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.

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.

Uso de gabapentina no tratamento da Síndrome SUNCT / Gabapentin in the treatment of SUNCT syndrome

Relatamos o caso clínico de duas mulheres com quadro compatível com síndrome SUNCT (cefaléia de curta duração, unilateral, neuralgiforme com hiperemia conjuntival e lacrimejamento). As duas apresentavam exames clínico e neurológico normais e RM com sinais de microangiopatia. A primeira apresentava cefaléia há três anos, que ocorria várias vezes por dia, sempre que mastigava ou bocejava. Havia feito uso várias medicações sem melhora. A dor foi controlada após o uso de 600 mg de gabapentina ao dia. A segunda paciente referia cefaléia há seis meses. A dor era diária, ocorrendo de 20-40 vezes por dia. Na ocasião da primeira avaliação no ambulatório, já fazia uso 600 mg de carbamazepina ao dia e 15 mg de clorpromazina, com melhora parcial. Após introdução de gabapentina- 1200 mg/ dia, a paciente evoluiu sem dor, porém com episódios de hiperemia conjuntival.

We report the cases of two women who presented a clinical condition compatible with the SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) syndrome. Both presented normal clinical and neurological examination and MRI compatible with microangiopathy. The first one related headache attacks for three years, occurring several times a day when she masticated or yawned. She had a history of multiple failed therapies. The pain was controlled after the use of gabapetin (600 mg per day). The second one related she had daily headaches for six months that occurred from 20 to 40 times per day. At the first visit to the ambulatory, she related she was using carbamazepine (600 mg per day) and chlorpromazine (15 mg per day) having a partial response. Administration of gabapentin (1200 mg per day) led to complete resolution of the pain attacks, but the patient continue to have episodes of conjunctival injection.

Headache: Response to Lamotrigine.

A number of patients attending a psychiatry out patient department present with headache and cranial autonomic symptoms (CAS) which include cluster headache (CH), shortlasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA) and paroxismal hemicrania (PH). The present case reports describe two cases of SUNCT which showed response to lamotrigine. In a day-today practice SUNCT should be kept in mind as effective therapy is available.

Black, white and shades of grey: SUNCT or short-lasting chronic paroxysmal hemicrania? / Preto, branco e tons de cinza: SUNCT ou hemicrania paroxística crônica de curta duração?

AIM OF THE STUDY: To report a case of unilateral headache with two possibilities of diagnosis. METHOD: Case report. RESULTS: Patient with unilateral, intense, stabbing periocular headache with conjuntival injection and tearing. Although the duration of attacks was typical of SUNCT, there was complete remission of the pain with indomethacin, suggesting that this was a case of chronic paroxysmal hemicrania with unusually short attack duration. CONCLUSION: Therapeutic trials of indomethacin on younger patients presenting clinical diagnosis of SUNCT could be tried on a more regular basis.

MOTIVO DO ESTUDO: Relatar um caso de cefaléia unilateral com duas possibilidades diagnósticas. MÉTODOS: Relato de caso. RESULTADOS: Paciente com cefaléia unilateral, intensa, em pontadas em região periocular, com congestão conjuntival e lacrimejamento. Embora a duração das crises fosse típica de SUNCT, houve remissão completa da dor com indometacina, sugerindo que se trate de um caso de hemicramia paroxística crônica com crises de duração atipicamente muito curta. CONCLUSÃO: Testes terapêuticos com indometacina em pacientes mais jovens com diagnóstico clínico de SUNCT poderiam ser tentados de forma mais regular.

Bilateral SUNCT syndrome associated to chronic maxillary sinus disease

A síndrome SUNCT (short lasting unilateral neuralgiform headache with conjuntival injection and tearing) é definida como curtos ataques de dor periorbital unilateral, acompanhada de lacrimejamento e hiperemia conjuntival ipsilateral. Apresentamos um raro caso de SUNCT com dor bilateral com evolução de cinco anos e iniciado após uma infecção de seio maxilar que evoluiu para sinusite crônica. Esta associação foi descrita em poucos casos de SUNCT, porém pouco esclarecida. O paciente era um homem de 58 anos que preencheu um diário de dor que demonstrou o típico padrão circadiano da síndrome, com pioras matinais e vespertinas, e apresentou melhora com uso de gabapentina. Submetido a cirurgia endoscópica funcional em seio maxilar e evoluiu com modulação da dor, sugerindo um potencial efeito benéfico após tratamento da sinusopatia.Na revisão de literatura encontramos 21 casos de SUNCT bilateral, cinco dos quais apresentavam história de sinusite; no entanto, a relação entre as duas entidades permanece ainda incerta.

SUNCT syndrome associated with pituitary tumor: case report

O paciente relatado neste artigo apresentou uma condição clínica compatível com síndrome SUNCT (cefaléia de curta duração, unilateral, neuralgiforme com hiperemia conjuntival e lacrimejamento). Ele referia dor diária, intensa, em facada, localizada na região pré-auricular e temporal esquerdas. Cada ataque de dor permanecia por cerca de um minuto, com freqüência de duas a oito vezes por dia. A dor se acompanhava de lacrimejamento ipsolateral, congestão conjuntival e rinorréia. A RM mostrou um tumor de hipófise com pouca extensão suprasselar. Dosagens de prolactina, GH, TSH e ACTH estavam em níveis normais. Foi então submetido a hipofisectomia transesfenoidal com remoção completa do tumor após o que a dor cessou completamente. O diagnóstico anátomo-patológico foi adenoma não secretor. Quatorze meses após a cirurgia, o paciente permanecia livre de dor.

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.