Geevarughese Sarah, Parikh Rajul
Clinical, Biomedical, and Educational Research, Edward Via College of Osteopathic Medicine, Spartanburg, USA.
Neurology, HCA Florida Memorial Hospital, Jacksonville, USA.
Cureus. 2024 Aug 8;16(8):e66447. doi: 10.7759/cureus.66447. eCollection 2024 Aug.
Frontotemporal dementia (FTD) is among the most common forms of dementia, with an average symptom onset in the fifth decade of life. Neuropathologic changes in FTD demonstrate degeneration in the frontal and/or temporal lobes, which is defined as frontotemporal lobar degeneration (FTLD). FTD is categorized into a subset of variants by symptomatic presentation and corresponding clinical workup. Primary progressive aphasia (PPA) is among these variants of FTD and is distinguished by its primary clinical presentation of language impairment with correlating neuropathology in the aforementioned areas of the brain. More specifically, the classification of PPA is further subdivided into three clinical variants, which has allowed for appropriate diagnostic and prognostic considerations within this patient population. Among these variants in PPA are the semantic (svPPA), non-fluent (navPPA), and logopenic (lvPPA) forms. Motor neuron disease (MND) is a progressive and irreversible process of neuronal degeneration that can lead to an upper motor neuron, a lower motor neuron, or a combination of these two symptomologies. FTD and its association with MND is a well-established spectrum, although more rarely among the PPA variant of FTD. Comparatively, there is a significant body of clinical knowledge on the association between the behavioral variant of FTD (bvFTD) and MND. This is the case of a 69-year-old female with navPPA who later presented with clinical symptoms of MND. Although the two clinical diagnoses, PPA and MND, are irreversible and progressive, this case serves to elucidate diagnostic and prognostic considerations in this rare patient population.
额颞叶痴呆（FTD）是最常见的痴呆形式之一，平均症状发作于生命的第五个十年。FTD的神经病理学变化表现为额叶和/或颞叶的退化，这被定义为额颞叶变性（FTLD）。FTD根据症状表现和相应的临床检查被分类为不同的变体子集。原发性进行性失语（PPA）是FTD的这些变体之一，其主要临床表现为语言障碍，并在大脑上述区域伴有相关的神经病理学改变。更具体地说，PPA的分类进一步细分为三种临床变体，这使得能够对该患者群体进行适当的诊断和预后考虑。PPA的这些变体包括语义性（svPPA）、非流利性（navPPA）和音韵性（lvPPA）形式。运动神经元病（MND）是一种神经元变性的进行性且不可逆的过程，可导致上运动神经元、下运动神经元或这两种症状的组合。FTD及其与MND的关联是一个已被充分证实的谱系，尽管在FTD的PPA变体中较为罕见。相比之下，关于FTD行为变体（bvFTD）与MND之间的关联有大量的临床知识。本文报道了一例69岁患有navPPA的女性患者，该患者后来出现了MND的临床症状。尽管PPA和MND这两种临床诊断都是不可逆且进行性的，但该病例有助于阐明这一罕见患者群体的诊断和预后考虑因素。
