Appeal type: Experimental/Investigational
Decision: Upheld

Diagnosis: Nueromyelitis Optica Spectrum Disorder (NMOSD).

Treatment: Gammaglobulin liquid (IVIG) infusion.

The insurer denied Gammaglobulin liquid (IVIG) infusion.

The denial was revered.

This patient was seen by a provider for follow up appointment after a recent hospitalization for neuromyelitis optica spectrum disorder (NMOSD). He noted that the patient had worsening of his neurological symptoms due the insurance delaying his treatment. He noted that the magnetic resonance imaging (MRI) showed enhancing lesions in the brain and spinal cord. He noted that the regimen contained IVIG and Rituximab. It was reported that the patient was severely affected by his condition and was using a walker to walk,  he had speech difficulties and severe disequilibrium with  lower extremities weakness. The patient had ongoing symptoms of diplopia, paresthesia, hypesthesia, fatigue, incoordination, limb weakness, dysarthria, and ataxia. The plan was to continue with IVIG and rituximab. He noted Devic's disease in the assessment. In early 2020, he noted that in mid 2019 the claimant symptoms; hiccup and weakness in the lower extremities with wide based gait were Devic's disease. The plan was to continue with IVIG and Rituximab. 

Per Lunemann, J. D., Quast, I., & Dalakas, M. C. (2016), "Neuromyelitis optica (Devic syndrome), long considered a clinical variant of MS, is now regarded as a distinct clinical entity. In a retrospective study, IVIG was described to be effective in some patients who experienced acute relapses and did not benefit from steroids or plasma exchange therapy [87], and might also be effective as long-term treatment [88]. While these data suggest that IVIG therapy could be an alternative for patients with contraindication to immunosuppressive prophylactic treatments such as azathioprine and rituximab or, particularly, in children, randomized controlled trials are needed to establish the efficacy of IVIG for treatment of relapses and prevention of disease progression in patients with this condition."

Per Viswanathan, S., Wong, A. H., Quek, A. M., & Yuki, N. (2015), "IVIG may be used to reduce the relapse frequency in patients with NMO/NMOSD."

Per Kowarik, M. C., Soltys, J., & Bennett, J. L. (2014), "Intravenous immunoglobulin (IVIG) and cyclophosphamide have also been used to treat acute NMO exacerbations and prevent relapses. IVIg frequently is substituted for PLEX in other neurologic disorders, including myasthenia gravis and GuillainBarre syndrome. In idiopathic ON, however, IVIg has failed to improve the outcome of steroid resistant cases (67,68), suggesting that caution should be exercised before automatically substituting IVIg for PLEX in NMO. Recently, 2 small case series have reported benefit in NMO patients receiving either acute or short-term prophylactic IVIg therapy (69,70), indicating that IVIG therapy may warrant further investigation in NMO (71). For acute exacerbations resistant to IVMP and PLEX, some patients with acute TM have benefited from cyclophosphamide infusion (72). Interestingly, a recent case series has reported lack of efficacy for the use of cyclophosphamide in NMO relapse prevention (73), indicating that distinct immunologic mediators may drive NMO lesion onset and propagation."      

Gammaglobulin liquid (IVIG) infusion is approved for similar diagnosis i.e. antibody positive autoimmune disease and should be available to patients with this condition. This is an auto-antibody disease and using IVIG, in conditions like this, has been proven to be beneficial.     

The patient did not improve with plasma exchange and steroids and he had some improvement with the combination of IVIG and Rituximab. Gammaglobulin liquid (IVIG) infusion is likely to be more beneficial than any of the standard treatments/procedures for this patient.                                                    

The carrier's denial of Gammaglobulin liquid (IVIG) infusion should be reversed.