病初期より慢性偽性腸閉塞を合併したmitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes（MELAS）の1例

Re: Comments on “A Case of MELAS With the m.3243A>G Variant of the MT-TL1 Gene Mimicking Acute Intermittent Porphyria”: The Authors Respond

“…Another interesting characteristic of IPO in our patient was that it appeared acutely during hospitalization and so could be immediately released by gastrointestinal decompression, which differs from some previous cases where chronic or subacute IPO occurred in a chronic phase or even represented the first complaint of the patients. 4 5 6 The characteristics of IPO in our patient are consistent with the statement made based on a cohort study in the UK that IPO can occur throughout the entire spectrum of m.3243A>G-related disease. 3 …”

Improvement of Intestinal Pseudo-Obstruction by Total Parenteral Nutrition in a Young Woman With Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes: A Case Report

“…Since then, the patient has received adequate nutritional management and there has been no IPO recurrence. In prior cases of IPO in patients with MELAS, parenteral nutrition was performed [4,[12][13][14]. However, there are no reported cases of early introduction of TPN.…”

“…In patients with MELAS, distigmine bromide [12] and neostigmine [13] have been shown to be effective for treating IPO, while prucalopride has demonstrated efficacy when the condition is characterized by acute onset [14]. There is one report of TPN being effective [6].…”