Doença de Moyamoya: impacto no desempenho da linguagem oral e escrita

Abstract

Moyamoya disease is an unusual form of occlusive, cerebrovascular disorder that affects the arteries of the central nervous system, causing acquired language alterations and learning difficulties. The study aim was to describe the oral/written language and cognitive skills in a seven-year-and-seven-month-old girl diagnosed with Moyamoya disease. The assessment consisted of interviews with her parents and application of the following instruments: Observation of Communicative Behavior, Peabody Picture Vocabulary Test, Academic Performance Test, Profile of Phonological Awareness, Raven's Progressive Matrices Test, Special Scale, Wechsler Intelligence Scale for Children, Bender Visual Motor Gestalt Test, and Wisconsin Card Sorting Test. Two episodes of stroke in the left and right temporal-parietal and left frontal areas occurred until the age of six years and five months. Revascularization surgery and medication treatment were conducted. The audiologic and ophthalmologic assessments indicated normality. At the time of the study, the girl was attending the second grade of elementary school. She presented changes in oral and written language (syllabic-alphabetic), non-naming of all graphemes, low arithmetic and writing means, reading skill below first grade level and psycholinguistic delay, and pre-school level phonological processing skills. The psychological evaluation indicated satisfactory intellectual level; however, it also showed cognitive performance impairment in verbal and execution tasks and limitations on graphic-perceptual-motor skills and sequential logic organization. The stroke episodes influenced the performance of learning processes, affecting the analysis, integration, and interpretation of relevant visual and auditory information.

Moyamoya: An Update and Review

“…Initial hemorrhagic presentation is seven times less likely in pediatric patients compared to adults [21]. Pediatric patients with MMD have a statistically significant increased incidence of psycholinguistic delay with, impairment of verbal, visual integration, auditory response, and executive function when compared to age-adjusted controls [22]. Headache is a common symptom in patients with MMD and is believed to be caused by irritation of the pain-sensitive fibers in the meninges by dilating neovascularization [23].…”

Neuropsychology of Moyamoya Disease

“…points in his intellectual functioning [42]. In the same line, Cusin-Lamonica et al reported the case of a girl of seven years old who suffered two episodes of stroke in the left and right temporal-parietal and left frontal areas that occurred until the age of six years and five months [43]. She presented signs of deterioration in oral and written language (syllabic-alphabetic), non-naming of all graphemes, low arithmetic and writing means, pre-first-grade reading skillsand psycholinguistic delay, and pre-school-level phonological processing skills.…”

Western Moyamoya Phenotype: A Scoping Review

“…The rates of hemorrhage reported in several large studies are presented in Table 4. In individual moyamoya patients, neurological symptoms secondary to moyamoya included laryngomalacia [118], hypotension encephalopathy [117], behavioral changes [119], oral and written language deficits [119,128], transient psychosis [121,129,173], hemiplegia [126,131], hearing loss [132], and cognitive difficulties [120]. Movement disorders as symptoms of moyamoya included chorea [122,123], dystonia [124,125], hemifacial spasms [130], and hemiballismus [127].…”

“…We found 16 (15%) papers describing an association between moyamoya and hematologic disorders. These include hereditary spherocytosis [124][125][126][127], beta-thalassemia [128,133,173], Fanconi anemia [78], sickle cell anemia [79] and trait [85,86], hemophilia A [131,197], hemoglobin Southamptom [132], and Henoch-Schonlein purpura [174].…”