ArticleBMC neurology2018
A case report of rigidity and recurrent lower limb myoclonus: progressive encephalomyelitis rigidity and myoclonus syndrome, a chameleon.
Article in BMC neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 11 citations in OpenAlex.
- A dual manifestation of GAD-antibody spectrum disorder: a case of progressive encephalomyelitis with rigidity, myoclonus and autoimmune epilepsy with mesial temporal sclerosis.BMC neurology · 2026Article
- Case report: Atypical anti-GlyR antibody-positive pediatric progressive encephalomyelitis with rigidity and myoclonus presenting as chronic joint pain.Frontiers in immunology · 2026Article
- Progressive encephalomyelitis with rigidity and myoclonus initially diagnosed as an anxiety disorder associated with anti-glycine receptor antibodies: A case report.The Journal of international medical research · 2025Article
- Diagnosis and Clinical Features in Autoimmune-Mediated Movement Disorders.Journal of movement disorders · 2022Article
- Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies.Internal medicine (Tokyo, Japan) · 2021Article
- Practical approach to respiratory emergencies in neurological diseases.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Review
- Progressive Encephalomyelitis With Rigidity and Myoclonus With Thymoma: A Case Report and Literature Review.Frontiers in neurology · 2020Article
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Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundProgressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome is a rare neurological condition. Its clinical characteristics include axial and limb muscle rigidity, myoclonus, painful spasms and hyperekplexia. Diagnosis of this disease can be very challenging and optimal long-term treatment is unclear. CASE PRESENTATION: We report a case of a 62 year old patient admitted for repetitive myoclonus and rigidity in the lower limbs progressing since 10 years, associated with a fluctuating encephalopathy requiring stays in Intensive Care Unit. Multiple diagnostics and treatment were proposed, unsuccessfully, before the diagnosis of PERM syndrome was established. In association with the clinical presentation, a strong positive result for GAD (glutamic acid decarboxylase) antibodies lead to the diagnosis of PERM syndrome.
conclusionsPERM syndrome is a rare disease and its diagnosis is not easy. Once the diagnosis is established, the correct treatment should follow and could be lifesaving, regardless of a delayed diagnosis. Maintenance of long-term oral corticotherapy is suggested to prevent relapses.
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