Profile of speech and cognitive impairments in a patient with moyamoya disease: a longitudinal case study

Moyamoya is a rare chronic brain vascular disease with a set of potential life-threatening consequences due to a high probability of stroke occurrence. Patients suffering from this disease are often presented with diverse and severe sensory, motor and cognitive deficits, which often lead to general inability to work and, in the most severe cases, to complete loss of self-serving skills and gross disturbance of vital homeostatic functions. Here we present a case of a patient with moyamoya disease, who has been dynamically observed over more than 4 years after the first manifestation of her symptoms. We have been tracking the appearance of patient’s new symptoms and resolution of existing ones after neurosurgical treatment (revascularization surgery) with in-depth neurological examination, neuropsychological assessment and fMRI/DTI sessions. This study represents one of the first attempts to rigorously estimate the patterns of deficits at different levels, connecting neural circuits affected by the disease to behavior. Our findings lend further support to the idea that revascularization surgery can improve cognitive performance and highlights the importance of long-term follow-up in understanding the effects of surgery on brain function.