BackgroundLeigh syndrome (LS) is a mitochondrial disorder that shows abnormal basal ganglia lesion and psychomotor regression. Although vitamins have been used for LS, we not found any effective drug.Case presentationA 26-year-old man who showed delay short stature at the age of 1 year was diagnosed with LS according to results cerebrospinal fluid high signal intensity in bilateral striatum on ...

To investigate emotion recognition in amyotrophic lateral sclerosis (ALS) patients without cognitive/behavioral deficits and its relationship with the integrity of basal ganglia, hippocampus amygdala.

Descriptions of symptomatic focal dystonia caused by focal lesions of the central nervous system (CNS) are rare in the literature. We report a 9-year-old child who experienced sudden-onset left-hand dystonia for 6 months. Brain magnetic resonance imaging showed a mass lesion involving the putamen, globus pallidus, head of caudate, and the anterior limb of the internal capsule. Histopathological...

The ability to suppress or override competing attentional and behavioral responses is a key component of cognitive processes. This ability continues to develop throughout childhood and appears to be disrupted in a number of childhood disorders (e.g., attention deficit/hyperactivity disorder and Tourette syndrome). At least two brain regions have been implicated repeatedly in these disorders--th...

Changes in synchronized neuronal oscillatory activity are reported in both cortex and basal ganglia of Parkinson's disease patients. The origin of these changes, in particular their relationship with the progressive nigrostriatal dopaminergic denervation, is unknown. Therefore, in the present study we studied interregional neuronal synchronization in motor cortex and basal ganglia during the de...

Progress in our understanding of the mechanisms underlying the cardinal motor abnormalities of Parkinson’s disease (PD), in particular akinesia and bradykinesia and their treatment, has been remarkable. Notable accomplishments include insights into the functional organization of the basal ganglia and their place in the motor system as components of a family of parallel cortico-subcortical circu...