Decrease in myocardial 123I-MIBG radioactivity in REM sleep behavior disorder: two patients with different clinical progression

Although decrease in myocardial iodine-123 metaiodobenzylguanidine ((123)I-MIBG) radioactivity has been reported in patients with rapid eye movement (REM) sleep behavior disorder (RBD), its pathophysiology has not been thoroughly disclosed. We report two RBD patients with differing clinical progression, in whom myocardial (123)I-MIBG scintigraphy was performed. One 69-year-old patient had more than a 20-year history of idiopathic RBD and showed a decrease in myocardial (123)I-MIBG radioactivity. The other 69-year-old patient started to manifest nocturnal behaviors at age 62, then mild parkinsonism at age 68, and showed a similar decrease in myocardial (123)I-MIBG radioactivity both before and after the onset of parkinsonism. These cases suggest that RBD could develop in diverse patterns of clinical progression even if signs of underlying Lewy body pathology are uniformly indicated.     

Myasthenia gravis after allogeneic bone marrow transplantation treated with mycophenolate mofetil monitored by peripheral blood OX40+ CD4+ T cells

A patient who developed myasthenia gravis (MG) 25 months after allogeneic bone marrow transplant was immunologically analyzed. OX40+CD4+ T cells in the peripheral blood prominently increased one month before the onset of MG. CD4/CD8 ratios, usually abnormally inverted in patients with chronic graft-vs.-host disease (cGVHD), showed pseudonormalization during the course of MG. We succeeded in uneventful rapid tapering of prednisolone (PSL) using mycophenolate mofetil (MMF). Monitoring of OX40+CD4+ T cells supported the tapering of PSL and MMF as a marker of cGVHD activity. This case suggested the utility of MMF and monitoring of OX40+CD4+ T cells in the management of cGVHD-associated autoimmune diseases.     

Dehiscence of levator aponeurosis in ptosis after sub-Tenon injection of triamcinolone acetonide

Objective: To report the incidence, intraoperative findings, and surgical outcome of secondary ptosis that developed after a sub-Tenon injection of triamcinolone acetonide (TA).Study Design: Retrospective, cross-sectional study.Participants: One hundred forty-seven cases with a total of 286 sub-Tenon TA injections.Methods: The medical records of 163 eyes of 147 cases treated with a sub-Tenon injection of 10 mg or 20 mg TA were reviewed. The incidence of secondary ptosis (palpebral fissure >2 mm narrower than that of the fellow eye) after a sub-Tenon TA injection was determined. The preoperative levator function and margin reflex distance (MRD) of the affected eyes, and the intraoperative findings in eyes that underwent reconstructive surgery, were evaluated.Results: Eight eyes (5%) developed secondary ptosis after the injection and 6 eyes were treated by reconstructive surgery. The preoperative levator function of the affected eyes did not differ from that of the fellow eyes. Intraoperatively, no septal disruption or fat herniation was noted, but an aponeurotic disinsertion was identified and repaired with an advancement of the leading edge to the anterior tarsal plate. The surgery led to satisfactory results, with improvement of the MRD from −1.3 (SD 1.5) mm preoperatively to 2.3 (SD 0.5) mm postoperatively (p = 0.027). Additional sub-Tenon TA injections were required in 2 eyes after eyelid surgery but the ptosis did not worsen.Conclusions: A sub-Tenon TA injection can occasionally cause ptosis by inducing a disinsertion of the levator aponeurosis. However, surgical reconstruction can lead to successful resolution of the ptosis.     

Valosin‐containing protein immunoreactivity in tauopathies,synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease

Valosin‐containing protein (VCP) is associated with multiple cellular functions, including ubiquitin‐dependent protein degradation. Mutations in VCP are known to cause inclusion body myopathy with Paget's disease and frontotemporal dementia and familial amyotrophic lateral sclerosis (fALS; ALS14), both of which are characterized by trans‐activation response DNA protein 43 (TDP‐43)‐positive neuronal cytoplasmic and nuclear inclusions. Recently, immunoreactivity for fALS‐associated proteins (TDP‐43, fused in sarcoma (FUS), optineurin and ubiquilin‐2) were reported to be present in cytoplasmic and nuclear inclusions in various neurodegenerative diseases. However, the extent and frequency of VCP‐immunoreactive structures in these neurodegenerative diseases are uncertain. We immunohistochemically examined the brains of 72 cases with neurodegenerative diseases and five control cases. VCP immunoreactivity was present in Lewy bodies in Parkinson's disease and dementia with Lewy bodies, and neuronal nuclear inclusions in five polyglutamine diseases and intranuclear inclusion body disease, as well as in Marinesco bodies in aged control subjects. However, other neuronal and glial cytoplasmic inclusions in tauopathies and TDP‐43 proteinopathies were unstained. These findings suggest that VCP may have common mechanisms in the formation or degradation of cytoplasmic and nuclear inclusions of neurons, but not of glial cells, in several neurodegenerative conditions.     

Temporal change in human nicotinic acetylcholine receptor after smoking cessation: 5IA SPECT study.

Nicotinic acetylcholine receptors (nAChRs) are of great interest because they are implicated in various brain functions. They also are thought to play an important role in nicotine addiction of smokers. Chronic (-)-nicotine, a nAChR agonist, treatment in mice and rats elicits a dose-dependent increase in nAChRs in the brain. Upregulation of nAChRs in postmortem human brains of smokers has also been reported. However, changes in nAChRs after cigarette smoking cessation in humans are poorly understood. The aim of this study was to detect the dynamic changes of nAChRs after smoking and smoking cessation in the brains of living subjects. METHODS: We performed 5-(123)I-iodo-A-85380 ((123)I-5IA) SPECT on nonsmokers and smokers (n = 16) who had quit smoking for 4 h, 10 d, and 21 d and calculated and compared distribution volumes (Vt) of (123)I-5IA. RESULTS: The binding potential of nAChRs (Vt of (123)I-5IA) in the brains of smokers decreased by 33.5% +/- 10.5% after 4 h of smoking cessation, increased by 25.7% +/- 9.2% after 10 d of smoking cessation, and decreased to the level of nonsmokers after 21 d of smoking cessation. CONCLUSION: Because the upregulation of the nAChRs of the smokers after chronic exposure of the nicotine was downregulated to the nonsmokers' level by around 21 d after smoking cessation, the upregulation is a temporary effect. The decrease in nicotinic receptors to nonsmoker levels may be the breaking point during the nicotine withdrawal period.