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Efficacy of apheresis in antibody-negative progressive encephalomyelitis with rigidity and myoclonus
Yohei Aoki1, Mayumi Sakata1, Hidekazu Suzuki2
1Department of Neurology, Wakayama Medical University, Wakayama, Japan.
Introduction:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is characterized by brainstem symptoms, muscle rigidity, and myoclonus. While autoantibodies to inhibitory neurons have been associated with the pathology, about 30% of cases are negative for autoantibodies. There are few reported cases of antibody-negative PERM and its clinical course and prognosis are unknown.
Methods:
We report three cases of antibody-negative PERM in which plasma exchange was effective.
Results:
Case 1 was a 68-year-old man, case 2 was a 27-year-old woman, and case 3 was a 47-year-old woman. In all three cases, steroid pulse therapy and intravenous immunoglobulin had limited effect and plasma exchange was markedly effective. All patients experienced disease relapse, which was effectively treated with plasma exchange.
Conclusion:
In the present cases of antibody-negative PERM, plasma exchange was most effective and was maintained long-term despite repeated relapses. Plasma exchange should be actively chosen in antibody-negative PERM.
Insights
Plasma exchange effectively treated antibody-negative Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM). This treatment was successful even with relapses, suggesting it should be considered for PERM cases lacking autoantibodies.
Area of Science:
- Neurology
- Immunology
- Neuroimmunology
Background:
- Progressive encephalomyelitis with rigidity and myoclonus (PERM) presents with brainstem symptoms, rigidity, and myoclonus.
- Autoantibodies to inhibitory neurons are linked to PERM, but approximately 30% of cases are antibody-negative.
- Limited data exists on the clinical course and prognosis of antibody-negative PERM.
Purpose of the Study:
- To investigate the efficacy of plasma exchange in antibody-negative PERM.
- To describe the clinical course and treatment outcomes in antibody-negative PERM cases.
Main Methods:
- Case series reporting on three patients with antibody-negative PERM.
- Evaluation of treatment responses to plasma exchange, steroid pulse therapy, and intravenous immunoglobulin.
Main Results:
- Plasma exchange demonstrated marked effectiveness in all three antibody-negative PERM cases.
- Steroid pulse therapy and intravenous immunoglobulin showed limited efficacy.
- Patients experienced relapses, which were successfully managed with plasma exchange.
Conclusions:
- Plasma exchange was the most effective treatment for antibody-negative PERM in these cases.
- Long-term plasma exchange was beneficial despite recurrent disease relapses.
- Plasma exchange should be proactively considered for antibody-negative PERM.
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