ABSTRACT
At present, language therapy is the only available treatment for childhood aphasia (CA). Studying new interventions to augment and hasten the benefits provided by language therapy in children is strongly needed. CA frequently emerges as a consequence of traumatic brain injury and, as in the case of adults, it may be associated with dysfunctional activity of neurotransmitter systems. The use of cognitive-enhancing drugs, alone or combined with aphasia therapy, promotes improvement of language deficits in aphasic adults. In this study we report the case of a 9-year-old right-handed girl, subject P, who had chronic anomic aphasia associated with traumatic lesions in the left temporal-parietal cortex. We performed a single-subject, open-label study encompassing administration of the cholinergic agent donepezil (DP) alone during 12 weeks, followed by a combination of DP and intensive naming therapy (INT) for 2 weeks and thereafter by a continued treatment of DP alone during 12 weeks, a 4-week washout period, and another 2 weeks of INT. Four comprehensive language and neuropsychological evaluations were performed at different timepoints along the study, and multiple naming evaluations were performed after each INT in order to assess performance in treated and untreated words. Structural magnetic resonance imaging (MRI) was performed at baseline. MRI revealed two focal lesions in the left hemisphere, one large involving the posterior inferior and middle temporal gyri and another comprising the angular gyrus. Overall, baseline evaluation disclosed marked impairment in naming with mild-to-moderate compromise of spontaneous speech, repetition, and auditory comprehension. Executive and attention functions were also affected, but memory, visuoconstructive, and visuoperceptive functions were preserved. Treatment with DP alone significantly improved spontaneous speech, auditory comprehension, repetition, and picture naming, in addition to processing speed, selective, and sustained attention. Combined DP-INT further improved naming. After washout of both interventions, most of these beneficial changes remained. Importantly, DP produced no side effects and subject P attained the necessary level of language competence to return to regular schooling. In conclusion, the use of DP alone and in combination with INT improved language function and related cognitive posttraumatic deficits in a child with acquired aphasia. Further studies in larger samples are warranted.
ABSTRACT
The acquisition and evolution of speech production, discourse and communication can be negatively impacted by brain malformations. We describe, for the first time, a case of developmental dynamic dysphasia (DDD) in a right-handed adolescent boy (subject D) with cortical malformations involving language-eloquent regions (inferior frontal gyrus) in both the left and the right hemispheres. Language evaluation revealed a markedly reduced verbal output affecting phonemic and semantic fluency, phrase and sentence generation and verbal communication in everyday life. Auditory comprehension, repetition, naming, reading and spelling were relatively preserved, but executive function was impaired. Multimodal neuroimaging showed a malformed cerebral cortex with atypical configuration and placement of white matter tracts bilaterally and abnormal callosal fibers. Dichotic listening showed right hemisphere dominance for language, and functional magnetic resonance imaging (fMRI) additionally revealed dissociated hemispheric language representation with right frontal activation for phonology and bilateral dominance for semantic processing. Moreover, subject D also had congenital mirror movements (CMM), defined as involuntary movements of one side of the body that mirror intentional movements of the other side. Transcranial magnetic stimulation and fMRI during voluntary unimanual (left and right) hand movements showed bilateral motor cortex recruitment and tractography revealed a lack of decussation of bilateral corticospinal tracts. Genetic testing aimed to detect mutations that disrupt the development of commissural tracts correlating with CMM (e.g., Germline DCC mutations) was negative. Overall, our findings suggest that DDD in subject D resulted from the underdevelopment of the left inferior frontal gyrus with limited capacity for plastic reorganization by its homologous counterpart in the right hemisphere. Corpus callosum anomalies probably contributed to hinder interhemispheric connectivity necessary to compensate language and communication deficits after left frontal involvement.
