Amyotrophic Lateral Sclerosis with a Priority Request for a Postmortem Kidney Donation to a Relative.

The patient was 57 years old when he was diagnosed with amyotrophic lateral sclerosis (ALS) at 1 year after developing bulbar symptoms. At 58 years old, he stated that he was considering donating his kidney to his son suffering from diabetic nephropathy. We confirmed the patient's intentions through repeated interviews before his death at 61 years old. Nephrectomy was performed 30 min after his cardiac death. Organ donation spontaneously proposed by an ALS patient should be considered in order to meet the requests of patients who want their families and other patients to live longer, thereby imparting a beneficial legacy through their deaths.

Long-term outcomes after surgery to prevent aspiration for patients with amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons selectively. In particular, weakness in respiratory and swallowing muscles occasionally causes aspiration pneumonia and choking, which can be lethal. Surgery to prevent aspiration, which separates the trachea and esophagus, can reduce the associated risks. Central-part laryngectomy (CPL) is a relatively minimally invasive surgery to prevent aspiration. No studies have been conducted on the long-term outcomes of surgery to prevent aspiration in patients with ALS. This case series aimed to determine the long-term outcomes of surgery to prevent aspiration and the use of a continuous low-pressure aspirator in patients with ALS by evaluating the frequency of intratracheal sputum suctions performed per day, intra- and postoperative complications, oral intake data, and satisfaction of patients and their primary caregiver to predict improvement in patients' quality of life (QOL). METHODS: We report a case series of six patients with ALS who underwent CPL along with tracheostomy to prevent aspiration between January 2015 and November 2018. We evaluated their pre- and postoperative status and administered questionnaires at the time of last admission to the patients and their primary caregivers. RESULTS: The mean follow-up period after CPL was 33.5 months. Aerophagia was a common postoperative complication. The use of a continuous low-pressure aspirator resulted in reduced frequency of intratracheal sputum suctions. All cases avoided aspiration pneumonia. Oral intake was continued for 2-4 years after the tracheostomy and CPL. The satisfaction levels of the patient and primary caregiver were high. CONCLUSION: Our case series suggests that the use of a continuous low-pressure aspirator in patients undergoing CPL improves oral intake and reduces the frequency of intratracheal sputum suctions, which improves the QOL of patients with ALS and their families and caregivers. CPL and continuous low-pressure aspiration should be considered as a management option for ALS with significant bulbar and respiratory muscle weakness/dysfunction.

Rapid Treatment of Acute Ischemic Stroke Using a Computed Tomography-Based Reperfusion Protocol: The Reality of a Local Community Hospital with Limited Resources.

Objective: In patients with acute ischemic stroke (AIS), prognosis strongly depends on the onset-to-recanalization time. The Ishinomaki protocol for rapid recanalization has been used since October 2017. This protocol determines the indication for reperfusion therapy based on computed tomography (CT)/three-dimensional CT angiography (3DCTA) findings and intends to reduce the onset-to-recanalization time. We aimed to compare the outcomes before and after protocol introduction. Methods: Our hospital is the only thrombectomy-capable center in Ishinomaki, Tome, and Kesennuma medical area. Before protocol introduction (April 2014-June 2016), both CT and magnetic resonance imaging were performed to determine the indications for intravenous (IV) recombinant tissue-plasminogen activator (rt-PA) or mechanical thrombectomy within 6 hours of disease onset. However, after protocol introduction (from October 2017), plain CT and 3DCTA were used. We collected data on patients who underwent mechanical thrombectomy and/or IV rt-PA before (n = 13) and after (n = 34) the protocol introduction. The required time from onset to door (OTD), door to needle (DTN), needle to puncture (NTP), puncture to recanalization (PTR), and door to recanalization (DTR) were compared before and after protocol introduction. Furthermore, thrombolysis in cerebral infarction (TICI) grades and modified Rankin scale (mRS) scores at discharge were compared. Results: The outcomes before and after protocol introduction were as follows: OTD: 105 ± 73.8 (mean ± standard deviation) vs. 120 ± 68.1 min (p = 0.376, Mann-Whitney U test); DTN: 62.9 ± 15.9 vs. 41 ± 17 min (p <0.01); NTP: 112 ± 69.8 vs. 39.9 ± 33.7 min (p <0.01); PTR: 87.9 ± 45.4 vs. 52.5 ± 27.9 min (p <0.01); and DTR, 230 ± 69.9 vs. 110 ± 40.3 min (p <0.0001). Before and after protocol introduction, the proportion of patients with TICI grade 2b-3, mRS score of 0-2 at discharge, and mRS score of 5-6 were 54% vs. 50% (p = 0.815, Fisher's exact test), 23% vs. 21% (p = 0.854), and 15% vs. 50% (p = 0.046), respectively. Conclusion: The Ishinomaki protocol reduced the mean DTR time by 120 min. The reduction in treatment time was due to the change in CT-based recanalization and collaboration with emergency physicians and paramedics. There was no increase in good outcomes, but there was a significant increase in poor outcomes at discharge. Patients who could not be salvaged were indicated for reperfusion therapy as CT and 3DCTA cannot detect the ischemic core.

Hybrid Assistive Limb® for sporadic inclusion body myositis: A case series.

We evaluated the efficacy of rehabilitation therapy with Hybrid Assistive Limb® (HAL; hereafter HAL therapy) in three patients diagnosed with sporadic inclusion body myositis (sIBM) who were hospitalized to undergo HAL therapy. Among them, one patient participated in eight courses and the other two in two courses of HAL therapy between 2017 and 2020. We determined the mean rate of improvement in two-minute walking distance and 6 m walking speed at the time of hospital discharge. After HAL therapy, we confirmed the patients' desire to continue the use of HAL. In one patient, we observed improvements of 146.0% and 120.0% in two-minute walk and 6 m walking speed, respectively, after the first course of HAL therapy; these values are 133.7% and 130% after the eighth course of HAL therapy. These values exceeded 90% in the other two patients after the second course of HAL therapy. HAL therapy maintained both quantity and quality of ambulation and showed positive psychological effects on patient conditions because it reduces exercise load and facilitates safety. While HAL therapy might be effective in maintaining and improving ambulation in patients with sIBM, we should consider to discontinue HAL therapy as it increased risk of falling.

An Acute Case of Granulomatous Amoebic Encephalitis-Balamuthia mandrillaris Infection.

A 74-year-old woman who exhibited drowsiness was referred to our hospital. Enhanced head magnetic resonance imaging (MRI) revealed multiple ring-enhancing lesions and lesions showing partial mild hemorrhaging. The patient gradually progressed to a comatose condition with notable brain deterioration of unknown cause on follow-up MRI. On day nine, the patient inexplicably died, although brain herniation was suspected. Autopsy and histopathology revealed numerous amoebic trophozoites in the perivascular spaces and within the necrotic tissue. Brain immunostaining tested positive for Balamuthia mandrillaris. Infection due to free-living amoeba is rare in Japan; however, it may increase in the near future due to unknown reasons.