Progressive encephalomyelitis with rigidity: A Taiwanese case and review of literature.
Clin Neurol Neurosurg
; 208: 106807, 2021 Sep.
Article
en En
| MEDLINE
| ID: mdl-34325335
ABSTRACT
INTRODUCTION:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder. However, the outcome is still variable with different serological and tumor associations, and the elements to good response with less relapse is yet to be elucidated.METHOD:
We present a case and obtain a literature review of patients with PERM and make comparisons based on different serological groups. We also analyze patients with idiopathic PERM that had detailed medical records.RESULTS:
81 patients were collected and analyzed. The largest group were glycine receptor-antibody (GlyR-Ab)-positive (70%), and the seropositive-GlyR-Ab-negative group had better response to immunotherapy. Malignancy can occur up to 2 years from the presentation of PERM. Among the 18 cases with detailed records, the patients who had good outcome initiate immunotherapy within 2 months from presentation. 9 of the 12 patients who experienced no relapse had non-steroid immunotherapy. The maximal interval time of relapse was 24 months.CONCLUSION:
We recommend tumor surveillance up to 2 years in patients with PERM and early administration of immunotherapies and maintain with non-steroid immunotherapy with or without oral corticosteroid for a minimum of 2 years to reduce the risk of relapse in GlyR-Ab-positive patients.Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Autoanticuerpos
/
Receptores de Glicina
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Encefalomielitis
/
Rigidez Muscular
Límite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
Clin Neurol Neurosurg
Año:
2021
Tipo del documento:
Article
País de afiliación:
Taiwán