OnabotulinumtoxinA treatment for chronic migraine: experience in 52 patients treated with the PREEMPT paradigm.

OnabotulinumtoxinA (OnabotA) was approved for treatment of chronic migraine (CM) after publication of PREEMPT trials. Thus, we set out to evaluate the efficacy of OnabotA in a series of patients with CM treated according to the PREEMPT protocol. In May 2012 we began to offer OnabotA to patients with CM who did not respond to topiramate and at least one other preventive therapy (beta blocker and/or calcium channel antagonist). We prospectively recorded demographic data and the characteristics of migraine, and we assessed the modifications in monthly headache and migraine days, as well as the number of days of symptomatic medication and triptan intake. By September 2014 we had treated 52 patients (8 male, 44 female), whose age at treatment onset was 42.8 ± 12.7 years (range: 16-71) and age at migraine onset was 16.8 ± 7.8 years (3-32). In 43 of these patients (82.7%) symptomatic overuse of medication was observed at the onset of treatment. A total of 168 procedures were performed and after the first session, we observed a significant reduction in all the variables considered. Twelve (23.1%) patients failed to perceive a positive effect after the first procedure and it was not repeated in 4 of them. By contrast, there was a significant decreasing in all the variables evaluated compared to the baseline in the 39 patients that received a second series of injections. The use of OnabotA according to the PREEMPT paradigm is an effective treatment in patients with chronic migraine in a real-life setting.

Posterior reversible encephalopathy syndrome as presenting form of very early systemic sclerosis.

Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers. Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known. Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed "active pattern," considered typical of SSc. Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

Atypical nummular headache or circumscribed migraine: the utility of pressure algometry.

A peripheral mechanism has been proposed for nummular headache; however, there have been descriptions of atypical features resembling migraine. The authors describe a case in which algometry assessment facilitated the discrimination between atypical nummular headache and circumscribed migraine. A 21-year-old woman presented with a history of focal episodic pain in a circumscribed area on the left frontal region. The algometry study showed a unilateral and diffuse decrease of the pain pressure thresholds with frontal predominance, as has been proposed for migraine patients. This result led the authors to introduce a more specific preventive therapy with topiramate, with significant relief. In conclusion, cartographic investigation of pressure pain sensitivity is a simple tool that can help to differentiate between nummular headache and migraine. Further confirmatory investigations are needed.

From wakefulness to sleep: migraine and hypnic headache association in a series of 23 patients.

OBJECTIVES/BACKGROUND: We analyzed characteristics of hypnic headache (HH), migraine and the relationship between both headaches in 23 patients. HH is an uncommon primary headache characterized by exclusively sleep-related attacks. Concurrence of other headaches, mainly migraine, has been reported, but relationship between both syndromes has rarely been considered. METHODS: We prospectively collected data in a headache outpatient office from January 2008 to September 2013. Demographic data and migraine and hypnic headache mean features were assessed. RESULTS: Twenty-three out of 2500 (0.92%) were diagnosed with HH or probable HH, and 16 of them (69.5%) had a history of migraine. Mean age at onset of HH and migraine was 56.2 ± 9.3 and 24.6 ± 12.2 years, respectively. In 12 cases, migraine attacks disappeared at 56.7 ± 9.8 years old. Regarding the relationship between both syndromes, in 10 patients, migraine disappeared and HH began immediately after. In 1 case there was a pain-free period, and in 5 an overlap between both headaches was registered. CONCLUSION: A history of migraine is common in HH patients in our series. Most frequent transition pattern was an immediate change between both syndromes. Hypnic headache and migraine might share a common pathophysiological predisposition.

Neuralgia occipital: características clínicas y terapéuticas de una serie de 14 pacientes / Occipital neuralgia: clinical and therapeutic characteristics of a series of 14 patients

Introducción. La neuralgia occipital es un dolor en la distribución de los nervios occipitales, acompañado de hipersensibilidad al tacto en el territorio correspondiente. Objetivos. Presentamos la serie de neuralgia occipital de la consulta monográfica de cefaleas de un hospital terciario y analizamos sus características clínicas y su respuesta terapéutica. Pacientes y métodos. Se recogen variables de los casos de neuralgia occipital diagnosticados en dicha consulta entre enero de 2008 y abril de 2013. Resultados. Serie de 14 pacientes(10 mujeres, 4 varones) con neuralgia occipital sobre un total de 2.338 (0,59%). Edad al inicio del cuadro: 53,4 ± 20,3 años (rango: 17-81 años), y tiempo hasta el diagnóstico de 35,5 ± 58,8 meses (rango: 1-230 meses). Se descartó apropiadamente en cada caso patología intracraneal o cervical. En 13 de ellos (92,8%) se observó dolor basal de carácter generalmente opresivo e intensidad 5,3 ± 1,3 (4-8) en la escala analógica verbal. Once (78,5%) presentaban exacerbaciones, generalmente de carácter punzante, frecuencia variable (4,6 ± 7 al día) e intensidad 7,8 ± 1,7 (rango: 4-10) en la escala analógica verbal. En cuatro no se llevó a cabo bloqueo anestésico (dos por patrón remitente y dos por deseo del paciente); en los restantes, se realizó bloqueo con eficacia completa de duración entre dos y siete meses. Cuatro casos habían recibido anteriormente tratamiento preventivo (amitriptilina en tres y gabapentina en uno), sin respuesta. Conclusiones. En esta serie de una consulta monográfica de cefaleas, la neuralgia occipital es una entidad infrecuente y que afecta principalmente a pacientes mayores de 50 años. Ha de tenerse en cuenta, dada su respuesta escasa a preventivos, y completa y prolongada a bloqueos anestésicos (AU)

Introduction. Occipital neuralgia is a pain in the distribution of the occipital nerves, accompanied by hypersensitivity to touch in the corresponding territory. Aims. We present the occipital neuralgia series from the specialised headache unit at a tertiary hospital and analyse its clinical characteristics and its response to therapy. Patients and methods. Variables were collected from the cases of occipital neuralgia diagnosed in the above-mentioned headache unit between January 2008 and April 2013. Results. A series of 14 patients (10 females, 4 males) with occipital neuralgia was obtained out of a total of 2338 (0.59%). Age at onset of the clinical signs and symptoms: 53.4 ± 20.3 years (range: 17-81 years) and time elapsed to diagnosis was 35.5 ± 58.8 months (range: 1-230 months). An intracranial or cervical pathology was ruled out by suitable means in each case. Baseline pain of a generally oppressive nature and an intensity of 5.3 ± 1.3 (4-8) on the verbal analogue scale was observed in 13 of them (92.8%). Eleven (78.5%) presented exacerbations, generally stabbing pains, a variable frequency (4.6 ± 7 a day) and an intensity of 7.8 ± 1.7 (range: 4-10) on the verbal analogue scale. Anaesthetic blockade was not performed in four of them (two due to a remitting pattern and two following the patient’s wishes); in the others, blockade was carried out and was completely effective for between two and seven months. Four cases had previously received preventive treatment (amitriptyline in three and gabapentin in one), with no response. Conclusions. In this series from a specialised headache unit, occipital neuralgia is an infrequent condition that mainly affects patients over 50 years of age. Given its poor response to preventive treatment, the full prolonged response to anaesthetic blockades must be taken into account (AU)

[Occipital neuralgia: clinical and therapeutic characteristics of a series of 14 patients]. / Neuralgia occipital: características clinicas y terapéuticas de una serie de 14 pacientes.

INTRODUCTION. Occipital neuralgia is a pain in the distribution of the occipital nerves, accompanied by hypersensitivity to touch in the corresponding territory. AIMS. We present the occipital neuralgia series from the specialised headache unit at a tertiary hospital and analyse its clinical characteristics and its response to therapy. PATIENTS AND METHODS. Variables were collected from the cases of occipital neuralgia diagnosed in the above-mentioned headache unit between January 2008 and April 2013. RESULTS. A series of 14 patients (10 females, 4 males) with occipital neuralgia was obtained out of a total of 2338 (0.59%). Age at onset of the clinical signs and symptoms: 53.4 ± 20.3 years (range: 17-81 years) and time elapsed to diagnosis was 35.5 ± 58.8 months (range: 1-230 months). An intracranial or cervical pathology was ruled out by suitable means in each case. Baseline pain of a generally oppressive nature and an intensity of 5.3 ± 1.3 (4-8) on the verbal analogue scale was observed in 13 of them (92.8%). Eleven (78.5%) presented exacerbations, generally stabbing pains, a variable frequency (4.6 ± 7 a day) and an intensity of 7.8 ± 1.7 (range: 4-10) on the verbal analogue scale. Anaesthetic blockade was not performed in four of them (two due to a remitting pattern and two following the patient's wishes); in the others, blockade was carried out and was completely effective for between two and seven months. Four cases had previously received preventive treatment (amitriptyline in three and gabapentin in one), with no response. CONCLUSIONS. In this series from a specialised headache unit, occipital neuralgia is an infrequent condition that mainly affects patients over 50 years of age. Given its poor response to preventive treatment, the full prolonged response to anaesthetic blockades must be taken into account.

TITLE: Neuralgia occipital: caracteristicas clinicas y terapeuticas de una serie de 14 pacientes.Introduccion. La neuralgia occipital es un dolor en la distribucion de los nervios occipitales, acompañado de hipersensibilidad al tacto en el territorio correspondiente. Objetivos. Presentamos la serie de neuralgia occipital de la consulta monografica de cefaleas de un hospital terciario y analizamos sus caracteristicas clinicas y su respuesta terapeutica. Pacientes y metodos. Se recogen variables de los casos de neuralgia occipital diagnosticados en dicha consulta entre enero de 2008 y abril de 2013. Resultados. Serie de 14 pacientes (10 mujeres, 4 varones) con neuralgia occipital sobre un total de 2.338 (0,59%). Edad al inicio del cuadro: 53,4 ± 20,3 años (rango: 17-81 años), y tiempo hasta el diagnostico de 35,5 ± 58,8 meses (rango: 1-230 meses). Se descarto apropiadamente en cada caso patologia intracraneal o cervical. En 13 de ellos (92,8%) se observo dolor basal de caracter generalmente opresivo e intensidad 5,3 ± 1,3 (4-8) en la escala analogica verbal. Once (78,5%) presentaban exacerbaciones, generalmente de caracter punzante, frecuencia variable (4,6 ± 7 al dia) e intensidad 7,8 ± 1,7 (rango: 4-10) en la escala analogica verbal. En cuatro no se llevo a cabo bloqueo anestesico (dos por patron remitente y dos por deseo del paciente); en los restantes, se realizo bloqueo con eficacia completa de duracion entre dos y siete meses. Cuatro casos habian recibido anteriormente tratamiento preventivo (amitriptilina en tres y gabapentina en uno), sin respuesta. Conclusiones. En esta serie de una consulta monografica de cefaleas, la neuralgia occipital es una entidad infrecuente y que afecta principalmente a pacientes mayores de 50 años. Ha de tenerse en cuenta, dada su respuesta escasa a preventivos, y completa y prolongada a bloqueos anestesicos.

[Radiological and surgical characteristics of a case of recurring spinal hydatidosis]. / Caracteristicas radiologicas y quirurgicas de un caso de hidatidosis espinal recurrente.

INTRODUCTION: Hydatidosis is an illness that is still to be found in our setting. The lungs and liver are the organs most frequently involved and it rarely extends to bones. Involvement of the spine is infrequent, but it can give rise to a high degree of disability and may recur despite apparently complete excision. Here we report a case of recurring spinal hydatidosis with radiological and surgical particularities. CASE REPORT: Our case involves a 63-year-old male living in a rural area of the northern part of Spain's central plateau. The patient had a history of surgery for spinal hydatidosis and was on treatment with mebendazole. In the months before his visit, the patient had experienced difficulty in walking and urgency incontinence. The examination revealed paraparesis that predominantly affected the right-hand side and the patient required bilateral support to be able to walk. A dorso-lumbar magnetic resonance scan showed post-surgery changes with pedicular instrumentation added later in another centre, and a hydatid cyst with involvement of the body of the D10 vertebra, epidural extension and compression of the spinal cord. The patient was submitted to a surgical procedure involving the extraction of the system of fixation and full macroscopic excision of the cyst, with partial resection of the body of the vertebra. Initially the patient displayed motor improvement and treatment with mebendazole was reintroduced in high doses. CONCLUSIONS: Spinal hydatidosis is a rare condition that, in spite of correct surgical and medical treatment, often recurs. Magnetic resonance scans show characteristic 'honeycomb' images, which are sometimes large and have their origins in the body of the vertebra. Although successive surgical interventions can become increasingly more difficult because of old instrumentation or the conservation of compromised vertebral bodies, aggressive excision is indicated while the patient still has useful neurological functioning.

Características radiológicas y quirúrgicas de un caso de hidatidosis espinal recurrente / Radiological and surgical characteristics of a case of recurring spinal hydatidosis

Introducción. La hidatidosis es una enfermedad aún presente en nuestro entorno. El pulmón y el hígado son los órganos implicados con más frecuencia y rara vez hay una extensión ósea. La afectación espinal es infrecuente y puede dar lugar a gran discapacidad y recurrir pese a exéresis aparentemente completas. Presentamos un caso de hidatidosis espinal recurrente con particularidades radiológicas y quirúrgicas. Caso clínico. Varón de 63 años, residente en un área rural de la meseta norte española. Antecedente de hidatidosis espinal intervenida y en tratamiento con mebendazol. Consultó por empeoramiento en los meses previos de su dificultad para la marcha y urgencia esfínteriana. En la exploración se objetivó una paraparesia de predominio derecho y marcha con requerimiento de apoyo bilateral. La resonancia magnética dorsolumbar mostró cambios posquirúrgicos con instrumentación pedicular posterior realizada en otro centro y quiste hidatídico con afectación del cuerpo vertebral D10, extensión epidural y compresión medular. Se llevó a cabo una intervención quirúrgica consistente en la extracción del sistema de fijación y exéresis macroscópica completa del quiste, con resección parcial del cuerpo vertebral. Se apreció una mejoría motora inicial y se reintrodujo el tratamiento con mebendazol en dosis altas. Conclusiones. La hidatidosis espinal es una entidad poco frecuente que, pese a un adecuado tratamiento quirúrgico y médico, recurre con frecuencia. La resonancia magnética muestra imágenes características en ‘panal de abeja’, en ocasiones de gran tamaño y con origen en el cuerpo vertebral. Aunque las intervenciones quirúrgicas sucesivas plantean dificultades relacionadas con instrumentaciones antiguas o la conservación de cuerpos vertebrales afectos, la exéresis agresiva está indicada mientras el paciente mantenga una función neurológica útil(AU)

Introduction. Hydatidosis is an illness that is still to be found in our setting. The lungs and liver are the organs most frequently involved and it rarely extends to bones. Involvement of the spine is infrequent, but it can give rise to a high degree of disability and may recur despite apparently complete excision. Here we report a case of recurring spinal hydatidosis with radiological and surgical particularities. Case report. Our case involves a 63-year-old male living in a rural area of the northern part of Spain’s central plateau. The patient had a history of surgery for spinal hydatidosis and was on treatment with mebendazole. In the months before his visit, the patient had experienced difficulty in walking and urgency incontinence. The examination revealed paraparesis that predominantly affected the right-hand side and the patient required bilateral support to be able to walk. A dorsolumbar magnetic resonance scan showed post-surgery changes with pedicular instrumentation added later in another centre, and a hydatid cyst with involvement of the body of the D10 vertebra, epidural extension and compression of the spinal cord. The patient was submitted to a surgical procedure involving the extraction of the system of fixation and full macroscopic excision of the cyst, with partial resection of the body of the vertebra. Initially the patient displayed motor improvement and treatment with mebendazole was reintroduced in high doses. Conclusions. Spinal hydatidosis is a rare condition that, in spite of correct surgical and medical treatment, often recurs. Magnetic resonance scans show characteristic ‘honeycomb’ images, which are sometimes large and have their origins in the body of the vertebra. Although successive surgical interventions can become increasingly more difficult because of old instrumentation or the conservation of compromised vertebral bodies, aggressive excision is indicated while the patient still has useful neurological functioning(AU)

[Primary stabbing headache: clinical characteristics and response to treatment in a series of 67 patients]. / Cefalea primaria punzante: caracteristicas clínicas y respuesta al tratamiento en una serie de 67 pacientes.

INTRODUCTION: Primary stabbing headache (PSH) is defined by the presence of short stabbing pains in the first branch of the trigeminal nerve. According to population-based studies, it is very prevalent, but most cases present stabbing pains with low frequencies and intensities that do not lead the patient to seek medical attention. AIMS: We report on 67 cases of PSH attended in the headache service of a tertiary hospital. In the study, the demographic and clinical characteristics are studied, treatment response is reviewed and the features of PSH are compared in terms of whether it was the only headache or was accompanied by others. PATIENTS AND METHODS: The study involved 67 patients (51 females and 16 males) diagnosed with PSH between January 2008 and January 2012, of a total number of 1668 (4%) patients attended in the above-mentioned service. RESULTS: Age at onset: 34.5 ± 16.7 years. Forty-nine cases (73.1%) were associated to another headache, above all migraine. Stabbing pains were often bilateral; 38 (56.7%) patients suffered more than one a day and 11 (16.4%) had more than 10 per day. They lasted less than five seconds in 48 patients (71.6%) and more than 10 seconds in 11 of them (16.4%), with an intensity of 6.8 ± 1.5. The age of onset of PSH was higher if it was the only type of headache than if it was accompanied by others. Twenty-six (38.8%) patients required preventive treatment for the associated headache and 16 (23.8%) took indomethacin, with a similar response in the two groups (73 versus 75%). CONCLUSIONS; PSH is not infrequent in headache clinics, but its phenotype differs from that reported in population-based studies. The characteristics of PSH vary depending on whether it is the only headache or is associated with others. Preventive treatment is often required and patients respond well to it.

Cefalea primaria punzante: características clínicas y respuesta al tratamiento en una serie de 67 pacientes / Primary stabbing headache: clinical characteristics and response to treatment in a series of 67 patients

Introducción. La cefalea primaria punzante (CPP) se define por la presencia de punzadas breves localizadas en la primera rama del nervio trigémino. Según estudios de base poblacional, es muy prevalente, pero la mayoría de casos presenta punzadas de baja frecuencia e intensidad que no requieren consulta médica. Objetivos. Presentar 67 casos de CPP de la consulta de cefaleas de un hospital terciario, analizar las características demográficasy clínicas, revisar la respuesta al tratamiento y comparar las características de la CPP según sea cefalea única o se acompañe de otras. Pacientes y métodos. Muestra de 67 pacientes (51 mujeres y 16 varones) diagnosticados de CPP entre enero de 2008 y enero de 2012, de un total de 1.668 (4%) atendidos en dicha consulta. Resultados. Edad al inicio: 34,5 ± 16,7 años. Cuarenta y nueve casos (73,1%) asociaban otra cefalea, sobre todo migraña. Las punzadas eran frecuentemente bilaterales; 38 pacientes (56,7%) sufrían más de una al día y 11 (16,4%) más de 10 al día. Su duración era menor de cinco segundos en 48 (71,6%) pacientes y mayor de 10 segundos en 11 (16,4%), con una intensidad de 6,8 ± 1,5. La edad de inicio de la CPP era mayor si era cefalea única que si acompañaba a otras. Veintiséis (38,8%) pacientes requirieron un preventivo para la cefalea asociada y 16 (23,8%) indometacina con respuesta similar en los dos grupos (73 frente a 75%). Conclusiones. La CPP no es infrecuente en una consulta de cefaleas, pero su fenotipo difiere del descrito en estudios de base poblacional. Las características de la CPP son diferentes en función de si es cefalea única o asocia otras. Se requiere tratamiento preventivo con frecuencia y la respuesta es buena (AU)

Introduction. Primary stabbing headache (PSH) is defined by the presence of short stabbing pains in the first branch of the trigeminal nerve. According to population-based studies, it is very prevalent, but most cases present stabbing pains with low frequencies and intensities that do not lead the patient to seek medical attention. Aims. We report on 67 cases of PSH attended in the headache service of a tertiary hospital. In the study, the demographic and clinical characteristics are studied, treatment response is reviewed and the features of PSH are compared in terms of whether it was the only headache or was accompanied by others. Patients and methods. The study involved 67 patients (51 females and 16 males) diagnosed with PSH between January 2008 and January 2012, of a total number of 1668 (4%) patients attended in the above-mentioned service. Results. Age at onset: 34.5 ± 16.7 years. Forty-nine cases (73.1%) were associated to another headache, above all migraine. Stabbing pains were often bilateral; 38 (56.7%) patients suffered more than one a day and 11 (16.4%) had more than 10 per day. They lasted less than five seconds in 48 patients (71.6%) and more than 10 seconds in 11 of them (16.4%), with an intensity of 6.8 ± 1.5. The age of onset of PSH was higher if it was the only type of headache than if it was accompanied by others. Twenty-six (38.8%) patients required preventive treatment for the associated headache and 16 (23.8%) took indomethacin, with a similar response in the two groups (73 versus 75%). Conclusions. PSH is not infrequent in headache clinics, but its phenotype differs from that reported in population-based studies. The characteristics of PSH vary depending on whether it is the only headache or is associated with others. Preventive treatment is often required and patients respond well to it (AU)

Nummular headache with and without exacerbations: comparative characteristics in a series of 72 patients.

OBJECTIVES: Apart from the characteristic chronic head pain in a coin-shaped circumscribed area, superimposed exacerbations have been described from early reports of nummular headache (NH). In a prospective series, we aim to compare the demographic and clinical characteristics between cases of exacerbations (ENH) and non-exacerbations (NENH) in NH. METHODS AND RESULTS: Seventy-two NH patients (44 female, 28 male) attending a headache outpatient office. As eight patients presented with bifocal NH we analysed 80 painful areas; 47 (58.8%) presented in situ exacerbations. Mean intensity of exacerbations was 7.5 ± 1.6 and they lasted 5.7 ± 11.6 minutes. Exacerbation quality was mostly stabbing. We found no differences between ENH and NEHN groups in age at onset, baseline pain intensity, size of painful area, allodynia or other sensory symptoms, or baseline pain quality. There were no differences between populations with respect to relief with symptomatic therapy, requirement of preventative therapy and its response to preventatives. CONCLUSION: In situ exacerbations superimposed on baseline pain are frequent in NH and might be included in diagnostic criteria. No statistically significant differences were found between ENH and NENH cases in demographic and nosological characteristics, or needing or response to therapy, but these sample sizes are small.

Peripheral nerve blocks: a therapeutic alternative for hemicrania continua.

OBJECTIVES: A complete response to indomethacin is required for the diagnosis of hemicrania continua (HC). Nevertheless, patients may develop side effects leading to withdrawal of this drug. Several alternatives have been proposed with no consistent effectiveness. Both anaesthetic blocks of peripheral nerves and trochlear corticosteroid injections have been effective in some case reports. METHODS: Twenty-two patients with HC were assessed in a headache outpatient office. Physical examination included palpation of the supraorbital nerve (SON) and greater occipital nerve (GON) as well as of the trochlear area. RESULTS: In 14 patients, at least one tender point was detected. Due to indomethacin intolerance, at least one anaesthetic block of the GON or SON, or an injection of corticosteroids in the trochlear area, were performed in nine patients. Four of them were treated with a combination procedure. All these patients experienced total or partial improvement lasting from 2 to 10 months. CONCLUSION: Anaesthetic blocks or corticosteroid injections may be effective in HC patients showing tenderness of the SON, GON or trochlear area.