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Autoimmune Voltage-Gated Potassium Channelopathy Presenting With Catecholamine Excess

K Amy Stepp1, Christin Folker1, Marie Tanzer2

  • 1Department of Pediatrics, Maine Medical Center's Barbara Bush Children's Hospital, Portland, Maine.

Pediatric Neurology
|May 18, 2017
PubMed

Insights

Autoimmune voltage-gated potassium channelopathies can cause neurological symptoms and elevated catecholamines. Prompt treatment with immunotherapy resolved a pediatric case, highlighting the importance of considering these antibodies in diagnosis.

Area of Science:

  • Neurology
  • Immunology
  • Endocrinology

Background:

  • Autoimmune voltage-gated potassium channelopathies present with diverse neurological symptoms.
  • These conditions can affect central, peripheral, and autonomic nervous systems.

Observation:

  • A 12-year-old boy experienced pain, anxiety, weight loss, hypertension, and tachycardia.
  • Elevated plasma and urine metanephrines and catecholamines were detected.
  • Investigations for common causes of catecholamine excess were negative.

Findings:

  • The patient tested positive for voltage-gated potassium channel antibodies.
  • Treatment with intravenous immunoglobulin and pulse methylprednisolone led to symptom resolution.
  • Vital signs, weight, and metanephrine levels normalized post-treatment.

Implications:

  • Voltage-gated potassium channel antibodies are a crucial consideration in patients with unexplained catecholamine excess.
  • This case underscores the link between autoimmune channelopathies and endocrine dysfunction.
  • Immunotherapy can effectively manage neurological and autonomic symptoms associated with these antibodies.
Abstract

Keywords:
Voltage-gated potassium channelsautonomic nervous system diseasescatecholamine excessneuronal autoimmunityparaneoplastic syndromespediatric

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