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- W2900450737 abstract "Objective: To present a case demonstrating progressive encephalomyelitis with rigidity and myoclonus (PERM) following West Nile Virus (WNV) encephalitis. Background: PERM is a rare, likely autoimmune process within the central nervous system, which is included on the stiff person syndrome spectrum. PERM is associated with several autoantibodies. The most common autoantibody within the disease spectrum is against glutamic acid decarboxylase isoform 65 (GAD-65). The clinical features of PERM include transient axial, truncal or limb spasms, encephalopathy, oculomotor dysfunction, autonomic dysfunction and upper and lower motor neuron dysfunction. A similar case of GAD-65 autoimmunity has been reported following WNV infection. Design/Methods: NA Results: We report a case of a 55 year old Caucasian woman with a recent history of West Nile Virus encephalitis who presented with three days of progressive body spasms, confusion and fevers. She had autonomic instability with labile blood pressure and intermittent diaphoresis. She was intubated for airway protection. On examination, she demonstrated a left gaze preference, axial and limb rigidity, diffuse hyperreflexia including a positive jaw jerk, Hoffman sign and a right Babinski. She had frequent neck, arm and leg spasms especially with stimulation. MRI of her brain and spine were normal. CSF studies found elevated protein (122 mg/dL) with pleocytosis (74 nucleated cells/mcl). Serum and CSF anti-GAD-65 antibodies were positive at 74.1 nmol/L and 3.92 nmol/L, respectively (ref. range Conclusions: This case presents an example of anti-GAD-65 associated PERM following a WNV encephalitis. Future studies are needed to evaluate a potential parainfections association between WNV infection and GAD-65 autoimmunity. Disclosure: Dr. Loeb has nothing to disclose. Dr. Devine has nothing to disclose. Dr. Perven has nothing to disclose." @default.
- W2900450737 created "2018-11-29" @default.
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- W2900450737 date "2018-04-10" @default.
- W2900450737 modified "2023-09-26" @default.
- W2900450737 title "Anti-GAD-65 associated Progressive Encephalomyelitis with Rigidity and Myoclonus following West Nile Virus Encephalitis. (P5.404)" @default.
- W2900450737 hasPublicationYear "2018" @default.
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