Erythroprosopalgia G44.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Bing-Horton Syndrome; Histamine headache; Horton neuralgia

History
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Tulp, 1641; Willis, 1685; Harris, 1926; Horton, 1939; Bing, 1913

Definition
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Mostly nocturnal, unilateral cluster headaches lasting only a few hours or minutes.

Localization
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Hemicranial, especially temporal or periorbital emphasis.

Clinical features
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  • Homolateral swelling of the face; severe periorbital pain, unilateral lacrimation, aqueous secretion from the nose, swelling of the nasal mucosa and acute facial sweating. This is followed by pronounced redness and a feeling of heat on the affected half of the face. Possibly narrowing of the pupil and narrowing of the eyelid until the seizure subsides. Motor restlessness during the pain attacks (patients walk around until the pain subsides).
  • Frequently long asymptomatic or pain-free intervals (months to years).
  • Attack of pain triggered by sublingual administration of 1 mg Nitrolingual (after 30-50 minutes), flickering and flickering light, flashes of light, stay at high altitudes, histamine and nitroglycerine or alcohol.

Differential diagnosis
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Trigeminal neuralgia; temporal arteritis.

Therapy
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  • Acute therapy:
    • Oxygen inhalation 6-8 l/min
    • Therapeutic blocking of the supraorbital nerve, e.g. with 0.5 ml bupivacaine 0.5%.
    • Intranasal instillation of 1 ml lidocaine gel 2-4% (e.g. xylocaine gel).
    • Sumatriptan (e.g. Imigran Tbl.) 50-100 mg/day p.o. or 6 mg s.c.
    • In persistent cases: shock therapy with systemic glucocorticoids (e.g. Prednisolon Tbl.). Initially 80 mg/day p.o. for 4 days, then dose reduction by 10-20 mg/day.
    • Secale preparation like Ergotamin (Ergo-Kranit Tbl.).
  • prophylaxis:
    • Verapamil (e.g. Isoptin Tbl.) up to 3-4 times 80 mg/day, creeping in during the first week.
    • Alternatively: Trial with lithium (e.g. Hypnorex Tbl., Quilonum Tbl.) with individual dose design and control of the drug effect levels in the blood.

Diet/life habits
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Alcohol withdrawal, nicotine withdrawal. No consumption of food containing glutamate.

Literature
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  1. Bing R (1913) Textbook of Nervous Diseases. (Berlin)
  2. Bing R (1930) On traumatic erythromelalgia and erythroprosopalgia. Neurologist, (Berlin) 3: 506-512
  3. Edvinsson L (2001) Pathophysiology of primary headaches. Curr Pain Headache Rep 5: 71-78
  4. Ekbom K et al (2002) Cluster headache: aetiology, diagnosis and management. Drugs 62: 61-69
  5. Harris BT (1936) Ciliary (migrainous) neuralgia and its treatment. BMJ 1: 457-460
  6. Horton BT (1939) A new syndrome of vascular headache: report of treatment with histamine: preliminary report. Proceedings of the Staff Meetings of the Mayo Clinic (Rochester, MN) 14: 257-260
  7. Horton BT (1941) The use of histamine in the treatment of specific types of headaches. JAMA (Chicago) 116: 377-383
  8. Horton BT (1961) Histamine cephalgia (Horton's headache or syndrome). Maryland State Medical Journal 10:178-203Peres MF (2000) Cluster headache and melatonin. Lancet 355: 147
  9. Tulp N (1641) Observationum medicarum libri tres. (Amsterdam)
  10. Willis T (1685) The London Practice of Physick. 1st edition (London; Thomas Basset and William Crooke) p 386-387

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020