Appeal type: Medical necessity
Decision: Upheld

Diagnosis: Stiff person syndrome

Treatment: Bivigam injection (code J1556)

The insurer denied coverage for Bivigam injection (code J1556)

The denial is overturned

 
This case involves an adult diagnosed with stiff person syndrome who presented to the neurologist office for consultation complaining of posturing of the toes on the right foot, making it difficult to walk.  He claimed to have slowness to the right side of the body with reduced dexterity and changes to the voice. The symptoms have been ongoing. Exam findings noted a raspy speech with tight heel cords bilaterally, increased tone and rigidity in the left biceps with left toe dystonia and right toe flexor dystonia. He also had dystonic gastrocnemius on the right but was without significant pronator drift. Reflexes were 3/4 at the triceps, biceps, brachioradialis, and knee jerk and 1/4 at the ankles. The patient's right arm did not move during gait assessment. The physician had concerns regarding neurological autoimmune antibody as a cause of the underlying symptoms. The report stated the patient failed to respond to Zanaflex, baclofen, and Flexeril and was being recommended Robaxin.

The treating physician stated the patient was suffering from progressive dystonia, rigidity, and gait dysfunction. The patient does not have Parkinson's or extrapyramidal disorder. Clinically, the patient has stiff person syndrome by repeated and exhaustive work-up. The physician noted that many patients can have seronegative and non-oncology basis as noted in literature. The patient has done muscle relaxants and steroids with marginal response. The physician was recommending intravenous IG (immune globulin) given that the patient's symptoms were affecting his daily function, causing pain, affecting work, and were escalating. The patient received a notice of adverse determination regarding Bivigam injections. The report indicated that while the patient has a diagnosis of stiff person syndrome, there was no presence of anti-glutamic acid decarboxylase (GAD) autoantibody to approve the request.

Current medical literature states that there are 2 main treatments for stiff person syndrome (SPS) to include GABAergic (gamma-aminobutyric acid) therapy and immunotherapy. Literature indicates that most patients with stiff person syndrome have GAD-antibodies. The patient received a notice of adverse determination regarding Bivigam injections given that there was no presence of anti-GAD antibodies and laboratory test results. SPS is often misdiagnosed as Parkinson's disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.  However, the treating physician stressed that not all patients will have anti-GAD antibodies with this condition and that other conditions have been ruled out on extensive work-up of the patient. The patient has had progressive muscle rigidity with painful muscle spasms, has difficulty walking and performing activities of daily living, and has tried multiple medications without success. The physician stressed that without IG treatment, the patient will continue to suffer and decline. Given this information, it was determined that the Bivigam injection (code J1556) is medically necessary.

Based on the above, the insurer's denial must be overturned. The health care plan did not act reasonably and with sound medical judgment and in the best interest of the patient. 

The medical necessity for Bivigam injection (code J1556) services is substantiated.