IgLON5 antibodies disease
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Clinic
- Anti-IgLON5 disease is a neurodegenerative autoimmune disease.
- It is marked by parasomnias and chorea
- Patients are severely affected by bulbar impairment and its respiratory consequences.
- Sleep-related breathing disorders and parasomnias are common.
- All patients showed clinical or EEG signs of motor neuron disease without fulfilling the diagnostic criteria for ALS. [1]
Pathophysiology
- IgLON proteins are a family of five cell-adhesion molecules IgLON 1, 2, 3, 4 & 5, which assist in neuronal growth and connections among nerve cells. and help in brain evolution and maturation to maintain integrity of the blood brain barrier.
- Abnormal pTau deposits seen in several brains, brain-stems and upper cervical cords without inflammatory cells differentiate this entity from other autoimmune encephalitis.
- IgLON5 refers to a cell surface protein involved in promoting connections among nerve cells.
- Parasomnias may be seen in both REM and NREM sleep.
- Respiratory problems related to sleep disorder such as obstructive sleep apnea (OSA) and jerky stertorous breathing (50%)
Related disease
- ↑ Anti-IgLON5 Disease A New Bulbar-Onset Motor Neuron Mimic Syndrome Jana Werner, Ilijas Jelcic, Esther Irene Schwarz, Elisabeth Probst-Müller, Jakob Nilsson, Bernhard Schwizer, Konrad Ernst Bloch, Andreas Lutterotti, Hans-Heinrich Jung, Bettina Schreiner Neurol Neuroimmunol Neuroinflamm Mar 2021, 8 (2) e962; DOI: 10.1212/NXI.0000000000000962