Recently uncovered ailment ravages cerebellum.
A youth develops a critical health issue, showing symptoms like double vision, walking issues, and speech impairment within two weeks. Doctors first thought it's an infection, but their suspicion shifted after analyzing the cerebrospinal fluid.
A medical team in Hannover Medical School uncovered a new autoimmune disease in which the immune system targets the cerebellum. A 18-year-old patient's case helped with this discovery. He started experiencing severe movement, vision, and speech disturbances.
Doctors initially suspected infections with bacteria or viruses based on the symptoms and profuse immune cells in the cerebrospinal fluid. "We couldn't find any viruses or bacteria, so we examined our MHH-owned cerebrospinal fluid lab," said Professor Kurt-Wolfram Sühs, a senior specialist at MHH's Neurology Clinic with Clinical Neurophysiology.
A new autoimmune disease is found
This led to the speculation that an autoantibody induced the symptoms – a suspicion confirmed later. The anti-DAGLA antibody was detected in the patient's cerebrospinal fluid, as mentioned in the statement. The same antibody was present in the cerebrospinal fluid of three more patients with severe gait, speech, and vision troubles. They analyzed more than 101 samples from sick individuals and 102 from healthy ones.
They noticed that all the patients were youngsters between 18 and 34, before they got ill. MRT scans revealed a noticeable reduction in the substance of the affected cerebellum at the back of the brain, also known as the "conductor" of movements and balance.
The uncovered cerebellar disorder which stems from the immune system is called a new form of cerebellar ataxia. The DALAG antibodies are key markers for rapid diagnosis and timely treatment. "Early detection of Anti-DAGLA antibodies in the cerebrospinal fluid is critical for diagnosing this fast-developing cerebellar encephalitis quickly and initiating immediate treatment," Sühs said.
Treatment helped three patients recover
The four patients were treated with anti-inflammatory medication and immunotherapy with the drug Rituximab — which has treated autoimmune diseases successfully for years. The health of three patients persistently improved.
The team intends to do more research on more patients for better treatments, according to their statement. They collaborated with healthcare facilities in Belgium, Germany, Luxembourg, and Austria, and their findings were published in the journal "Journal of Neurology, Neurosurgery & Psychiatry".
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The autoimmune disease targeting the cerebellum, discovered by the medical team, has been linked to the immune system and brain. Specifically, the immune system produces antibodies against DAGLA, a protein essential for brain health, leading to symptoms like gait, speech, and vision issues.
Early detection of these autoantibodies in the cerebrospinal fluid, as advised by Professor Kurt-Wolfram Sühs, plays a crucial role in diagnosing this immune-related brain disorder promptly, enabling effective treatment and potentially reducing its impact on a person's education and daily activities.
