SUNCT syndrome responding absolutely to steroids in two cases with different etiologies

Figuerola, María de Lourdes; Bruera, Osvaldo; Pozzo, M.J.; Leston, Jorge

doi:10.1007/s10194-008-0080-4

Cited by 20 publications

(12 citation statements)

References 8 publications

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“…Open‐label studies and case reports suggest that lamotrigine, topiramate, gabapentin, and CMZ may be useful in prevention of SUNCT 10 . IV lidocaine 10 and, recently, steroids 11 have been used successfully to abolish the attacks. Hypothalamic stimulation may be an option in intractable cases 12 …”

Section: Discussionmentioning

confidence: 99%

A Case of MS Presenting With SUNCT Status

2010

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Various primary headache syndromes are seen in a higher proportion of individuals with MS. 1 SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing) is a rare type of trigeminal autonomic cephalgia (TAC). It is characterized by brief (5-240 seconds), unilateral attacks of stabbing or lancinating pain centered around the eye or temple, that are associated with marked autonomic symptoms. While initially thought to be idiopathic, with the development of modern neuroimaging, SUNCT is now recognized to be associated with a variety of acquired structural abnormalities. To our knowledge, there are only 2 cases reported of SUNCT caused by demyelinating lesions, one because of MS 2 with diffuse lesions in the pons, medulla, and c-spine, and the other because of Devic's disease 3 with a lesion in the upper cervical cord. The patient with MS had experienced attacks of SUNCT 18 and 24 years after the diagnosis of MS. Here we present a case of SUNCT as the presenting symptom leading to the diagnosis of MS with a demyelinating lesion in the left trigeminal nucleus and tract. CASE REPORTMs. H is a 61-year-old woman with a history of hypertension, obesity, psoriasis, and trigeminal neuralgia 2 years prior to presenting to us. At that time she had pain in the distribution of the left maxillary nerve. There were no associated autonomic symptoms; neurological examination was normal and no brain imaging was performed. She was treated carbamazepine (CMZ) with complete abolition of the pain until April 2008 when, after missing 2 doses of CMZ, she developed attacks of excruciating electrical pain on the left side of the forehead occurring an average of 30 times an hour, and lasting seconds each time. There was complete resolution of pain between the attacks without a refractory period, as one attack could be immediately followed by another. Marked ipsilateral lacrimation and rhinorrhea accompanied the attacks and persisted to a lesser degree in between. Her neurological examination, including facial sensation, was normal. High-flow oxygen, intravenous (IV) metoclopramide, parenteral sumatriptan, corticosteroids, indomethacin, and lidocaine patch were given in the emergency department 141 without relief. CMZ level was 3.7. She was admitted to the neurology service for treatment and workup of what appeared to be SUNCT status. Steroids, CMZ at a higher dose, and indomethacin were continued. The attacks of pain ceased the next day.Cerebrospinal fluid (CSF) had no white cells, normal glucose, and mildly elevated protein of 54. CSF tests for Lyme, HSV,VZV, CMV, EBV,West Nile, Eastern Equine, and LaCrosse viruses, Cryptococcus, ACE, cytology and flow cytometry were negative. In the serum, RPR was nonreactive, ESR 31, and CRP 13.8. Magnetic resonance imaging (MRI) showed a nonenhancing, nonrestricting lesion within the pontine portion of the left trigeminal nucleus and tract ( Fig.). There were also numerous periventricular and callosal white matter T2 hyperintensities that were consistent with demyelinat...

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Section: Discussionmentioning

confidence: 99%

A Case of MS Presenting With SUNCT Status

2010

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“…We summarized 69 cases of SUNCT-like conditions associated with certain etiologies in 62 English-language studies reported on from 1991 to 2017 and for which there were detailed descriptions of the clinical features and imaging results of the patients. These cases comprised 17 with neoplasm, 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 35 with neurovascular disease, 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 2 with trauma, 44 45 10 with infection, 46 47 48 49 50 51 52 53 54 3 with inflammatory disease, 55 56 57 and 2 with congenital malformation ( Table 1 , 2 , and 3 ). 58 59 …”

Section: Resultsmentioning

confidence: 99%

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

et al. 2018

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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.

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“…Most cases were defined as probably symptomatic SUNCT/SUNA. The cause found in patients with probably symptomatic SUNCT was most often compression of the trigeminal nerve by an artery (8 out of 14), followed by malignancies as a mixed gangliocytoma, an epidermoid tumour, and prolactinomas [ 3 •, 37 , 39 , 42 , 46 , 49 ]. Furthermore, an aneurysm and cavernous sinus dural fistula were found [ 38 , 40 ].…”

Section: Resultsmentioning

confidence: 99%

Symptomatic Trigeminal Autonomic Cephalalgias

Coo

Wilbrink

Haan

2015

Curr Pain Headache Rep

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Trigeminal autonomic cephalalgias (TACs) are primary headache syndromes that share some clinical features such as a trigeminal distribution of the pain and accompanying ipsilateral autonomic symptoms. By definition, no underlying structural lesion for the phenotype is found. There are, however, many descriptions in the literature of patients with structural lesions causing symptoms that are indistinguishable from those of idiopathic TACs. In this article, we review the recent insights in symptomatic TACs by comparing and categorizing newly published cases. We confirm that symptomatic TACs can have typical phenotypes. It is of crucial importance to identify symptomatic TACs, as the underlying cause will influence treatment and outcome. Our update focuses on when a structural lesion should be sought.

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SUNCT syndrome responding absolutely to steroids in two cases with different etiologies

Abstract: SUNCT is a syndrome of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. We are presenting now two cases absolutely responders to steroid therapy, one of them a SUNCT-like secondary to a prolactinoma and the other primary.

Cited by 20 publications

References 8 publications

A Case of MS Presenting With SUNCT Status

A Case of MS Presenting With SUNCT Status

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

Symptomatic Trigeminal Autonomic Cephalalgias

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