Short communicationA case of autoimmune autonomic ganglionopathy with prolonged delirium
Introduction
Delirium is common syndrome among elderly people with physical comorbidities, and it is associated with greater mortality risk and major burden to health care cost (Inouye, 2006; Witlox et al., 2010). The care of the patients with delirium is often difficult, owing to their multiple predisposing and precipitating factors (Inouye, 2006; Martins and Fernandes, 2012; Witlox et al., 2010). Prescription of antipsychotic drugs is occasionally required for acute severe agitation (Inouye, 2006; Oh et al., 2017), and some patients showed complex medical conditions for overlapping symptoms of physical disorders and side effects of antipsychotic drugs.
Autoimmune autonomic ganglionopathy (AAG) is a rare acquired immune-mediated disorder that leads to autonomic failure (Vernino et al., 2000). This disorder is associated with autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR) (Vernino et al., 2008). In its typical course, patients with AAG present severe panautonomic dysfunction including orthostatic hypotension, irregular heart rate, abnormal perspiration, dysuria, and constipation (Nakane et al., 2015; Vernino et al., 2008).
AAG should be more widely recognized in psychiatric field because it is sometimes complicated by mental and behavioral symptoms (Hayashi and Ishii, 2009; Nakane et al., 2017). It is especially important to accumulate case information from Asian countries because some Japanese AAG cases with mental symptoms have been reported (Hayashi and Ishii, 2009; Nakane et al., 2017). Here, we report a case of a patient with AAG who was admitted to our psychiatric department for prolonged severe delirium following appearance of serious autonomic dysfunction involving syncope, bradycardia, and dysuria.
Section snippets
Case report
A 72-year-old male had medical history of hypertension, diabetes mellitus and paroxysmal atrial fibrillation. He had worked as an office worker and retired at the age of 65. No cognitive decline interfered with his daily living. When he was 72 years-old, loss of consciousness sometimes occurred when in an upright position. Sudden dyspnea appeared at home within a month, and he was admitted to the hospital for two weeks with diagnosis of bradycardic heart failure. Approximately six weeks later,
Discussion
This case of severe prolonged delirium and comorbidity of AAG was diagnosed during the patient’s general care in hospital. Repeated loss of consciousness attributed to severe orthostatic hypotension disturbed the patient’s recovery from delirium. Intracerebral hemorrhage occurring during hospitalization may be substantially affected by unstable blood pressure due to AAG. This case suggests the importance of differential diagnosis of AAG in patients with mental and behavioral symptoms
Role of funding source
None.
Conflict of interests
The authors declare that there are no conflicts of interest regarding the publication of this paper.
Acknowledgement
We thank Benjamin Phillis from Wakayama Medical University for proofreading and editing the manuscript.
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Antibodies to the α3 subunit of the ganglionic-type nicotinic acetylcholine receptors in patients with autoimmune encephalitis
2020, Journal of NeuroimmunologyCitation Excerpt :It remains unclear whether Abs are causative antibodies for autonomic dysfunction in AE. However, we previously reported the case of AAG presenting with acute encephalitis and case of AAG with prolonged severe delirium (Kuki et al., 2016; Uenishi et al., 2019), and we identified approximately 30% of AAG patients had CNS involvements i.e. personality change, cognitive impairment, and encephalopathy (Nakane et al., 2017; Nakane et al., 2018; Nakane et al., 2020). Nicotinic AChRs belong to the ligand-gated ion channel super-family and participate in many critical physiological reactions.
gAChR antibodies in children and adolescents with acquired autoimmune dysautonomia in Japan
2021, Annals of Clinical and Translational Neurology