Rapidly progressive cognitive impairment resulting in heavy psychosocial burden in a patient with Fabry disease undergoing hemodialysis: a case report.

BACKGROUND: Long-term enzyme replacement therapy (ERT) may improve prognosis in the patients with Fabry disease (FD), however, detail psychosocial burden has not been focused on long life expectancy. We experienced a male case of FD under ERT, he was placed on hemodialysis and presented rapidly progressive cognitive function. CASE PRESENTATION: A 51-year-old male patient with FD has been receiving ERT from age of 38 years. Hemodialysis was initiated at the age of 47 years. The patient experienced several attacks of cerebral infarction, and brain images demonstrated wide-spread asymptomatic ischemic lesions. His behavior became problematic at the age of 51 years. He often exhibited restlessness during hemodialysis sessions and failure to communicate effectively. The patient experienced impairment of attention and executive function, topographical disorientation, and amnesia. Consequently, it was necessary for medical staff and family members to monitor his behavior for safe extracorporeal circulation and daily life activities. Annual standardized neuropsychiatric testing revealed worsening of cognitive performance. CONCLUSIONS: Despite treating with long-term ERT, it is necessary to determine the psychosocial burden derived from the progression of cognitive impairment in patients with FD undergoing hemodialysis.

[A Case of Cerebral Tuberculoma].

Cerebral tuberculoma is a rare and serious form of tuberculosis. Despite advancements in imaging and laboratory diagnostics, it is challenging to diagnose cerebral tuberculoma due to its insidious nature and nonspecific findings. A 56-year-old woman was referred to our hospital for headaches. The patient had previously undergone treatment for pulmonary tuberculosis, which had been completely cured 2 months prior to presentation. Brain MRI revealed an enhanced mass lesion with surrounding edema in the right frontal lobe. Although a mild increase in the serum carcinoembryonic antigen(CEA)level and a moderate accumulation of FDG on FDG-PET indicated inflammatory changes or a malignant brain tumor, other imaging and laboratory findings were nonspecific. The mass lesion was indistinguishable from a brain tumor. Hence, the patient underwent surgical removal, and the pathological diagnosis was tuberculoma. In patients with a history of tuberculosis, cerebral tuberculoma should be considered in the differential diagnosis of intracranial mass lesions, even if the original lesion is completely cured. A mild increase in the serum CEA level and a moderate accumulation of FDG on FDG-PET were considered indicative of intracranial inflammation and consistent with cerebral tuberculoma.

[Postpartum chronic subdural hematoma following spinal anesthesia: case report].

Chronic subdural hematoma is a rare complication of epidural anesthesia. This report describes the case of a 34-year-old woman who presented with postpartum headache after she received epidural anesthesia for labor pain. The anesthesiologist's record did not show any anesthesia-related complication. Two days after the delivery (on day 2), the patient complained of headache. Postdural puncture headache was diagnosed, so she was administered analgesics, hydration therapy, and bed rest. On day 4, she reported a slight improvement and was discharged. The postural headache persisted even on day 7 after delivery. During the conservative treatment, she had suffered a mild head trauma. On day 13, she started to feel a non-postural and severe throbbing headache. On day 24, she was referred to our department. Bilateral chronic subdural hematoma was confirmed by a computed tomography scan. Physical examination revealed only mild right hemiparesis. Left burr hole trepanation was performed and this was followed by uneventful postoperative course. Right chronic subdural hematoma was managed by conservative treatment, and it completely recovered after 4 weeks. Chronic subdural hematoma should be considered when postpartum patients who have received epidural anesthesia present with mild to severe, persistent, and non-postural headache.

Rehabilitation improves prognosis and activities of daily living in hemodialysis patients with low activities of daily living.

BACKGROUND: Activities of daily living (ADL) in aged hemodialysis patients decrease by many factors as hemodialysis therapy, various disease-related complications and underlying disease for rehabilitation. But the correlation between low ADL and mortality remains unclear. We assessed the levels of ADL and effects of rehabilitation in hemodialysis patients with low ADL. Moreover, the association between the baseline functional independence measure (FIM) or rehabilitation treatment effects and all-cause mortality were investigated. METHODS: This prospective cohort study included 182 inpatients on maintenance hemodialysis, who underwent rehabilitation for a decline in ADL. Before and after initiating rehabilitation, ADL were assessed using FIM. RESULTS: The total baseline FIM was 65.1±26.9 (motor items: 39.5±18.7; cognitive items: 25.6±10.7). After rehabilitation, the total FIM increased to 77.1±33.1 (motor items: 50.9±24.4; cognitive items: 26.1±10.8). The baseline FIM, presence or absence of FIM increase, and albumin were significantly associated with mortality. Moreover, the mortality hazard ratio in patients with FIM ≤67 and no FIM increase was 20-fold significantly higher than that in patients with FIM ≥68 and FIM increase. The cognitive items and albumin were significantly associated with the rehabilitation effects in multivariate analysis. CONCLUSIONS: Although the FIM decreased by half in hemodialysis patients, rehabilitation improved their FIM (particularly the motor items). The FIM was a novel predictive marker of 3-year mortality in these patients, and an increased FIM after rehabilitation resulted in better prognosis. Moreover, the effectiveness of rehabilitation may depend on maintaining cognitive functions.