eMedicine Specialties > Neurology > Headache and Pain

Chronic Paroxysmal Hemicrania: Differential Diagnoses & Workup

Author: Manish K Singh, MD, Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience
Coauthor(s): Jashvant Patel, MD, Medical Director, Department of Pain Medicine and Comprehensive Rehabilitation, Medical College of Pennsylvania Hahnemann University
Contributor Information and Disclosures

Updated: Sep 3, 2009

Differential Diagnoses

Cluster Headache
Migraine Headache
Migraine Variants
Trigeminal Neuralgia

Other Problems to Be Considered

Differential diagnosis should include other headaches with major autonomic features (eg, SUNCT syndrome) and persistent headaches with milder autonomic features (eg, hemicrania continua [HC]).

Several paroxysmal indomethacin-responsive headache syndromes have been described, including HC, jabs-and-jolts syndrome, icepick headache, hypnic headache syndrome, benign cough headache syndrome, benign exertional headache, coital headache, and thunderclap headache.

Cervicogenic headache, facial neuralgia, and other rare disorders, such as the red ear syndrome, also are considered in the differential diagnosis.

Simultaneous occurrences of CPH and other disorders, including CH, trigeminal neuralgia (CPH-tic syndrome), and migraine, also have been reported. The crucial factor in the differential diagnosis is the absolute response to indomethacin.

The trigeminal-autonomic cephalgias include CH and paroxysmal hemicranias in which head pain and cranial autonomic symptoms are prominent. CH is the most important differential diagnosis.

CH has male preponderance, unlike CPH, which is more common in females. In CPH, frequency of attacks is higher, usually more than 15 in 24 hours, whereas CH has an attack frequency of 1-4 (maximum 8) in 24 hours. The duration of headaches is shorter in CPH (2-25 min) than in CH (15-60 min).
Finally, CPH can be distinguished by its response to indomethacin therapy, which is not seen in CH.

HC is a rare disorder characterized by continuous, unilateral, indomethacin-responsive hemicrania of moderate severity. These headaches last from minutes to several days and sometimes are associated with autonomic features. A noncontinuous form also has been described.

The SUNCT syndrome is a distinctive rare condition with male preponderance characterized by less severe pain but marked autonomic activation during attacks. The pain, usually in the frontal and periocular area, is intractable to medications, including indomethacin.

Recently, a unique, stereotypical, episodic headache disorder marked by long-lasting autonomic symptoms with associated hemicrania (LASH) has been reported. The autonomic symptoms clearly overshadowed the headache as the major component of the syndrome. Both types of symptoms responded to indomethacin.

Other disorders that may mimic CPH include infarcts, arteriovenous malformation, brain tumor (eg, frontal lobe tumor, meningioma of the roof of the cavernous sinus, pathological process in the region of the cavernous sinus, gangliocytoma near sella turcica, parasellar pituitary microadenoma), maxillary cyst, collagen vascular disorders, cervical radiculopathy, disk herniation, and carotidynia.

Workup

Laboratory Studies

  • Perform lab studies to evaluate structural, metabolic, and other secondary causes of headache and facial pain.
  • Baseline routine blood tests may be needed to exclude contraindications to certain drugs and to avoid complications from long-term use of various medications.

Imaging Studies

  • CT scan, or preferably MRI, of the brain may be needed to rule out structural pathology.
  • Findings of neuroimaging studies, including MRI, are usually normal in patients with CPH.
  • Consider MR angiogram or arteriogram, if necessary, for atypical presentations.
  • Electroencephalography, brain mapping, and other radiologic studies are not required for patients with typical presentations.

Other Tests

  • Indotest (indomethacin 50 mg IM test dose) may be a useful tool in assessment of unilateral headache. The diagnosis of CPH is extremely important because it may imply lifelong treatment with a potentially noxious drug. Perform indotest in a standardized manner.
  • No characteristic electrocardiographic patterns were found during attacks, but marked variations in heart rate and rhythm abnormalities, including bradycardia, sinoatrial block, bundle branch block with episodes of atrial fibrillation, and multiple extrasystoles, have been observed.
  • Orbital phlebography may be abnormal in some patients, but the significance of this finding has not been established.
  • In one study of 3 patients with CPH, a slightly lower cerebral vasomotor reactivity was observed in the medial and posterior cerebral arteries on both sides and in the anterior cerebral artery on the symptomatic side than in healthy subjects. These observations may imply an abnormal vascular reactivity in CPH.
  • As compared to CH, CPH attacks did not demonstrate any changes in visually evoked event-related potentials (ERPs), latencies, and amplitudes.12
  • Perform ophthalmic evaluation, if needed, to assess ocular pathology such as glaucoma or orbital pseudotumor.

Procedures

  • Consider lumbar puncture, if necessary, for atypical presentations.

More on Chronic Paroxysmal Hemicrania

Overview: Chronic Paroxysmal Hemicrania
Differential Diagnoses & Workup: Chronic Paroxysmal Hemicrania
Treatment & Medication: Chronic Paroxysmal Hemicrania
Follow-up: Chronic Paroxysmal Hemicrania
References
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References

  1. Sjaastad O, Dale I. Evidence for a new (?), treatable headache entity. Headache. Jul 1974;14(2):105-8. [Medline].

  2. Sjaastad O, Dale I. A new (?) Clinical headache entity "chronic paroxysmal hemicrania" 2. Acta Neurol Scand. Aug 1976;54(2):140-59. [Medline].

  3. Russell D, Vincent M. Chronic paroxysmal hemicrania. In: The Headaches. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2000:741-9.

  4. Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain. Jan 1997;120 ( Pt 1):193-209. [Medline].

  5. Matharu M, May A. Functional and structural neuroimaging in trigeminal autonomic cephalalgias. Curr Pain Headache Rep. Apr 2008;12(2):132-7. [Medline].

  6. Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. Aug 2009;8(8):755-64. [Medline].

  7. Sjaastad O, Apfelbaum R, Caskey W, et al. Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Ups J Med Sci Suppl. 1980;31:27-33. [Medline].

  8. Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache. May 1989;29(5):280-1. [Medline].

  9. Gladstein J, Holden EW, Peralta L. Chronic paroxysmal hemicrania in a child. Headache. Oct 1994;34(9):519-20. [Medline].

  10. Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicrania in a young child: possible relation to ipsilateral occipital infarction. J Child Neurol. Jul 1993;8(3):235-6. [Medline].

  11. Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia. 1988;8 Suppl 7:1-96. [Medline].

  12. Evers S, Bauer B, Suhr B. Cognitive processing is involved in cluster headache but not in chronic paroxysmal hemicrania. Neurology. Jul 22 1999;53(2):357-63. [Medline].

  13. Shabbir N, McAbee G. Adolescent chronic paroxysmal hemicrania responsive to verapamil monotherapy. Headache. Apr 1994;34(4):209-10. [Medline].

  14. Dahlöf C. Subcutaneous sumatriptan does not abort attacks of chronic paroxysmal hemicrania (CPH). Headache. Apr 1993;33(4):201-2. [Medline].

  15. Hannerz J, Jogestrand T. Intracranial hypertension and sumatriptan efficacy in a case of chronic paroxysmal hemicrania which became bilateral. (The mechanism of indomethacin in CPH). Headache. Jun 1993;33(6):320-3. [Medline].

  16. Pascual J, Quijano J. A case of chronic paroxysmal hemicrania responding to subcutaneous sumatriptan. J Neurol Neurosurg Psychiatry. Sep 1998;65(3):407. [Medline].

  17. Antonaci F. Chronic paroxysmal hemicrania and hemicrania continua: orbital phlebography and MRI studies. Headache. Jan 1994;34(1):32-4. [Medline].

  18. Antonaci F, Pareja JA, Caminero AB. Chronic paroxysmal hemicrania and hemicrania continua: anaesthetic blockades of pericranial nerves. Funct Neurol. Jan-Feb 1997;12(1):11-5. [Medline].

  19. Antonaci F, Pareja JA, Caminero AB. Chronic paroxysmal hemicrania and hemicrania continua: lack of efficacy of sumatriptan. Headache. Mar 1998;38(3):197-200. [Medline].

  20. Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua. Parenteral indomethacin: the 'indotest'. Headache. Feb 1998;38(2):122-8. [Medline].

  21. Bill A, Stjernschantz J, Mandahl A. Substance P: release on trigeminal nerve stimulation, effects in the eye. Acta Physiol Scand. Jul 1979;106(3):371-3. [Medline].

  22. Bogucki A, Szymanska R, Braciak W. Chronic paroxysmal hemicrania: lack of pre-chronic stage. Cephalalgia. Sep 1984;4(3):187-9. [Medline].

  23. Bordini C, Antonaci F, Stovner LJ, Schrader H, Sjaastad O. "Hemicrania continua": a clinical review. Headache. Jan 1991;31(1):20-6. [Medline].

  24. Buzzi MG, Sakas DE, Moskowitz MA. Indomethacin and acetylsalicylic acid block neurogenic plasma protein extravasation in rat dura mater. Eur J Pharmacol. Jun 20 1989;165(2-3):251-8. [Medline].

  25. Caminero AB, Pareja JA, Dobato JL. Chronic paroxysmal hemicrania-tic syndrome. Cephalalgia. Apr 1998;18(3):159-61; discussion 124. [Medline].

  26. Centonze V, Bassi A, Causarano V. Simultaneous occurrence of ipsilateral cluster headache and chronic paroxysmal hemicrania: a case report. Headache. Jan 2000;40(1):54-6. [Medline].

  27. Cohen AS, Matharu MS, Goadsby PJ. Trigeminal autonomic cephalalgias: current and future treatments. Headache. Jun 2007;47(6):969-80. [Medline].

  28. Di M, Zhu Y. [Studies on cardiac function in children aged 8-12 with simple obesity]. Chung Hua Yu Fang I Hsueh Tsa Chih. May 1995;29(3):162-4. [Medline].

  29. Di Monda V. Chronic paroxysmal hemicrania and SUNCT syndrome. Ital J Neurol Sci. Apr 1999;20(2 Suppl):S49-52. [Medline].

  30. Drummond PD. Thermographic and pupillary asymmetry in chronic paroxysmal hemicrania. A case study. Cephalalgia. Sep 1985;5(3):133-6. [Medline].

  31. Feigen LP, King LW, Ray J. Differential effects of ibuprofen and indomethacin in the regional circulation of the dog. J Pharmacol Exp Ther. Dec 1981;219(3):679-84. [Medline].

  32. Foerderreuther S, von Maydell R, Straube A. A CPH-like picture in two patients with an orbitocavernous sinus syndrome. Cephalalgia. Aug 1997;17(5):608-11. [Medline].

  33. Forderreuther S, Straube A. [A rare headache syndrome. SUNCT syndrome, hemicrania continua and red ear syndrome]. Nervenarzt. Aug 1999;70(8):754-8. [Medline].

  34. Goadsby PJ. Short-lasting primary headaches: focus on trigeminal automatic cephalgias and indomethacin-sensitive headaches. Curr Opin Neurol. Jun 1999;12(3):273-7. [Medline].

  35. Goadsby PJ, Edvinsson L. Neuropeptide changes in a case of chronic paroxysmal hemicrania--evidence for trigemino-parasympathetic activation. Cephalalgia. Oct 1996;16(6):448-50. [Medline].

  36. Hannerz J. Trigeminal neuralgia with chronic paroxysmal hemicrania: the CPH-tic syndrome. Cephalalgia. Oct 1993;13(5):361-4. [Medline].

  37. Hannerz J, Jogestrand T. Intracranial hypertension and sumatriptan efficacy in a case of chronic paroxysmal hemicrania which became bilateral. (The mechanism of indomethacin in CPH). Headache. Jun 1993;33(6):320-3. [Medline].

  38. Horven I. Dynamic tonometry. II. Methods of corneal indentation pulse registration. Acta Ophthalmol (Copenh). 1970;48(1):23-38. [Medline].

  39. Joubert J, Powell D, Djikowski J. Chronic paroxysmal hemicrania in a South African black. A case report. Cephalalgia. Sep 1987;7(3):193-6. [Medline].

  40. Kudrow L. Cluster headache: diagnosis and management. Headache. Apr 1979;19(3):142-50. [Medline].

  41. Lainez JM, Pareja Martinez AI. [Hemicrania continua]. Neurologia. Dec 1997;12 Suppl 5:50-5. [Medline].

  42. Martinez-Salio A, Porta-Etessam J, Perez-Martinez D. Case reports: chronic paroxysmal hemicrania-tic syndrome. Headache. Sep 2000;40(8):682-5. [Medline].

  43. May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiologic implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab. Feb 1999;19(2):115-27. [Medline].

  44. Medina JL. Organic headaches mimicking chronic paroxysmal hemicrania. Headache. Feb 1992;32(2):73-4. [Medline].

  45. Moncada E, Graff-Radford SB. Benign indomethacin-responsive headaches presenting in the orofacial region: eight case reports. J Orofac Pain. Summer 1995;9(3):276-84. [Medline].

  46. Morales Asin F. [Chronic paroxysmal hemicrania]. Neurologia. Dec 1997;12 Suppl 5:44-9. [Medline].

  47. Moskowitz MA. Basic mechanisms in vascular headache. Neurol Clin. Nov 1990;8(4):801-15. [Medline].

  48. Newman LC, Herskovitz S, Lipton RB, et al. Chronic paroxysmal headache: two cases with cerebrovascular disease. Headache. Feb 1992;32(2):75-6. [Medline].

  49. Palmieri A, Mainardi F, Maggioni F, et al. Cluster-like headache secondary to cavernous sinus metastasis. Cephalalgia. Sep 2005;25(9):743-5. [Medline].

  50. Pareja J, Pareja J. Chronic paroxysmal hemicrania coexisting with migraine. Differential response to pharmacological treatment. Headache. Feb 1992;32(2):77-8. [Medline].

  51. Pareja JA. Chronic paroxysmal hemicrania: dissociation of the pain and autonomic features. Headache. Feb 1995;35(2):111-3. [Medline].

  52. Pollmann W, Pfaffenrath V. Chronic paroxysmal hemicrania: the first possible bilateral case. Cephalalgia. Mar 1986;6(1):55-7. [Medline].

  53. Price RW, Posner JB. Chronic paroxysmal hemicrania: a disabling headache syndrome responding to indomethacin. Ann Neurol. Feb 1978;3(2):183-4. [Medline].

  54. Quintana A, Raczka E, Giralt MT. Effects of aspirin and indomethacin on cerebral circulation in the conscious rat: evidence for a physiological role of endogenous prostaglandins. Prostaglandins. Apr 1983;25(4):549-56. [Medline].

  55. Rapoport AM, Sheftell FD, Baskin SM. Chronic paroxysmal hemicrania--case report of the second known definite occurrence in a male. Cephalalgia. Jun 1981;1(2):67-9. [Medline].

  56. Raskin NH. Short-lived head pains. Neurol Clin. Feb 1997;15(1):143-52. [Medline].

  57. Raskin NH, Schwartz RK. Icepick-like pain. Neurology. Feb 1980;30(2):203-5. [Medline].

  58. Rozen TD. LASH: a syndrome of long-lasting autonomic symptoms with hemicrania (A new indomethacin- responsive headache). Headache. Jun 2000;40(6):483-6. [Medline].

  59. Russell D. Chronic paroxysmal hemicrania: severity, duration and time of occurrence of attacks. Cephalalgia. Mar 1984;4(1):53-6. [Medline].

  60. Saunte C. Chronic paroxysmal hemicrania: salivation, tearing and nasal secretion. Cephalalgia. Mar 1984;4(1):25-32. [Medline].

  61. Saunte C, Russell D, Sjaastad O. Chronic paroxysmal hemicrania. IX. On the mechanism of attack-related sweating. Cephalalgia. Sep 1983;3(3):191-9. [Medline].

  62. Sawrasewicz-Rybak M. [Chronic paroxysmal hemicrania]. Wiad Lek. 1999;52(1-2):56-60. [Medline].

  63. Sjaastad O. Chronic paroxysmal hemicrania. In: Sjaastad O, ed. Cluster Headache Syndrome. 1992. London: WB Saunders; 291-92.

  64. Sjaastad O. Chronic paroxysmal hemicrania. In: Vinken PI, Bruyn OW, Klawans HL, et al, eds. Handbook of Clinical Neurology. 48. Amsterdam: Elsevier; 1986:257-66.

  65. Sjaastad O, Antonaci F. A piroxicam derivative partly effective in chronic paroxysmal hemicrania and hemicrania continua. Headache. Oct 1995;35(9):549-50. [Medline].

  66. Sjaastad O, Kruszewski P. Trigeminal neuralgia and "SUNCT" syndrome: similarities and differences in the clinical pictures. An overview. Funct Neurol. Mar-Apr 1992;7(2):103-7. [Medline].

  67. Sjaastad O, Russell D, Saunte C, et al. Chronic paroxysmal hemicrania. VI. Precipitation of attacks. Further studies on the precipitation mechanism. Cephalalgia. Dec 1982;2(4):211-4. [Medline].

  68. Sjaastad O, Saunte C, Graham JR. Chronic paroxysmal hemicrania. VII. Mechanical precipitation of attacks: new cases and localization of trigger points. Cephalalgia. Jun 1984;4(2):113-8. [Medline].

  69. Sjaastad O, Saunte C, Russell D. Cluster headache. The sweating pattern during spontaneous attacks. Cephalalgia. Dec 1981;1(4):233-44. [Medline].

  70. Sjaastad O, Saunte C, Salvesen R. Shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating, and rhinorrhea. Cephalalgia. Jun 1989;9(2):147-56. [Medline].

  71. Sjaastad O, Spierings EL. "Hemicrania continua": another headache absolutely responsive to indomethacin. Cephalalgia. Mar 1984;4(1):65-70. [Medline].

  72. Sjaastad O, Spierings EL, Saunte C. "Hemicrania continua". An indomethacin responsive headache. II. Autonomic function studies. Cephalalgia. Dec 1984;4(4):265-73. [Medline].

  73. Sjaastad O, Stovner LJ, Stolt-Nielsen A. CPH and hemicrania continua: requirements of high indomethacin dosages-- an ominous sign?. Headache. Jun 1995;35(6):363-7. [Medline].

  74. Souza Souza Carvalho D, Salvesen R, Sand T, et al. Chronic paroxysmal hemicrania. XIII. The pupillometric pattern. Cephalalgia. Sep 1988;8(3):219-26. [Medline].

  75. Stein HJ, Rogado AZ. Headache rounds. Chronic paroxysmal hemicrania: two new patients. Headache. Mar 1980;20(2):72-6. [Medline].

  76. Tehindrazanarivelo AD, Visy JM, Bousser MG. Ipsilateral cluster headache and chronic paroxysmal hemicrania: two case reports. Cephalalgia. Oct 1992;12(5):318-20. [Medline].

  77. Vijayan N. Symptomatic chronic paroxysmal hemicrania. Cephalalgia. Apr 1992;12(2):111-3. [Medline].

  78. Wennmalm A, Carlsson I, Edlund A, et al. Central and peripheral haemodynamic effects of non-steroidal anti-inflammatory drugs in man. Arch Toxicol Suppl. 1984;7:350-9. [Medline].

  79. Zukerman E, Peres MF, Kaup AO. Chronic paroxysmal hemicrania-tic syndrome. Neurology. Apr 11 2000;54(7):1524-6. [Medline].

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Further Reading

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Keywords

Sjaastad syndrome, IHS code: 3.2 chronic paroxysmal hemicrania, ICD-9 code: 346.9 hemicrania, CPH, headache, indomethacin, chronic paroxysmal hemicrania, unilateral headache, headaches with autonomic activation, headaches without autonomic activation, chronic and episodic paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing, SUNCT syndrome

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Contributor Information and Disclosures

Author

Manish K Singh, MD, Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience
Manish K Singh, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American Association of Physicians of Indian Origin, American Headache Society, American Medical Association, and American Society of Regional Anesthesia and Pain Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Jashvant Patel, MD, Medical Director, Department of Pain Medicine and Comprehensive Rehabilitation, Medical College of Pennsylvania Hahnemann University
Jashvant Patel, MD is a member of the following medical societies: Alberta Medical Association, American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Medical Association, American Society of Regional Anesthesia and Pain Medicine, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Medical Editor

Jorge E Mendizabal, MD, Consulting Staff, Corpus Christi Neurology
Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

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Disclosure: eMedicine Salary Employment

Managing Editor

James H Halsey, MD, Professor, Department of Neurology, University of Alabama Medical Center
James H Halsey, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neuroimaging, Medical Association of the State of Alabama, New York Academy of Sciences, Pan American Medical Association, Sigma Xi, Society for Neuroscience, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Howard A Crystal, MD, Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center
Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association
Disclosure: Medivations Honoraria Consulting

 
 
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