecido principalmente por sua descrição de uma nova doença, logo designado com seu nome. Entretanto, sua ampla gama de interesses, compreendia também doenças vasculares cerebrais. Descreveu a Demência senil, uma condição muito heterogênea, e foi capaz, não somente distingui-la da doença cerebral sifilítica, como também separar dois subtipos clinicopatológicos que podem ser rotulados como um "subtipo arteriosclerótico", que pode ser relacionado ao atual continuum clinicopatológico do "Comprometimento cognitivo vascular", e um "subtipo neurodegenerativo", caracterizado por degeneração [cortical] primária de células ganglionares [células nervosas], possivelmente prenunciando uma doença peculiar pré-senil, que ele descreveu alguns anos depois e que portaria seu nome. Considerou a possibilidade de uma apresentação senil deste subtipo da doença, que seria descrita por Oskar Fischer pouco tempo depois. Levando em conta os aspectos de sobreposição clinicopatológica do "subtipo arteriosclerótico" da Demência senil com a Atrofia arteriosclerótica do cérebro, poderia ser possível considerar que ambas representariam uma só condição.

Alois Alzheimer and vascular brain disease: arteriosclerotic atrophy of the brain / Alois Alzheimer e doença vascular cerebral: atrofia arteriosclerótica do cérebro

Alois Alzheimer is best known for his description of neurofibrillary changes in brain neurons of a demented patient, identifying a novel disease, soon named after him by Kraepelin. However, the range of his studies was broad, including vascular brain diseases, published between 1894 and 1902. Alzheimer described the clinical picture of Arteriosclerotic atrophy of the brain, differentiating it from other similar disorders. He stated that autopsy allowed pathological distinction between arteriosclerosis and syphilis, thereby achieving some of his objectives of segregating disorders and separating them from syphilis. His studies contributed greatly to establishing the key information on vascular brain diseases, predating the present state of knowledge on the issue, while providing early descriptions of what would be later regarded as the dimensional presentation of the now called ?Vascular cognitive impairment?, constituted by a spectrum that includes a stage of ?Vascular cognitive impairment not dementia? and another of ?Vascular dementia?.

Alois Alzheimer é conhecido principalmente pela sua descrição de alterações neurofibrilares em neurônios cerebrais de uma paciente demenciada, identificando uma nova doença logo denominada com seu nome por Kraepelin. Entretanto, o âmbito de seus estudos foi amplo, incluindo doenças vasculares cerebrais, publicados entre 1894 e 1902. Alzheimer descreveu o quadro clínico da Atrofia arteriosclerótica do cérebro, diferenciando-a de outros transtornos semelhantes. Ele afirmou que a autópsia permitia distinguir patologicamente entre arteriosclerose e sífilis, alcançando alguns de seus objetivos, segregar transtornos e separá-los da sífilis. Seus estudos contribuíram fortemente para estabelecer as informações-chave sobre doença vascular cerebral, que antecederam o estado do conhecimento atual sobre o assunto, e antecipando o que mais tarde seriavisto como a apresentação dimensional do atualmente denominado ?Comprometimento cognitivo vascular?, constituído por um espectro que inclui um estágio de ?Comprometimento cognitivo vascular não demência? e outro, a ?Demência vascular?.

Prevalence of dementia subtypes in a developing country: a clinicopathological study

OBJECTIVES: To assess the distribution of dementia subtypes in Brazil using a population-based clinicopathological study. METHOD: Brains from deceased individuals aged ≥50 years old were collected after the next of kin signed an informed consent form and provided information through standardized questionnaires. Post-mortem clinical diagnoses were established in consensus meetings, and only cases with moderate or severe dementia or without cognitive impairment were included in the analysis. Immunohistochemical neuropathological examinations were performed following the universally accepted guidelines. A diagnosis of Alzheimer's disease was made when there were at least both a moderate density of neuritic plaques (Consortium to Establish a Register for Alzheimer's disease B or C) and Braak stage III for neurofibrillary tangle distribution. For the diagnosis of vascular dementia, at least three zones or strategic areas had to be affected by infarcts, lacunae, or microinfarcts. RESULTS: From 1,291 subjects, 113 cases were classified as having moderate or severe dementia, and 972 cases were free of cognitive impairment. The neuropathological diagnoses of the dementia sub-group were Alzheimer's disease (35.4%), vascular dementia (21.2%), Alzheimer's disease plus vascular dementia (13.3%), and other causes of dementia (30.1%). Small-vessel disease, which alone was not considered sufficient for a vascular dementia diagnosis, was present in 38.9% of all of the dementia cases and in 16.8% of the group without cognitive impairment (odds ratio = 2.91; 95% confidence interval, 1.53-5.51), adjusted for age, sex, and education. CONCLUSIONS: The relatively high frequencies of vascular dementia and small-vessel disease in the dementia sub-group constitute relevant findings for public health initiatives because control of vascular risk factors could decrease the prevalence of dementia in developing countries. .

Non-inflammatory cerebral amyloid angiopathy as a cause of rapidly progressive dementia: a case study / Angiopatia amilóide cerebral não inflamatória como causa de demência rapidamente progressiva: estudo de caso

Abstract ? A 77 year-old men developed a subacute-onset, rapidly progressive cognitive decline. After 6 months of evolution, he scored 6 on the Mini-Mental State Examination and had left hemiparesis and hemineglect. The patient died 11 months after the onset of cognitive symptoms. Brain MRI showed microhemorrhages on gradient-echo sequence and confluent areas of white matter hyperintensities on T2-weighted images. Brain biopsy revealed amyloid-? peptide deposition in vessel walls, some of them surrounded by micro-bleeds. In this case report, we discuss the role of cerebral amyloid angiopathy (CAA) in cognitive decline, due to structural lesions associated with hemorrhages and infarcts, white matter lesions and co-morbidity of Alzheimer?s disease, as well as the most recently described amyloid angiopathy-related inflammation.

Resumo ? Um homem de setenta e sete anos desenvolveu quadro de início subagudo de demência rapidamente progressiva. Após seis meses de evolução, sua pontuação do Mini exame do estado mental foi de 6, e no exame neurológico apresentava heminegligência e hemiparesia esquerda. O paciente faleceu 11 meses após início dos sintomas. RM do encéfalo mostrou microhemorragias em sequência gradiente-echo e áreas confluentes de hiperintensidade de substância branca nas imagens pesadas em T2. Na biópsia cerebral observou-se deposição de peptídeo ?-amilóide em vasos sanguíneos, alguns rodeados por microhemorragias. Neste relato de caso, discutimos o papel da angiopatia amiloide cerebral no declínio cognitivo, devido a lesões estruturais associadas a hemorragias e infartos, lesões de substância branca e comorbidade com doença de Alzheimer, assim como a mais recentemente descrita inflamação relacionada a angiopatia amiloide.