High-dose chemotherapy followed by autologous peripheral blood stem cell transplantation for recurrent primary mediastinal malignant germ cell tumor: a case report.

A 15-yr-old boy presented with an anterior mediastinal mass, multiple lung metastases and obstruction of the left brachiocephalic vein, the superior vena cava and the subclavian vein. Tumor biopsy by CT guidance confirmed a diagnosis of GCT. Five courses of BEP therapy were performed, and CT of the chest revealed reduction in the anterior mediastinal mass and disappearance of the multiple lung metastases. We performed the anterior mediastinal mass extraction followed by adjuvant chemotherapy consisting of ICE and TIP. However, the AFP levels became elevated soon after. Abnormal accumulation was observed in the right upper lung by DW-MRI. After the operation, two courses of TI chemotherapy and two courses of HDCT followed by auto-PBSCT were performed. He was complicated with auditory disorder and renal dysfunction. Although HDCT followed by auto-PBSCT was effective for the relapsed primary mediastinal GCT, a treatment strategy avoiding late complications is warranted.

Microtubule association of a neuronal RNA-binding protein HuD through its binding to the light chain of MAP1B.

RNA-binding proteins (RBPs) play a vital role in the post-transcriptional regulation of gene expression during neuronal differentiation and synaptic plasticity. One such RBP family, the neuronal Hu protein family, serves as an early marker of neuronal differentiation and targets several mRNAs containing adenine/uridine-rich elements. Recently, we reported that one of the neuronal Hu proteins, HuD stimulates cap-dependent translation through interactions with eIF4A and poly (A) tail. Nevertheless, little is known with respect to how neuronal Hu proteins contribute to the local translation of target mRNAs in neuronal differentiation. Here, we found that neuronal Hu proteins, but not the ubiquitously expressed HuR protein, directly interact with the light chain of microtubule-associated proteins MAP1B (LC1). We also show that HuD simultaneously binds both RNA and LC1 in vitro and that it tightly associates with microtubules in cells in an LC1-dependent manner, raising the possibility that HuD recruits target mRNAs to microtubules. These results uncover the neuronal binding partners for neuron-specific Hu proteins and suggest the involvement of Hu proteins in microtubule-mediated regulation of mRNA expression within neuronal processes.

Epilepsies and epileptic syndromes starting in the neonatal period.

As seizures in the neonatal period have generally been identified only by direct clinical observation, there is frequently a lack of objectivity as to whether seizures are categorized as epilepsies or non-epilepsies. A major characteristic of neonatal seizures is electro-clinical dissociation and some electro-graphic seizures do not produce clinical symptoms. It is difficult to correctly identify real epilepsies or epileptic syndromes in the neonatal period without ictal electroencephalogram (EEG). Some epileptic syndromes starting in the neonatal period such as early myoclonic encephalopathy, Ohtahara syndrome, or migrating partial seizures in infancy are categorized as malignant epilepsies. A suppression-burst EEG pattern (SBP) is usually seen in neonates with serious brain damage, malignant epileptic syndromes or other neurological conditions. However SBP has not been consistently defined in the literature. We review malignant epilepsies and benign familial and non-familial neonatal seizures starting in the neonatal period and propose the characteristics of SBP in Ohtahara syndrome. Epileptic encephalopathies with SBP in the neonatal period are known to evolve into relatively few types of epileptic syndromes. We emphasize the importance of ictal EEG for diagnosis and treatment of malignant epilepsies and epileptic syndromes in the neonatal period.

Somatosensory-related gamma-, beta- and alpha-augmentation precedes alpha- and beta-attenuation in humans.

OBJECTIVE: Several human studies have demonstrated that the amplitudes of cortical oscillations are altered by various sensorimotor and cognitive tasks. Event-related augmentation of gamma oscillations and attenuation of alpha and beta oscillations have been often used as surrogate markers of cortical activation elicited by tasks especially in presurgical identification of eloquent cortices. In the present study, we addressed a question whether somatosensory-related gamma augmentation 'precedes' or 'co-occurs with' somatosensory-related attenuation of alpha-beta oscillations. METHODS: We studied 10 patients who underwent intracranial electrocorticography for epilepsy surgery, and determined the temporal and spatial characteristics of median-nerve somatosensory-related amplitude changes at gamma- (30-100Hz), beta- (14-28Hz) and alpha-band (8-12Hz) oscillations. RESULTS: We found that somatosensory-related gamma augmentation involving the post- and pre-central gyri evolved into beta and alpha augmentation, which was subsequently followed by beta and alpha attenuation involving the post- and pre-central gyri. CONCLUSIONS: These observations support the hypothesis that somatosensory-related gamma augmentation but not alpha-beta attenuation represents the initial cortical processing for external somatosensory stimuli. Somatosensory-related alpha-beta attenuation appears to represent a temporally distinct stage of somatosensory processing. SIGNIFICANCE: The present study has increased our understanding of event-related gamma augmentation and alpha-beta attenuation seen on electrocorticography.

Short-latency median-nerve somatosensory-evoked potentials and induced gamma-oscillations in humans.

Recent studies have suggested that cortical gamma-oscillations are tightly linked with various forms of physiological activity. In the present study, the dynamic changes of intracranially recorded median-nerve somatosensory-evoked potentials (SEPs) and somatosensory-induced gamma-oscillations were animated on a three-dimensional MR image, and the temporal and spatial characteristics of these activities were analysed in 10 children being evaluated for epilepsy surgery. Visual and quantitative assessments revealed that short-latency SEPs and somatosensory-induced gamma-oscillations predominantly involved the post-central gyrus and less intensely involved the pre-central gyrus and the anterior parietal lobule. Formation of a dipole of N20 peak with opposite polarities across the central sulcus was well delineated in animation movies. High-frequency (100-250 Hz) somatosensory-induced gamma-oscillations emerged in the post-central gyrus at 13.6-17.5 ms after median-nerve stimulation, gradually slowed down in frequency around and below 100 Hz, and progressively involved the neighbouring areas. A substantial proportion of somatosensory-induced gamma-oscillations was initially phase-locked and the proportion of a non-phase-locked component gradually increased over time. The primary motor hand areas proven by cortical stimulation frequently coincided with the sites showing the largest N20 peak and the largest somatosensory-induced gamma oscillations. In vivo animation of SEPs and somatosensory-induced gamma oscillations both may be utilized to localize the primary sensory-motor hand area in pre-surgical evaluation. The dipole on SEPs is consistent with the previously accepted notion that the cortices along the central sulcus are activated. The high-frequency somatosensory-induced gamma-oscillations in the post-central gyrus may represent the initial neural processing for external somatosensory stimuli, whereas the subsequent lower-frequency oscillations might represent the reafferent cortical activity occurring in larger cortical networks.