Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report

Yasuaki Mizutani; Shunsuke Adachi; Shoko Nakano; Kazutaka Hayashi; Atsuhiro Higashi; Koichi Kikuchi; Toshiki Maeda; Kenichiro Murate; Sayuri Shima; Takahiro Iizuka; Akihiro Ueda; Mizuki Ito; Hirohisa Watanabe

doi:10.1016/j.autneu.2021.102910

Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report

Yasuaki Mizutani, Shunsuke Adachi, Shoko Nakano, Kazutaka Hayashi, Atsuhiro Higashi, Koichi Kikuchi, Toshiki Maeda, Kenichiro Murate, Sayuri Shima, Takahiro Iizuka, Akihiro Ueda, Mizuki Ito, Hirohisa Watanabe

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3 Citations (Scopus)

Abstract

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder. We report a 59-year-old man who presented with progressive encephalomyelitis causing diplopia, bulbar palsy, severe dysautonomia, followed by stiffness and myoclonic cluster. Laboratory tests showed mild pleocytosis, with markedly elevated plasma levels of norepinephrine, epinephrine, and arginine vasopressin. Glycine-receptor antibodies were identified in both serum and CSF. Despite a poor response to methylprednisolone, immunoglobulins, and plasma exchange, α-blocker stabilized dysautonomia. Dysautonomia is presumed to be due to antibody-mediated disinhibited sympathetic hyperactivity; however, this case suggests that concomitant use of α-blocker with immunotherapy may ameliorate dysautonomia.

Original language	English
Article number	102910
Journal	Autonomic Neuroscience: Basic and Clinical
Volume	237
DOIs	https://doi.org/10.1016/j.autneu.2021.102910
Publication status	Published - 01-2022

All Science Journal Classification (ASJC) codes

Endocrine and Autonomic Systems
Clinical Neurology
Cellular and Molecular Neuroscience

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Mizutani, Y., Adachi, S., Nakano, S., Hayashi, K., Higashi, A., Kikuchi, K., Maeda, T., Murate, K., Shima, S., Iizuka, T., Ueda, A., Ito, M., & Watanabe, H. (2022). Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report. Autonomic Neuroscience: Basic and Clinical, 237, Article 102910. https://doi.org/10.1016/j.autneu.2021.102910

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title = "Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report",

abstract = "Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder. We report a 59-year-old man who presented with progressive encephalomyelitis causing diplopia, bulbar palsy, severe dysautonomia, followed by stiffness and myoclonic cluster. Laboratory tests showed mild pleocytosis, with markedly elevated plasma levels of norepinephrine, epinephrine, and arginine vasopressin. Glycine-receptor antibodies were identified in both serum and CSF. Despite a poor response to methylprednisolone, immunoglobulins, and plasma exchange, α-blocker stabilized dysautonomia. Dysautonomia is presumed to be due to antibody-mediated disinhibited sympathetic hyperactivity; however, this case suggests that concomitant use of α-blocker with immunotherapy may ameliorate dysautonomia.",

author = "Yasuaki Mizutani and Shunsuke Adachi and Shoko Nakano and Kazutaka Hayashi and Atsuhiro Higashi and Koichi Kikuchi and Toshiki Maeda and Kenichiro Murate and Sayuri Shima and Takahiro Iizuka and Akihiro Ueda and Mizuki Ito and Hirohisa Watanabe",

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year = "2022",

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doi = "10.1016/j.autneu.2021.102910",

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Mizutani, Y, Adachi, S, Nakano, S, Hayashi, K, Higashi, A, Kikuchi, K, Maeda, T, Murate, K, Shima, S, Iizuka, T, Ueda, A , Ito, M & Watanabe, H 2022, 'Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report', Autonomic Neuroscience: Basic and Clinical, vol. 237, 102910. https://doi.org/10.1016/j.autneu.2021.102910

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T1 - Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM)

T2 - A case report

AU - Mizutani, Yasuaki

AU - Adachi, Shunsuke

AU - Nakano, Shoko

AU - Hayashi, Kazutaka

AU - Higashi, Atsuhiro

AU - Kikuchi, Koichi

AU - Maeda, Toshiki

AU - Murate, Kenichiro

AU - Shima, Sayuri

AU - Iizuka, Takahiro

AU - Ueda, Akihiro

AU - Ito, Mizuki

AU - Watanabe, Hirohisa

PY - 2022/1

Y1 - 2022/1

N2 - Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder. We report a 59-year-old man who presented with progressive encephalomyelitis causing diplopia, bulbar palsy, severe dysautonomia, followed by stiffness and myoclonic cluster. Laboratory tests showed mild pleocytosis, with markedly elevated plasma levels of norepinephrine, epinephrine, and arginine vasopressin. Glycine-receptor antibodies were identified in both serum and CSF. Despite a poor response to methylprednisolone, immunoglobulins, and plasma exchange, α-blocker stabilized dysautonomia. Dysautonomia is presumed to be due to antibody-mediated disinhibited sympathetic hyperactivity; however, this case suggests that concomitant use of α-blocker with immunotherapy may ameliorate dysautonomia.

AB - Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder. We report a 59-year-old man who presented with progressive encephalomyelitis causing diplopia, bulbar palsy, severe dysautonomia, followed by stiffness and myoclonic cluster. Laboratory tests showed mild pleocytosis, with markedly elevated plasma levels of norepinephrine, epinephrine, and arginine vasopressin. Glycine-receptor antibodies were identified in both serum and CSF. Despite a poor response to methylprednisolone, immunoglobulins, and plasma exchange, α-blocker stabilized dysautonomia. Dysautonomia is presumed to be due to antibody-mediated disinhibited sympathetic hyperactivity; however, this case suggests that concomitant use of α-blocker with immunotherapy may ameliorate dysautonomia.

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Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report

Abstract

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