CACNA1H antibodies associated with headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL)

Cephalalgia. 2013 Jan;33(2):123-9. doi: 10.1177/0333102412463494. Epub 2012 Oct 30.

Abstract

Background: Patients with the syndrome of headache with neurological deficits and lymphocytosis (HaNDL) typically present with recurrent and temporary attacks of neurological symptoms and cerebrospinal fluid lymphocytosis.

Aim and methods: To identify potential HaNDL-associated antibodies directed against neuronal surface and/or synapse antigens, sera of four HaNDL patients and controls were screened with indirect immunohistochemistry, immunofluorescence, cell-based assay, radioimmunoassay, protein macroarray and enzyme-linked immunosorbent assay (ELISA).

Results: Although HaNDL sera did not yield antibodies to any of the well-characterized neuronal surface or synapse antigens, protein macroarray and ELISA studies showed high-titer antibodies to a subunit of the T-type voltage-gated calcium channel (VGCC), CACNA1H, in sera of two HaNDL patients.

Conclusion: Our results support the notion that ion channel autoimmunity might at least partially contribute to HaNDL pathogenesis and occurrence of neurological symptoms.

MeSH terms

  • Adult
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Calcium Channels, T-Type / blood
  • Calcium Channels, T-Type / immunology*
  • Cerebrospinal Fluid / immunology*
  • Female
  • Headache / blood
  • Headache / immunology*
  • Humans
  • Lymphocytosis / cerebrospinal fluid*
  • Lymphocytosis / immunology*
  • Male
  • Middle Aged
  • Nervous System Diseases / blood
  • Nervous System Diseases / immunology*

Substances

  • Autoantibodies
  • CACNA1H protein, human
  • Calcium Channels, T-Type