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Published on: June 2, 2022
[Limbic encephalitis with severe sleep disorder associated with voltage-gated potassium channels (VGKCs) antibodies]
1Service de neurologie, centre hospitalier de Belfort-Montbéliard, 2, 25209 Montbéliard cedex, France.
Introduction:
Voltage-gated potassium channels (VGKCs) antibodies are associated with neuromyotonia, limbic encephalitis and Morvan syndrome.
Case Report:
We report the case of a patient who, after three weeks of fever, presented an anamnestic syndrome, associated with confusion and partial seizures. MRI showed left hyperintensity of mesial temporal structures on Flair images and right hippocampal atrophy on T1 weighted sequences. Laboratory tests only showed high level of anti-TPO antibodies. Thus, the patient was considered as having Hashimoto's encephalopathy. She was treated with intravenous methylprednisolone with no improvement of symptoms. On the contrary, the patient suffered from insomnia, deep diurnal drowsiness and complete disappearance of REM sleep. Episodes of hypothermia and severe hyponatremia were recorded. Serum VGKC antibodies were found at high level. After intravenous immunoglobulin treatment followed by methylprednisolone, we noted remarkable improvement of clinical status. Polysomnography showed reappearence of REM sleep.
Conclusion:
This case report broadens the spectrum of clinical manifestations associated with VGKC antibodies and suggests that VGKC are implicated in regulation of sleep. The potential pathophysiological mechanisms linking sleep disturbances and VGKC antibodies are discussed.
Insights
Voltage-gated potassium channel (VGKC) antibodies can cause neurological disorders. This case highlights their potential role in regulating sleep, with treatment improving REM sleep.
Area of Science:
- Neurology
- Immunology
Background:
- Voltage-gated potassium channels (VGKCs) are implicated in neurological disorders like limbic encephalitis.
- Antibodies targeting VGKCs are associated with specific autoimmune conditions.
Observation:
- A patient presented with an anamnestic syndrome, confusion, seizures, and sleep disturbances including REM sleep absence.
- Initial treatment for suspected Hashimoto's encephalopathy was ineffective.
- High levels of VGKC antibodies were detected, alongside hypothermia and hyponatremia.
Findings:
- Treatment with intravenous immunoglobulin and methylprednisolone led to significant clinical improvement.
- Post-treatment polysomnography confirmed the reappearance of REM sleep.
Implications:
- This case expands the known clinical spectrum of VGKC antibody-associated diseases.
- VGKC antibodies may play a role in the regulation of human sleep architecture.
- Further research into the pathophysiology linking VGKC antibodies and sleep disturbances is warranted.
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