Abstract
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder that presents with one or more of meningitis, encephalitis and myelitis. 95% of patients present with either meningoencephalitis or meningo-encephalo-myelitis. Myelitis without cerebral involvement is rare and thought to represent only about 5% or less of cases.
We report, along with review of literature, a rare presentation of autoimmune GFAP astrocytopathy, who presented with myelitis without encephalitis and experienced initial misdiagnosis and a delay in the diagnosis.
A 25-year-old male, kiwi packer, migrant from India presented with meningism (fevers, headache, neck stiffness, photophobia, nausea and vomiting) with subsequent development of urinary retention and progressive weakness and sensory change in the limbs. CSF examination revealed the GFAP-IgG with significantly elevated lymphocytes and protein. Magnetic resonance imaging revealed a rare finding of longitudinally extensive myelitis extending from the C2 to T11 level without any brain lesions. He had significantly elevated lymphocytes and protein in the CSF with the presence of GFAP-IgG. Interestingly, He was initially diagnosed with viral meningitis and had multiple re-presentations to the hospital with ongoing deterioration in clinical status despite antibiotic and antiviral therapy. This led to further investigations and immunotherapy (IV steroids and plasma exchange) with good recovery.
This is a valuable case for learning, which reports an uncommon presentation of a rare disorder. It highlights the importance of detailed history and examination, having broad differentials in mind and early re-evaluation of diagnosis when things do not go as planned.
