A case of Crow-Fukase syndrome with the preservation of pain sensation and a favorable clinical course.

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A 48-year-old woman with Crow-Fukase syndrome suffering from polyneuropathy, generalized dark brown pigmentation of the skin, hirsutism, edema, diabetes mellitus and amenorrhoea is reported. In this case, sensory disturbances of temperature, touch and vibration with grove and stocking type were recognized but only pain sensation was not disturbed.A small amount of M-protein of IgA (λ) and Bence-Jones protein were detected, but myeloma was not found by bone survey with roentogenography, bone and Garium scintigraphy. The cerebrospinal fluid protein was increased initially (150mg/dl), then gradually decreased to the normal level and the symptom of polyneuropathy was gradually improved. On rural nerve biopsy, demyelination in addition to axonal degeneration was shown, and large fibers were mainly disturbed but small fibers were preserved. This fact might be related to the absence of disturbance of pain sensation.Although the patient has not been on prednisolone, the clinical course of this case has been favorable for 3 years.
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