Clinical, Cognitive and Anatomical Evolution from Nonfluent Progressive Aphasia to Corticobasal Syndrome: A Case Report

Abstract

Recent clinical and pathological studies have suggested that frontotemporal lobar degeneration (FTLD) and corticobasal syndrome (CBS) show clinical and pathological overlap. We present four years of longitudinal clinical, cognitive and anatomical data in the case of a 56-year-old woman, AS, whose clinical picture evolved from FTLD to CBS. For the first three years, AS showed a progressive speech and language disorder compatible with a diagnosis of the nonfluent aphasia variant of FTLD. At year four, 10 years after her first symptom, AS developed the classical clinical signs of CBS, including alien limb phenomenon and dystonia. Voxel-based morphometry (VBM) applied to AS's four annual scans showed progression of atrophy from the inferior posterior frontal gyrus, to the left insula and finally to the medial frontal lobe. This case demonstrates the clinical overlap between FTLD and CBS and shows that the two can appear in the same patient at different stages of the disease in relation to the progression of anatomical damage.

Screening for Cognitive Dysfunction in Corticobasal Syndrome: Utility of Addenbrooke’s Cognitive Examination

“…It is remarkable that patients presenting with an isolated language disorder, PNFA, and those with a syndrome originally conceptualized as a movement disorder sparing cognition, CBS, actually performed at a similar level on the ACE-R, further adding to evidence of overlap between these disorders that share the same range of underlying neuropathologies [6,25]. The most notable difference was on the visuospatial component of the ACE-R.…”

Primary progressive aphasia: conceptual evolution and challenges

“…This deficit is encountered in lv-PPA cases with presumed AD and has been proposed as a putative clinical marker of lv-PPA. 105 Interestingly, this deficit is associated with left superior temporal gyrus atrophy, a region involved in phonological processing that then can potentially be the anatomical signature of lv-PPA. 108 Taken together, this converging clinical and anatomical evidence points to phonologic disintegration as the main neurocognitive system affected in lv-PPA, which could explain the co-occurrence of phonologic errors and the reduced phonologic short-term memory that causes impairment of sentence repetition.…”

“…While some highlight the anterior left insula, 102 others suggest a role for the superior lateral premotor cortex, bilateral supplementary motor area, 51,103,104 and basal ganglia. 105 Although the clinical identification of a single neurocognitive process that underpins apraxia of speech remains not entirely defined, behavioral reaction time and neuroimaging analyses suggest that impaired specification of temporal articulatory goals for sequences of speech sounds 104,106 possibly is due to weak connectivity between right and left premotor regions, thereby preventing high fidelity integration of feedback and feedforward motor commands. 107 Another attempt in identifying coherent clinicalneuroanatomical-pathological markers in PPA is posited by phonological errors or phoneme misplacement in otherwise well-articulated words.…”

A review on primary progressive aphasia

“…With progression, these processes lead to more generalized clinical involvement resulting in recognizable diseases that can additionally include the behavioral variant of frontotemporal lobar degeneration, corticalbasal degeneration (CBD) (Kertesz et al 1994;Ikeda et al 1996;Gorno-Tempini, Murray et al 2004;McMonagle et al 2006), and even amyotrophic lateral sclerosis (ALS) (Bak et al 2001;Caselli et al 1993). Occasionally, Alzheimer's disease (Pogacar and Williams 1984;Greene et al 1990Greene et al , 1996 or Creutzfeldt-Jakob disease (Shuttleworth et al 1985;Mandell et al 1989;Ghorayeb et al 1998) is diagnosed.…”