Royal Free Disease is a rare disorder characterized by the inflammation and destruction of nerve cells in the brain, leading to a variety of neurological symptoms. This condition is also known as "acute necrotizing encephalitis" or "acute hemorrhagic leukoencephalitis." It is an autoimmune illness that occurs when the body's immune system mistakenly attacks and damages its own brain cells.
The disease is named after the Royal Free Hospital in London where it was initially described in the 1950s. It primarily affects children and young adults, with a higher incidence in females. The exact cause of Royal Free Disease remains unknown, although it is believed to be triggered by an infection or a viral illness.
Symptoms of Royal Free Disease typically emerge abruptly and progress rapidly. Patients may experience a sudden onset of fever, severe headache, confusion, seizures, and focal neurological deficits. Imaging studies of the brain often reveal areas of inflammation and necrosis. Moreover, cerebrospinal fluid analysis may show elevated protein levels.
The prognosis for individuals with Royal Free Disease is generally poor, as the condition can rapidly lead to severe disability and even death. Treatment mainly revolves around supportive care, including management of symptoms and prevention of complications. High-dose corticosteroids and immunotherapy may be employed to suppress the immune response, although their effectiveness remains limited.
In conclusion, Royal Free Disease is a rare autoimmune disorder characterized by inflammation and destruction of brain cells, predominantly affecting young individuals. It causes a range of neurological symptoms and is associated with a poor prognosis.
