Alternating hemiplegia of childhood in chinese following long-term treatment with flunarizine or topiramate.

OBJECTIVE: Alternating hemiplegia of childhood (AHC) is a rare and intractable disorder. The etiology and standard therapy of AHC remain unknown. The long-term effects of flunarizine or topiramate on patients with AHC are still not clear. METHODS: Fifteen patients were investigated in this study. Their neurological disturbance and mental retardation after drug therapy were evaluated. RESULTS: Nine patients treated with flunarizine therapy and three children with topimarate treatment presented with shorter duration or less frequency of the hemiplegic attacks. These drug responsive patients also showed improvements on neurological disturbance including eye movement disorder, choreoathetotic movements, dystonia, and ataxia. However, seizure episodes and cognitive impairments were not alleviated in AHC with long-term drug therapy. CONCLUSIONS: The findings from the present study support flunarizine or topitamate as the rational treatment for AHC.

Effects of the group II mGlu receptor agonist 2R,4R-APDC on dentate gyrus cell proliferation in the adult rat brain after diffuse brain injury.

OBJECTIVES: Diffuse brain injury (DBI) has been shown to increase the proliferation of granule cell precursors in the adult dentate gyrus (DG). However, the mechanism by which DBI-induced cell proliferation in the DG may enhance seizure susceptibility remains largely unknown. MATERIALS AND METHODS: Using bromodeoxyuridine (BrdU) immunohistochemistry, we examined the effects of group II metabotropic glutamate receptor (mGluR) agonist, 2R,4R-4-aminopyrrolidine-2,4-dicarboxylate (2R,4R-APDC), on cell proliferation in the DG after DBI. RESULTS: It has been found that 2R,4R-APDC significantly blocked DBI-induced increase in the number of BrdU-positive cells in the DG, especially in hilus. In addition, double-label immunofluorescence staining showed that treatment with APDC did not affect the differentiation of newborn cells into neurons or astrocytes. Taken together, our findings indicate that the activation of mGluR system may inhibit the DBI-induced cell proliferation in the DG, but not the differentiation of newborn cells. DISCUSSION: It is suggested that 2R,4R-APDC has potential neuroprotection via inhibiting the aberrant neurogenesis induced by DBI, which is an important pathological basis of seizure or other abnormalities following DBI.

The dual role of tumor necrosis factor-alpha in the pathophysiology of spinal cord injury.

Recent studies have demonstrated that tumor necrosis factor-alpha (TNF-alpha) is one of the most important mediators in spinal cord injury (SCI). However, the role of TNF-alpha in this process is still under debate due to conflicting evidence. Here, we utilized TNF-alpha transgenic (tg) rats and wild-type (wt) littermates to further investigate the role of TNF-alpha in SCI. We observed that, in the acute phase post-SCI (< or =3 days), TNF-alpha tg rats showed higher expression of TNF-alpha protein and more apoptotic cells in the spinal cord than wt rats, while in the chronic period (> or =7 days), TNF-alpha tg rats exhibited persistent baseline level of TNF-alpha protein, better tissue healing, and more activated astrocytes in the border of the lesion than wt rats. These data further demonstrate that TNF-alpha plays a dual role in SCI and its role probably depends on when it is released after SCI and on which cellular population it acts on.

Poly(ADP-ribose) signal in seizures-induced neuron death.

Poly(ADP-ribose) is found to be involved in many physiological or pathological processes. It is mainly modulated by poly(ADP-ribose) polymerase (PARP) and poly(ADP-ribose) glycohydrolase (PARG). Either PARP or PARG is associated with the neuronal death in a variety of neurodegenerative diseases. Cumulative data have suggested that poly(ADP-ribose) regulation might have a therapeutic value in neurotoxicity-induced neuron damage, probably due to the inhibition of apoptosis, suppressing of inflammation and activation of cell survival signaling. We hypothesize poly(ADP-ribose) play an important role in seizures-induced neuron death. Seizures can lead to neuron degeneration as for the exitotoxity of glutamate. Recently, it is indicated seizures also can trigger PARP activation. Further investigation is needed to determine whether poly(ADP-ribose) signal is a therapeutic target for seizures-induced injury.

Meningioma without dural attachment: case report, classification, and review of the literature.

BACKGROUND: The primary occurrences of meningiomas without attachment to the dura are rare. Clinical considerations and pathophysiologic mechanisms about these tumors have not been sufficiently explored, and a complete classification has not been accomplished. CASE DESCRIPTION: A 16-year-old adolescent boy presented with epileptic seizure for 9 years. Neurologic deficits were not found on admission. Magnetic resonance imaging revealed a 25 x 23-mm mass lesion without dural attachment located in the parietooccipital region. The tumor was iso-intense on T1-weighted and hyperintense on T2-weighted images, and became clearly and heterogenously enhanced with gadolinium. During surgery, a right parietooccipital craniotomy revealed the tumor was completely buried in the sulcus occipitalis anterior. Total removal of the tumor was accomplished. Histologic examination indicated that the lesion was an atypical meningioma. CONCLUSION: According to sites of the tumor, supratentorial meningiomas without dural attachment are classified into 5 varieties, and posterior fossa meningiomas without dural attachment into 4 categories. Except for intraventricular ones, meningiomas without dural attachment predominantly occur in males. The average age is about 20 years younger than that of meningiomas in general. Fibroblastic meningiomas constitute the major subtype. Intraparenchymal or subcortical meningiomas should be considered as one type, which may arise from arachnoid cells of the pia mater within brain sulcus.