Hereditary Coproporphyria Mimicking Guillain-Barré Syndrome After COVID-19 Infection.

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.

Resolution of cryptogenic new onset refractory status epilepticus with tocilizumab.

New onset refractory status epilepticus (NORSE) was defined by the International League Against Epilepsy as occurring in patients presenting without a prior diagnosis of epilepsy or other neurological disease, with seizures that persist beyond 24 h. There is still a need to develop new treatment strategies for NORSE, particularly for those patients who are least responsive to conventional medical therapies. We present a case of a young female patient without any medical history presenting with status epilepticus, which was refractory not only to anti-seizure medications and anesthetics, but also to conventional immunomodulatory therapies. After nine weeks of electroclinical seizure activity, the patient responded to two doses of tocilizumab.