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Nervous system ORGAN SYSTEMS

Short-lasting unilateral neuralgiform headache attacks (SUNHA)

Characterized by strictly unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features.

Characterized by strictly unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features.

  • Type of trigeminal autonomic cephalalgias (TACs)

Classification

International Classification of Headache Disorders (ICHD-3β) (2013):

  • SUNHA with conjunctival injection and tearing (SUNCT):
    • Characterized by short-lasting unilateral headache attacks accompanied by ipsilateral lacrimation and conjunctival injection
  • SUNHA with cranial autonomic symptoms (SUNA)
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing-related pathways and structures. Shadow A represents the dorsolateral medulla and upper cervical spinal cord where the spinal nucleus of the trigeminal nerve was located, which was often affected by cerebral infarction and demyelination. Vascular compression was likely to occur in the area of Shadow B. The neoplasm and infection had a widespread focus, and were mostly located at the preganglionic fibers of the trigeminal nerve (Shadow C). | Cao, Y., Yang, F., Dong, Z., Huang, X., Cao, B., & Yu, S. (2018). Secondary Short-Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing: A New Case and a Literature Review. Journal of clinical neurology (Seoul, Korea), 14(4), 433–443. https://doi.org/10.3988/jcn.2018.14.4.433

Clinical features

Severe unilateral headache

Sudden brief attacks of severe unilateral headache in orbital, periorbital, or temporal regions, accompanied by ipsilateral cranial autonomic symptoms.

Ipsilateral autonomic symptoms:

  • SUNCT: Both conjunctival injection and lacrimation present
  • SUNA: Either conjunctival injection or lacrimation, but not both
  • Common cranial autonomic symptoms: Nasal congestion, rhinorrhea, miosis, ptosis, eyelid edema, forehead and facial sweating and/or flushing, and a sensation of fullness in the ear

Diagnosis

ICHD-3 beta criteria for SUNHA:

Specifies an attack length of 1–600s, with an attack frequency of at least once a day for more than half the time when the disorder is active
International Headache Society diagnostic criteria for short-lasting unilateral neuralgiform headache attacks | Levy, A., & Matharu, M. S. (2018). Short-Lasting Unilateral Neuralgiform Headache Attacks. Annals of Indian Academy of Neurology, 21(Suppl 1), S31–S38. https://doi.org/10.4103/aian.AIAN_356_17

MRI:

Facial photograph during headache attack and brain and upper cervical cord MRI: (A) Facial photograph during headache attack showing rhinorrhea and lacrimation on the left side (arrows). Written informed consent was obtained to show part of the face without masking. (B) Sagittal fluid-attenuated inversion recovery (FLAIR), (C) an axial FLAIR image at the level of the lower medulla oblongata, and (D) gadolinium-enhanced T1-weighted images of the brain. Three-dimensional double inversion recovery images in sagittal (E), coronal (F), and axial planes (G, medulla oblongata; H, lower medulla oblongata). Arrowheads indicate lesions. | Mizuno, Y., Shinoda, K., Watanabe, M., Matsushita, T., Yamasaki, R., & Kira, J. I. (2018). Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms in NMOSD. Neurology(R) neuroimmunology & neuroinflammation, 5(3), e447. https://doi.org/10.1212/NXI.0000000000000447

Differential diagnosis:

Other trigeminal autonomic cephalalgias (TACs) & trigeminal neuralgia (TN)
  • Cluster headache
  • Paroxysmal hemicrania
  • Hemicrania continua
  • TN; primary stabbing headache; and secondary causes of SUNHA.
Clinical features of the trigeminal autonomic cephalalgias (TACs) | Levy, A., & Matharu, M. S. (2018). Short-Lasting Unilateral Neuralgiform Headache Attacks. Annals of Indian Academy of Neurology, 21(Suppl 1), S31–S38. https://doi.org/10.4103/aian.AIAN_356_17
Differentiating features of short-lasting unilateral neuralgiform headache attacks and trigeminal neuralgia | Levy, A., & Matharu, M. S. (2018). Short-Lasting Unilateral Neuralgiform Headache Attacks. Annals of Indian Academy of Neurology, 21(Suppl 1), S31–S38. https://doi.org/10.4103/aian.AIAN_356_17

Management

Medical management:

Medical management mainly consists of preventive treatments since the attacks are very short-lasting, attack therapy strategies are not useful in SUNHA. There is no available abortive treatment for the individual attacks.
Proposed algorithm for medical treatment of short-lasting unilateral neuralgiform headache attacks | Levy, A., & Matharu, M. S. (2018). Short-Lasting Unilateral Neuralgiform Headache Attacks. Annals of Indian Academy of Neurology, 21(Suppl 1), S31–S38. https://doi.org/10.4103/aian.AIAN_356_17

Surgical management:

  • Ablative procedures of the trigeminal nerve
  • MVD of the trigeminal nerve
  • Neurostimulation techniques

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