ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Recent research has led to increased understanding of hereditary muscle diseases. This review covers recent progress in the molecular genetics of Duchenne and Becker muscular dystrophies, myotonic dystrophy, facioscapubhumeral muscular dystrophy, and other muscle diseases.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Remarkable progress has occurred in the metabolic myopathies, especially in the area of mitochohdrial encephalomyopathies, where: a point mutation in the transfer RNALeu(UUR)gene of mitochondrial DNA (mtDNA) was found in mitochorvdrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS); a new pathogenetic mechanism has been attributed to depletion of mtDNA in infants with fatal myopathy or hepatopathy; the functional consequences of a mtDNA mutation have been studied in mtDNA-less (rho°) cells repopulated with mutant genomes; and deletions of mtDNA have been documented in non-dividing tissues of old people. In the area of lipid disorders, availability of a full-length complementary DNA for human liver carnitine palmitoyttransferase (CPT) will help clarify the molecular basis of CPT deficiency, the most common cause of recurrent myoglobinuria.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

The definition of acute and chronic idiopathic demyelinating polyneuro-pathy and the nature of the underlying immunological mechanisms continue to generate interest. The Guillain-Barre syndrome includes cases with prominent axonal degeneration, some of which may follow Campylobacter infection, the causation of the axonal degeneration being uncertain: Amongst hereditary neuropathies, major advances have occurred in the molecular genetics of hereditary amyloid neuropathy, hereditary motor and sensory neuropathy and neurofibromatosis.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Acetylcholine receptors, anti-acetylcholine-receptor antibodies, T-cell responses, immunogenetics and management in myasthenia gravis are discussed in this review. Also covered are anti-voltage-gated-calcium-channel antibodies in the Lambert-Eaton myasthenic syndrome and an antibody-mediated mechanism in acquired neuromyotonia (Isaacs' syndrome).

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Contributions on three main topics have been published during the last year, the neuropathy of the eosinophilia-myalgia syndrome (EMS), which is due to consumption of L-tryptophan-containing products; the. neuropathies associated with Sjögren's syndrome, which constitute a heterogeneous group that include trigeminal sensory neuropathy, ataxic sensory neuropathy, and mononeuropathy multiplex resulting from vasculitis; and cytomegatovirus (CMV) opportunistic infection, which represents a life-threatening virus in patients with the acquired immune deficiency syndrome (AIDS), which can provoke severe neuropathies that may respond well to early treatment.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

The inflammatory myopathies are a heterogeneous group of disorders, mediated by immune mechanisms. This review summarizes the current literature on these diseases, including polymyositis (PM), dermatomyositis (DM), the eosinophilia-myalgia syndrome (EMS), inclusion body myositis (IBM), and Other miscellaneous inflammatory muscle disorders. The review focuses on significant studies on induction and immunopathogenetic effector mechanisms, but literature discussing important clinical and diagnostic features is also reviewed.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Progress in research related to amyotrophic lateral sclerosis and related diseases seems to be accelerating. In 1990–1991, the major themes in studies of pathogenesis were autoimmunity, gene mapping and excitotbxins. Another major question is the identity and treatment of a motor neuropathy that resembles adult-onset spinal muscular atrophy.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

The role of metabolic factors in diabetic neuropathy and human-immunp-deficiency-viois-related myeloneuropathy is described. A novel amino acid and botulinum toxin precipitate the immunological response resulting in the eosinophilia-myalgia syndrome and brachial plexopathy, respectively. Clinical and electrophysiobgical features of neuropathies that result from sodium channel blockers, thallium, suramin, arsenic, human immunodeficiency virus and nitrous oxide are also reviewed. The role of ischemia in the etiology of diabetic neuropathies is discussed.

ISSN:0951-7383    

出版商:OVID    

年代:1991

数据来源: OVID