title: Tension-type headache as the unique pain experience of a patient with congenital insensitivity to pain.
abstract: Congenital insensitivity to pain (CIP) is a rare clinical syndrome characterized by dramatic impairment of pain perception since birth and is generally caused by a hereditary sensory and autonomic neuropathy (HSAN) with loss of the small-calibre, nociceptive nerve fibres. We report the case of a 32-year-old woman with CIP and a presumptive diagnosis of HSAN type V, who experienced physical pain for the first and unique time in her life shortly after the sudden loss of her brother. This patient had sustained innumerable painless injuries during childhood, including bone fractures and severe burns. The only pain she ever felt consisted in an intense headache, which took place in a context of strong emotional overload and anxiety, 3 weeks after her younger brother died suddenly in a car accident. The description of this inaugural episode of headache fulfilled the diagnostic criteria of episodic tension-type headache. This case strongly suggests that the transcription of the grief of bereavement into physical pain may sometimes occur independently of the peripheral mechanisms of nociception and despite the lack of previous pain experience. In the light of recent experimental data showing that the same neural mechanisms that regulate physical pain may also control the expression of separation distress and the feeling of social exclusion, this unique case helps to better understand why some patients may feel physically hurt after the loss of someone they love.
authors.affiliation: Département de Neurophysiologie Clinique, Centre Hospitalo-Universitaire, Pitié-Salpêtrière, Paris, France Consultation de la Douleur, Centre Hospitalo-Universitaire, Pitié-Salpêtrière, Paris, France INSERM U713, Centre Hospitalo-Universitaire, Pitié-Salpêtrière, Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: Danziger
authors.forename: C-J
authors.initials: CJ
authors.lastname: Willer
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008016:Life Change Events; D009460:Neurologic Examination; D010146:Pain / Q000523:psychology*; D000699:Pain Insensitivity, Congenital / Q000150:complications* / Q000523:psychology*; D010147:Pain Measurement; D012867:Skin / Q000294:innervation / Q000503:physiopathology; D018781:Tension-Type Headache / Q000209:etiology*