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Background and ObjectivesPrimary progressive aphasia (PPA) is a neurodegenerative condition that predominantly impairs language. Most investigations of how focal atrophy affects language consider 1 time point compared with healthy controls. However, true atrophy quantification requires comparing individual brains over time. In this observational cohort study, we identified areas where focal atrophy was associated with contemporaneous decline in naming in the same individuals.MethodsCross-sectional analyses–related Boston Naming Test (BNT) performance and volume in 22 regions of interests (ROIs) at each time point using Least Absolute Shrinkage and Selection Operator (LASSO) regression. Longitudinal analysis evaluated changes in BNT performance and change in volume in the same ROIs.ResultsParticipants (N = 62; 50% female; mean age = 66.8 ± 7.4 years) with PPA completed the BNT and MRI twice (mean = 343.9 ± 209.0 days apart). In cross-sectional left inferior frontal gyrus pars opercularis, superior temporal pole, middle temporal gyrus, and inferior temporal gyrus were identified as critical for naming at all time points. Longitudinal analysis revealed that increasing atrophy in the left supramarginal gyrus and middle temporal pole predicted greater naming decline, as did female sex and longer intervals between time points.DiscussionAlthough cross-sectional analyses identified classic language areas that were consistently related to poor performance at multiple time points, it was not increasing atrophy in these areas that lead to further decline: longitudinal analysis of each person's atrophy over time instead identified nearby but distinct regions where increased atrophy was related to decreasing performance. The results demonstrate that directly examining atrophy (in each individual) over time furthers understanding of decline in PPA and reveal the importance of left supramarginal gyrus and middle temporal pole in maintaining naming when areas normally critical for language degenerate. The novel results provide insight into how the underlying disease progresses to result in the clinical decline in naming, the deficit most common among all 3 PPA variants.

Background and Objectives:It is widely agreed that primary progressive aphasia (PPA) is a clinical syndrome with at least three distinct variants that differ in phenotype, areas of atrophy, and most common underlying neurodegenerative disease. The distinction between logopenic variant PPA and other variants is important for prognosis and medical management. However, differentiating logopenic from nonfluent agrammatic variant can be difficult. We aimed to identify a novel behavioral assessment that (1) distinguishes logopenic from the other variants with a high degree of accuracy and (2) correlates with left superior temporal-inferior parietal atrophy (previously shown to be associated with this variant). Location of atrophy was measured using a novel, clinically useful imaging analysis.Methods:In this observational cohort study of 227 individuals with PPA, participants were administered sentence reading and repetition subtests from a standard battery. A subset of 41 participants were administered enhanced reading and repetition subtests with 5 longer sentences, of which 13 had brain imaging. Ratios of word-level and sentence-level accuracy in reading:repetition were calculated. We used one-way ANOVA to determine if either or both ratios of reading:repetition independently discriminated between variants and t-tests to determine if the ratios distinguished between nonfluent and logopenic variants. We identified Receiver Operating Characteristics and Pearson correlations between the reading:repetition ratios and ratio of left:right superior temporal-inferior parietal volume.Results:Sentence reading:repetition ratio using the new stimuli significantly differed across the three variants (pDiscussion:Results provide an efficient and reliable clinical assessment, and a novel imaging analysis, to distinguish the clinical syndrome of logopenic variant from other variants of PPA. Results also support the proposal that logopenic variant PPA reflects a defect in phonological short-term memory caused by atrophy in superior temporal-inferior parietal cortex.Classification of Evidence:This study provides Class III evidence that the sentence reading:repetition ratio distinguished logopenic PPA from other PPA variants.

We analyzed computerized tomography (CT) findings in six patients with Fahr's syndrome. They presented calcifications in basal ganglia, dentate nucleus, subcortical region and semioval center, due to alteration in calcium metabolism or due to senile relative hypoxemic state. The image pattern was not strictly related with etiology, although some differences in dystrophic senile calcifications (the only one present in semioval center and absent in subcortical region). CT is an easy exam, has maximum sensitivity and allows diagnosis, contributing to early treatment of many etiologies of Fahr's syndrome. Analisamos os achados de tomografia computadorizada (TC) de seis pacientes com síndrome de Fahr. Eles apresentaram calcificações nos gânglios da base, núcleo denteado, região subcortical e centro semi-oval, devidas a distúrbios no metabolismo do cálcio ou a estado de hipóxia relativa, por senilidade. O padrão de imagem não apresenta relação clara com a etiologia, apesar de algumas diferenças no caso das calcificações distróficas senis (as únicas presentes nos centros semi-ovais e ausentes na região subcortical). TC é um exame de fácil realização, máxima sensibilidade e permite o diagnóstico, contribuindo para o tratamento precoce de muitas das etiologias da síndrome de Fahr.

To advance our understanding about the emotional and cognitive deficits of patients with frontotemporal dementia with behavioral variant (bvFTD), the current study examined comprehension and expression of emotions from prosodic and facial cues in a 66-year-old woman. The patient diagnosed with bvFTD is compared to six patients with acute right hemisphere stroke. Recognition of emotion from prosodic cues was assessed using an identification task in four conditions with decreasing verbal demands (neutral sentences, language-like pseudo sentences, monosyllables, and asyllabic vowel sounds). Repetition of utterances with emotional connotations and self-generated conversations were analyzed to measure relative changes in mean fundamental frequency (f0), f0 variance, speech rate, and intensity along with the facial musculature pattern. The patient showed a marked deficit in identifying emotions in all four prosody conditions; and she did not show much variation in modulating mean f0, f0 variance, speech rate and intensity for all emotion categories when compared to neutral utterances. In addition, this patient demonstrated little to no facial expressions during emotionally provoking tasks, but demonstrated no difficulty recognizing emotions from facial expressions or verbal scenarios. Results show that the patient seems to have selective impairment in recognition of emotions from prosody and expression of emotions using both prosodic and facial features. Impaired processing of emotional prosody and facial expressions could be important for detecting bvFTD with greater right hemisphere atrophy.

Cerebellar glioblastoma multiforme (GBM) is a rare tumor. This is the third case published in Brazilian literature and, the last one has been described more than 15 years ago. The aggressive behavior of GBM prompts for fast treatment, which can be hampered by the fact that the diagnosis of GBM requires a high degree of suspicion. We describe a case of GBM in a 46 years old man. In conjunction, we present a literature review including particular issues, clinical data, advances in imaging studies, pathological characteristics, treatment options and the behavior of such malignant tumor. Glioblastoma multiforme (GBM) cerebelar é um tumor raro. Apresentamos o terceiro caso revisado em literatura neurológica brasileira, sendo o último descrito há mais de 15 anos. Devido a sua agressividade, o tratamento deve ser instituído rapidamente, porém certa indefinição quanto à conduta pode ocorrer, pois o diagnóstico de GBM pode não ser lembrado. Apresentamos um caso de GBM cerebelar em um homem de 46 anos. Também fazemos uma revisão a respeito de seu comportamento, quadro clínico e avanços quanto à investigação por imagem, aspectos histopatológicos, formas de tratamento e suas características peculiares.