Posterior Cortical Atrophy (PCA) and Logopenic progressive aphasia (LPA) are nonamnestic variants of Alzheimer's disease (AD). Language deficits are a hallmark of LPA but not PCA. Studies have revealed the presence of language deficits in PCA similar to LPA, however it has not been a consistent observation. Only alexia and agraphia are the language deficits listed in the latest consensus criteria for classification of PCA. In this case report we present the clinical, cognitive-linguistic, and neuroimaging features of a patient presenting with an unusual overlap of LPA-PCA. Although the diagnostic characteristics for LPA is fulfilled, the probable progression of the disease to exhibit most of the characteristics similar to PCA is highlighted. Thus, it contributes to the notion of a continuum between the two atypical variants of AD. The possibility of patients with PCA to exhibit language deficits with the progression of the disease similar to that of LPA that primarily involves the phonological short-term memory deficits is emphasized. The need to not undermine the language deficits irrespective of the stage of the disease in PCA is weighed upon for a better identification and management via speech-language intervention programs.
Audiological manifestations of patients with PLA2G6 associated neurodegeneration (PLAN) are limited. We analyzed the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD). Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry and Brainstem Auditory Evoked Potentials (BAEP’s). Audiological studies revealed abnormal findings in 10 out of 13 children (77%). The findings indicated sensorineural hearing loss in six and auditory neuropathy spectrum disorder in four children. This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease is required before making appropriate recommendations for aural rehabilitation.
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