Hypothalamic Neurochemical and Microglial Suppression in Long-Standing Human Hibernation-Like Syndrome.
Wei Jiang, Andries Kalsbeek, Roelof A Hut et al.
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Case in brief
A 57-year-old woman had a more than 30-year history of winter-associated hypothermia, neuroendocrine instability, psychomotor suppression and circannual rhythmicity. Postmortem examination showed preserved hypothalamic and basal forebrain anatomy but near-complete loss of immunoreactivity for AVP, OXT, CRH and TRH neurons in the SCN, SON and PVN, with reduced VIP and neurotensin immunoreactivity in the SCN and reduced synaptophysin across examined hypothalamic regions. Catecholaminergic neurons, myelination, microvasculature, GFAP and AQP4 were relatively preserved; microglial immunoreactivity was nearly absent. The findings were associated with a long-standing hibernation-like phenotype.
What made this case unusual
Despite profound, selective hypothalamic neurochemical abnormalities and near-absent microglial immunoreactivity, the relevant anatomy and several other neural and tissue features were preserved. The patient’s recurrent hypothermia was tolerated over decades without systemic organ failure.
Diagnostic / clinical pearl
In a long-standing hibernation-like syndrome, preserved hypothalamic anatomy does not exclude profound dysfunction: postmortem findings showed marked loss of key circadian and neuroendocrine peptide immunoreactivity.