Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease.

Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II).

Corticostriatopallidal neuroprotection by adenovirus-mediated ciliary neurotrophic factor gene transfer in a rat model of progressive striatal degeneration.

Ciliary neurotrophic factor (CNTF) is a potent protective factor for striatal neurons in animal models of Huntington's disease (HD). Clinical application of this potential therapeutic still requires the design and optimization of delivery systems. In the case of HD, spatial spread in the vast volume occupied by the striatum and long-term delivery of the factor are particular challenges for these systems.

Migration pathways of human glioblastoma cells xenografted into the immunosuppressed rat brain.

Diffuse invasion of the brain by tumor cells is a hallmark of human glioblastomas and a major cause for the poor prognosis of these tumors. This phenomenon is only partially reproduced by rodent models of gliomas that display a very high rate of proliferation and limited cell migration. We have analyzed the development of human glioblastoma cells (GL15) xenografted into the brain of immunosuppressed rats, in order to define the characteristics of tumor cell invasion.

Hypertrophic neuropathy of the facial nerve.

Hypertrophic neuropathy is a peripheral nerve lesion that is histologically characterized by onion bulb formations around axons. This histologic picture, which is usually seen in generalized hypertrophic neuropathies, can occasionally be observed in single nerves as localized hypertrophic neuropathy. Cranial involvement of such localized hypertrophic neuropathy represents a very rare entity; only a few cases have been reported in the literature.

Safety and tolerability assessment of intrastriatal neural allografts in five patients with Huntington's disease.

This study describes issues related to the safety and tolerability of fetal striatal neural allografts as assessed in five patients with Huntington's disease. Huntington's disease (HD) is characterized by motor, cognitive, and behavioral disturbances. The latter include psychological disturbances and, as a consequence, we took particular care to analyze behavioral changes, in addition to the usual "safety" follow-up.

Phenotypic alteration of astrocytes induced by ciliary neurotrophic factor in the intact adult brain, As revealed by adenovirus-mediated gene transfer.

Synthesis of the ciliary neurotrophic factor (CNTF) and its specific receptor (CNTFRalpha) is widespread in the intact CNS, but potential biological roles for this system remain elusive. Contradictory results have been obtained concerning a possible effect on the morphological and biochemical phenotype of astrocytes. To reassess this question, we have taken advantage of adenovirus-mediated gene transfer into the rat brain to obtain the local release of CNTF.

Transforming growth factor alpha expression as a response of murine motor neurons to axonal injury and mutation-induced degeneration.

We previously showed that degenerating adult motor neurons of the murine mutant wobbler, a model of spinal muscular atrophy, express Transforming Growth Factor alpha (TGF alpha), a growth factor endowed with glio- and neurotrophic activities. Here, we evaluated whether TGF alpha expression is a general response of adult motor neurons to injury. Synthesis of its precursor (pro-TGF alpha) was investigated in another model of motoneuronal degeneration, the murine mutant muscle deficient, and in hypoglossal motor neurons following axonal crush and cut.

Long-term histological follow-up of genetically modified myoblasts grafted into the brain.

Although primary muscle cells have been used as intracerebral vehicles for transgene expression in the past, data concerning their long-term survival after grafting into the brain, and the reaction of the host tissue to their implantation are lacking. In order to study these aspects, we have implanted, into the brain, primary muscle cells infected ex vivo with recombinant retroviruses carrying the E. coli LacZ gene.

Adenovirus-mediated gene transfer to the brain: methodological assessment.

The purpose of this short review is to analyse major advantages and limitations of the adenovirus (Ad), specifically with relevance to its use as a vector for gene transfer to the brain. The characteristics of Ad transduction include: the relative absence of cell type specificity; the limited spatial spread of the virus; and the long-term expression of the transgene. In the central nervous system, in contrast to that which occurs in other organs, Ad transduction in the adult does not systematically provoke cell death.

[Beta-2 microglobulin in cerebrospinal fluid in neurology].

Objectives: beta 2-microglobulin (beta 2-m) is a small molecular weight protein (11 800 Daltons) which can transudate into the cerebrospinal fluid (CSF) in the same manner than albumin. Intrathecal synthesis is a sign of local immuno-stimulation and is correlated with immunoglobulin G. The aim of this study was to ascertain the relationship between beta 2-microglobulin levels in the CSF and neurological diseases.

In vivo transfer of a marker gene to study motoneuronal development.

Adenovirus vectors containing a marker gene (lacZ from Escherichia coli) are potent for transferring the gene to neurones after intraparenchymal injections. Expression of the marker gene may lead to the synthesis of an enormous amount of beta-galactosidase which diffuses throughout the entire neurone, providing a 'Golgi-like' staining. This suggested that the technique may be used to study the morphology of specific neuronal populations.

Stimulation of the cyclic GMP pathway by NO induces expression of the immediate early genes c-fos and junB in PC12 cells.

Stimulation of several second messenger pathways induces the expression of immediate early genes such as c-fos, c-jun, junB, and junD, but little is known about their induction via the stimulation of the cyclic GMP pathway. Here we looked at the expression of early genes in pheochromocytoma PC12 cells after activation of cytosolic guanylate cyclase by sodium nitroprusside. This compound spontaneously releases NO, a molecule known to be involved in cell communication.

Left tuberothalamic artery territory infarction: neuropsychological and MRI features.

We examined a 29-year-old woman who had language disturbances and memory impairment after a left thalamic infarction. MRI showed injury that was limited to the tuberothalamic artery territory. Beside reduced voice volume and verbal memory trouble, she presented with aspontaneity, loss of psychic self-activation and affective drive.

Hot-water epilepsy in an adult: ictal EEG, MRI and SPECT features.

Reflex epilepsy constitutes a rare form of epileptic seizures. We observed a 20-year-old man who presented with seizures induced by immersion in hot water. The trigger stimulus was specific.

Induction of D2 dopamine receptor mRNA synthesis in a 6-hydroxydopamine parkinsonian rat model.

By injecting 6-hydroxydopamine into the pars compacta of the substantia nigra, we produced a hemiparkinsonian rat model in which there is almost complete destruction of the dopaminergic nigrostriatal pathway but sparing of the dopaminergic mesolimbic system. The lesion has been characterized by several criteria: a rotational behavior in response to apomorphine, a complete loss of tyrosine hydroxylase immunoreactivity in the lesioned substantia nigra, a near total depletion of dopamine and metabolites in the striatum ipsilateral to the lesion, and a supersensitivity of the dopamine D2 receptors in the ipsilateral striatum. Dopaminergic striatal deafferentation was accompanied by an increase of D2 receptor mRNA synthesis in the striatum ipsilateral to the lesion, suggesting that the increased D2 receptor density observed after the lesion is due to an increase of the synthesis of receptor molecules.

[Meningioma with pseudo-vascular disclosure].

In a retrospective study of 125 case reports of intracranial supratentorial meningiomas, the authors have isolated 10 patients whose meningioma had been revealed by pseudo-vascular syndromes. Mean age and sex ratio were the same in this group of patients as in those patients whose meningioma had been more classically disclosed by an epileptic seizure, a motor or a progressive sensorimotor deficit. The site of the tumour exerted an influence on the nature of clinical manifestations.

Contralateral disappearance of parkinsonian signs after subthalamic hematoma.

A man with Parkinson's disease (PD) suddenly developed a left hemiballismus, and the CT showed a hematoma of the right subthalamic nucleus. After the ballistic movements had disappeared, akinesia and the other parkinsonian signs did not reappear on the left. This clinical case confirms the involvement of the subthalamic nucleus in the akinesia of PD, as suggested by recent experimental data.

[Arachnoid cyst of the temporal fossa. Subdural hematoma. Contribution of MRI].

A 20-year old man presented with an arachnoid cyst of the middle fossa and a subdural hematoma (SDH). An intra-cystic hemorrhage masked the presence of the SDH on CT. MRI demonstrated a communication between cyst and SDH.