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We present the structural and functional neuroimaging of one patient with the infantile myoclonic encephalopathy (IME) and controls. We used a genetic model to develop behavioral abnormalities accompanied by encephalopathy. A 19-month-old girl with a history of developmental delay, ataxia, orofacial dyskinesia and generalized myoclonus was diagnosed with IME. She showed increased automatic speech and speech stuttering, with impaired communication skills, as well as an inability to stop automatic speech. MRI revealed increased signal in the subcortical region of the right frontoparietal and temporoparietal lobes. Neuropsychological assessment showed severe verbal and non-verbal memory impairment with no improvements over time. Prefrontal and parietal cortical subplate on diffusion tensor imaging showed preserved microstructure in WM. Single-cell recording from the medial prefrontal cortex (mPFC) showed cortical neurons with an increased rate of firing, but normal basic electrophysiological properties. The diminished inhibition in the mPFC could contribute to the activation in the subcortical regions. The excitatory response mediated by nitric oxide in the mPFC could also be related to the increase in the firing rate of neurons. The results suggest that a conductive disturbance in this early network contributes to learning and memory impairment in IME, and may be related to impairment in speech and communication.Q:

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