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SUNCT Syndrome

Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing


Presentation

  • He presented a stabbing and intense daily pain located in the left pre-auricular and temporal regions. Each of these intense pain attacks lasted around one minute and presented a frequency of two to eight times per day.[ncbi.nlm.nih.gov]
Hunting
  • The phlebography findings were similar to those observed in the Tolosa-Hunt syndrome and in cluster headache.[ncbi.nlm.nih.gov]
  • Hunt, CH, Dodick, DW, Bosch, EP. SUNCT responsive to gabapentin. Headache 2002 ; 42: 525 – 6. DOI 10.1046/j.1526-46102002.02129.x Google Scholar Crossref Medline ISI 11. Porta-Etessam, J, Benito-Leon, J, Martinez-Salio, A, Berbel, A.[doi.org]
  • Hunt, CH, Dodick, DW, Bosch, EP. SUNCT responsive to gabapentin. Headache 2002 ; 42: 525 – 6. Google Scholar Crossref Medline ISI 27. Matharu, MS, Boes, CJ, Goadsby, PJ. SUNCT syndrome: prolonged attacks, refractoriness and response to topiramate.[cep.sagepub.com]
Eye Swelling
  • The pain attacks are associated with watery eyes, swelling of the eyelids, sweaty forehead, and nasal congestion.[fpnotebook.com]
Conjunctival Hyperemia
  • Other signs and symptoms that may be seen include conjunctival hyperemia, excessive tearing, apparent enophthalmos, and decreased IOP (4,7).[eyewiki.org]
Chemosis
  • A ptosis and chemosis of the right eye was noticed.[doi.org]
Unilateral Facial Pain
  • This evolution suggests that SUNCT might correspond, at least in this case, to a "transformed" trigeminal neuralgia and emphasizes the close relationship between these unilateral facial pain syndromes.[ncbi.nlm.nih.gov]
Headache
  • Headaches in patients with brain tumors: a study of 111 patients. Neurology 1993;43:1678-1683. [ Links ] 21. Suwanwela N, Phanthumchinda K, Kaoropthum S. Headache in brain tumor: a cross-sectional study. Headache 1994;34:435-438. [ Links ] 22.[doi.org]
  • These headache syndromes are compared with other short-lasting headache disorders, such as hypnic headache, and persistent headache with milder autonomic features such as hemicrania continua.[ncbi.nlm.nih.gov]
Neglect
  • Over a two-year period, the attacks came back immediately whenever the patient reduced the dose or neglected treatment. We concluded that lamotrigine is effective in treating SUNCT syndrome when used in high doses for a prolonged period of time.[ncbi.nlm.nih.gov]

Workup

  • Diagnostic criteria IHS diagnostic criteria for CPH include the following (see Presentation, Workup, Treatment, and Medication) [4, 5, 6] : At least 50 attacks fulfilling the criteria mentioned in the bullet points below Attacks of severe, unilateral[emedicine.medscape.com]

Treatment

  • […] drug could be considered as an option for the treatment of SUNCT. 1.[doi.org]
  • OnabotulinumtoxinA should be added to the limited therapeutic arsenal available for the treatment of refractory cases of SUNCT.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis There is no cure for these headaches. The disorder is not fatal but can cause considerable discomfort. x Prognosis There is no cure for these headaches. The disorder is not fatal but can cause considerable discomfort.[ninds.nih.gov]
  • SUNCT/SUNA Syndromes Diagnosis, Prognosis, and Treatment SUNCT/SUNA syndrome is commonly incorrectly diagnosed as trigeminal neuralgia. Brain MRI is generally recommended for work up to rule out structural lesions responsible for the pain syndrome.[doctorstrizhak.com]
  • What is the prognosis? “There is no cure for these headaches. The disorder is not fatal but can cause considerable discomfort.” NEUROMUSCULAR DENTISTRY MAY PROVIE EFFECTIVE PREVENTIVE TREATMENT FOR SOME PATIENTS.[sleepandhealth.com]
  • (See Prognosis.) Before the development of chronic symptoms, many patients (42%) pass through a nonchronic stage, with attacks separated by intervals of complete remission.[emedicine.medscape.com]

Etiology

  • Indeed, an observation period of more than 5 years speaks against a symptomatic etiology of SUNCT in these cases. Although rarely seen, clinical status may be a feature of the SUNCT syndrome.[ncbi.nlm.nih.gov]
  • Etiology Etiopathology remains unclear. Trauma, arteriovenous malformations and pituitary adenomas may have a causative role. Diagnostic methods Diagnosis is clinical. Differential diagnosis Trigeminal neuralgia is the major differential diagnosis.[orpha.net]
  • SUNCT syndrome responding absolutely to steroids in two cases with different etiologies. J Headache Pain 2009 ; 10: 55 – 7. DOI 10.1007/S10194-008-0080-4 Google Scholar Crossref Medline ISI 9. Graff-Radford, SB.[doi.org]
  • The etiology and pathology of SUNA has yet to be documented. We report an 18-year-old man with SUNA. Lomerizine hydrochloride which is used as a preventive medicine for migraine, improved his headaches.[joi.jlc.jst.go.jp]

Epidemiology

  • Summary Epidemiology The prevalence is estimated at approximately 1 in 15,000. It is slightly more predominant in males (male-to-female ratio of 1.5:1), with a mean age of onset of around 50 years.[orpha.net]
  • The Vågå study; epidemiology of headache I: the prevalence of ultrashort paroxysms. Cephalalgia 2001; 21: 207–15 PubMed CrossRef Google Scholar 18. Harris W.[doi.org]
  • The Vågå study; epidemiology of headache I. the prevalence of ultrashort paroxysms. Cephalalgia 2001 ; 21: 207 – 15. Google Scholar SAGE Journals ISI[cep.sagepub.com]
Sex distribution
Age distribution

Pathophysiology

  • We report a patient with prolactinoma and cabergoline-induced SUNCT attacks and the literature is reviewed for a better understanding of the pathophysiology.[ncbi.nlm.nih.gov]
  • The pathophysiology of SUNCT is unknown.[neurology.org]
  • Coexistence of TACS and trigeminal neuralgia: Pathophysiological conjectures. Headache 2006 ; 46 : 1565 – 70. 30. SUNCT syndrome: The first German case series. Cephalalgia 2003 ; 23 : 398 – 400. American Academy of Pain Medicine[doi.org]

Prevention

  • Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention.[ncbi.nlm.nih.gov]
  • Trauninger, A. and Alkonyi, B. and Kovács, N. and Komoly, S. and Pfund, Z. (2010) Methylprednisolone therapy for short-term prevention of SUNCT syndrome. CEPHALALGIA, 30 (6). pp. 735-739.[real.mtak.hu]
  • However, a few short-term treatments are available and can relieve and possibly prevent some symptoms of attacks.[en.wikipedia.org]

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