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Periodic fever associated with intermittent rhythmic delta activity: a syndrome of hypothalamic origin?

J J van Hilten1, F Roelfsema, J W van der Meer

  • 1Department of Neurology, Leiden University Hospital, The Netherlands.

Insights

This case study describes a patient experiencing periodic fever attacks every 30 days. These infradian cycles and associated symptoms suggest a rare periodic hypothalamic syndrome.

Area of Science:

  • Neurology
  • Endocrinology
  • Internal Medicine

Background:

  • Periodic fever syndromes are a group of autoinflammatory disorders characterized by recurrent episodes of fever.
  • Hypothalamic dysfunction can manifest with various symptoms, including alterations in body temperature regulation.

Observation:

  • A patient presented with a 16-year history of recurrent fever attacks occurring in a regular 30-day cycle.
  • Clinical features associated with the fever attacks were systematically documented.
  • Electroencephalogram (EEG) recordings were obtained during two separate fever episodes.

Findings:

  • The observed infradian cycle (occurring less than once per week) of fever attacks is a key characteristic.
  • The combination of the cyclical fever, associated clinical manifestations, and specific EEG findings during attacks strongly points towards a diagnosis.
  • The findings are suggestive of a periodic hypothalamic syndrome, a condition involving the hypothalamus's role in regulating cyclical physiological events.

Implications:

  • This case highlights the importance of recognizing infradian fever cycles in diagnosing rare hypothalamic disorders.
  • Further research into the neuroendocrine mechanisms underlying periodic hypothalamic syndromes is warranted.
  • Accurate diagnosis can guide targeted therapeutic strategies for patients with unexplained recurrent fevers.

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