Epidermal and fibroblast growth factors behave as mitogenic regulators for a single multipotent stem cell-like population from the subventricular region of the adult mouse forebrain.

The subventricular zone (SVZ) of the adult mammalian forebrain contains kinetically distinct precursor populations that contribute new neurons to the olfactory bulb. Because among forebrain precursors there are stem-like cells that can be cultured in the presence of mitogens such as epidermal growth factor (EGF) and fibroblast growth factor 2 (FGF2), we asked whether distinct subsets of stem-like cells coexist within the SVZ or whether the proliferation of a single type of SVZ stem-like cell is controlled by several GFs. We show that the latter is the case.

Isolation and cloning of multipotential stem cells from the embryonic human CNS and establishment of transplantable human neural stem cell lines by epigenetic stimulation.

Stem cells that can give rise to neurons, astroglia, and oligodendroglia have been found in the developing and adult central nervous system (CNS) of rodents. Yet, their existence within the human brain has not been documented, and the isolation and characterization of multipotent embryonic human neural stem cells have proven difficult to accomplish. We show that the developing human CNS embodies multipotent precursors that differ from their murine counterpart in that they require simultaneous, synergistic stimulation by both epidermal and fibroblast growth factor-2 to exhibit critical stem cell characteristics.

Partial seizures associated with antiphospholipid antibodies in childhood.

We report antiphospholipid antibody positivity in three of a consecutive series of 23 children presenting partial epileptic seizures. There was no clinical or serological evidence of systemic lupus erythematosus or other connective-tissue disease. Neither computed tomography nor magnetic resonance imaging revealed ischemic alteration.

Biofeedback-assisted relaxation training for young adolescents with tension-type headache: a controlled study.

Tension-type headache is common in children and adolescents and is generally treated with medication, but emerging literature suggests that various behavioral treatments may provide efficacious alternatives to medication. Juvenile tension-type headache sufferers were randomly assigned to biofeedback-assisted relaxation continued to improve and were superior to the control condition at a 6- and 12-month follow-up (86% versus 50%). Biofeedback-assisted relaxation appears to be an efficacious and durable treatment for juvenile tension-type headache and merits further exploration.

Immunocytochemical investigation on dysplastic human tissue from epileptic patients.

In this report we describe three patients with developmental cortical abnormalities (generally referred as cortical dysplasia), revealed by MRI and operated on for intractable epilepsy. Tissue, removed for strictly therapeutic reasons, was defined as the epileptogenic area by electroclinical data and stereo EEG (SEEG) recordings. Tissue samples were processed initially for histology, and selected sections were further processed for immunocytochemical investigation in order to determine whether the region of cortical dysplasia was co-extensive with the epileptogenic area.

Identification of the gene encoding the human mitochondrial RNA polymerase (h-mtRPOL) by cyberscreening of the Expressed Sequence Tags database.

A gene cloning strategy based on the screening of the Expressed Sequence Tags database (dbEST) using sequences of mitochondrial housekeeping proteins of yeast was employed to identify the cDNA encoding the precursor of the human mitochondrial RNA polymerase (h-mtRPOL). The 3831 bp h-mtRPOL cDNA is located on chromosome 19p13.3 and encodes a protein of 1230 amino acid residues.

Emerging treatments in myopathies.

Outstanding improvement has been made in the molecular understanding of several muscle diseases with the application of molecular biological techniques to the investigation of human disorders. The identification of genetic mutations has improved considerably the diagnostic approach to muscular dystrophies, mitochondrial myopathies and ion channel disorders. Important results have been achieved in the field of inflammatory myopathies.

Acetylcholine receptor alpha-subunit isoforms are differentially expressed in thymuses from myasthenic patients.

A central role of the thymus in autosensitization to the acetylcholine receptor has been proposed to explain the immunopathogenetic processes in myasthenia gravis (MG). Two isoforms of the alpha-subunit of the acetylcholine receptor are known; they differ by a 25-amino-acid insertion coded by the P3A exon. We investigated the expression of the P3A exon by RNA polymerase chain reaction in fetal and adult human myoblasts and TE671 cells; both isoforms were expressed.

Acquired hemidystonia in childhood: a clinical and neuroradiological study of thirteen patients.

A retrospective study of 13 patients (4 males/9 females) with acquired hemidystonia in childhood is reported. The mean age of onset of hemidystonia was 6.4 years (range 1-13.

Neurological disorders, other than stroke, associated with antiphospholipid antibodies in childhood.

We report the results of a systematic study on the association of antiphospholipid antibodies (aPLs) with some neurological disease other than stroke in a childhood population. Patients affected by migraine, benign intracranial hypertension (BIH) or unilateral movement disorders, such as hemichorea and hemidystonia with acute-subacute onset, were screened for aPLs. None of them had clinical or serological evidence of Systemic Lupus Erythematosus (SLE) or other connective tissue disease.

DMD and BMD in the same family due to distinct mutations.

We report on a family with a boy affected by Duchenne muscular dystrophy (DMD) and an asymptomatic cousin with a Becker-type dystrophin abnormality, diagnosed by chance. Dystrophin gene analysis showed that these conditions were caused by two distinct deletions with breakpoints in different exons. In Xp21 families, DNA analysis and dystrophin testing of asymptomatic males with high CK plasma levels might detect different dystrophin mutations in separate haplotypes as in our family, although we stress there should be clear clinical or familial indications for such testing.

Neuronal ceroid-lipofuscinosis: a clinical and morphological study of 19 patients.

We report on clinical, electrophysiological, neuroradiological, and morphological data from 19 patients with different types (late infantile, juvenile, and adult) of neuronal ceroid-lipofuscinosis (NCL), observed in the last 10 years at the Neurological Institute of Milan. Late Infantile NCL (LINCL) (8 patients, 4m/4f). Age at onset: 2-4 1/2 years.

Basic fibroblast growth factor supports the proliferation of epidermal growth factor-generated neuronal precursor cells of the adult mouse CNS.

Stem cells isolated from the CNS of both embryonic and adult mice undergo extensive proliferation in the presence of epidermal growth factor (EGF). Removal of EGF determines the differentiation of these cells into neurons and glia. We have recently demonstrated that basic fibroblast growth factor (bFGF) regulates the proliferation of EGF-generated progenitors of the embryonic mouse striatum.

Cellular aspects of myositis.

Polymyositis, dermatomyositis, and inclusion-body myositis are characterized by muscle cell infiltration and specific alterations on or within muscle fibers. Infiltrating immune cells (ie, T or B lymphocytes, macrophages, and natural killer cells) have distinctive distributions in these conditions: increased presence of CD8+/MHC I-restricted T lymphocytes at endomysial sites in polymyositis and more B than T lymphocytes perivascularly in muscles of dermatomyositis patients. Muscle-infiltrating T lymphocytes mainly express alpha beta T cell receptors (TCRs) in polymyositis; they also express TCRs characterized by oligoclonal V beta repertoire, with a consensus motif indicating a conventional antigen as target of the immune attack.

Removal of antiacetylcholine receptor antibodies by protein-A immunoadsorption in myasthenia gravis.

Myasthenia Gravis is an autoimmune disease in which autoantibodies to the acetylcholine receptor interfere with neuromuscular transmission. Plasma exchange is effective in temporarily relieving the symptoms of the disease, but for repeated use the lack of selectivity and need for replacement fluids (which increases the risk of contracting viral diseases) are important drawbacks. Staphylococcal protein A, a potent ligand for immunoglobulins, that interacts negligibly with other plasma proteins, appears to be an optimal candidate for removing antiacetylcholine receptor antibodies, which are mostly IgG.

Primary antiphospholipid syndrome and neurologic events.

The occurrence of lupus anticoagulant and anticardiolipin antibodies was demonstrated in a girl affected by recurrent episodes of visual disturbances, with ophthalmologic evidence of visual impairment and sometimes accompanied by migraine. Systemic lupus erythematosus was excluded on the basis of both clinical and serologic criteria and the diagnosis of primary antiphospholipid syndrome was made. Vascular pathogenesis was suggested by the characteristic symptoms.

Neurogenic arthrogryposis multiplex congenita: clinical and MRI findings.

A clinical and magnetic resonance imaging (MRI) study on a selected group of 11 children, with a diagnosis of neurogenic arthrogryposis multiplex congenita (AMC) based on clinical, electromyographic, and muscle biopsy findings, is presented to determine the extent of central nervous system involvement in AMC. Family history, pregnancy, perinatal problems, other abnormalities, and epileptic seizures were reviewed. Neurologic examination, electroencephalography, intellectual assessment, and MRI study both of spinal cord and brain were performed.

Italian experience of electromyographic-biofeedback treatment of episodic common migraine: preliminary results.

Electromyographic biofeedback is commonly used successfully to treat tension-type headache, while thermal biofeedback is considered the best behavioral therapy for migraine. Ten female patients with episodic common migraine (age 20-35 years, age of disease onset 16.2 +/- 5.

Cognitive and motor performance in multiple system atrophy and Parkinson's disease compared.

Nineteen patients with multiple system atrophy (MSA) of striato-nigral degeneration type were tested to examine cognitive and motor performance. Parkinson's disease (PD) patients and healthy subjects served as controls. The MSA and PD patients showed similar cognitive dysfunction and motor impairment, performing poorly in the visuo-spatial organization, the construction tests and motor assessment tests.