A Case of MS Presenting With SUNCT Status

Abstract

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...

SUNCT in a Patient With Multiple Sclerosis: A Case Report

“…Follow‐up MRI T2WI revealed hyperintensities at the left trigeminal tract topography. These neuroimages support previous explanations of SUNCT's pathophysiology …”

Multiple sclerosis presenting initially with a worsening of migraine symptoms

“…This variation in frequency might be explained by differences in study design and patient inclusion criteria. Ophthalmoplegic migraine-like headache [10], complicated migraine [11], short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) [12], and cluster tic syndrome [13] have also been reported in single MS cases. Fragoso and Brooks [14] described a single case and Yetimalar et al [15] reported the cases of two young women with migraine onset as the only symptom of the first episode of MS.…”

Tics in TACs: A Step into an Avalanche? Systematic Literature Review and Conclusions

“…Secondary SUNCT/SUNA in a stricter sense has been related to vascular and infectious disorders, neoplasms, and other disorders (Table ) . Most frequently, SUNCT seems to be associated with pituitary adenomas showing a prevalence of 7% (three of 43 patients) in the case series of Cohen et al and 100% (five of five patients) in a series reported by Chitsantikul and Becker .…”