RT Journal Article
SR Electronic
T1 Longitudinally Extensive Myelitis Associated With Immune Checkpoint Inhibitors
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e967
DO 10.1212/NXI.0000000000000967
VO 8
IS 3
A1 Alberto Picca
A1 Giulia Berzero
A1 Kevin Bihan
A1 Vincent Jachiet
A1 Edouard Januel
A1 Marc Coustans
A1 Cecile Cauquil
A1 Julie Perrin
A1 Pablo Berlanga
A1 Nora Kramkimel
A1 Bethsabée Garel
A1 Perrine Devic
A1 François Ducray
A1 Marion Benazra
A1 Flavie Bompaire
A1 Delphine Leclercq
A1 Jean-Marie Michot
A1 Samy Ammari
A1 Dimitri Psimaras
YR 2021
UL http://nn.neurology.org/content/8/3/e967.abstract
AB Objective To define the characteristics and the outcome of myelitis associated with immune checkpoint inhibitors (ICIs).Methods We performed a retrospective research in the databases of the French Pharmacovigilance Agency and the OncoNeuroTox network for patients who developed myelitis following treatment with ICIs (2011–2020). A systematic review of the literature was performed to identify similar cases.Results We identified 7 patients who developed myelitis after treatment with ICIs (anti-PD1 [n = 6], anti-PD1 + anti-CTLA4 [n = 1]). Neurologic symptoms included paraparesis (100%), sphincter dysfunction (86%), tactile/thermic sensory disturbances (71%), and proprioceptive ataxia (43%). At the peak of symptom severity, all patients were nonambulatory. MRI typically showed longitudinally extensive lesions, with patchy contrast enhancement. CSF invariably showed inflammatory findings. Five patients (71%) had clinical and/or paraclinical evidence of concomitant cerebral, meningeal, caudal roots, and/or peripheral nerve involvement. Despite the prompt discontinuation of ICIs and administration of high-dose glucocorticoids (n = 7), most patients needed second-line immune therapies (n = 5) because of poor recovery or early relapses. At last follow-up, only 3 patients had regained an ambulatory status (43%). Literature review identified 13 previously reported cases, showing similar clinical and paraclinical features. All patients discontinued ICIs and received high-dose glucocorticoids, with the addition of other immune therapies in 8. Clinical improvement was reported for 10 patients.Conclusion Myelitis is a rare but severe complication of ICIs that shows limited response to glucocorticoids. Considering the poor functional outcome associated with longitudinally extensive myelitis, strong and protracted immune therapy combinations are probably needed upfront to improve patient outcome and prevent early relapses.CTLA4=cytotoxic T-lymphocyte-associated protein 4; ICI=immune checkpoint inhibitor; irAE=immune-related adverse event; NSCLC=non–small-cell lung cancer; PD-1=programmed death 1; PDL-1=programmed death-ligand 1