Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Autoimmune Encephalitis.
Treatment: Immunoglobulin (J1561) (6 Visits).
The insurer denied: Immunoglobulin (J1561) (6 Visits).
The denial is overturned.

This patient is an adult male with history of autoimmune encephalitis. He was initially seen 7 years ago with post-viral fatigue syndrome and positive antinuclear antibodies (ANA). Then 3 years ago he had cyclic fatigue and gastrointestinal functional disorder. Also, he had Raynaud's and mild dysphagia with arthralgias. The patient later developed bipolar disorder and abnormal white matter changes on magnetic resonance image (MRI) of the brain that was consistent with possible autoimmune encephalitis versus neuropsychiatric lupus. The patient was hospitalized for this 3 months ago. He received intravenous immunoglobulin (IVIG) and intravenous Solumedrol 1000 milligrams (mg) for three days. The patient did not have much improvement from this treatment, but the provider feels the patient just may need longer treatment. The patient has a negative lumbar puncture. The patient had a negative computed tomography (CT) scan of the chest, abdomen and pelvis along with brain magnetic resonance image (MRI). He also had a positron emission tomography (PET) scan of the brain that did not show any malignancy. His positron emission tomography scan was consistent with autoimmune encephalitis. The patient has severe memory deficits. The request for intravenous immunoglobulin was initially denied due to documentation not indicating that the patient had been evaluated for possible neoplasm associated with encephalitis and documentation not indicating that the patient has a specific autoantibody associated with autoimmune encephalitis. The reason the provider is appealing the determination is because the patient has been ruled out for malignancy. The patient has neurological symptoms of memory deficits and other etiologies such as infection and other neurological disorders or other autoimmune conditions have been ruled out.

The proposed treatment is medically necessary.

Autoimmune encephalitis (AE) comprises a group of non-infectious immune-mediated inflammatory disorders of the brain parenchyma often involving the cortical or deep grey matter with or without involvement of the white matter, meninges or the spinal cord. The documentation does not indicate that the patient has autoantibodies that are positive, but the patient can still have autoimmune encephalitis despite not having antibodies positive. The patient has imaging consistent with autoimmune encephalitis. Intravenous immunoglobulin (IVIG) at a dose of two grams per kilogram (g/kg) over two to five days is a relatively easy-to-use and timely option for fast immunomodulation treatment for autoimmune encephalitis. 
In a study by Lee et al, 23 patients received intravenous immunoglobulin (intension-to-treat, ITT), and 18 patients completed the study according to the protocol (per-protocol, PP). Modified Rankin Scale (mRS) score improved significantly at days eight and 29 compared to baseline in both the intension-to-treat and per-protocol populations. Other secondary outcomes also improved significantly at day eight, 15, and 29 versus baseline. In the per-protocol population, 6/18 patients achieved favorable outcomes with intravenous immunoglobulin alone (modified Rankin Scale [mRS] score = 0~2 at day eight), and 12/18 patients received rescue immunotherapy. Five adverse events were reported in relation to intravenous immunoglobulin, all of which were mild. Their conclusion of the study was that intravenous immunoglobulin improved neurological functional outcomes, and the improvement was evident by day eight. Adverse effects were tolerable. These data provide the prospective evidence regarding the efficacy of intravenous immunoglobulin (IVIG) in improving the functional outcomes of autoimmune encephalitis. Therefore, at this time despite the documentation not supporting that the patient has a history of an autoantibody positive encephalitis, the documentation does support that the patient does have autoimmune encephalitis and intravenous immunoglobulin is standard of care for next treatment. Therefore, the proposed treatment is medically necessary.