A transformation of trigeminal neuralgia into SUNCT/SUNA: A case report and literature review / Uma transformação da neuralgia do trigêmeo em SUNCT/SUNA: relato de caso e revisão da literatura

Introduction: Trigeminal neuralgia and Short-lasting Unilateral Neuralgiform Headache with Conjunctival injection and Tearing (SUNCT)/Short-lasting Unilateral Neuralgiform Headache Attacks with Cranial Autonomic Symptoms (SUNA) are characterized by similar clinical manifestations, which may lead to diagnostic confusion. However, the transformation of trigeminal neuralgia into SUNCT/SUNA is a rare phenomenon. This report describes a case of trigeminal neuralgia transformation into SUNCT/SUNA due to neurovascular compression and reviews all previously published cases of trigeminal neuralgia to SUNCT/SUNA transformation in the literature. Case presentation: A 49-year-old Thai male patient presented with progressive right facial pain for a period of three months. One year prior, he developed trigeminal neuralgia along the maxillary branch of the trigeminal nerve, characterized by electrical shock-like pain in the right upper molar, exacerbated by eating. His symptoms were effectively managed with carbamazepine. Nine months later, he began experiencing recurrent electrical shock-like pain along the ophthalmic division of the right trigeminal nerve, accompanied by lacrimation, which failed to respond to continued treatment with carbamazepine. Three months prior to presentation, his symptoms evolved into SUNCT/SUNA, characterized by electrical shock-like pain in the right periorbital area and conjunctival injection, lacrimation. Neuroimaging revealed high-grade neurovascular compression of the right trigeminal nerve by the right superior cerebellar artery. The patient's symptoms resolved following microvascular decompression. Conclusion: Clinicians should be aware that patients with longer disease duration of trigeminal neuralgia who develop new neuralgic pain in the ophthalmic branch division with mild autonomic symptoms may be at risk for transformation into SUNCT/SUNA.

Introdução: Neuralgia do trigêmeo e Cefaléia neuralgiforme unilateral de curta duração com injeção e lacrimejamento conjuntival (SUNCT)/Crises de cefaléia neuralgiforme unilateral de curta duração com sintomas autonômicos cranianos (SUNA) são caracterizadas por manifestações clínicas semelhantes, o que pode levar à confusão diagnóstica. Contudo, a transformação da neuralgia do trigêmeo em SUNCT/SUNA é um fenômeno raro. Este relato descreve um caso de transformação de neuralgia do trigêmeo em SUNCT/SUNA devido à compressão neurovascular e revisa todos os casos de neuralgia do trigêmeo para transformação SUNCT/SUNA publicados anteriormente na literatura. Apresentação do caso: Paciente tailandês de 49 anos, sexo masculino, apresentou dor facial progressiva à direita há três meses. Há um ano, ele desenvolveu neuralgia do trigêmeo ao longo do ramo maxilar do nervo trigêmeo, caracterizada por dor tipo choque elétrico no molar superior direito, exacerbada pela alimentação. Seus sintomas foram controlados de forma eficaz com carbamazepina. Nove meses depois, ele começou a sentir dor recorrente semelhante a choque elétrico ao longo da divisão oftálmica do nervo trigêmeo direito, acompanhada de lacrimejamento, que não respondeu ao tratamento continuado com carbamazepina. Três meses antes da apresentação, os sintomas evoluíram para SUNCT/SUNA, caracterizado por dor tipo choque elétrico na região periorbital direita e injeção conjuntival, lacrimejamento. A neuroimagem revelou compressão neurovascular de alto grau do nervo trigêmeo direito pela artéria cerebelar superior direita. Os sintomas do paciente foram resolvidos após descompressão microvascular. Conclusão: Os médicos devem estar cientes de que pacientes com maior duração da neuralgia do trigêmeo que desenvolvem nova dor nevrálgica na divisão do ramo oftálmico com sintomas autonômicos leves podem estar em risco de transformação em SUNCT/SUNA.

Lidocaína endovenosa no tratamento da síndrome SUNCT secundária a tumor de hipófise: seguimento de uma paciente por um período de quatro anos. Relato de caso / Intravenous lidocaine to treat SUNCT syndrome secondary to pituitary tumor: follow up of a patient for four years. Case report

JUSTIFICATIVA E OBJETIVOS: A síndrome SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome) é descrita como dor de localização orbitária, supraorbitária ou temporal unilateral, caracterizada como em pontada ou pulsátil, de forte intensidade, incapacitante, de curta duração, variando entre 5 e 240 segundos. É uma síndrome dolorosa de difícil tratamento, no entanto, no presente caso obteve-se resposta analgésica satisfatória com lidocaína endovenosa. RELATO DO CASO: Paciente do gênero feminino, 32 anos portadora de síndrome SUNCT secundária a tumor de hipófise (submetida a hipofisectomia transesfenoidal e radiocirurgia), passou a apresentar 30 crises de dor diariamente. Não obteve resposta analgésica com o procedimento cirúrgico. Foi iniciado tratamento farmacológico com lamotrigina, clorpromazina e amitriptilina sem alivio da dor. Submetida a ciclos de infusão de lidocaína venosa obteve adequado alivio da dor. CONCLUSÃO: Neste caso de síndrome SUNCT, a lidocaína endovenosa foi capaz de proporcionar analgesia adequada e duradoura, podendo ser considerada como parte integrante nos pacientes portadores dessa de síndrome.

BACKGROUND AND OBJECTIVES: SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome) is described as unilateral orbitary, supraorbitary or temporal pain, characterized as stabbing or throbbing, severe, disabling, of short duration, varying from 5 to 240 seconds. This is a difficult to treat syndrome, however in our case there has been satisfactory analgesia with intravenous lidocaine. CASE REPORT: Female patient, 32 years old, with SUNCT syndrome secondary to pituitary tumor (submitted to transsphenoidal hypophysectomy and radio-surgery), started presenting 30 daily pain crises. There has been no analgesic response to surgical procedure. Pharmacological treatment was started with lamotrigine, chlorpromazine and amitriptyline without pain relief. Patient was submitted to intravenous lidocaine infusion cycles with adequate pain relief. CONCLUSION: In this case of SUNCT syndrome, intravenous lidocaine was able to promote adequate and long-lasting analgesia and may be considered integral part of the treatment of this syndrome.

Manejo analgésico y anestésico en la mastocitosis sistémica: a propósito de un caso / Analgesic and anesthetic management in systemic mastocytosis: a case report

Las mastocitosis son un grupo heterogéneo de enfermedades que se caracterizan por la proliferación de mastocitos en uno o más órganos o tejidos. Se denomina mastocitosis sistémica (MS) cuando hay afectación de un tejido distinto a la piel. La MS es una enfermedad poco frecuente, cuya incidencia y prevalencia se desconocen. El manejo anestésico de estos pacientes debe considerar que muchos de los fármacos empleados pueden causar una liberación masiva de mediadores químicos mastocitarios. Se presenta el caso de una mujer con MS programada para una histerectomía total, valorando la importancia del correcto estudio preoperatorio así como la técnica anestésica y el tipo de analgesia elegida en este caso. Se presenta nuestro protocolo de actuación de cara a la cirugía en estos pacientes.

Mastocytoses are a heterogeneous group of entities characterized by mast cell proliferation in one or more organs or tissues. When tissues other than the skin are involved, the disease is called systemic mastocytosis (SM). SM is a highly infrequent disease, whose incidence and prevalence are unknown. The anesthetic management of these patients must consider the fact that many drugs can trigger massive release of chemical mediators of mast cells. We report the case of a patient diagnosed with SM who underwent total hysterectomy and discuss the importance of thorough preoperative study, as well as the anesthetic technique and type of analgesia chosen. We also report our protocol for anesthetic management in this disease.

Cefaleas trigémino autonómicas: 3 casos de localización dentomaxilar / Trigeminal autonomic cephalalgias: 3 cases of dentomaxillar location

Las cefaleas trigemino autonómicas (CTAs: cefalea tipo cluster, hemicránea paroxística y el SUNCT) son un grupo de cefaleas primarias, caracterizadas por la presencia de dolor unilateral en la distribución somática del nervio trigémino, asociada a características autonómicas cráneofaciales ipsilaterales. A pesar de sus elementos comunes, de forma individual, difieren con respecto a su duración, frecuencia y la respuesta a indometacina. Se presentan tres casos de CTAs de localización primaria dentomaxilar, sus características comunes y particulares, y la necesidad del diagnóstico diferencial con cuadros dolorosos provenientes de estructuras estomatognáticas.

The Trigeminal Autonomic Cephalalgias (TACs: Cluster headache, paroxysmal hemicrania and SUNCT) are a primary headache grouped characterized by the presence of unilateral pain in the somatic distribution of the trigeminal nerve, associated with craniofacial ipsilateral autonomic features. Despite their common elements, individually these headaches differ with respect to attack duration, frequency, and response to indomethacin.A three cases of TACs of dentomaxilar location is presented, common and particular characteristics, and the need for differential diagnosis with pain from stomatognathic structures.

Patent foramen ovale in trigeminal autonomic cephalalgias and hemicrania continua: a non-specific pathophysiological occurrence? / Foramen oval patente em cefaleias trigêmino-autonômicas e hemicrania contínua: uma ocorrência fisiopatológica não específica?

Patent foramen ovale (PFO), a relatively common abnormality in adults, has been associated with migraine. Few studies also linked PFO with cluster headache (CH). To verify whether right-to-left shunt (RLS) is related to headaches other than migraine and CH, we used transcranial Doppler following microbubbles injection to detect shunts in 24 CH, 7 paroxysmal hemicrania (PH), one SUNCT, two hemicrania continua (HC) patients; and 34 matched controls. RLS was significantly more frequent in CH than in controls (54 percent vs. 25 percent, p=0.03), particularly above the age of 50. In the HC+PH+SUNCT group, RLS was found in 6 patients and in 2 controls (p=0.08). Smoking as well as the Epworth Sleepiness Scale correlated significantly with CH, smoking being more frequent in patients with RLS. PFO may be non-specifically related to trigeminal autonomic cephalalgias and HC. The headache phenotype in PFO patients probably depends on individual susceptibility to circulating trigger factors.

O forame oval patente (FOP), uma anormalidade cardíaca relativamente comum em adultos, tem sido associado à enxaqueca, mas raramente às cefaléias trigêmino-autonômicas (TACs). Utilizamos o Doppler transcraniano (DTC) para detecção de shunt direito-esquerdo (SDE) em 24 pacientes com cefaléia em salvas (CS), sete com hemicrania paroxística (HP), dois com hemicrania continua (HC) e um com SUNCT; alem de 34 controles. O SDE foi mais frequente nos pacientes com CS do que nos controles (54 por cento vs. 25 por cento p=0,03), particularmente acima de 50 anos. No grupo HP+HC+SUNCT, o SDE foi encontrado em seis pacientes e dois controles (p=0,08). O hábito de fumar, bem como sonolência excessiva diurna foram mais frequentes em paciente com CS. O FOP pode ter importância inespecífica na fisiopatologia das TACs e HC, na dependência da susceptibilidade individual a fatores desencadeantes.

Qué debe conocer el neurólogo de las cefaleas en salvas y otras hemicráneas autonomotrigeminales / What must the neurologist know about cluster headaches, and other trigeminal autonomic hemicranias

Aunque la migraña y los dolores de cabeza de tipo tensión son las cefalalgias más frecuentes en la práctica clínica, es muy importante el reconocimiento de otros síndromes dolorosos menos comunes como la cefalea en salvas, la hemicránea paroxística crónica y otros síndromes autonomotrigeminales. Estos síndromes dolorosos comparten algunos rasgos clínicos. El diagnóstico diferencial se basa en el número y la frecuencia de los episodios y la intensidad del dolor, así como en la respuesta a medicamentos específicos. La cefalea en salvas, las hemicráneas paroxísticas, la hemicránea continua y otros síndromes se incluyeron en la clasificación internacional de dolor de cabeza modificada por la IHS en el 2004. Esta revisión muestra los aspectos epidemiológicos, fisiopatológicos y clínicos y el tratamiento de estas cefaleas.

Although migraine and tension type headaches are the most frequent disorders in clinical practice, it is very important to recognize other painful syndromes such as cluster, paroxysmal hemicranias and trigeminal autonomic cephalgias wich are less common. These painful syndromes share some clinical features, and the main differential diagnoses are based on the number and the frequency of the episodes, and the intensity of pain, as well as in the response to specific medications. Cluster headache, paroxysmal hemicranias, hemicrania continua and other syndromes were included in the International Classification of Headache modified by the IHS in 2004. This review shows the epidemic aspects, pathophysiopatology, clinical manifestations and treatment of these headaches.

SUNCT syndrome: report of a possible symptomatic case

SUNCT is one of the rarest and least known primary headache disorders. Although its pathogenesis has been partially understood by functional neuroimaging and reports of secondary cases, there is limited understanding of its cause. We report a case of SUNCT in a 54-years-old man, that could not be strictly classified as secondary SUNCT; however, the time lag of pain onset suggests a new theory in which neuroplasticity could be involved in the origin and duration of the pain in SUNCT syndrome.

SUNCT é uma das mais raras e menos conhecidas cefaléias primárias. Embora sua patogênese esteja parcialmente compreendida por neuroimagem funcional e relatos de casos secundários, há insuficiente conhecimento a respeito de sua causa. Nós relatamos um caso de SUNCT em um homem de 54 anos, que não poderia ser estritamente classificado como SUNCT secundário; entretanto, o lapso de tempo para o início da dor sugere uma nova hipótese na qual a neuroplasticidade possa esta envolvida na origem e duração da dor na síndrome SUNCT.

Diagnosis and therapy of trigeminal autonomic cephalalgias / 中国实用内科杂志

This paper introduces the classification of trigeminal autonomic cephalalgias (TAC) and their clinical manifestation,diagnostic criteria and therapy of main type.TAC share the clinical features of headache and prominent cranial parasympathetic autonomic features.The most effective therapy for acute cluster headache is oxygen inhalation and Sumatriptan subcutaneous injection.Ergotamine is the first choice drug for the preventive treatment of episodic cluster headache and Verapamil coupled with Lithium salt is preventive treatment for chronic cluster headache.Indomethacin is specific treatment for paroxysmal hemicrania.Lamotrigine,Topiramate and Gabapentin are all useful to SUNCT.

Verapamil-responsive SUNCT Syndrome

SUNCT syndrome is characterized by short-lasting, unilateral, neuralgiform headache attacks, with conjuntival injec-tion and tearing. Distinct from trigeminal neuralgia, cluster headache, or paroxysmal hemicrania, this rare syndrome is SUNCT syndrome is characterized by short-lasting, unilateral, neuralgiform headache attacks, with conjuntival injec-tion and tearing. Distinct from trigeminal neuralgia, cluster headache, or paroxysmal hemicrania, this rare syndrome is generally refractory to various medications. We report a 63-year-old man diagnosed with SUNCT syndrome, whose symptoms were relieved by verapamil. The therapeutic effects of verapamil on SUNCT syndrome remain to be verified by further experiences. (J Korean Neurol Assoc 19(4):407~409, 2001)

A Case of SUNCT Syndrome which Showed Marked Improvement with Carbamazepine and Discussion on Nosologic Aspect of SUNCT Syndrome

SUNCT syndrome is a headache syndrome characterized by short-lasting(usually 15-120 sec), unilateral head paroxysms localized in the peri-ocular area, accompanied by conjunctival injection, lacrimation, nasal stuffiness, rhinorrhea, and subclinical forehead sweating, all on the symptomatic side. We experienced a new SUNCT syndrome case. 57-year-old patient had been suffering from right orbital pain attacks since he was 54. The pain, of an ""electrical"" or ""burning"" character, was moderate to severe in intensity. Single attacks lasted for 30-60 seconds each and were accompanied by prominent ipsilateral conjunctival injection and tearing as well as slight rhinorrhea. His symptoms were relieved by carbamazepine. Carbamazepine treatment seemingly brought about marked decrease in the frequency and severity of attacks. This case may be the first reported SUNCT syndrome case in Korea. Furthermore this case may shed light on the nosologic point view of SUNCT syndrome. The suspected pathophysiology of trigeminal neuralgia is expansion of the low threshold portions of the receptive fields owing to loss of surround inhibition. Carbamazepine facilitate afferent inhibition and depress excitatory transmission in the trigeminal nucleus. So the effectiveness to carbamazepine has been considered as one of diagnostic criteria of trigeminal neuralgia. Through the survey of previous reported cases including our case, it is suspected that SUNCT syndrome be closer to trigeminal neuralgia rather than cluster headache. We discussed several viewpoints for the nosologic aspect of SUNCT syndrome in this report.