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Biomedical subjects

R Nemni

Publications and source records attributed to R Nemni.

85 records · Page 5Linked to original sources
PubMed1983

Chronic progressive external ophthalmoplegia (CPEO): clinical, morphologic, and biochemical studies.

We studied skeletal muscles from eight chronic progressive external ophthalmoplegia patients with ragged-red fibers (group A), five CPEO patients without ragged-red fibers (group B), and five controls. The EM morphometric fraction of structurally abnormal mitochondria was increased in group A, and there was a similarly increased fraction of normal-appearing mitochondria in group B. State 3 respiration and uncoupled respiration were severely decreased in both groups. The morphometric mitochondrial fraction and respiratory functions were inversely related in all three groups. The cytochrome content in group A was normal; cytochromes b and cc1 were decreased in group B. These studies point to a central role of mitochondrial dysfunction in all types of CPEO, but the basic abnormalities remain to be elucidated.

Adult↗
PubMed1983

Metabolic and morphologic changes induced by serotonin in skeletal muscle of rats after abdominal aorta ligation.

Myopatic changes observed in muscle of rats after abdominal aorta ligation and/or serotonin injection have been regarded as a suitable model to explore the possibility that vasoactive treatments induce muscular damage similar to human myopathies. In the present study, we investigated the effect of serotonin administration to normal and aorta-ligated rats on cAMP-dependent protein-kinase (PK) and hydrolytic enzymes in skeletal muscle.

Animals↗
PubMed1982

Peripheral neuropathy in multiple system atrophy with autonomic failure.

Two patients with multiple system atrophy, autonomic failure, and peripheral neuropathy are reported. EMG conduction study in both muscle and sural nerve histology in one patient documented the involvement of the neuromuscular system. Morphologic study of the biopsied nerve showed marked reduction of large myelinated fibers, whereas the unmyelinated axons were totally spared. The latter finding provides evidence that the syspathetic nervous system contributes few, if any, axons to the total population of unmyelinated fibers in the human sural nerve. Peripheral nerve damage may be common in the Shy-Drager syndrome.

Atrophy↗
PubMed1981

Symmetric sarcoid polyneuropathy: analysis of a sural nerve biopsy.

A 20-year-old woman with sarcoidosis had uveitis, subacute symmetric sensorimotor neuropathy, and noncaseous granulomas in biopsies of gastrocnemius muscle and sural nerve. Morphologic studies of the nerve confirmed the electrodiagnostic impression of an acute axonal and demyelinating neuropathy. Of 100 teased myelinated nerve fibers, 15% contained myelin ovoids and 24% demonstrated segmental demyelination. Quantitative analysis showed a reduction in the numerical density of myelinated fibers. By electronmicroscopy, unmyelinated fibers were largely spared. The exact mechanism of nerve fiber damage was not determined, but a local effect of the granuloma seemed likely, because most lesions were found in the endoneurium.

Adult↗
PubMedDate not supplied

Antineuronal antibodies in patients with neuroblastoma: relationships with clinical features.

We evaluated the frequency of serum antineuronal antibodies in a cohort of 39 neuroblastoma patients and related their presence to clinical features. Twelve patients displayed antineuronal antibodies at immunocytochemistry. Only one of these 12 patients suffered from a clinically overt paraneoplastic syndrome. No significant differences emerged between autoantibody-positive and autoantibody-negative patients in terms of progression-free and overall survival, although when only patients evolving to disease progression were considered, the time interval between diagnosis and progression was slightly longer in autoantibody-positive patients.

Adolescent↗
PubMedDate not supplied

Peripheral motor-sensory neuropathy in membranous lipodystrophy (Nasu's disease): a case report.

A case of membranous lipodystrophy (ML) with peripheral polyneuropathy (PN) is described. A 40-year-old woman presented with dementia, multiple lytic bone lesions with typical membranous material in adipose tissues and neurophysiological and pathological findings of peripheral neuropathy. We describe the first case of ML with PN. It is interesting since nerve involvement might be an additional cause of symptoms usually attributed to bone lesions.

Adipose Tissue↗
PubMedDate not supplied

Neuropathies associated with monoclonal gammapathies.

There is increasing evidence that monoclonal proteins are implicated in the development of peripheral neuropathy. Approximately ten percent of patients with peripheral neuropathy of unknown cause have a monoclonal protein and this rate is significantly higher than prevalence rates of monoclonal protein in comparable segments of the general population. Extensive clinical, electrophysiological and immunopathological evidences indicate that peripheral neuropathy associated with monoclonal protein are heterogeneous, including: 1. the demyelinating, predominantly sensory neuropathies associated with anti-MAG antibodies; 2. the axonal, sensory neuropathies associated with anti-sulfatide and anti-chondroitin sulfate antibodies; 3. the motor neuropathies associated with anti-GM1 antibodies. Patients with chronic polyneuropathies should be evaluated for underlying plasma cell dyscrasia.

Adult↗
PubMedDate not supplied

Peripheral neuropathy in scleroderma.

Nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type I. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.

Adult↗