Rivista di neurologia最新文献

The Authors present a case of post-anoxic coma accompanied by myoclonic status. They describe the clinical picture and instrumental data. The outcome seems to be determined by the serious anoxic-pathological damage rather than the myoclonic jerks. They discuss the problem concerning preventive treatment by use of thiopental sodium (T.P.S.) in such cases.

Miller Fisher Syndrome (MFS), which is characterized by ophthalmoplegia, ataxia and tendon areflexia, is generally considered as a clinical variant of Guillain-Barré Syndrome. However some features of the disease are still debated, particularly regarding possible central nervous system involvement. After presenting two new cases of MFS, the authors provide a critical review of the literature and discuss the nosographical position of the disease. The main conclusions can be summarized as follows: MFS is a predominantly axonal inflammatory neuropathy with prevailing involvement of oculomotor nerves. It is associated to spinal multi or polyneuropathy, which in mildly affected cases is manifested by areflexia, while in severe ones it can be responsible of sense and/or motor impairment. In addition to peripheral neuropathy CNS involvement, exclusive or more marked in posterior fossa, occurs not infrequently. The prognosis of the disease is often benign, but disabling or even fatal outcome is possible. Corticosteroid treatment, possibly because of antiinflammatory and/or immunosuppressive action, could be effective in some patients. Finally, in spite of some similarities with GBS, MFS should be considered as a separate entity with its own nosographical position.

Large scale deletions of mitochondrial DNA (mtDNA) or altered inter-genomic regulation in skeletal muscle have been demonstrated in patients with mitochondrial encephalomyopathies due to Cytochrome C oxidase (COx) deficiency. We have analyzed by Southern blotting and Polymerase Chain Reaction (PCR) the mtDNA in primary muscle cultures (myoblast-myotube stages and at clonal densities) and in fibrogenic subclones obtained from 9 patients with partial COx deficiency who had in their muscle biopsy a subpopulation of mtDNA showing deletions of variable size (between 2.1 and 6.5 Kb). Only in the cultures from one patient, southern analysis revealed in myoblasts and myotubes a mtDNA almost identical to that found in the original muscle biopsy and persistence of deletion in muscle cells grown at clonal densities. The deletion was detectable in fibrogenic lineage only by PCR amplification. The deleted mtDNA molecules were detectable in myogenic or fibrogenic cultures from other patients only by PCR amplification. The different amounts of deleted mtDNA in the various tissues could be due either to an unequal distribution of the altered mtDNA during embryogenesis with amplification of deleted molecules in myogenic lineage or could result from negative selection against the altered mtDNA in rapidly proliferating cells, such as fibroblasts.

44 cases are reported of definite femoral mononeuropathy, by clinical and neurophysiological criteria. In 11 patients there was diabetes mellitus, in 10 compressions, in 23 no determined etiology was identified. Among these 23 patients, 15 showed glucose intolerance. The value of diabetes mellitus and the glucose intolerance in the etiology of femoral mononeuropathy is discussed.

The authors reviewed recent literature reports on Gilles de la Tourette syndrome. Clinical aspects, etiopathogenetic theories and therapeutic trials were taken into account, to better define the disease and its rational therapeutic approach.

Five families with late onset spinocerebellar ataxia (SCA) were studied. A high association was found between the disease and HLA. A stronger association results with a marker called D6S89. Clinical data of 26 patients and neuropathological study in two are reported. The clinical phenotypes of other HLA-linked SCA kindreds shows differences when comparison is made. The Authors suggest that the phenotype might appear more homogeneous if disease duration is taken into account.

A Collet-Sicard syndrome was observed in a 53-year-old patient with a coiling of the left internal carotid artery just below the skull base. Although impairment of cranial nerves by tortuous vessel compression has frequently been reported, a combined palsy of the last four cranial nerves related to such a mechanism has never been described before.

A striking dissociation between the inability to identify living things plus food, along with a preserved ability to identify inanimate objects has been observed in patients who are recovering from herpes simplex encephalitis. In order to find out if a similar dissociation is also present in Alzheimer's disease (where the suspected initial site of parenchymal atrophy involves the same areas affected by herpes simplex virus), we carried out five experimental linguistic tasks to compare Alzheimer's patients' performance therein with that of vascular dementia patients and controls. Our results indicate a constant parallel between the category-specific semantic impairment of Alzheimer's patients and that described in patients recovering from herpes simplex encephalitis. The dissociation of living things+food vs inanimate objects in Alzheimer's disease appears to be of diagnostic value: it is not present in vascular dementia.

The effect on peripheral T-lymphocytes of Thymopentin (TP-5), asynthetic pentapeptide reproducing the biological activity of Thymopoietin, is known in Herpes Simplex infections and in Rheumatoid Arthritis. The aim of this study was to observe the effect of TP-5 on the OKT4 and OKT8 lymphocytes in Multiple Sclerosis. The AA. have studied this effect in two patients affected by definite MS, whose lymphocytes subpopulation, observed for 33 and 13.5 months respectively, showed a constant OKT4/OKT8 ratio greater than 2.5 in peripheral blood and whose clinical course was chronically progressive. TP-5 was administered during a period of one month. A decrease of the OKT4/OKT8 ratio in both patients (significant in one, p less than 0.01) due to the increase of OKT8 was observed. Also the clinical symptomatology improved in one patient.