SUNCT/SUNA

2020 ◽  
pp. 196-202
Author(s):  
Juan A. Pareja ◽  
Leopoldine A. Wilbrink ◽  
María-Luz Cuadrado
Keyword(s):  
Deep Brain Stimulation ◽  
Trigeminal Nerve ◽  
Preventive Treatment ◽  
Gasserian Ganglion ◽  
Trigeminal Autonomic Cephalalgias ◽  
Sphenopalatine Ganglion ◽  
Conjunctival Injection ◽  
Intravenous Lidocaine ◽  
Drug Of Choice ◽  
Ablative Procedures

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA) are currently classified in the section of the trigeminal autonomic cephalalgias as separate subtypes of the same disorder (short-lasting unilateral neuralgiform headache attacks). There is no therapy consistently effective for SUNCT or SUNA. During the worst periods intravenous lidocaine or phenytoin may decrease the frequency of SUNCT/SUNA attacks. Lamotrigine is the drug of choice for the preventive treatment of SUNCT, but it is less effective for SUNA. Gabapentin may be also effective for both SUNCT and SUNA. Topiramate can be effective in SUNCT, but it has not shown an effect in SUNA. Some medically refractory cases have obtained benefit from surgical procedures. These include microvascular decompression of the trigeminal nerve, ablative procedures involving the trigeminal nerve, Gasserian ganglion or sphenopalatine ganglion, occipital nerve stimulation, and hypothalamic deep brain stimulation.

scholarly journals Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome secondary to an epidermoid tumor in the cerebellopontine angle

2013 ◽  
Vol 34 (3) ◽  
pp. E1 ◽  
Author(s):  
Shaun D. Rodgers ◽  
Bryan J. Marascalchi ◽  
Russell G. Strom ◽  
Paul P. Huang
Keyword(s):  
Medical Treatment ◽  
Trigeminal Nerve ◽  
Cerebellopontine Angle ◽  
Signs And Symptoms ◽  
Definitive Treatment ◽  
Trigeminal Autonomic Cephalalgias ◽  
Conjunctival Injection ◽  
Total Resection ◽  
Sunct Syndrome ◽  
Epidermoid Tumor

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection and lacrimation, suggestive of SUNCT syndrome. After failed medical treatment an MRI sequence was obtained in this patient, demonstrating an epidermoid tumor in the left cerebellopontine angle. The patient's symptoms completely resolved after a gross-total resection of the tumor. This case demonstrates the effectiveness of resection as definitive treatment for SUNCT syndrome associated with tumoral compression of the trigeminal nerve. Early MRI studies should be considered in all patients with SUNCT, especially those with atypical signs and symptoms.

Familial SUNCT in mother and son

Cephalalgia ◽  
2016 ◽  
Vol 36 (10) ◽  
pp. 993-997 ◽  
Author(s):  
Isabel Pavão Martins ◽  
Pedro Viana ◽  
Patricia Pita Lobo
Keyword(s):  
Trigeminal Nerve ◽  
Case Reports ◽  
Heterogeneous Group ◽  
Familial Case ◽  
Trigeminal Autonomic Cephalalgias ◽  
Conjunctival Injection ◽  
Historical Account ◽  
Autonomic Symptoms ◽  
Genetic Nature

Background Trigeminal autonomic cephalalgias comprise a heterogeneous group of lateralized headaches associated with ipsilateral autonomic symptoms. They are usually localized within the territory of one or more rami of the trigeminal nerve, but may be localized outside its cutaneous territory. Although these headaches are considered primary disorders, the evidence supporting their genetic nature is lacking, particularly concerning their neuralgic forms, with the exception of a familial case described partly based on a historical account. Case reports We report on a mother and son with episodic, short-lasting, intense, paroxysmal headaches, with the same localization in the left retroauricular region, associated with prominent conjunctival injection and tearing, which are consistent with the diagnosis of SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing). Discussion These cases corroborate the existence of hereditary forms of this disorder, thus supporting its primary nature.

Responsiveness of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing to hypothalamic deep brain stimulation

2009 ◽  
Vol 110 (2) ◽  
pp. 279-281 ◽  
Author(s):  
Mark K. Lyons ◽  
David W. Dodick ◽  
Virgilio Gerald H. Evidente
Keyword(s):  
Deep Brain Stimulation ◽  
Functional Imaging ◽  
Brain Stimulation ◽  
Primary Headache ◽  
Headache Disorder ◽  
Trigeminal Autonomic Cephalalgias ◽  
Conjunctival Injection ◽  
Imaging Studies ◽  
Primary Headache Disorder ◽  
Deep Brain

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe primary headache disorder that is often refractory to medical therapy. Although the pathogenesis of this and other trigeminal autonomic cephalalgias is not completely understood, ipsilateral activation of the posterior and inferior hypothalamus has been identified on functional imaging studies during attacks. The authors report on a case of SUNCT syndrome successfully treated with hypothalamic deep brain stimulation and discuss the current literature.

scholarly journals Termination of SUNCT with intravenous lignocaine followed by oral mexiletine

2018 ◽  
Vol 1 ◽  
pp. 251581631876882
Author(s):  
Eoin Mulroy ◽  
Oliver Armstrong-Scott ◽  
Peter Bergin
Keyword(s):  
Side Effects ◽  
Trigeminal Nerve ◽  
Facial Pain ◽  
Trigeminal Autonomic Cephalalgias ◽  
Conjunctival Injection ◽  
Autonomic Symptoms ◽  
First Report ◽  
Successful Transition

Background: The trigeminal autonomic cephalalgias (TACs) are a group of debilitating, pathophysiologically similar headache syndromes characterized by facial pain and autonomic symptoms in areas supplied by the trigeminal nerve. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is among the rarest of the TAC syndromes and can be particularly recalcitrant to treatment. Case: We describe the case of a 50-year old woman with difficult-to-control SUNCT whose pain was completely aborted within hours of commencing intravenous lignocaine therapy and was maintained pain-free after transitioning to oral mexiletine. Conclusion: This is the first report of successful transition from intravenous lignocaine to oral mexiletine in SUNCT, and we suggest that this treatment should be tried early in difficult-to-control SUNCT. This therapy is safe, effective and with minimal side effects if administered in an appropriate manner.

Extended middle fossa approach to lateralized pontine cavernomas in children

2018 ◽  
Vol 21 (4) ◽  
pp. 384-388 ◽  
Author(s):  
Robert C. Rennert ◽  
Reid Hoshide ◽  
Mark Calayag ◽  
Joanna Kemp ◽  
David D. Gonda ◽  
...  
Keyword(s):  
Trigeminal Nerve ◽  
Internal Auditory Canal ◽  
Csf Leak ◽  
Geniculate Ganglion ◽  
Gasserian Ganglion ◽  
Middle Fossa ◽  
Cavernous Malformations ◽  
Middle Fossa Approach ◽  
Cerebellar Artery ◽  
Mandibular Branch

OBJECTIVETreatment of hemorrhagic cavernous malformations within the lateral pontine region demands meticulous surgical planning and execution to maximize resection while minimizing morbidity. The authors report a single institution’s experience using the extended middle fossa rhomboid approach for the safe resection of hemorrhagic cavernomas involving the lateral pons.METHODSA retrospective chart review was performed to identify and review the surgical outcomes of patients who underwent an extended middle fossa rhomboid approach for the resection of hemorrhagic cavernomas involving the lateral pons during a 10-year period at Rady Children’s Hospital of San Diego. Surgical landmarks for this extradural approach were based on the Fukushima dual-fan model, which defines the rhomboid based on the following anatomical structures: 1) the junction of the greater superficial petrosal nerve (GSPN) and mandibular branch of the trigeminal nerve; 2) the lateral edge of the porus trigeminus; 3) the intersection of the petrous ridge and arcuate eminence; and 4) the intersection of the GSPN, geniculate ganglion, and arcuate eminence. The boundaries of maximal bony removal for this approach are the clivus inferiorly below the inferior petrosal sinus; unroofing of the internal auditory canal posteriorly; skeletonizing the geniculate ganglion, GSPN, and internal carotid artery laterally; and drilling under the Gasserian ganglion anteriorly. This extradural petrosectomy allowed for an approach to all lesions from an area posterolateral to the basilar artery near its junction with cranial nerve (CN) VI, superior to the anterior inferior cerebellar artery and lateral to the origin of CN V. Retraction of the mandibular branch of the trigeminal nerve during this approach allowed avoidance of the region involving CN IV and the superior cerebellar artery.RESULTSEight pediatric patients (4 girls and 4 boys, mean age of 13.2 ± 4.6 years) with hemorrhagic cavernomas involving the lateral pons and extension to the pial surface were treated using the surgical approach described above. Seven cavernomas were completely resected. In the eighth patient, a second peripheral lesion was not resected with the primary lesion. One patient had a transient CN VI palsy, and 2 patients had transient trigeminal hypesthesia/dysesthesia. One patient experienced a CSF leak that was successfully treated by oversewing the wound.CONCLUSIONSThe extended middle fossa approach can be used for resection of lateral pontine hemorrhagic cavernomas with minimal morbidity in the pediatric population.

scholarly journals Severe Headache with Eye Involvement from Herpes Zoster Ophthalmicus, Trigeminal Tract, and Brainstem Nuclei

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Sasitorn Siritho ◽  
Wadchara Pumpradit ◽  
Wiboon Suriyajakryuththana ◽  
Krit Pongpirul
Keyword(s):  
Trigeminal Nerve ◽  
Maculopapular Rash ◽  
Cervical Cord ◽  
Gasserian Ganglion ◽  
Herpes Zoster Ophthalmicus ◽  
Trigeminal Nuclei ◽  
Upper Cervical ◽  
Sensory Nucleus ◽  
The Right ◽  
Orbital Imaging

A 43-year-old female presented with severe sharp stabbing right-sided periorbital and retroorbital area headache, dull-aching unilateral jaw pain, eyelid swelling, ptosis, and tearing of the right eye but no rash. The pain episodes lasted five minutes to one hour and occurred 10–15 times per day with unremitting milder pain between the attacks. She later developed an erythematous maculopapular rash over the right forehead and therefore was treated with antivirals. MRI performed one month after the onset revealed small hypersignal-T2 in the right dorsolateral mid-pons and from the right dorsolateral aspect of the pontomedullary region to the right dorsolateral aspect of the upper cervical cord, along the course of the principal sensory nucleus and spinal nucleus of the right trigeminal nerve. No definite contrast enhancement of the right brain stem/upper cervical cord was seen. Orbital imaging showed no abnormality of bilateral optic nerves/chiasm, extraocular muscles, and globes. Slight enhancement of the right V1, V2, and the cisterna right trigeminal nerve was detected. Our findings support the hypothesis of direct involvement by virus theory, reflecting rostral viral transmission along the gasserian ganglion to the trigeminal nuclei at brainstem and caudal spreading along the descending tract of CN V.

Melanotic nerve sheath tumor of the gasserian ganglion and trigeminal nerve

1979 ◽  
Vol 133 (1) ◽  
pp. 142-144 ◽  
Author(s):  
RM Quencer ◽  
NA Stokes ◽  
D Wolfe ◽  
LK Page
Keyword(s):  
Trigeminal Nerve ◽  
Nerve Sheath Tumor ◽  
Gasserian Ganglion ◽  
Nerve Sheath

Hypothalamic stimulation for trigeminal neuralgia in multiple sclerosis patients: efficacy on the paroxysmal ophthalmic pain

2009 ◽  
Vol 15 (11) ◽  
pp. 1322-1328 ◽  
Author(s):  
R. Cordella ◽  
A. Franzini ◽  
L. La Mantia ◽  
C. Marras ◽  
A. Erbetta ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Deep Brain Stimulation ◽  
Trigeminal Neuralgia ◽  
Posterior Hypothalamus ◽  
Corneal Reflex ◽  
Multiple Sclerosis Patients ◽  
Ablative Procedures ◽  
Paroxysmal Pain ◽  
Deep Brain

Trigeminal neuralgia is a disorder characterized by paroxysmal pain arising in one or more trigeminal branches; it is commonly reported in multiple sclerosis. In multiple sclerosis patients the ophthalmic branch may be frequently involved and the risks carried by neurosurgical ablative procedures are higher including major adverse effects such as corneal reflex impairment and keratitis. The objective of this works is to assess the role of posterior hypothalamus neuromodulation in the treatment of trigeminal neuralgia in multiple sclerosis patients. Five multiple sclerosis patients suffering from refractory recurrent trigeminal neuralgia involving all three trigeminal branches underwent deep brain stimulation of the posterior hypothalamus. The rationale of this intervention emerges from our earlier success in treating pain patients suffering from trigeminal autonomic cephalalgias. After follow-up periods that ranged from 1 to 4 years after treatment, the paroxysmal pain arising from the first trigeminal branch was controlled, whereas the recurrence of pain in the second and third trigeminal branches necessitated repeated thermorhizotomies to control in pain in two patients after 2 years of follow-up. In conclusion, deep brain stimulation may be considered as an adjunctive procedure for treating refractory paroxysmal pain within the first trigeminal division so as to avoid the complication of corneal reflex impairment that is known to follow ablative procedures.

scholarly journals Neurosurgery for Chronic Neuropathic Pain

2000 ◽  
Vol 5 (1) ◽  
pp. 101-106
Author(s):  
Jung Y Park ◽  
Andres M Lozano
Keyword(s):  
Neuropathic Pain ◽  
Deep Brain Stimulation ◽  
Motor Cortex ◽  
Chronic Neuropathic Pain ◽  
Pain Mechanisms ◽  
Surgical Interventions ◽  
Spinal Motor ◽  
Limited Efficacy ◽  
Ablative Procedures ◽  
Deep Brain

Neurosurgery can play a role in the management of patients with refractory chronic neuropathic pain. However, selecting patients as candidates for surgery and choosing the most appropriate surgical procedure is challenging, and surgical interventions often have limited efficacy. When considering surgery, neuroaugmentative or neuromodulative procedures (eg, peripheral, spinal, motor cortex or deep brain stimulation) are generally preferred over ablative procedures as initial modalities. With better understanding of specific pain mechanisms, surgery will have more to offer patients with chronic neuropathic pain.

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