Biomaterial Database

[Paraneoplastic diseases of the motor end-plate and muscle]. 2000

M Rosich-Estrago

Servei de Neurologia, Hospital Psiquiàtric Universitari Institut Pere Mata, Ctra. de l'Institut Pere Mata, s/n, E-43206 Reus, Tarragona. mre@ctv.es

BACKGROUND Some forms of myasthenia gravis and many cases of Eaton-Lambert syndrome are disorders of neuromuscular transmission possibly of paraneoplastic origin. Paraneoplastic necrotizing myopathy and some cases of inflammatory myopathy (polymyositis-dermatomyositis) are paraneoplastic myopathies. METHODS These are all autoimmune processes. The diagnosis is clinical, based mainly on the characteristics of the muscle weakness and motor disorders, confirmed by serological methods (demonstration of antibodies) pharmacological and electromyographic tests, by imaging studies and in some cases biopsy. Treatment includes specific symptomatic measures (aimed at correcting the defect in neuromuscular transmission in the myasthenic syndromes, reducing axonal auto-excitability in the neuromuscular hyperexcitability syndromes, etc.) and pathogenic treatment (basically immunosuppression). A search for the hidden tumour should also be part of the diagnostic investigation. The paraneoplastic disease may remit if the associated tumour is treated.

UI	MeSH Term	Description	Entries
D009045	Motor Endplate	The specialized postsynaptic region of a muscle cell. The motor endplate is immediately across the synaptic cleft from the presynaptic axon terminal. Among its anatomical specializations are junctional folds which harbor a high density of cholinergic receptors.	Motor End-Plate,End-Plate, Motor,End-Plates, Motor,Endplate, Motor,Endplates, Motor,Motor End Plate,Motor End-Plates,Motor Endplates
D009157	Myasthenia Gravis	A disorder of neuromuscular transmission characterized by fatigable weakness of cranial and skeletal muscles with elevated titers of ACETYLCHOLINE RECEPTORS or muscle-specific receptor tyrosine kinase (MuSK) autoantibodies. Clinical manifestations may include ocular muscle weakness (fluctuating, asymmetric, external ophthalmoplegia; diplopia; ptosis; and weakness of eye closure) and extraocular fatigable weakness of facial, bulbar, respiratory, and proximal limb muscles. The disease may remain limited to the ocular muscles (ocular myasthenia). THYMOMA is commonly associated with this condition.	Anti-MuSK Myasthenia Gravis,MuSK MG,MuSK Myasthenia Gravis,Muscle-Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle-Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Generalized,Myasthenia Gravis, Ocular,Anti MuSK Myasthenia Gravis,Generalized Myasthenia Gravis,Muscle Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Anti-MuSK,Myasthenia Gravis, MuSK,Ocular Myasthenia Gravis
D009382	Neoplasms, Unknown Primary	Metastases in which the tissue of origin is unknown.	Neoplasm Metastasis, Unknown Primary,Neoplasms, Occult Primary,Occult Primary Neoplasms,Unknown Primary Tumors,Unknown Primary Neoplasm Metastasis,Unknown Primary Neoplasms,Neoplasm, Occult Primary,Neoplasm, Unknown Primary,Occult Primary Neoplasm,Primary Neoplasm, Occult,Primary Neoplasm, Unknown,Primary Neoplasms, Occult,Primary Neoplasms, Unknown,Primary Tumor, Unknown,Primary Tumors, Unknown,Tumor, Unknown Primary,Tumors, Unknown Primary,Unknown Primary Neoplasm,Unknown Primary Tumor
D009468	Neuromuscular Diseases	A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA.	Amyotonia Congenita,Oppenheim Disease,Cramp-Fasciculation Syndrome,Fasciculation-Cramp Syndrome, Benign,Foley-Denny-Brown Syndrome,Oppenheim's Disease,Benign Fasciculation-Cramp Syndrome,Benign Fasciculation-Cramp Syndromes,Cramp Fasciculation Syndrome,Cramp-Fasciculation Syndromes,Fasciculation Cramp Syndrome, Benign,Fasciculation-Cramp Syndromes, Benign,Foley Denny Brown Syndrome,Neuromuscular Disease,Oppenheims Disease,Syndrome, Cramp-Fasciculation,Syndrome, Foley-Denny-Brown,Syndromes, Cramp-Fasciculation
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
D003882	Dermatomyositis	A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)	Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013945	Thymoma	A neoplasm originating from thymic tissue, usually benign, and frequently encapsulated. Although it is occasionally invasive, metastases are extremely rare. It consists of any type of thymic epithelial cell as well as lymphocytes that are usually abundant. Malignant lymphomas that involve the thymus, e.g., lymphosarcoma, Hodgkin's disease (previously termed granulomatous thymoma), should not be regarded as thymoma. (From Stedman, 25th ed)	Carcinoma, Thymic,Carcinomas, Thymic,Thymic Carcinoma,Thymic Carcinomas,Thymomas
D013953	Thymus Neoplasms	Tumors or cancer of the THYMUS GLAND.	Cancer of Thymus,Thymus Cancer,Thymus Tumors,Cancer of the Thymus,Neoplasms, Thymic,Neoplasms, Thymus,Thymic Cancer,Thymic Neoplasms,Thymic Tumors,Cancer, Thymic,Cancer, Thymus,Cancers, Thymic,Cancers, Thymus,Neoplasm, Thymic,Neoplasm, Thymus,Thymic Cancers,Thymic Neoplasm,Thymic Tumor,Thymus Cancers,Thymus Neoplasm,Thymus Tumor,Tumor, Thymic,Tumor, Thymus,Tumors, Thymic,Tumors, Thymus
D015624	Lambert-Eaton Myasthenic Syndrome	An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)	Eaton-Lambert Syndrome,Myasthenic Syndrome, Lambert-Eaton,Eaton-Lambert Myasthenic Syndrome,Lambert-Eaton Syndrome,Myasthenic-Myopathic Syndrome of Eaton-Lambert,Myasthenic-Myopathic Syndrome of Lambert-Eaton,Myopathic-Myasthenic Syndrome of Eaton-Lambert,Myopathic-Myasthenic Syndrome of Lambert-Eaton,Eaton Lambert Myasthenic Syndrome,Eaton Lambert Syndrome,Eaton-Lambert Myasthenic-Myopathic Syndrome,Eaton-Lambert Myopathic-Myasthenic Syndrome,Eaton-Lambert Myopathic-Myasthenic Syndromes,Lambert Eaton Myasthenic Syndrome,Lambert Eaton Syndrome,Lambert-Eaton Myasthenic-Myopathic Syndrome,Lambert-Eaton Myasthenic-Myopathic Syndromes,Lambert-Eaton Myopathic-Myasthenic Syndrome,Lambert-Eaton Myopathic-Myasthenic Syndromes,Myasthenic Myopathic Syndrome of Eaton Lambert,Myasthenic Myopathic Syndrome of Lambert Eaton,Myasthenic Syndrome, Eaton-Lambert,Myasthenic Syndrome, Lambert Eaton,Myopathic Myasthenic Syndrome of Eaton Lambert,Syndrome, Eaton-Lambert,Syndrome, Eaton-Lambert Myasthenic,Syndrome, Lambert-Eaton,Syndrome, Lambert-Eaton Myasthenic