Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of encephalopathy showing various psychiatric and autonomic symptoms with positive anti-ganglionic acetylcholine receptor (gAChR) antibody

Yukiko Ohkubo, M.D.1), Attila Mori, M.D.2), Tomoo Nakayama, M.D.1), Susumu Chiba, M.D., Ph.D.1) and Shunya Nakane, M.D., Ph.D.3)

1)Department of Neurology, Sapporo Nishimaruyama Hospital
2)Department of Neurology, Ibaraki Rehabilitation Hospital
3)Department of Molecular Neuroscience and Therapeutics, Kumamoto University Hospital

An 84-year-old woman developed spontaneous recurring mutism. During the periods in which she was able to speak, she described that she had a peculiar delusion where her body was melting away. She did not obey orders although she was able to move her limbs spontaneously. Severe fluctuations in blood pressure measurements were observed; they were unaffected by postural changes. She also had urinary retention and constipation. Her psychiatric and autonomic symptoms showed marked daily and diurnal fluctuations. The brain MRI showed no abnormality in the limbic system or temporal lobes. The cerebrospinal fluid showed slightly elevated protein with normal cells counts. This case was initially thought to be an encephalopathy of unknown etiology. On subsequent testings she was shown to have positive antiganglionic acetylcholine receptor (gAChR) antibodies. Although the initial steroid pulse and intravenous immunoglobulin therapies markedly improved both psychiatric and autonomic symptoms, they turned ineffective in subsequent recurrences. We were not able to treat her with plasmapheresis or with other immunisuppressive drugs because of her poor general status, thus their effectiveness could not be determined. Judging from her clinical course, in which immunotherapy was effective although somewhat limited, a possible involvement of an autoimmune mechanism was suspected; however, the exact pathogenesis remains undetermined. It is possible that in this case there may have been an involvement of the immune system and that the patient might have had an encephalopathy with anti-gAChR antibodies.
Full Text of this Article in PDF (1367K)

(CLINICA NEUROL, 59: 631|635, 2019)
key words: anti-ganglionic acetylcholine receptor (gAChR) antibody, encephalopathy, psychiatric symptoms, autonomic symptoms

(Received: 4-Feb-19)