A Case of Anti-Glial Fibrillary Acidic Protein (GFAP) Antibody-associated Arachnoiditis Presenting as Cauda Equina Syndrome
Sahithi Chintha1, Gopikrishnan Unnikrishnan1, Pula Rohan1, Sudheeran Kannoth1, Vivek Nambiar1, Anand Kumar Anandakuttan1, Siby Gopinath1, Udit Saraf1, Annamma Mathai2, Jyothi Leelamaniamma2
1Neurology, Amrita Institute of Medical Sciences, 2Amrita Institute of Medical Sciences
Background:
Glial fibrillary acidic protein (GFAP) is a predominant intermediate filament protein present in adult astrocytes. Autoimmune GFAP astrocytopathy mainly presents as meningoencephalitis or encephalitis or myelitis. But, Anti-GFAP Antibody associated Arachnoiditis is not described yet.
Results:
We present a 50 year old female with no comorbidities who came with radicular pain and weakness of left lower limb, numbness of both thighs and urge incontinence since 45 days. Neurological examination revealed asymmetric paraparesis. MRI spine done revealed abnormal enhancement along cauda equina as well as dorsal cord-suggestive of early arachnoiditis.CSF analysis revealed lymphocytic pleocytosis (TC-282 cells /mm3), hypoglycorrychea-35 mg/dl and elevated proteins -173 mg/dl without any atypical cells. All the workup done for infective etiology (CSF gram stain, bacterial culture, KOH mount, fungal culture,TB gene Xpert, Cryptococcal Antigen, CSF Extended meningoencephalitis panel) was negative. Workup done for Autoimmune etiology revealed positive CSF GFAP antibody, ANA positivity with fine speckled pattern and negative serum NMOSD antibody panel. Whole body PET CTdone revealed no evidence of malignancy. Patient was treated with 1gm IV Methylprednisolone for 5 days and had significant improvement- able to walk without support. Repeat CSF analysis done 2 weeks later, showed improvement- lymphocytic pleocytosis was decreased (TC-77 cells /mm3), CSF proteins and glucose levels were normal. On 2 months follow up with Mycophenolate mofetil and she has no neurological deficits.
Conclusions:
Autoimmune GFAP Astrocytopathy can also present as Arachnoiditis.It will be worthwhile to look for CSFAnti-Glial Fibrillary Acidic Protein (GFAP) antibody as it a treatable condition which responds to Immunotherapy.