Amyotrophic lateral sclerosis with dementia: case report

Abstract

-A patient is described in whom a profound and rapidly progressive dementia occurred in association with clinical features of amyotrophic lateral sclerosis. A magnetic resonance imaging showed signs of frontal and especially left temporal atrophy. The pattern of dementia indicated impaired frontotemporal lobe functions, evidenced by reduced tracer uptake in the frontotemporal lobes on brain single photon emission computed tomography. Neuropathological examination in this patient revealed mild frontotemporal atrophy with spongiform changes and neuronal loss affecting mainly layers II and III of the frontotemporal cortices. There was atrophy of the hypoglossal nuclei. The spinal cord changes were consistent with motor neuron disease. The patient showed an irreversible and progressive course. A review of the relevant literature was made.KEY WORDS: dementia, amyotrophic lateral sclerosis, hypothyroidism, brain SPECT.Esclerose lateral amiotrófica com demência: relato de caso RESUMO -Demência de evolução rápida e progressiva associada com esclerose lateral amiotrófica ocorreu em uma paciente de 68 anos. A ressonância magnética mostrou sinais de atrofia frontal e, principalmente, temporal bilateral mais acentuada à esquerda. A demência se caracterizou como de tipo fronto-temporal, como sugere por hipoperfusão moderada nos lobos fronto-temporais através da tomografia cerebral computadorizada por emissão de fóton simples. O exame neuropatológico revelou atrofia leve fronto-temporal com alterações esponjiformes e perda neuronal afetando principalmente as camadas II e III dos córtices fronto-temporais. Havia importante perda de neurônios em ambos os núcleos do hipoglosso. A medula espinhal mostrou alterações consistentes com doença do neurônio motor. O caso teve curso de quatro anos até o óbito. PALAVRAS-CHAVE: demência, esclerose lateral amiotrófica, hipotireoidismo, SPECT cerebral.There has been considerable progress in the understanding of unusual dementias, often referred to as non-Alzheimer or rare dementias. These developments fall into three major areas: definition of clinicopathological entities, investigations into the cellular and molecular mechanisms, and genetic discoveries. Some of the unusual dementias have been previously considered to be subcortical, but now the widespread involvement of the cerebral cortex has been recognized. Most unusual dementia are associated with frontotemporal degeneration including Pick's disease, motor neuron disease (MND), corticobasal degeneration, frontal lobe degeneration of non-Alzheimer type, and the more recently defined frontotemporal degeneration with parkinsonism linked to chromosome-17. In MND, dementia is associated with ubiquitin-positive inclusions in the cortex and in the hippocampal formation. More recently it was recognized that neuronal loss affects not only the motor, but also non-motor cortical areas 1 .

Noninvasive optical estimation of CSF thickness for brain-atrophy monitoring

“…With these new human-head structures we do not have the ambition to reproduce an exact model of the AD development: they offer the opportunity to predict a general trend of realistic tr-NIR measurements, keeping an eye at the nature of the BAP. In fact, our erosion method preserves the complex structure of the brain surface, with grooves and ridges evolving similarly to the neurodegeneration in a real individual affected by dementia [ 2 , 25 , 33 ]. Of course, further details could have been taken into account, such as the modelling of the ventricle size variation, but at the first step of our work there is already a broad spectrum of useful information arising from this study.…”

“…Unaffected by atrophy, the temporal lobe is distant a few mm in agreement with what we measured in our model at Stage 0. An extreme example, such as the case reported by DeBrito-Marques [ 33 ], shows a patient having amyotrophic lateral sclerosis with dementia ( Fig. 2 ), exhibiting an impressive temporal lobe atrophy.…”

Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis

“…Atrophy of the precentral gyrus may be detected in PLS and ALS with significant UMN involvement, but generally requires quantitative morphometric analysis and is of limited clinical utility [178]. Frontotemporal atrophy may be seen on proton density MRI in patients with ALS or PLS and FTD, especially in advanced disease (Figure 14.4) [179][180][181][182][183].…”

Frontoparietal cortical atrophy with gliosis in the gray matter of cerebral cortex: case report

“…The most common of these disorders is AD 3 . However, there are several presenile frontotemporal dementia types, which are much less common than AD, like amyotrofic lateral sclerosis (ALS) 4 , classic Pick's disease (PD) 5 , Creutzfeld-Jakob disease and the Heidenhain variant 1 , prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17 6 , presenile dementia with diffuse calcified neurofibrillary tangles 7 , dementia with ALS features and diffuse Pick body-like inclusions 8 , and non-specific familial presenile dementia 9 . Most of these diseases are characterized by an insidious but invariably progressive deterioration of mental and physical capacities, culminating on a purely vegetative existence in which all patients need must be cared for 1 .…”