Appeal type: Medical Necessity
Decision: Overturned Decision of Health Plan

Nature of Statutory Criteria/Case Summary: A 53-year-old male enrollee has requested authorization and coverage for intravenous immunoglobulin therapy (IVIG) 1.4 g/kg for five days followed by a single dose every two weeks for treatment of the enrollee who has been diagnosed with autoimmune encephalitis and epilepsy.  Findings:  The physician reviewer found that The submitted documentation as well as the medical literature support the medical necessity of the requested services in this clinical setting. Rasmussens encephalitis is a rare neurological condition.  It is characterized as a chronic, progressive, focal (typically unilateral), and drug resistant with cognitive deterioration.  It is suspected that this disease entity is immune mediated. Typically, it is a disease that affects the pediatric population. However, adult cases have also been identified (10% of cases).  In the older population, the clinical course is usually slower and seizures semiology can correlate with temporal lobe dysfunction. A large proportion of the patient with Rasmussens encephalitis have epilepsia partialis continua.  Depending on the affected area, different focal semiologies may present themselves.  Patients who do not receive or respond to treatment can have significant morbidity with sequelae including but not limited to hemiparesis, hemianopia, cognitive decline, and dysphagia. According to the documentation, the patient has medically refractory epilepsy, focal damage and atrophy, cognitive loss, and identified episode of EPC correlating with the diagnosis of Rasmussens encephalitis. With Rasmussen's encephalitis the treatment paradigm includes treating the seizures as well as treatment against the underlying inflammatory process.  The patient is currently on a significant anti-epileptic regimen and with the addition of Onfi seems to have stabilized.  The proposed treatment for the underlying process is IVIG. Due to the rare nature of the disease, long term immunotherapy in Rasmussens encephalitis  is typically found in case reports or small uncontrolled patient series.  However, findings from these publications typically show good results with IVIG.  The available evidence supports a treatment approach that addresses the clinical manifestations of seizures as well as immune therapy against the disease process.  In the data, IVIG has been shown to be effective for treatment of Rasmussens encephalitis. For all these reasons, IVIG 1.4 g/kg for five days followed by a single dose every two weeks is medically necessary for treatment of this patients medical condition. Final Result: The reviewer determined that the requested services are medically necessary for treatment of the patients medical condition. Therefore, the Health Plans denial should be overturned. Credentials/Qualifications: The reviewer is board certified in neurology and is actively practicing. The reviewer is an expert in the treatment of the enrollees medical condition and knowledgeable about the proposed treatment through recent or current actual clinical experience treating those with the same or a similar medical condition.