OnabotulinumtoxinA in chronic migraine: predictors of response. A prospective multicentre descriptive study.

BACKGROUND AND PURPOSE: OnabotulinumtoxinA is a treatment specifically approved for the prophylaxis of chronic migraine in adults. The aim of this study was to assess the effectiveness of OnabotulinumtoxinA in chronic migraine after 1 year of treatment in a real-life setting and to identify clinical predictors of outcome. METHODS: We designed a prospective multicentre study performed in 13 hospitals in Spain. Patients underwent a complete medical history and examination. They were treated with OnabotulinumtoxinA every 12 weeks for 1 year. Data about outcome, adverse events, abortive medication use, emergency room use and disability were collected at 3 and 12 months. RESULTS: A total of 725 subjects completed the study. At 12 months, 79.3% showed >50% reduction in number of headaches per month and 94.9% reported no adverse events. Unilaterality of pain, fewer days of disability per month and milder headache at baseline were correlated with good outcome. Duration of disease <12 months increased the chances of response to treatment with OnabotulinumtoxinA (odds ratio, 1.470; 95% confidence interval, 1.123-2.174; P = 0.045). CONCLUSIONS: This study confirmed the effectiveness of treatment with OnabotulinumtoxinA after 1 year of treatment. The chances of a good outcome may be increased by starting treatment in the first 12 months after chronic migraine diagnosis.

[Competency: general principles and applicability in dementia]. / Competencia: conceptos generales y aplicación en la demencia.

INTRODUCTION: Competency means the capacity to make responsible and balanced decisions. This may be performed in clinical settings (decision-making abilities on treatment or risky diagnostic procedures) and also in daily-life activities (financial matters, nursing home admittance, contracts, etc.). Competency is linked to the ethical principle of autonomy and to a horizontal doctor-patient interaction, far from ancient paternalistic relationships. It is contemplated in the Spanish law as the patient's right to be informed and to make free choices, particularly in cases of dementia. DEVELOPMENT: The competency that we assess is the so-called natural or working capacity. It is specific for an action or task. The level of required capacity depends on the decision: higher for critical ones, lower for low-risk decisions. The assessment process requires noting the patient's capacity to understand, analyse, self-refer and apply the information. There are some guides available that may be useful in competency assessments, but nevertheless the final statement must be defined by the physician in charge of the patient and clinical judgement. Capacity is directly related to the level of cognitive deterioration. Nevertheless, specific cognitive tests like MMSE (mini-mental) have a low predictive value. The loss of competency is more associated with the so-called legal standards of incapacity (LS). These encompass a five steps range (LS1-LS5), which may detect the incapacity from the mild levels of dementia. The cortical functions that are the best predictors of incapacity are language and executive dysfunctions. These explain the incapacity in cases of Alzheimer's and Parkinson's disease, and have been studied more. CONCLUSIONS: Incapacity is common and it influences the clinical decision-making process. We must be particularly cautious with clinical trials of dementia. It also involves other areas of daily life, particularly financially related ones, where limitations are present from the mild cognitive impairment level. The neurological community has already produced specific and invaluable documents like the one from Sitges, although in our opinion this community has to increase its awareness, and also its involvement as much in the clinical as in the research sides of this field.

Nummular headache: a series with symptomatic and primary cases.

Nummular headache (NH) is a primary headache adopting the form of local pain in a circumscribed area of < 7 cm in diameter in the tuber parietale, albeit it may also be located in other areas of the head. Although it is chronic, it is commonly associated with exacerbations and short periods of remission. Here we report four cases. Two of them could not be considered primary: in one the pain was related to an underlying, pointed and benign lesion disclosed only by magnetic resonance imaging (case 1); the second one had persistent NH days after trans-sphenoidal surgery for a pituitary adenoma, similar to a postcraniotomy headache (case 2). The two final patients suffered from typical forms of primary NH, one associated with migraine without aura, the other with chronic tension-type headache. The response to pain-related treatments and to preventive drugs was poor in the symptomatic as well as in the primary cases. The mechanisms are not clear, and peripheral (case 1) and also central pathways (case 2) could be involved. In the end, secondary forms of NH might coexist with classical primary NH. Particular attention should be paid to tiny skull lesions and to key events preceding the pain.

[Non tumoural cerebral biopsies: an analysis of 50 cases]. / Biopsias cerebrales no tumorales: an lisis de 50 casos.

OBJECTIVE: Cerebral biopsy is an invasive technique with limited, specific indications in view of the potential risk of complications. PATIENTS AND METHODS: We reviewed a series of 50 cases of biopsies via craniotomy done over a period of 10 years to investigate suspected non tumoural disorders. RESULTS: There was a predominance of space occupying lesions and treatable infections (36%) and of non diagnostic biopsies (40%). There was poor correlation between the initial clinical diagnosis and the histological findings (50 71%). There were no complications. CONCLUSIONS: We conclude that the indications should be better defined and, in view of the satisfactory tolerance of this procedure, the study group should be increased by using other less invasive procedures (stereotaxia).

[Dementia with frontal lobe atrophy: clinical study from 18 patients with cognitive impairment and frontal lobe involvement detected by structural and functional neuroimaging]. / Demencia con atrofia frontal: estudio clínico de 18 pacientes con deterioro intelectual y degeneración de los lóbulos frontales según la neuroimagen.

OBJECTIVE: To characterize clinically patients with cognitive impairment and frontal lobe degeneration at neuroimaging. PATIENTS AND METHODS: Patients diagnosed of dementia or mild neurocognitive impairment by DSM IV criteria and neuropsychological tests with frontal lobe atrophy and hipoperfusion detected by computed tomography and single photon emission computed tomography. RESULTS: 18 patients, 5:1 on behalf of women; mean age at onset, 74 years; hereditary for dementia, 38%; mean duration of illness at first testing, 2 years; the most common initial symptoms, memory loss. At initial examination, psychotic or behavioral impairment was found in 61% and parkinsonism in 38%, the commonest cognitive troubles at this moment was the executive function, language and anterograde memory dysfunctions at the same rate. Frontal lobe atrophy and hypoperfusion was found in 100%, temporal in 88% and parietal in 38%. Electroencephalogram was pathological in 33%. The course of the disease was progressive but with fluctuation in 27%. Frontotemporal dementia clinical criteria in 13 patients, 4 of them clinical criteria of dementia with Lewy bodies too, and clinical criteria of Alzheimer s disease in 5.

[Hospitalizations for acute cerebrovascular accidents and transient ischemic attacks in Spain: temporal stability and spatial heterogeneity, 1998-2003]. / Hospitalizaciones por accidentes cerebrovasculares agudos y ataques isquémicos transitorios en España: estabilidad temporal y heterogeneidad espacial en el período 1998-2003.

INTRODUCTION: Stroke is a leading cause of hospitalisation. Ageing and differences in management and specialisation between health centres could explain the variability in hospitalisation and prognosis between areas. We analyse the number of hospitalisations due to stroke and TIA (Spain), 1998-2003, and the differences between regions. METHODS: The Spanish Ministry of Health, through its MBDS (Minimum Basic Data Set Office), provided data for stroke and TIA hospitalisation. Diagnoses were extracted according to ICD-9 (International Classification of Diseases) -codes 430-439- and to GRD (Group Related Diagnoses) -codes 14-17, 532, 810-. We included: a) autonomous community identification; b) average stay; c) age, sex, type of discharge (mortality); d) number of diagnoses, weight/cost for GRD. RESULTS: There was ateady temporal incidence of hospitalisation for stroke (GRD14, 160/100,000) and TIA (GRD15, 55/100,000). Weight and costs increased between 2000 and 2003, and reached 3,400 euro (GRD14) and 2,400 euro (GRD15). Average stay decreased from 12 to 10.1 days (stroke) and 8.6 to 7.3 days (TIA). Mortality also decreased from 12.7% to 9.2% for stroke (GRD14). The average number of diagnoses was similar for stroke and TIA: both increased from 4 to 5 over the 6 years. There were wide variations between autonomous communities in hospitalisations for stroke and TIA: from 250/100,000 to less than 120/100,000. There was a correlation between the ageing of the populations and these differences. There were also wide variations (up to 40%) in average stay and mortality between communities. CONCLUSIONS: Hospitalisation rate for stroke and TIA is very high and sustained; despite increasing complexity, average stay and mortality improved, which points to better management. Variability among regions is highlighted. Further prospective studies are required.

[In-hospital stroke: clinical profile and expectations for treatment]. / Accidentes cerebrovasculares intrahospitalarios: perfil clínico y expectativas terapéuticas.

INTRODUCTION: In-hospital strokes have been poorly reported. They provide an opportunity to shorten intervals for thrombolysis. Our proposals were: a) to describe their clinical features and neurological assessment, and b) regarding thrombolysis, to analyze potential candidates and exclusions at a general tertiary hospital, just before its approval/implementation at the center. METHODS: Cases were retrospectively recruited between May 2001-May 2004. They were identified from discharching diagnosis (ICD-9: 430-439; GRD: 14, 15, 16, 17, 532, 810) and from consultations required to the neurology service. Data collected were: a) admitting diagnosis and service; b) mechanism of stroke (Trial of Org 10172 in Acute Stroke Treatment, TOAST) and clinical syndrome (Oxfordshire Community Stroke Project, OCSP); c) vascular risk factors and previous symptomatic artherioesclerotic disease (PSAD), and d) prognosis, functional status at discharge (mRankin scale, mRS) and timing for neurological assessment. Every case was considered regarding thrombolytic treatment according to Safe Implementation of Trombolysis in Stroke-Monitoring Study (SIST-MOST) criteria. Potential criteria for exclusion were registered. RESULTS: 183 cases were included (26 transient ischemic accident, 149 ischemic strokes, 5 haemorrhages). Mean age: 74.5 years, 25.5%, above 80 years. Main sources of patients were cardiology plus related services (31.8%) and internal medicine (18%). Dominant mechanism was cardioembolism (40%). 18 cases (11.77%) were yatrogenic. 55.8% had had PSAD (stroke: 41; ischemic cardiopathy: 31). Mortality reached 33%. 36% were discharged pointing 3 or above in the mRS. Expert neurological assessment was requested in 89%, but just for 25% it was considered an emergency. From 149 ischemic strokes, 5 cases (3.2%) were potential candidates for thrombolysis. Mayor surgery, ageing (>80 years), severe acute disorders or combinations of them precluded thrombolysis. CONCLUSIONS: In-hospital strokes are particularly prevalent in patients with PSAD. Prognosis is poor. In 3.2% thrombolysis could be administered. To make this possible, a right perception of the timing and emergency should be encouraged among hospital staff.

[Neurological disorders in the narrative works of Benito Pérez Galdós]. / Trastornos neurológicos en la obra narrativa de Benito Pérez Galdós.

INTRODUCTION: Benito Pérez Galdós was a realistic writer. As such, he was devoted to displaying reality in its full complexity. He shared the traits of experimental medicine of Claude Bernard (Introduction à l'étude de la médecine expérimentale). The writer was a close friend of famous contemporary doctors (Gregorio Marañón, Manuel Tolosa Latour), had textbooks with neurological content, and was familiar with Charcot. METHODS: With this background, we have foreseen neurological descriptions in his works. To search for them, we have reviewed three paramount novels: Fortunata y Jacinta, La de Bringas and Tormento. RESULTS: We found: a) headache, usually migraine with and without aura; common precipitants and inheritance are present. The novelist himself suffered from severe migraine; b) movement disorders: Parkinson syndrome and hemifacial spasm; c) convulsive and non-convulsive epilepsy in a girl as well as syncope and psychogenic loss of consciousness; d) congenital syphilis, based on a typical physical appearance in a character with madness (paranoid schizophrenia); the reason was probably that syphilis was regarded as the most common cause of "dementia praecox"; e) alcoholism: acute intoxication, deprivation, behavior disorders, hepatic encephalopathy and a likely pellagra; f) sleep disorders: parasomnias (somnambulism, somniloquy) and sleep paralysis, and g) stroke and also inverted metamorphopsia of psychogenic origin. CONCLUSION: Ailments and disease pervaded life in the 19th century. Neurological disorders were highly prevalent and are fully integrated into Galdos realistic works. Many of them fulfill criteria of disorders contemplated according to degeneration theories. Nevertheless, humanitarian features raise the characters above their tragic destiny. Biographical factors, particularly his many love affairs and his political implication as a liberal, could have contributed to the plethora of precise descriptions.

[The turn of the screw: complex visual hallucinations in the Henry James' novel]. / Otra vuelta de tuerca: alucinaciones visuales complejas en la novela de Henry James.

The turn of the screw is one of the most celebrated stories by Henry James. It is also a top writing within the so-called fantastic literature, whose narrative strength comes from the intermittent visions suffered by the main character. The vividness and dramatic content that represent the firstly unidentified human figures, that moreover recur as brief, stereotyped and fragmentary images, are constitutive of complex visual hallucinations. These characteristics, alongside acute premonitory symptoms such as emotional changes (fear, anxiety) or altered thinking (forced, "dejà vu", "jamais vu"), and the final altered awareness or loss of consciousness, allow us to infer an epileptic nature of the ten episodes described. Postictal psychosis, that follows a lucid interval and may last up to the several weeks encompassed by the story, would account for the paranoia featured, in the setting of a temporal lobe epilepsy. The accurate descriptions prompted us to search for autobiographical, scientific or literary influences: The alcoholism and visual hallucinations suffered by his father, the knowledge on hallucinations provided by his brother Williams on his paramount and former The Principles of Psychology, and an early devotion to Poe's writings, an epileptic himself with excellent descriptions of seizures in his writings, might have enabled the author to perform his story with such a hallmark of neurological details.

[Importance of the clinical-radiological complementarity in superficial cerebral siderosis]. / Relevancia de la complementariedad clinicorradiológica en la siderosis superficial cerebral.

We report two cases of superficial siderosis of central nervous system. In one case it was idiopathic and in the other secondary to an unidentified subarachnoid hemorrhage. The symptoms that characterized the clinical picture of both were gait disturbance and hypoacusis. The MRI study showed a superficial rim of hypointensity that covered the cerebellum and brainstem, and extended along the cranial nerves and the brain surface. The findings were clearer in the T2 spin echo series. Due to the high sensitivity for hemosiderin deposits, MRI made it possible to make the final diagnosis of this rare disease.

[Spinal cord dural arteriovenous fistulae: analysis of a series with surgical curative treatment]. / Fístulas arteriovenosas durales espinales: análisis de una serie con tratamiento quirúrgico curativo.

BACKGROUND: Spinal dural arteriovenous fistulae (SDAVF) are elusive to an early diagnosis. Otherwise, there is no agreement regarding the best treatment. AIM: To review our series of spinal arteriovenous malformations to illustrate the treatment and outcome of FAVDE. PATIENTS AND METHODS: Descriptive series of cases diagnosed and treated at our hospital within the last ten years. Ten suspicious MRI, followed by angiograms, got the next diagnosis: 3 intraspinal malformations, 2 cavernoms and 5 FAVDE, reported here. RESULTS: Four out of 5 FAVDE were men. Mean age was 61year (53-77), and mean interval to diagnosis 10.8 months (1-24). The clinical course consisted of progressive paraparesis, wich was acute in one case, with wheelchair confinement. Sensory involvement and sphinter dysfunction were also always present; severe pain affected 2 cases. MRI revealed the FAVDE at low thoracic or lumbar levels, with tortuous flow voids over the surface of the affected area. Angiograms disclosed the single feeding vessel between D8-L3. Laminectomy and interruption of the draining vein was the chosen treatment. Postoperative complications were not found. Improvement followed until full recovery, that occurred in 4 cases. One patient, previously embolized, was the only one with just modest recovery, although the angiogram proved that deferred surgery ran well. Mean follow-up was 3.8 years (1.5-6); neither clinical deterioration nor suspicious MR finding were found. Postoperative angiogram, performed in two cases, confirmed the fistulae as occluded. CONCLUSIONS: FAVDE mainly involves old males with progressive paraparesis. Suitable MR findings and characteristic angiograms allow the diagnosis. Novel patients are subsidiary to selective and simple surgery, that is usually curative without recurrencies.