COVID-19 triggers AIP attack in woman, 31, case report says
Diagnosis was associated with rare condition marked by seizures, brain swelling
COVID-19 triggered an attack of acute intermittent porphyria (AIP) in a 31-year-old woman, according to a report in Japan.
The woman started having seizures after contracting COVID-19 and was later diagnosed with AIP associated with posterior reversible encephalopathy syndrome (PRES), a rare condition marked by brain swelling and seizures.
“This case highlights the potential for COVID-19 to trigger acute attacks in patients with underlying porphyria, potentially leading to complications such as PRES,” the researchers wrote.
The case study, “Unmasked acute intermittent porphyria in a patient with COVID-19-associated posterior reversible encephalopathy syndrome,” was published in BMC Neurology.
AIP is the most common form of acute porphyria, a group of conditions caused by the lack of one of the enzymes needed to produce heme, a molecule that enables red blood cells to transport oxygen. As a result, porphyrins and other heme precursors build up to toxic levels in parts of the body, driving disease symptoms.
Diagnosis follows coma
Here, researchers described the case of a Vietnamese woman who suddenly started having seizures after contracting COVID-19 and was ultimately diagnosed with PRES secondary to AIP.
The woman was transported to the hospital in a coma after experiencing two generalized tonic-clonic seizures — seizures marked by muscle stiffening, twitching, and loss of consciousness — that day. A week earlier, she had started having fever, abdominal pain, and vomiting. She had also been diagnosed with COVID-19 two days before going to the hospital.
When she was first examined, she had high blood pressure (hypertension) and a fast heart rate (tachycardia). Lab tests revealed severely low levels of sodium and mildly low levels of magnesium in the blood. On the second day of hospitalization, she regained consciousness. Despite the correction of her sodium levels, on the 10th day of hospitalization, she had another episode of generalized tonic-clonic seizures and temporary mild weakness in the left side of her body.
After that episode, she was mildly agitated and depressed, with hypertension, tachycardia, and generalized muscle pain. An MRI scan revealed brain lesions on both sides of the brain, which were indicative of PRES. Her hypertension was successfully managed with amlodipine, and a follow-up MRI showed a reduction in the abnormal findings.
Considering her combination of symptoms, particularly PRES, neuropsychiatric issues (agitation, depression), and gastrointestinal problems, acute porphyria was suspected.
Further analyses revealed her urine darkened when exposed to sunlight, consistent with the accumulation of porphyrins. That was confirmed by subsequent tests showing the presence of elevated levels of porphyrins and heme precursors, including aminolevulinic acid, coproporphyrin, and uroporphyrin, in the urine.
A genetic analysis also showed the woman had a mutation in the HMBS gene, confirming the diagnosis of AIP.
She was given hemin for four days, which eased her symptoms. Additionally, her medication for hypertension was discontinued, with no recurrence of high blood pressure.
“Clinicians should consider the possibility of porphyria in patients with COVID-19-associated PRES, especially when they present with gastrointestinal and neuropsychiatric symptoms,” the researchers wrote.
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