色素沈着・剛毛・浮腫・入型IgAの増量を呈した多発性神経炎の1症例における内分泌機能異常

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A syndrome which is known as plasma cell dyscrasia with polyneuropathy and various endocrine manifestations or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein and skin changes is very interesting because this syndrome has miscellaneous manifestations such as skin hyperpigmentation, hypertrichosis, polyneuropathy, M protein abnormality, plasma cell dyscrasia and endocrine disturbances. Miscellaneous endocrine abnormalities which have not been described so far are reported here. A 47 year old female was admitted with the chief complaints of edema and gait disturbance. Past and family histories were noncontributory. In April 1981, edema appeared in her face and legs. In June she noticed paresthesia in her legs. Edema increased gradually and she had difficulty walking. Her skin became pigmented and hairy. In October she was admitted because of polyneuropathy with increased cerebrospinal fluid protein without pleocytosis. Prednisolone was started. Walking improved slightly, but edema and paresthesia remained unchanged. Prednisolone was stopped at the end of the following March. In May 1982, she was admitted for further evaluation of edema and polyneuropathy. The patient was alert and cooperative. On standing the skin of her legs became cyanotic. There was hypertrichosis on the arms and legs. Her fingers were clubbed. A moderate swelling of the cervical lymph nodes was noted. There was mild hepatomegaly without splenomegaly. All tendon reflexes were lost. Plantar response was flexor. Muscular strength diminished mildly. She complained of paresthesia on the soles. Superficial sensation was normal. Vibratory sense decreased mildly. Cerebellar function and cranial nerves were normal. There was no sphincter disturbance. The examination of urine, stool and peripheral blood was normal. The repeatedly examined value of total cholesterol, total serum protein, CPK and choline esterase decreased. A mild increase in IgA (lambda type) and lambda type light chain was immunoelectrophoretically shown. Serum IgG were normal. Increased protein without pleocytosis was noted in the cerebrospinal fluid. Motor and sensory conduction velocities in the median nerve slowed. The evoked potential was not obtained in the sural nerve. Amyloid deposit could not be observed in the rectal biopsy. Myelin destruction (segmental demyelination) with relatively intact axon was microscopically and electron-microscopically shown in the sural nerve. The number of myelinated fibers was preserved. Antinuclear antibody (homogeneous pattern) was mildly positive. Endocrinologically, T3 was low and rT3 increased. The basal level of TSH was slightly increased and TSH was also mildly hyperresponsive for TRH. Elevated plasma ACTH and low urinary excretion of 17KS were present. Plasma DOC, lldeoxycortisol, corticosterone and serum DHEA-S decreased. A staircase increase of 17OHCS was observed for ACTH stimulation for three days. The circadian rhythm of plasma cortisol was reduced. These findings suggested the presence of latent adrenocortical failure. But whether this failure was due to the syndrome itself or the result of using prednisolone could not be concluded. It will be necessary to accumulate evidence and further investigate this syndrome. Urinary excretion of estrogens (E3 and occasionally E2) increased. Plasma testosterone and progesterone were mildly reduced. There was a mildly impaired glucose tolerance with a slowed peak of IRI. Plasma renin activity was low. Plasma aldosterone was normal. PRL was slightly hyperresponsive for TRH. The basal level of LH and FSH was high, and LH and FSH were hyperresponsive for LHRH. Urinary excretion of catecholamines, cPTH, nPTH, calcitonin and alpha MSH were normal. To our knowledge, this is the first report of this syndrome in which miscellaneous endocrine examinations have been performed with abnormal results.
日本内分泌学会の論文

色素沈着・剛毛・浮腫・入型IgAの増量を呈した多発性神経炎の1症例における内分泌機能異常

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