Teen Is First Known Angelman Patient With COVID-19, per Report

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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Researchers have reported the case of a teenager with Angelman syndrome (AS) in Brazil who developed severe COVID-19 but recovered after nearly three weeks in intensive care.

This is the first published report of COVID-19 in a person with Angelman, according to the investigators.

While it is impossible to draw broad conclusions from a single case, the team speculated that people with Angelman may be at increased risk of severe COVID-19 if they become infected with the virus.

At issue, they said, is that Angelman patients “lack a competent immune response and could suffer severe consequences of SARS-CoV-2 infection.”

Titled “Case Report: A Severe SARS-CoV-2 Infection in a Teenager With Angelman Syndrome,” the report was published in Frontiers in Medicine.

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COVID-19, the disease caused by the coronavirus SARS-CoV-2, first emerged more than a year ago, leading to a global pandemic and patient deaths. Since its appearance, scientists have been working to better understand who is at risk of severe disease if they become infected.

In Angelman and other rare diseases, the fact that the patient populations are so small can make it difficult to determine whether having the disorder increases the risk of severe COVID-19.

Now, researchers in Brazil describe the case of a 14-year-old boy with Angelman and asthma who was admitted to their hospital in April 2020 with symptoms that included fever and difficulty breathing. The teen had tried taking his previously prescribed asthma medications at home, but they did not ease his symptoms. His mother also had flu-like symptoms, per the report.

The patient was examined for a variety of viruses, and he tested positive for SARS-CoV-2, confirming a diagnosis of COVID-19.

“To our knowledge, no reports to date have described COVID-19 in patients with AS,” the researchers wrote.

In the hospital, the boy’s condition worsened. On the second day, he was transferred to an intensive care unit (ICU), and the next day, because breathing was still so difficult, he was put on mechanical ventilation. He remained on ventilation for nearly three weeks.

The patient continued to develop new symptoms. As he did, his care team implemented strategies to control them and to keep his condition from worsening. For example, when heart imaging showed evidence of a dangerous blood clot, he received anticoagulants, which are medications that prevent clotting.

During his time in the ICU, various laboratory tests and imaging studies were done of the patient’s lungs. These showed evidence of inflammation and lung damage, generally consistent with what has been described in other cases of severe COVID-19, the researchers said.

After two weeks of hospitalization, the boy’s COVID-19 tests came back negative. With continual care, his condition gradually improved.

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“The boy was discharged from hospital negative for SARS-CoV-2 and in good general condition and controlled asthma, without the need for home oxygen therapy,” the researchers wrote. “In total, he had been hospitalized for 36 days, with 20 days in intensive care.”

Testing subsequent to his hospital discharge showed that the boy had neutralizing antibodies against SARS-CoV-2, indicative of an immune response that can effectively fight the virus.

The researchers noted that no single case is sufficient to support broad conclusions. However, they speculated that other Angelman patients might be at increased risk for severe COVID-19 if they become infected with SARS-CoV-2.

Generally, neurological conditions are associated with a higher risk of certain lung infections, they noted. In addition, people with Angelman, including the boy in this report, often experience atypical sleep patterns that may interfere with the immune system’s ability to fight off the virus.

“Chronic sleep deprivation leads to sustained activation of the inflammatory response and increased risk of infectious disease, with major impairment of the antiviral response,” the researchers wrote.

In addition, they noted, people with Angelman often have difficulty with communication, which could lead to delayed diagnoses.

“The knowledge of COVID-19 infection in children is still sparse, and future studies must report the interaction between COVID-19 and particular syndromes,” the researchers concluded, adding that this study “might contribute to the understanding of COVID-19 and viral infection in patients with AS.”