Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.

Background/Aims: Esophageal motility disorders (EMDs) contribute to the pathophysiology of gastroesophageal reflux disease. However, the causes of EMDs and their impact on gastroesophageal reflux disease-associated symptoms remain unknown. This study aims to elucidate clinical features associated with various types of EMDs in patients with heartburn symptoms. Methods: Of the 511 patients who underwent high-resolution manometry, 394 who were evaluated for heartburn symptoms were examined. Patients subjected to high-resolution manometry were classified into 4 groups: outflow obstruction group, hypermotility group, hypomotility group, and normal motility group. Symptoms were evaluated using 3 questionnaires. Patient characteristics and symptoms for each EMD type were compared with those of the normal motility group. Results: Of the 394 patients, 193 (48.9%) were diagnosed with EMDs, including 71 with outflow obstruction, 15 with hypermotility, and 107 with hypomotility. The mean dysphagia score was significantly higher in each of the 3 EMD groups compared with those with normal motility. The mean acid reflux and dyspepsia scores were significantly lower in the outflow obstruction group (P < 0.05). The mean body mass index and median Brinkman index were significantly higher in the hypermotility group (P = 0.001 and P = 0.018, respectively), whereas the mean diarrhea and constipation scores were significantly lower in the hypomotility group (P < 0.05). Conclusions The results of our study indicate that different EMDs have distinct characteristics. Cigarette smoking and high body mass index were associated with esophageal hypermotility. Assessment of the dysphagia symptom scores may help identify patients with EMDs.

Herein, we report a case of a 49-year-old man with a history of bilateral hemiplegia caused by severe traumatic brain injury. During his stay in the convalescent rehabilitation hospital, he developed a flexion deformity of the 4th and 5th fingers of his right hand. Elbow palpation and ultrasonography showed that the ulnar nerve was dislocated from the elbow canal, and a nerve conduction study revealed that the conduction velocity was low in the right elbow. Thus, a diagnosis of cubital tunnel syndrome was made and an ulnar nerve transfer was performed to prevent progression. Prior to onset, an over-table had been used by the patient for daily activities such as sitting, training, and eating. Therefore, it was considered that the repeated use of the over-table plausibly exerted pressure on the dislocated ulnar nerve, leading to the onset of the cubital tunnel syndrome.As over-tables and wheelchair armrests are often used during rehabilitation, it is imperative to pay attention to elbow compression when using them in patients with ulnar nerve dislocation.

Background/Aims: Esophageal motility disorders (EMDs) contribute to the pathophysiology of gastroesophageal reflux disease. However, the causes of EMDs and their impact on gastroesophageal reflux disease-associated symptoms remain unknown. This study aims to elucidate clinical features associated with various types of EMDs in patients with heartburn symptoms. Methods: Of the 511 patients who underwent high-resolution manometry, 394 who were evaluated for heartburn symptoms were examined. Patients subjected to high-resolution manometry were classified into 4 groups: outflow obstruction group, hypermotility group, hypomotility group, and normal motility group. Symptoms were evaluated using 3 questionnaires. Patient characteristics and symptoms for each EMD type were compared with those of the normal motility group. Results: Of the 394 patients, 193 (48.9%) were diagnosed with EMDs, including 71 with outflow obstruction, 15 with hypermotility, and 107 with hypomotility. The mean dysphagia score was significantly higher in each of the 3 EMD groups compared with those with normal motility. The mean acid reflux and dyspepsia scores were significantly lower in the outflow obstruction group (P < 0.05). The mean body mass index and median Brinkman index were significantly higher in the hypermotility group (P = 0.001 and P = 0.018, respectively), whereas the mean diarrhea and constipation scores were significantly lower in the hypomotility group (P < 0.05). Conclusions The results of our study indicate that different EMDs have distinct characteristics. Cigarette smoking and high body mass index were associated with esophageal hypermotility. Assessment of the dysphagia symptom scores may help identify patients with EMDs.

Paraparesis is a rare and serious complication following epidural anesthesia. Abnormal findings on magnetic resonance imaging (MRI) help with the diagnosis；however, diagnosis is challenging in the absence of MRI abnormalities.We present the case of a 26-year-old pregnant woman who received combined spinal-epidural anesthesia for cesarean delivery. The following day, she developed muscle weakness and sensory disturbances in the right lower extremity, which were attributable to the continuous epidural analgesia with ropivacaine that was used during the cesarean delivery. MRI revealed no spinal canal abnormalities；however, skeletal muscle MRI performed in the subacute phase revealed abnormally high signals on T2 and fat suppression T2-weighted imaging in the right paraspinal muscle below the L3 level. Nerve conduction studies revealed reduced amplitude of compound muscle action potentials and sensory nerve action potentials recorded in the lower extremities. After a 4-month period of rehabilitation, the patient could walk independently with a right ankle-foot orthosis and crutch；however, her right leg palsy and sensory disturbances persisted. Here, we report the clinical course of a patient who developed lumbosacral radiculopathy attributable to ropivacaine-induced neurotoxicity. Additionally, we present a review of the literature.

Paraparesis is a rare and serious complication following epidural anesthesia. Abnormal findings on magnetic resonance imaging (MRI) help with the diagnosis；however, diagnosis is challenging in the absence of MRI abnormalities.We present the case of a 26-year-old pregnant woman who received combined spinal-epidural anesthesia for cesarean delivery. The following day, she developed muscle weakness and sensory disturbances in the right lower extremity, which were attributable to the continuous epidural analgesia with ropivacaine that was used during the cesarean delivery. MRI revealed no spinal canal abnormalities；however, skeletal muscle MRI performed in the subacute phase revealed abnormally high signals on T2 and fat suppression T2-weighted imaging in the right paraspinal muscle below the L3 level. Nerve conduction studies revealed reduced amplitude of compound muscle action potentials and sensory nerve action potentials recorded in the lower extremities. After a 4-month period of rehabilitation, the patient could walk independently with a right ankle-foot orthosis and crutch；however, her right leg palsy and sensory disturbances persisted. Here, we report the clinical course of a patient who developed lumbosacral radiculopathy attributable to ropivacaine-induced neurotoxicity. Additionally, we present a review of the literature.

Herein, we report a case of a 49-year-old man with a history of bilateral hemiplegia caused by severe traumatic brain injury. During his stay in the convalescent rehabilitation hospital, he developed a flexion deformity of the 4th and 5th fingers of his right hand. Elbow palpation and ultrasonography showed that the ulnar nerve was dislocated from the elbow canal, and a nerve conduction study revealed that the conduction velocity was low in the right elbow. Thus, a diagnosis of cubital tunnel syndrome was made and an ulnar nerve transfer was performed to prevent progression. Prior to onset, an over-table had been used by the patient for daily activities such as sitting, training, and eating. Therefore, it was considered that the repeated use of the over-table plausibly exerted pressure on the dislocated ulnar nerve, leading to the onset of the cubital tunnel syndrome.As over-tables and wheelchair armrests are often used during rehabilitation, it is imperative to pay attention to elbow compression when using them in patients with ulnar nerve dislocation.

This study sought to elucidate the relationship between units of rehabilitative training time and Functional Independence Measure (FIM) at time of discharge in femoral neck fracture patients. The subjects were 795 patients with femoral neck fracture from 19 hospitals registered in the Japan Rehabilitation Database who satisfied the inclusion criteria. Separating these into 15 hospitals (371 cases) where surgery was performed at outside institutions and 14 hospitals (424 cases) where surgery was performed in-house, multiple regression analysis was performed using six explanatory variables including hospital dummy and training time units, and motor FIM score at discharge as an objective variable. In the outside surgery group, whose training time units ranged from 0.8 to 8.6, the coefficient was not significant. However, it became significant when narrowed to two hospitals comprising more than 50 cases (B=2.187). The in-house surgery group's training time units represented a significantly positive coefficient (1.427). It is thought that if the number of training time units used for patients with femoral neck fractures increases by one unit, then the patient's motor FIM score at discharge will rise about 1.4 to 2 points.