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 upheld.

This is a male patient who was in the physician's office in mid 2020 after a recent hospitalization for an NMOSD (Neuromylitis Optica Spectrum Disorder) Devic's disease.  The patient had been worsening regards to his neurologic disease.  The patient received no treatment after his hospital discharge.  While admitted in the hospital he received 2 g/kg of IVIG and 1000 mg of rituximab.  The patient had a magnetic resonance imaging (MRI) of the brain and spinal cord that revealed persistent abnormal contrast-enhancing lesions throughout the brainstem, cerebellum, and upper cervical spinal cord.  In addition, there were multiple T2 hyperintense lesions in the thoracic spinal cord.  The patient tolerated the infusions well and had some improvement.  The patient was severely affected by the inflammatory neurologic process.  He required a walker to walk.  His speech was abnormal, and he had severe dizziness, disequilibrium, and had near syncopal events.  The patient received IV steroids at home in early 2020; he tolerated the treatments well.  The patient was scheduled again for 2 days of IV steroids every 4 weeks.  The patient was being seen for an MO SD, not multiple sclerosis.  The patient had symptoms of diplopia, paresthesias, hyperesthesia, fatigue, incoordination, limb weakness, ataxia, loss of balance, and dysarthria, but no vision loss.  The active problems included abnormal gait, abnormal MRI, acquired dysarthria, Devic's disease incoordination, muscle spasm, and muscle weakness.  The request was received for IVIG.

The scientific evidence in peer-reviewed literature does not support a result of improvement in health outcomes.  Intravenous immune globulin has not been specifically evaluated for acute attacks of NMOSD and is rarely used in this setting. This treatment is also not supported for attack prevention. 

Gammaglobulin liquid (IVIG) infusion does not have final approval from the appropriate regulatory bodies for this diagnosis. IVIg is not FDA approved for treatment of Neuromylitis Optica Spectrum Disorder. 

Based on current peer-reviewed medical literature, gammaglobulin liquid (IVIG) infusion is not supported for treatment of the patient's condition. And is not the best available treatment for this patient at this time. 

For attack prevention, long-term immunotherapy is indicated to reduce the risk of relapse as soon as the diagnosis of NMOSD is made. For patients with NMOSD who are seropositive for aquaporin-4 (AQP4) IgG antibodies, eculizumab is recommended. For patients who meet diagnostic criteria for NMOSD without AQP4-IgG or who meet criteria with unknown AQP4-IgG status, alternative agents include rituximab, azathioprine, or mycophenolate. There is no current peer-reviewed medical literature to support that gammaglobulin liquid (IVIG) infusion is likely to be more beneficial than these standard treatments. Therefore, Gammaglobulin liquid (IVIG) infusion is not 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 upheld.