Successful treatment with immunoadsorption therapy in four patients with severe and refractory
Yu Yang1, Bingjun Zhang1, Min Li1
1Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong Province, China.
Abstract:
There is still no optimal treatment for patients with severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis refractory to first-line therapy (including intravenous methylprednisolone [IVMP] and intravenous immunoglobulin [IVIG]). A small study has shown that immunoadsorption (IA) is effective in treating anti-NMDAR encephalitis. However, the effectiveness and safety of IA in the treatment of patients with refractory and severe anti-NMDAR encephalitis is not fully known. Four patients with severe anti-NMDAR encephalitis are reported, which were refractory to the first-line immunotherapy including IVMP and IVIG. Immunoadsorption is performed during the fulminant stage of disease, and the effectiveness and safety of IA are assessed. The modified Rankin Scale (mRS) is used to assess neurological conditions before and after IA. Four patients with the most severe form of anti-NMDAR encephalitis (two with teratoma and two with unknown origin) did not respond to one or more rounds of IVMP plus IVIG. They all required intensive care unit (ICU) support including long-term mechanical ventilation, and thus developed ICU-related complications. Gradual and steady improvement was observed after IA treatment. Except for mild hypotension in patient 1, no other adverse events were observed during IA. Two patients had good early overall recovery on discharge. The other two patients had a good outcome with mRS of 2 at the 12-month follow-up. This small case series suggests that IA may be an effective treatment option to accelerate the recovery of patients with severe and refractory anti-NMDAR encephalitis.
Insights
Immunoadsorption (IA) offers a promising treatment for severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis when standard therapies fail. This case series shows IA can accelerate recovery and improve outcomes in refractory NMDAR encephalitis patients.
Area of Science:
- Neuroimmunology
- Neurology
- Immunotherapy
Background:
- Severe anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis refractory to first-line treatments like IVMP and IVIG lacks optimal therapeutic options.
- Immunoadsorption (IA) has shown potential but its efficacy and safety in severe, refractory cases remain incompletely understood.
Observation:
- Four patients with severe, refractory anti-NMDAR encephalitis requiring intensive care and mechanical ventilation were treated with IA during the fulminant stage.
- These patients had not responded to standard immunotherapy, including IV methylprednisolone (IVMP) and IV immunoglobulin (IVIG).
Findings:
- Gradual neurological improvement was observed in all four patients following IA treatment.
- IA was generally safe, with only mild hypotension noted as an adverse event in one patient.
- Two patients showed good early recovery, while the other two achieved a modified Rankin Scale (mRS) score of 2 at 12-month follow-up.
Implications:
- IA may be an effective and safe therapeutic option to expedite recovery in patients with severe, refractory anti-NMDAR encephalitis.
- This approach could potentially reduce ICU stay and associated complications in critically ill patients.
- Further research is warranted to confirm these findings in larger cohorts.
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