To describe a patient with GFAP astrocytopathy resulting in treatment refractory myeloneuritis

Antibodies against glial fibrillary acid protein have been identified as a meningoencephalomyeloneuritis characterized by acute to subacute onset fever, headaches, optic neuritis, encephalopathy, seizures, and peripheral neuropathy. Coexisting autoimmune disorders or neoplasm have been identified with this disease. Typically, GFAP Astrocytopathy is a monophasic disease responsive to corticosteroids. GFAP IgG in CSF confirms the diagnosis.

Case Report

A 34 yo Caucasian male with history of type II diabetes mellitus and hypertension presented with subacute onset headaches, fever, diplopia, limb paresthesias and progressive weakness and inability to ambulate.  Neurologic exam demonstrated decreased level of awareness, inability to follow commands, right afferent pupillary defect, diffuse flaccid paralysis of limbs without response to noxious stimuli and areflexia. CSF studies showed lymphocytic pleocytosis with elevated protein, normal IgG index, no oligoclonal bands, negative West Nile, HIV, GQ1B, anti-MOG, anti-AQP4, anti NMDAR antibodies. Autoinmmune and paraneoplastic panel was positive for GFAP IgG. 

MRI brain demonstrated FLAIR hyperintensities in basal ganglia internal capsule, thalami, medial temporal lobes, brainstem, cerebellar peduncles and dentate nucleus. MRI cervical and thoracic spine showed multifocal hyperintensities in the lateral columns MRI Lumbar spine displayed lumbosacral radiculitis and denervation. Nerve conduction studies exhibited severe axonal sensorimotor neuropathy with active denervation in arm and leg. CT and ultrasound surveys were negative for malignancy. 

This novel case highlights the development of treatment refractory myeloneuritis as its main feature. Despite the patient being on high dose methylprednisolone followed by plasma exchange, intravenous immunoglobulin and maintenance prednisone with mycophenolate only his mentation improved 6 months after onset. Neither malignancy nor anti NMDAR antibodies which are associated with poor response to treatment were present.This case suggests the need of future studies to evaluate the impact of myeloneuritis on the outcome of the disease.