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Updated: Aug 17, 2025
Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
Jing Yuan1, Aihua Wang1, Yunfeng Hou2
1Department of Neurology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, China.
Abstract:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disease of unknown etiology, and most patients with PERM are positive for anti-glycine receptor (GlyR) antibody. In this case study, we report a clinical case of a varicella-zoster virus-infected patient who developed anti-GlyR antibody-positive PERM. He initially suffered from herpes zoster and gradually developed symptoms of impaired brainstem functions including hoarse voice and dysphagia, accompanied by paroxysmal sympathetic hyperactivity. The patient also suffered from severe spasms, which were easily triggered by external stimuli. Glycine receptor antibodies were then found to be positive in serum and cerebrospinal fluid, and the diagnosis of PERM was confirmed. Methylprednisolone and gamma globulin treatments were given, and spasms were improved after treatment. Unfortunately, the patient's family insisted on automatic discharge and the patient passed away several days later.
Insights
This case study details a patient with varicella-zoster virus infection who developed anti-glycine receptor (GlyR) antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM). Treatment improved spasms, but the patient later passed away.
Area of Science:
- Neurology
- Immunology
- Virology
Background:
- Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder.
- PERM is often associated with anti-glycine receptor (GlyR) antibodies.
- The etiology of PERM remains largely unknown.
Observation:
- A patient with varicella-zoster virus (VZV) infection developed PERM.
- Symptoms included brainstem dysfunction (hoarse voice, dysphagia), sympathetic hyperactivity, and severe spasms.
- Anti-GlyR antibodies were detected in serum and cerebrospinal fluid.
Findings:
- The patient was diagnosed with anti-GlyR antibody-positive PERM secondary to VZV infection.
- Treatment with methylprednisolone and gamma globulin led to improvement in spasms.
- Despite treatment, the patient experienced a fatal outcome after early discharge.
Implications:
- This case highlights a potential link between VZV infection and anti-GlyR antibody-mediated PERM.
- It underscores the importance of recognizing VZV as a potential trigger for autoimmune neurological disorders.
- Early diagnosis and management of anti-GlyR antibody-positive PERM are crucial, even in the context of viral infections.
