Evidence map›Paper›PMID 42045869›Full record

ArticleBMC neurology2026

Acute onset of progressive encephalomyelitis with rigidity and myoclonus complicated by paroxysmal respiratory failure: a case report.

Haiya Deng, Shujin Tang, Jiali Wen, Yuhua Fan, Jingjing Li

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in BMC neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Haiya Deng *Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, 510080, China.
Shujin Tang *Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, 510080, China.
Jiali WenDepartment of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, 510080, China.
Yuhua Fan *Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, 510080, China. fanyuhua@mail.sysu.edu.cn.
Jingjing Li *Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, 510080, China. lijj7@mail.sysu.edu.cn.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundProgressive encephalomyelitis with rigidity and myoclonus (PERM) is a variant form of stiff person spectrum disorders. Paroxysmal respiratory failure and paroxysmal sympathetic hyperactivity syndrome (PSH) had rarely been reported in PERM cases. CASE PRESENTATION: We report a PERM case with acute onset of limb stiffness followed by rapid progression to severe sound-induced myoclonus with respiratory failure (oxygen saturation decreased to 30% with generalized cyanosis). The patient also exhibited ptosis, restricted rightward and downward eye movement, peripheral facial paralysis, and PSH. Anti-glycine receptor (GlyR) antibodies were detected in both serum and cerebrospinal fluid. Brain MRI was normal, while cervical MRI at the upper cervical segment showed linear lesions. The aforementioned symptoms significantly improved after intravenous methylprednisolone, intravenous immunoglobulin, plasma exchange, and other symptomatic treatments such as phenobarbital. We summarized the patient's clinical presentations, cerebrospinal fluid and other laboratory test results, EEG, therapy, and 1-year follow-up. We also reviewed the literature and summarized the forms of onset of PERM, the presence of respiratory failure, and the effect on plasma exchange.

conclusionsWe describe a unique case of PERM characterized by acute onset, anti-GlyR antibodies positivity, PSH, and respiratory failure due to severe myoclonus. Early recognition and immunotherapy including plasma exchange may improve prognosis.

Indexed as

EncephalomyelitisMuscle RigidityMyoclonusRespiratory InsufficiencyAutoantibodiesHumansReceptors, GlycineAutoantibodiesReceptors, GlycineAnti-glycine receptor antibodiesImmunotherapyProgressive encephalomyelitis with rigidity and myoclonusStiff person spectrum disorder

Identifiers

PMID42045869
PMCPMC13267717

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