Stomach and Intestine(Tokyo) Volume 41, Issue 12 (November 2006)

Satoyoshi Syndrome with Gastrointestinal Lesions:With Special Reference to the Gastrointestinal Manifestation, Clinical Features, Histopathological Findings of Gastrointestinal Lesions, Report of Two Cases Takashi Nagahama 1 , Toshiyuki Matsui 1 , Hiroshi Tanabe 2 , Akinori Iwashita 2 , Kenshi Yao 1 , Yasuhiro Takaki 1 , Fumihito Hirai 1 , Suketo Sou 1 , Takashi Hisabe 1 , Sumio Tsuda 1 , Kaname Ohshige 2 , Tatsuhiro Oishi 3 , Eijiro Satoyoshi 4 1Department of Gastroenterology, Fukuoka University Chikushi Hospital, Chikushino, Japan 2Department of Pathology, Fukuoka University Chikushi Hospital, Chikushino, Japan 3Department of Gastroenterology, Oishi Hospital, Japan 4National Center of Neurology and Psychiatry, Japan Keyword: 里吉症候群 , 里吉病 , 全身こむら返り病 , gastroenterocolitis cystica polyposa , 吸収不良症候群 pp.1683-1697
Published Date 2006/11/25
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 We encountered 2 cases of Satoyoshi syndrome with gastrointestinal lesions. The first was a 36-year-old female patient who was referred to our hospital with the chief complaints of severe diarrhea, abdominal pain and nausea. She had universal alopecia and amenorrhea at the time of the initial examination. Examination findings on admission revealed evidence of malabsorption syndrome. Gastrointestinal examination revealed scirrhus-like findings, with poor distention and multiple submucosal protrusions in the stomach including the body and elimination of Kerckring folds, diffusely roughened mucosa, and multiple nodular protrusions extending from the duodenum to the small intestine. Despite obtaining multiple biopsy specimens, no definite histopathological diagnosis could be made. The patient was initiated on parenteral nutrition and treatment with adrenocortical steroid. However, she showed fibrous narrowing of both the small intestine and the large intestine approximately two years later. There was also evidence of sepsis, and the patient died. Histopathological findings on autopsy revealed the presence of gastroenterocolitis cystica polyposa extending across the length of the gastrointestinal tract, excluding the esophagus, and a definitive diagnosis of Satoyoshi syndrome was made. A review of the examination findings at a previous hospital showed that the patient had suffered from spasms of the fingers and of the dorsal muscles. In the subsequent course, muscle spasms had been confirmed only three times.

 The second case was a 39-year-old female patient with severe diarrhea and malabsorption syndrome who was referred to our hospital for thorough investigation. She gave a history of universal alopecia and amenorrhea since the age of 16 years, and she occasionally suffered from spasms of the fingers and legs. A radiogram of the gastrointestinal tract revealed, so-called “pumiceous appearance”, that is, elimination of Kerckring folds, diffuse roughening of the mucosa, and multiple nodular protrusions extending from the duodenum to the small intestine. A gastrointestinal endoscopic examination revealed irregular erythematous and fine granular changes in the mucosa with disseminated white spots in the first and second parts of the duodenum. The above-described findings bore close resemblance to the lesions of the duodenum and small intestine described in the first case. The patient was, therefore, strongly suspected to have Satoyoshi syndrome, and was started on treatment with dantrolene sodium, with clinical improvement of malabsorption syndrome.

 In both cases, the muscle spasms were extremely mild, and the primary presenting features were diarrhea and the malabsorption syndrome.

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41巻12号 (2006年11月)
電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院