TY - JOUR
T1 - A juvenile case of Lambert-Eaton myasthenic syndrome with severe emaciation
AU - Nakashima, Ichiro
AU - Kikuchi, Akio
AU - Onodera, Jun Ichi
AU - Shiga, Yusei
AU - Fujihara, Kazuo
AU - Motomura, Masakatsu
AU - Itoyama, Yasuto
PY - 1997/5
Y1 - 1997/5
N2 - Most of the patients with Lambert-Eaton myasthenic syndrome (LEMS) are over 40 years of age at onset and have a malignant neoplasm, especially pulmonary small cell carcinoma. Juvenile cases of LEMS are rare and have not been reported in Japan. We report a 21-year-old woman whose onset of LEMS was at 17 years of age. In the early stage of the disease she had limb muscle weakness and was diagnosed as having seronegative myasthenia gravis (MG) based on decrementing response in low frequency repetitive nerve stimulation, positive edrophonium test, and negative serum anti-acetylcholin receptor antibodies. Serum anti-voltage-dependent-calcium-channel (VGCC) antibody was negative with an available assay system. She was thymectomized and received anti-cholinesterases, corticosteroids, and cyclophosphamide, but their therapeutic effects were not evident. Two years later, she was confined to bed due to severe emaciation (Her body weight reduced from 47Kg to 27Kg.), bulbar palsy, and limb muscle weakness. She needed respiratory support and tube feeding. Type II fiber atrophy, which is non-specific, but is a common finding in LEMS, was seen in the biopsied muscle of the patient. On the other hand, postsynaptic folds and clefts of all seven motor end-plates in the specimen were normal, which is against the diagnosis of MG. Recently, she was found to have anti-VGCC antibody with an improved assay system. Despite vigorous examination, no malignancy or other autoimmune disorders have been found. 3, 4-Diaminopyridine was effective to improve limb muscle powers, but she is still bed-ridden.
AB - Most of the patients with Lambert-Eaton myasthenic syndrome (LEMS) are over 40 years of age at onset and have a malignant neoplasm, especially pulmonary small cell carcinoma. Juvenile cases of LEMS are rare and have not been reported in Japan. We report a 21-year-old woman whose onset of LEMS was at 17 years of age. In the early stage of the disease she had limb muscle weakness and was diagnosed as having seronegative myasthenia gravis (MG) based on decrementing response in low frequency repetitive nerve stimulation, positive edrophonium test, and negative serum anti-acetylcholin receptor antibodies. Serum anti-voltage-dependent-calcium-channel (VGCC) antibody was negative with an available assay system. She was thymectomized and received anti-cholinesterases, corticosteroids, and cyclophosphamide, but their therapeutic effects were not evident. Two years later, she was confined to bed due to severe emaciation (Her body weight reduced from 47Kg to 27Kg.), bulbar palsy, and limb muscle weakness. She needed respiratory support and tube feeding. Type II fiber atrophy, which is non-specific, but is a common finding in LEMS, was seen in the biopsied muscle of the patient. On the other hand, postsynaptic folds and clefts of all seven motor end-plates in the specimen were normal, which is against the diagnosis of MG. Recently, she was found to have anti-VGCC antibody with an improved assay system. Despite vigorous examination, no malignancy or other autoimmune disorders have been found. 3, 4-Diaminopyridine was effective to improve limb muscle powers, but she is still bed-ridden.
KW - Anti-voltage- dependent-calcium-channel antibody
KW - Emaciation
KW - Juvenile onset
KW - Lambert-Eaton myasthenic syndrome
KW - Myasthenia gravis
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M3 - Article
C2 - 9294328
AN - SCOPUS:0030775541
VL - 37
SP - 402
EP - 406
JO - Clinical Neurology
JF - Clinical Neurology
SN - 0009-918X
IS - 5
ER -