Multifocal motor neuropathy and chronic inflammatory demyelinating polyradiculoneuropathy. 1995

J M Léger
Department of Neurology, Hôpital de la Salpêtrière, Paris, France.

In multifocal motor or sensorimotor neuropathy with persistent conduction block, most of the recent research has concerned treatment. Open trials show that intravenous immunoglobulin is effective, providing a rapid and often marked clinical improvement, accompanied by a significant reduction of conduction block in some cases, but without any significant change in the titre of anti-ganglioside GM1 antibodies. However, long-term therapy with intravenous immunoglobulin seems to bring less improvement than expected, and further trials are needed to study the efficacy of other immunosuppressive drugs, such as oral or intravenous cyclophosphamide. These treatments may also benefit acquired lower motor neuron diseases with a progressive course, high anti-ganglioside GM1 titres and no conduction block. In chronic inflammatory demyelinating polyneuropathy, recent articles have reported interesting immunopathological, electrophysiological and immunochemical features. This syndrome seems to be heterogeneous. Therefore, better criteria need to be defined, particularly so that an informed choice can be made as to the most appropriate treatment. A recent controlled trial has shown that intravenous immunoglobulin infusion may be as effective as plasma exchange in the short-term therapy of chronic inflammatory demyelinating polyneuropathy.

UI MeSH Term Description Entries
D007249 Inflammation A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. Innate Inflammatory Response,Inflammations,Inflammatory Response, Innate,Innate Inflammatory Responses
D010523 Peripheral Nervous System Diseases Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. Peripheral Nerve Diseases,Peripheral Neuropathies,PNS (Peripheral Nervous System) Diseases,PNS Diseases,Peripheral Nervous System Disease,Peripheral Nervous System Disorders,Nerve Disease, Peripheral,Nerve Diseases, Peripheral,Neuropathy, Peripheral,PNS Disease,Peripheral Nerve Disease,Peripheral Neuropathy
D011129 Polyradiculoneuropathy Diseases characterized by injury or dysfunction involving multiple peripheral nerves and nerve roots. The process may primarily affect myelin or nerve axons. Two of the more common demyelinating forms are acute inflammatory polyradiculopathy (GUILLAIN-BARRE SYNDROME) and POLYRADICULONEUROPATHY, CHRONIC INFLAMMATORY DEMYELINATING. Polyradiculoneuritis refers to inflammation of multiple peripheral nerves and spinal nerve roots. Autoimmune Demyelinating Disease, Peripheral,Demyelinating Autoimmune Disease, Peripheral,Demyelinating Disease, Peripheral Autoimmune,Peripheral Autoimmune Demyelinating Disease,Polyradiculoneuritis,Polyradiculoneuritides,Polyradiculoneuropathies
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016472 Motor Neuron Disease Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089) Anterior Horn Cell Disease,Familial Motor Neuron Disease,Lateral Sclerosis,Motor Neuron Disease, Lower,Motor Neuron Disease, Upper,Lower Motor Neuron Disease,Motor Neuron Disease, Familial,Motor Neuron Disease, Secondary,Motor System Disease,Primary Lateral Sclerosis,Secondary Motor Neuron Disease,Upper Motor Neuron Disease,Lateral Scleroses,Lateral Scleroses, Primary,Lateral Sclerosis, Primary,Motor Neuron Diseases,Motor System Diseases,Neuron Disease, Motor,Neuron Diseases, Motor,Primary Lateral Scleroses,Scleroses, Lateral,Scleroses, Primary Lateral,Sclerosis, Lateral,Sclerosis, Primary Lateral

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