- •Trigeminal autonomic cephalalgias(TACs) could be the initial sole brainstem manifestation of neuromyelitis optica spectrum disorders (NMOSD).
- •An underlying cause for SUNCT /SUNA should be considered, especially if there is a limited response to anti-epileptic medication.
- •The activation of trigeminal-autonomic reflex and ephaptic coupling might be the underlying mechanism of symptomatic TACs in NMOSD.
The pathophysiology of trigeminal autonomic cephalalgias (TACs) is poorly understood at present. Symptomatic TACs are rarely reported in neuromyelitis optica spectrum disorders (NMOSD). To better clarify this distinct clinical manifestation in NMOSD and to investigate its possible pathophysiology, we reviewed articles describing such cases including our own case.
We performed a search of all clinical studies of TACs in NMOSD published up to September 1st, 2022. We put no restrictions on the year of English publication in our search. The following keywords were searched: trigeminal autonomic cephalalgias, cluster headache, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), hemicrania continua, paroxysmal hemicrania, neuromyelitis optica, neuromyelitis optica spectrum disorder, Devic's disease.
We reviewed six cases (five published reports and our own case study) that fulfilled the diagnosis of NMOSD and TACs. Four of them were SUNCT, one was SUNA, and one was paroxysmal hemicrania. In three of these cases, headache was the initial sole manifestation. Only one case had a good response to routine TACs’ treatment. All these patients had lesions in the medulla oblongata and cervical cord. Three cases’ TACs were side-locked, and two of them had a left dorsolateral medulla oblongata lesion that corresponded with the left side TACs, while three cases’ headaches happened on either side of the head. The phenomenon could be explained by the activation of trigeminal-autonomic reflex and ephaptic coupling.
TACs could be the initial sole brainstem manifestation of NMOSD. An underlying cause for SUNCT/SUNA should be considered, especially if there is a limited response to anti-epileptic medication. The activation of trigeminal-autonomic reflex and ephaptic coupling might be the underlying mechanism of symptomatic TACs in NMOSD.
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- The pathophysiology of the trigeminal autonomic cephalalgias, with clinical implications.Clin. Auton. Res. 2018; 28: 315-324
- Trigeminal autonomic cephalalgias.Neurol. Clin. 2019; 37: 847-869
- Secondary short-lasting unilateral neuralgiform headache with conjunctival injection and tearing: a new case and a literature review.J. Clin. Neurol. 2018; 14: 433-443
- Symptomatic trigeminal autonomic cephalalgias.Neurologist. 2009; 15: 305-312
- The fifth cranial nerve in headaches.J. Headache Pain. 2020; 21: 65
- Pathophysiology of cluster headache: a trigeminal autonomic cephalgia.Lancet Neurol. 2002; 1: 251-257
- The International Classification of Headache Disorders, 3rd edition.Cephalalgia. 2018; 38: 1-211
- Nonsynaptic modulation of neuronal activity in the brain: electric currents and extracellular ions.Physiol. Rev. 1995; 75: 689-723
- Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients.Mult. Scler. 2014; 20: 843-847
- SUNCT associated with Devic's syndrome.Cephalalgia. 2006; 26: 221-224
- A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.Lancet. 2004; 364: 2106-2112
- Trigeminal autonomic cephalalgia as a presenting feature of Neuromyelitis Optica: "A rare combination of two uncommon disorders".Mult. Scler. Relat. Disord. 2016; 6: 73-74
- Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms in NMOSD.Neurol. Neuroimmunol. Neuroinflamm. 2018; 5: e447
- Ephaptic transmission in chronically damaged peripheral nerves.Neurology. 1979; 29: 1061-1064
- A rare case of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing with progression to neuromyelitis optica spectrum disorder.J. Int. Med. Res. 2020; 48300060520964349
- The clinical course of neuromyelitis optica (Devic's syndrome).Neurology. 1999; 53: 1107-1114
- The spectrum of neuromyelitis optica.Lancet Neurol. 2007; 6: 805-815
- International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.Neurology. 2015; 85: 177-189
Published online: April 20, 2023
Accepted: April 16, 2023
Received in revised form: March 29, 2023
Received: December 14, 2022
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