Hereditary coproporphyria (HCP) is a rare disorder caused by a deficiency of an
enzyme,
coproporphyrinogen oxidase, in the
heme synthetic pathway. This disease has a highly variable clinical presentation with acute attacks of
neurologic symptoms that can last from days to months. Rarely, it and other
acute porphyrias may cause ascending
paralysis, which is difficult to distinguish from
Guillain-Barré syndrome (GBS). Acute attacks can be triggered by factors that increase the synthesis of
heme, such as hormonal changes, certain medications, dietary changes, and
infections. We report a 26-year-old female with HCP who presented with acute ascending flaccid
paralysis and
respiratory failure after
coronavirus disease 2019 (COVID-19)
infection and was initially misdiagnosed and treated for GBS. She was transferred to our neurosciences intensive care unit, where the diagnosis of
acute porphyria was established. Initial improvement occurred during treatment for several weeks with
hemin (Panhematin®) and continued with
givosiran (Givlaari®), which was recently introduced for the prevention of acute attacks. We suggest that
acute porphyria should be part of the differential diagnosis when GBS is suspected. To our knowledge, this is the first report of an attack of
acute hepatic porphyria (AHP) that developed after a
COVID-19 infection and the first with advanced
paresis to be treated with
givosiran. Her response suggests that
givosiran may contribute to recovery from advanced
neurological manifestations of
acute porphyrias.