J Clin Neurol.  2018 Oct;14(4):433-443. 10.3988/jcn.2018.14.4.433.

Secondary Short-Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing: A New Case and a Literature Review

Affiliations
  • 1Department of Neurology, Chinese PLA General Hospital, Beijing, China. yusy1963@126.com
  • 2Department of Neurology, General Hospital of Jinan Military Command, Jinan, China.

Abstract

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a primary headache syndrome with an unclear pathogenesis. However, there is increasing evidence in the literature for secondary SUNCT being attributable to certain known lesions. We explored the possible neurobiological mechanism underlying SUNCT based on all reported cases of secondary SUNCT for which detailed information is available. Here we report a case of neuromyelitis optica spectrum disorders that had typical symptoms of SUNCT that might have been attributable to involvement of the spinal nucleus of the trigeminal nerve. We also review cases of secondary SUNCT reported in the English-language literature and analyze them for demographic characteristics, clinical features, response to treatment, and imaging findings. The literature review shows that secondary SUNCT can derive from a neoplasm, vascular disease, trauma, infection, inflammation, or congenital malformation. The pons with involvement of the trigeminal root entry zone was the most commonly affected region for inducing secondary SUNCT. In conclusion, the neurobiology of secondary SUNCT includes structures such as the nucleus and the trigeminal nerve with its branches, suggesting that some cases of primary SUNCT have underlying mechanisms that are related to existing focal damage that cannot be visualized.

Keyword

secondary short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing; systematic short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing; pathogenesis

MeSH Terms

Headache Disorders
Headache*
Inflammation
Neurobiology
Neuromyelitis Optica
Pons
Tears*
Trigeminal Nerve
Vascular Neoplasms

Figure

  • Fig. 1 Medical history. AQP4-ab: aquaporin-4 antibody, CSF: cerebrospinal fluid, MBP-ab: myelin basic protein antibody, MRI: magnetic resonance imaging.

  • Fig. 2 MRI performed on August 9 showed slightly long T1-weighted (A), and T2-weighted (B) values for the splenium of the corpus callosum (arrowheads) with a high diffusion-weighted-imaging signal (C), and equal T1 signal (D), slightly high T2 (E), and T2 Flair values (F) for the right middle cerebellar peduncle (arrowheads).

  • Fig. 3 Enhanced lesion on October 16 located near the left ventral medulla (arrowhead) in axis (A) and extended from the dorsolateral of lower medulla oblongata to the C1 level (arrowhead) in the sagittal position (B).

  • Fig. 4 MRI in May showed the enhanced lesion in the optic nerve (arrowheads) of the right eye (A and B), while the left side was normal (C).

  • Fig. 5 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).


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