Use of plasmapheresis in acquired demyelinating syndromes

Background: Patients with acute inflammatory demyelination of the central nervous system (CNS) may present with severe neurological impairment, including flaccid quadriparesis and amaurosis. Plasmapheresis (PLEX) is an alternative treatment for patients who do not immediately improve clinically or for whom symptoms worsen despite corticosteroid dosing and is preferred in the context of serious events.

Objective: Describe the profile of the patients with demyelinating diseases that were submitted to PLEX from July 2012 until July 2022 in a tertiary center in the city of Sao Paulo.

Methods: Retrospective cohort study of patients <18 years with acute CNS demyelinating events seen at a single tertiary referral center who received PLEX as second- or third-line therapy between 2010 and 2022. Through chart review of clinical notes.

Results: Total of 80 patients who received diagnosis of demyelinating disease: Multiple Sclerosis (MS), Acute Disseminated Encephalomyelitis (ADEM), Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), Neuromyelitis Optica Spectrum Disorder (NMOSD) or optic neuritis (NO), 18 were to PLEX. From a total of 18 patients, the most prevalent diagnosis was MS, with 7 patients, followed by NMOSD with 5 patients, MOGAD 3 patients, ADEM 1 patient and 2 patients that presented a NO bilateral, that so far did not fulfil a specific disorder. The youngest patient submitted was 5 years old, and the oldest were 16. From the 18 patients, 11 were in its first clinical event. All received at least 5 days of metilprednisolone as first line therapy. The clinical neurology syndrome was 5 with NO bilateral, 3 with NO unilateral, 6 with myelitis and 4 patients with more then 1 syndrome (myelitis with NO or with a stem brain syndrome). Only one was submitted to PLEX more then once. None of our patients presented severe complications related to plasmapheresis, and all of them showed some improvement.

Conclusions: Demyelinating diseases acute events are potential cause of sequelae in young patients and sometimes require more aggressive therapeutics in order to prevent amaurosis or severe motor disfunction. Access to PLEX is not an easily available, and require trained personel, as the limitations are also related with weight and access to ICU. There is room for improvements over clinical protocols and categorization of patients eligible for PLEX.

 

Scroll to top

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
18 September 2023