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Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical condition characterized by arterial involvement of the intracranial vessels, manifesting with vasospasm. The most common clinical manifestation related to the syndrome is the thunderclap headache, which consists of a severe headache that reaches the peak of pain within minutes. The imaging study assumes a leading role in the complementary investigation. Laboratory tests and cerebrospinal fluid analysis are often nonspecific and without significant diagnostic importance. Non-contrast studies of the brain parenchyma reveal variable results that can often be normal. Angiographic findings, which initially may not reveal any changes, allow the visualization of diffuse narrowing of the vessels, with the posterior cerebral circulation being preferentially affected. The present study reports the case of a 19-year-old woman with no relevant medical history, except that she was a regular user of marijuana and a drug based on chloroform and ether. The patient showed clinical and imaging signs compatible with RCVS, and the narcotics used by her were considered precipitating factors.

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BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is defined as a clinical and radiological syndrome that comprises a group of disorders characterized by sudden-onset severe headache and segmental vasoconstriction of the cerebral arteries with resolution within 3 months. CASE PRESENTATION: A 51-year-old female patient with a 2-week history of sudden-onset severe headache, visual disturbances and cerebellum; no relevant imaging findings, except for an infundibular dilation at the origin of the posterior communicating artery, and so, angiography was performed. When symptoms persisted, a new imaging study was carried out with findings of RCVS as the cause of the symptoms from the beginning. CONCLUSIONS: Findings of RCVS can be obtained in various vasculopathies of the nervous system and vasculitis, being misdiagnosed, and so, clinical suspicion is essential; if vasoconstriction is not demonstrated on the initial image and other diagnoses have been excluded, the patient should be managed as having possible or probable RCVS. LEARNING POINTS: RCVS is a clinical and radiological syndrome that comprises a group of disorders characterized by sudden-onset severe headache and segmental vasoconstriction of the cerebral arteries.It can occur spontaneously or can be triggered by a specific "trigger" in 25 to 60% of cases.It commonly affects women between 20 and 50 years of age, but children and adolescents may also be affected.

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Reversible cerebral vasoconstriction syndrome (RCVS) is characterised by severe thunderclap headaches (with or without the presence of acute neurological symptoms) and segmental vasoconstriction of cerebral arteries that resolves spontaneously in a period of three months. Cases have been described in the literature with producing and non-producing masses of metanephrines. Within these reports, associations with cavernous haemangioma, medulloblastoma, colon cancer, paraganglioma, pheochromocytoma, uterine fibroids, among others were found. However, no association with adrenal masses which do not produce metanephrines was found. In this context, we reported the case of a woman with this type of tumour associated with RCVS which provided a treatment challenge, as well as we reviewed the literature on cases of RCVS associated with masses.

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RESUMEN El síndrome de vasoconstricción cerebral reversible es una entidad clínico-radiológica caracterizada por la presentación de cefalea severa de inicio hiperagudo tipo "trueno", con o sin signos y síntomas neurológicos adicionales en relación a una vasoconstricción arterial cerebral segmentaria que resuelve espontáneamente a los 3 meses. Por la superposición de las manifestaciones clínicas con otras entidades nosológicas, y por los múltiples factores etiológicos asociados, el diagnóstico se convierte en un reto; es imperativo realizarlo de forma temprana para la instauración de un tratamiento adecuado y la prevención de complicaciones. Se presenta el caso clínico de una paciente en quien se documentó como etiología la realización repetitiva de la maniobra de Valsalva sin otro factor concomitante, se exponen las intervenciones realizadas y se hace una revisión narrativa del tema con énfasis en el diagnóstico diferencial.

SUMMARY Reversible cerebral vasoconstriction syndrome is a clinical-radiological entity characterized by severe and hyperacute onset-thunderclap headache, with or without additional neurological signs and symptoms in relation to a segmental cerebral arterial vasoconstriction that resolves spontaneously at around 3 months. Its clinical manifestations are similar to other diseases, and additionally there are multiple associated etiological factors; early diagnosis becomes a challenge, but is essential to establish proper treatment and prevent complications. We present the case of a female patient in whom the repetitive performance of the Valsalva maneuver without another concomitant factor was documented as etiology, the interventions performed are presented and a narrative review of the topic is made with emphasis on differential diagnosis.

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Abstract Reversible vasoconstriction syndrome is a group of clinical-radiological alterations that are characterized by severe sudden-onset headaches and reversible multifocal narrowing of the cerebral arteries. Most patients do not present with focal neurological deficit, although it can be seen in a small group, associated with cerebral edema, stroke or seizures. It is considered to be a benign process that causes disability and death in a minority of patients. The term 'reversible vasoconstriction syndrome' has been proposed to unify a variety of clinical syndromes which are similar, but have different etiologies, and have originated various eponyms. The apparently low frequency of reversible vasoconstriction syndrome and the way it presents make it a diagnostic challenge in the emergency room, and it may go unnoticed without an adequate medical history. A case probably related to the use of isometeptene is presented. (Acta Med Colomb 2019; 44. DOI: https://doi.org/10.36104/amc.2019.1213)

Resumen El síndrome de vasoconstricción reversible es un grupo de alteraciones clínico-radiológicas que se caracterizan por cefaleas intensas de inicio brusco y estrechamiento multifocal reversible de las arterias cerebrales. La mayoría de los pacientes no presentan déficit neurológico focal, aunque puede verse en un grupo reducido asociándose con edema cerebral, ataque cerebrovascular o convulsiones. Es considerado un proceso benigno, en pocos casos originan discapacidad y muerte en una minoría de pacientes. El término de síndrome de vasoconstricción reversible se ha propuesto para unificar a una variedad de síndromes clínicos similares, pero de etiología diferentes y han originados diversos epónimos. La aparente baja frecuencia del síndrome de vasoconstricción reversible y su forma de presentación hace que se convierta en un reto diagnóstico en los servicios de urgencias y puede pasar desapercibido si no se tiene una historia clínica adecuada. Presentamos un caso probablemente relacionado al uso de isometepteno. (Acta Med Colomb 2019; 44. DOI:https://doi.org/10.36104/amc.2019.1213)

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Abstract Reversible cerebral vasoconstriction syndrome is a cerebrovascular disorder leading to multifocal arterial constriction and dilation. Reversible cerebral vasoconstriction syndrome is possibly caused by transient deregulation of cerebral vascular tone. We report a rare case of a patient with chief complain of postpartum headache, was later diagnosed as a case of reversible cerebral vasoconstriction syndrome. A young full term primigravida with good uterine contraction admitted to labour room. Later she complained of leaking per vagina and on examination meconium stained liquor was noted. Caesarean delivery under spinal anesthesia was done and intra-operative period was uneventful. Both mother and baby were normal and shifted to postoperative ward and nursery respectively. In postoperative ward, mother complained of severe headache after 1 h and later developed seizure. Midazolam was given intravenously and was intubated and transferred to critical care unit for further investigation and management. Non contrast computerised tomography scan of brain showed right occipital intracerebral as well as subarachnoid bleed. CT angiography showed right vertebral artery narrowing without any other vascular malformation. Patient was managed in critical care unit for 2 days and then extubated and shifted to high dependency ward after a day observation and discharged 3 days later after a full uneventful recovery.

Resumo A síndrome de vasoconstrição cerebral reversível é uma doença cerebrovascular que leva à constrição e dilatação arterial multifocal. A síndrome de vasoconstrição cerebral reversível é possivelmente causada pela desregulação transitória do tônus vascular cerebral. Relatamos um caso raro de uma paciente com queixa principal de cefaleia pós-parto, posteriormente diagnosticada como um caso de síndrome de vasoconstrição cerebral reversível. A jovem primigesta a termo apresentando boa contração uterina foi internada em sala de parto. Mais tarde, a parturiente queixou-se de perda de líquido pela vagina e, ao exame, líquido amniótico manchado foi observado. O parto cesariano sob raquianestesia foi realizado, e não houve intercorrência no período intraoperatório. Tanto a mãe quanto o bebê estavam normais e foram transferidos para a sala de recuperação pós-operatória e berçário, respectivamente. Na sala de recuperação, a mãe queixou-se de forte dor de cabeça após uma hora e depois desenvolveu convulsão. Midazolam foi administrado por via intravenosa, e a paciente foi intubada e transferida para uma unidade de terapia intensiva para posterior investigação e tratamento. A tomografia computadorizada sem contraste do cérebro mostrou hemorragia intracerebral occipital direita e subaracnoide. A angiotomografia mostrou estreitamento da artéria vertebral direita, sem qualquer outra malformação vascular. A paciente foi tratada em unidade de terapia intensiva por dois dias e, em seguida, foi extubada e transferida para a ala de alta dependência onde permaneceu um dia em observação, recebendo alta hospitalar três dias depois, após uma recuperação completa e sem intercorrências.

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Reversible cerebral vasoconstriction syndrome is a cerebrovascular disorder leading to multifocal arterial constriction and dilation. Reversible cerebral vasoconstriction syndrome is possibly caused by transient deregulation of cerebral vascular tone. We report a rare case of a patient with chief complain of postpartum headache, was later diagnosed as a case of reversible cerebral vasoconstriction syndrome. A young full term primigravida with good uterine contraction admitted to labour room. Later she complained of leaking per vagina and on examination meconium stained liquor was noted. Caesarean delivery under spinal anesthesia was done and intra-operative period was uneventful. Both mother and baby were normal and shifted to postoperative ward and nursery respectively. In postoperative ward, mother complained of severe headache after one hour and later developed seizure. Midazolam was given intravenously and was intubated and transferred to critical care unit for further investigation and management. Non contrast computerized tomography scan of brain showed right occipital intracerebral as well as subarachnoid bleed. CT angiography showed right vertebral artery narrowing without any other vascular malformation. Patient was managed in critical care unit for 2 days and then extubated and shifted to high dependency ward after a day observation and discharged 3 days later after a full uneventful recovery.

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Resumen ANTECEDENTES: El síndrome de vasoconstricción cerebral reversible, o síndrome de Call-Fleming, es una alteración excepcional, de origen incierto, caracterizada por cefalea, déficit neurológico y vasoespasmo cerebral reversible. En cuanto a su mecanismo fisiopatológico, se señala la relación con vasoconstricción segmentaria reversible en diferentes áreas cerebrales. CASO CLÍNICO: Paciente de 34 años, sin antecedentes de importancia para el padecimiento actual, de 40 semanas de embarazo, que ingresó a la unidad médica por actividad uterina irregular, con cifras tensionales de 120-80 mmHg, pulso 80 por minuto, frecuencia respiratoria 20 por minuto y temperatura de 37 ºC; la frecuencia cardiaca fetal fue de 140 latidos por minuto. Al no existir progresión del trabajo de parto, se decidió su interrupción por vía abdominal y se obtuvo un recién nacido sano. Durante el puerperio inmediato, después de la administración de ergonovina, la paciente manifestó hipertensión arterial y cefalea súbita relacionada con déficit neurológico, por lo que requirió el ingreso a la unidad de cuidados intensivos hasta su estabilización. Se estableció el diagnóstico de cefalea tipo "trueno"; la tomografía computada no mostró alteraciones aparentes, el líquido cefalorraquídeo fue claro, con concentración leucocitaria de 10 mm3 y eritrocitaria de 6 mm3. CONCLUSIÓN: El síndrome de Call-Fleming es una alteración poco frecuente durante el embarazo y puerperio; por tanto, es importante conocer, identificar y establecer el diagnóstico diferencial de la enfermedad.

Abstract BACKGROUND: Reversible cerebral vasoconstriction syndrome, or Call-Fleming syndrome, is an exceptional disorder of uncertain origin, characterized by headache, neurological deficit and reversible cerebral vasospasm. Regarding its physiopathological mechanism, the relationship with reversible segmental vasoconstriction in different brain areas has been postulated. CLINICAL CASE: A 34-year-old patient, in the 40 weeks pregnant, admitted to the medical unit due to irregular uterine activity, with blood pressure figures of 120/80 mmHg, pulse 80/min, respiratory rate 20/min and temperature of 37 ° C; the fetal heart rate was 140 beats per minute. Due to the non-progression of labor, it was decided to interrupt it by abdominal route, from which a healthy newborn was obtained. During the immediate puerperium, after the administration of ergonovine, the patient manifested arterial hypertension and sudden headache related to neurological deficit, for which she required admission to the Intensive Care Unit until stabilization. The diagnosis of "thunder" type headache was established; the computed tomography showed no apparent alterations, the cerebrospinal fluid was clear, and leukocyte concentration of 10 mm3 and erythrocyte of 6 mm3. CONCLUSION: Call-Fleming syndrome is a rare condition during pregnancy and puerperium; therefore, it is important to know, identify and establish the differential diagnosis of the disease.

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