Anza Zahid1, Beatriz Thames1, Osman Ozel1, Mohammad Nakawah2, Sheetal Shroff3
1Houston Methodist Neurological Institute, Department of Neurology, Houston, TX, 2Houston Methodist Neurological Institute, Department of Neurocognitive Disease, Houston, TX, 3Houston Methodist Neurological Institute, Department of Neuromuscular Disease, Houston, TX
Objective:
To report an atypical case with pseudo-bulbar affect (PBA) and tremors in GFAP Astrocytopathy.
Background:
Broader neurological manifestations of autoimmune encephalitis including movement disorders, episodic emotional liability, and autonomic dysfunction are becoming increasing recognized. Autoimmune glial fibrillary acidic protein (GFAP) is a steroid-responsive astrocytopathy characterized by one or more symptoms of acute to subacute encephalitis, meningitis, myelitis, and low risk association with neoplasms.
Design/Methods:
Literature review.
Results:
A 65 year-old man with Hypertension, Hyperlipidemia, and Hypothyroidism presented from an outside hospital with a 2 month history of progressively worsening encephalopathy, hallucinations, bilateral distal upper extremity kinetic tremors, and ataxia. Lumbar puncture showed frank lymphocytic pleocytosis (WBC: 1501) with elevated IgG synthesis (225.06), Q-albumin ratio (18.79), and presence of oligoclonal bands. Meningitis and infective encephalitis panels were negative twice. Serial MRI Brain, CT chest, abdomen, and pelvis were unremarkable for any abnormalities and overt malignancies. EEG was consistent with diffuse background disturbance without seizure activity. Meanwhile, the patient was started on IV methylprednisolone (IVMP) 1 g x 5 days followed by PLEX x 5 days with significant clinical improvement in mentation, tremors and ataxia. Patient was discharged to rehab where he developed interval worsening with recurrent sudden uncontrollable crying spells and outburst. ENC2 panel showed high positive titers for GFAP antibody (1: 256) in CSF only. Repeat lumbar puncture showed resolving lymphocytic pleocytosis of (WBC: 327) with active BBB. Patient was restarted on IVMP with plan for maintenance with a steroid sparing agent Rituxan with addition of mood stabilizers. Subsequent physical exam showed improvement in mood and tremors. Patient returned to physical therapy.
Conclusions:
Anti-GFAP astrocytopathy should be included in the differential diagnosis of patients who present with a relapsing-remitting, steroid responsive subacute encephalitis. Pseudo-bulbar affect and tremors may an atypical neurological manifestation associated with possible loss of somatosensory inhibition to cerebellum in GFAP astrocytopathy.