SUNCT/SUNA and neurovascular compression: New cases and critical literature review

IRCCS Institute of Neurological Sciences of Bologna, Headache Centre, Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy.
Cephalalgia (Impact Factor: 4.12). 06/2013; 33(16). DOI: 10.1177/0333102413494273
Source: PubMed

ABSTRACT Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes.
The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression.
We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases.
We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%.
We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.


Available from: Daniela Grimaldi, Apr 17, 2015
  • [Show abstract] [Hide abstract]
    ABSTRACT: Clusterkopfschmerz, paroxysmale Hemikranie und ,,short-lasting unilateral neuralgiform headache attacks with conjuntival injection and tearing“ (SUNCT-Syndrom) werden unter den trigeminoautonomen Kopfschmerzerkrankungen (,,trigemino autonomic cephalalgia; TAC) zusammengefasst. Die neue vorgeschlagene internationale Klassifikation der Kopfschmerzerkrankungen (ICDH-3 beta) zählt nun auch die Hemicrania continua zu dieser Gruppe, außerdem wurden die Diagnosekriterien der anderen TACs modifiziert. TACs sind durch ständige oder in Attacken auftretende Kopfschmerzen mit zusätzlichen kranial autonomen Begleitsymptomen charakterisiert. Sie unterscheiden sich in ihrer Attackendauer und müssen wegen unterschiedlicher pharmakologischer Therapiestrategien voneinander abgegrenzt werden.
    Der Nervenarzt 12/2013; 84(12). DOI:10.1007/s00115-012-3624-4 · 0.86 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Trigeminal autonomic cephalgias (TAC) are characterized by severe and strictly unilateral headaches with a frontotemporal and periorbital preponderance in combination with ipsilateral cranial autonomic symptoms, such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, and restlessness or agitation. One main differentiating factor is the duration of painful attacks. While attacks typically last 5 s to 10 min in SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), paroxysmal hemicrania lasts 2-30 min and cluster headaches 15-180 min. Hemicrania continua represents a continuous TAC variant. From a therapeutic view, TACs differ substantially. Lamotrigine is used as first-choice prevention in SUNCT syndrome and indometacin in paroxysmal hemicrania. For cluster headaches, acute therapy with inhaled pure oxygen and fast-acting triptans (sumatriptan s.c. and intranasal zolmitriptan) is equally important to short-term preventive therapy with methysergide and cortisone and long-term prophylactic treatment comprising verapamil as drug of first choice and lithium carbonate and topiramate as drugs of second choice. In refractory cases of chronic cluster headache, neuromodulatory approaches such as occipital nerve stimulation and sphenopalatine ganglion stimulation are increasingly applied.
    Bundesgesundheitsblatt - Gesundheitsforschung - Gesundheitsschutz 07/2014; 57(8). DOI:10.1007/s00103-014-2003-7 · 1.01 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: To report the first ever case of primary short-lasting unilateral neuralgiform headache attacks (SUNCT) syndrome completely responsive to clomiphene citrate. Case report. SUNCT is a primary headache disorder marked by frequent attacks of one-sided headache with cranial autonomic associated symptoms. When SUNCT is deemed medicinally treatment refractory, it can cause tremendous patient-related disability. Surgical treatment options are available including hypothalamic deep brain stimulation, occipital nerve stimulator placement or arterial decompression surgery, but these procedures carry significant morbidity. A patient presented with a 10 month complaint of multiple, daily short-lasting, right-sided headaches each lasting from 60 to 120 seconds in duration and occurring from 100 to 200 times per day. The head pain was associated with ipsilateral eyelid ptosis and conjunctival injection. The patient was diagnosed with SUNCT but was unresponsive to multiple recognized medicinal treatments. He had complete alleviation of his attacks with clomiphene citrate, a synthetic, non-steroidal, ovulatory stimulant that directly binds to hypothalamic estrogen receptors. The clomiphene was tolerated without any adverse events. A putative mechanism of action for clomiphene in the prevention of SUNCT will be presented. Clomiphene citrate is a unique treatment for SUNCT and appears to be very safe and effective.
    Cephalalgia 03/2014; 34(12). DOI:10.1177/0333102414527647 · 4.12 Impact Factor