Predicting postoperative hypocortisolism in patients with non-aldosterone-producing adrenocortical adenoma: a retrospective single-centre study.

BACKGROUND: Limited information exists on postoperative hypocortisolism and hypothalamus-pituitary-adrenal axis recovery in patients with adrenal incidentaloma following unilateral adrenalectomy. We evaluated frequency of postoperative hypocortisolism and predictors for recovery in non-aldosterone-producing adrenocortical adenoma patients after unilateral adrenalectomy. METHODS: A retrospective analysis of 32 adrenal incidentaloma patients originally included in the ITACA trial (NCT04127552) with confirmed non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy from September 2019 to April 2023 was conducted. Preoperative assessments included adrenal MRI, anthropometrics, evaluation of comorbidities, adrenal function assessed via ACTH, urinary free cortisol, and 1 mg dexamethasone suppression test. ACTH and serum cortisol or Short Synacthen test were performed within 6 days, 6 weeks, 6 months, and a year after surgery. RESULTS: Six days postoperative, 18.8% of patients had normal adrenal function. Among those with postoperative hypocortisolism, 53.8% recovered by 6 weeks. Patients with earlier adrenal recovery (6 weeks) had lower preoperative 1 mg dexamethasone suppression test (median 1 mg dexamethasone suppression test 76.2 [61.8-111.0] nmol/L vs 260.0 [113.0-288.5] nmol/L, p < 0.001). Univariate analysis showed preoperative 1 mg dexamethasone suppression test negatively related with baseline ACTH levels (r = - 0.376; p = 0.041) and negatively associated with the 6-week baseline (r = - 0.395, p = 0.034) and 30-min cortisol levels during Short Synacthen test (r = - 0.534, p = 0.023). Logistic regression analysis demonstrated preoperative 1 mg dexamethasone suppression test as the only biochemical predictor for 6-week adrenal recovery: ROC curve identified a 1 mg dexamethasone suppression test threshold of 131 nmol/L predicting 6-week recovery with 89.5% sensitivity and 72.7% specificity (AUC 0.87; 95% CI 66.9-98.7, p < 0.001). Other preoperative assessments (tumor size, ACTH levels and anthropometrics) were not associated with postoperative hypothalamus-pituitary-adrenal axis function, but the presence of diabetes was associated with a lower probability of recovery (OR = 24.55, p = 0.036). ACTH levels increased postoperatively in all patients but did not predict hypothalamus-pituitary-adrenal axis recovery. CONCLUSIONS: The preoperative 1 mg dexamethasone suppression test cortisol value and presence of diabetes are the only relevant predictor of hypothalamus-pituitary-adrenal axis recovery in patients with non-aldosterone- producing adrenocortical adenoma undergoing surgery, regardless other clinical and biochemical variables. Notably, pre- and postoperative ACTH levels did not predict hypothalamus-pituitary-adrenal axis recovery. These findings point towards the potential for saving resources by optimizing their allocation during follow-up assessments for patients with non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy.

Estudio observacional multicéntrico español de primeros episodios sugestivos de esclerosis múltiple: cohorte PREM / Spanish multi-centre observational study of the fi rst attacks suggestive of multiple sclerosis: the PREM cohort

Introducción. Estudios recientes han demostrado la necesidad de optimizar el manejo de los pacientes tras un primer brote sugerente de esclerosis múltiple (EM). Nuestro objetivo es comprobar si los resultados de seguimiento de estos estudios son reproducibles en un contexto multicéntrico español. Pacientes y métodos. El estudio PREM (estudio multicéntrico español prospectivo observacional a 24 meses) incluyó a pacientes en los tres primeros meses tras un primer brote sugerente de EM con al menos dos lesiones típicas en una resonancia magnética. Se obtuvo la Expanded Disability Status Scale (EDSS) y se valoró la presencia de brotes basalmente y a los 3, 6, 9, 12, 18 y 24 meses; se realizó una resonancia magnética basalmente y a los 6 y 24 meses, para el cálculo del volumen cerebral y de volúmenes lesionales (T1, T2 y T1 tras la administración de gadolinio). Se valoraron los criterios de McDonald y Poser durante el seguimiento. Un subgrupo de pacientes fue seguido hasta completar cuatro años. Resultados. Se incluyó a 110 pacientes (un 67% mujeres) de edad media de 30,2 años; 22 pacientes abandonaron prematuramente el estudio. Un 19% y un 45% de pacientes cumplían criterios de Poser a los 6 meses y 24 meses, respectivamente; un 63% y un 71% en el caso de los criterios de McDonald. La EDSS descendió signifi cativamente (–0,94; p < 0,001) y se observó desarrollo de atrofi a (–1,2%; p < 0,001) a los 24 meses. Conclusión. Los resultados de seguimiento de pacientes con primeros brotes sugerentes de EM en un contexto multicéntrico español son superponibles a los de los ensayos clínicos internacionales realizados en estos pacientes (AU)

Introduction. Recent studies have shown the need to optimise the management of patients after a fi rst attack suggestive of multiple sclerosis (MS). Our aim is to determine whether the results from follow-ups in these studies are reproducible within a Spanish multi-centre context. Patients and methods. The PREM study (observational prospective Spanish multi-centre study at 24 months) included patients in the fi rst three months following a fi rst event suggestive of MS with at least two typical lesions in a magnetic resonance scan. The Expanded Disability Status Scale (EDSS) was obtained and the presence of attacks was evaluated basally and at 3, 6, 9, 12, 18 and 24 months; a magnetic resonance scan was performed basally and at 6 and 24 months so as to be able to calculate the brain volume and the volumes of the lesions (T1, T2 and T1 after administering gadolinium). McDonald and Poser criteria were evaluated during the follow-up. A subgroup of patients was followed up for a total eriod of four years. Results. Altogether 110 patients (67% females) with a mean age of 30.2 years were included in the study; 22 patients dropped out of the study before it fi nished. Poser criteria were met by 19% and 45% of patients at 6 months and 24 months, respectively; 63% and 71% satisfi ed McDonald criteria. The EDSS decreased signifi cantly (–0.94; p < 0.001) and development of atrophy was observed (–1.2%; p < 0.001) at 24 months. Conclusions. Results of the follow-up of patients with fi rst attacks suggestive of MS within a Spanish multi-centre context are wholly comparable with those from international clinical trials performed in these patients (AU)

[Multiple sclerosis epidemiological situation update: pertinence and set-up of a population based registry of new cases in Catalonia]. / Situación epidemiológica actual de la esclerosis múltiple: pertinencia y puesta en marcha de un registro poblacional de nuevos casos en Cataluña.

INTRODUCTION: The first epidemiological studies on multiple sclerosis (MS) around the world pictured a north to south latitudinal gradient that led to the first genetic and environmental pathogenic hypothesis. MS incidence seems to be increasing during the past 20 years based on recent data from prospective studies performed in Europe, America and Asia. This phenomenon could be explained by a better case ascertainment as well as a change in causal factors. The few prospective studies in our area together with the increase in the disease in other regions, justifies an epidemiological MS project in order to describe the incidence and temporal trends of MS. DEVELOPMENT: A prospective multicenter MS registry has been established according to the actual requirements of an epidemiological surveillance system. Case definition is based on the fulfillment of the McDonald diagnostic criteria. The registry setting is the geographical area of Cataluna (northeastern Spain), using a wide network of hospitals specialized in MS management. CONCLUSION: Recent epidemiological studies have described an increase in MS incidence. In order to contrast this finding in our area, we consider appropriate to set up a population based registry.

Situación epidemiológica actual de la esclerosis múltiple: pertinencia y puesta en marcha de un registro poblacional de nuevos casos en Cataluña / Multiple sclerosis epidemiological situation update: pertinence and set-up of a population based registry of new cases in Catalonia

Introducción. Los primeros estudios epidemiológicos de esclerosis múltiple (EM) de ámbito mundial caracterizaron un patrón geográfico latitudinal, con prevalencias más altas en las zonas más alejadas del ecuador. A raíz de esta distribución, se plantearon hipótesis causales de índole genética y ambiental. Según los datos de estudios prospectivos desarrollados en diversas regiones de Europa, América y Asia, la incidencia de la enfermedad ha aumentado a lo largo de los últimos 30 años, lo cual podría indicar una mejor detección de casos o un cambio en los factores causales subyacentes. Los escasos estudios prospectivos disponibles en nuestro entorno y el aumento de la enfermedad descrito en otras regiones justifican la pertinencia de un proyecto epidemiológico dirigido a conocer las tasas de incidencia y la tendencia temporal de EM. Desarrollo. De acuerdo con los requisitos actuales de un sistema de vigilancia epidemiológica, se ha establecido un registro prospectivo de carácter multicéntrico. Para la definición de nuevo diagnóstico se emplean los criterios establecidos por McDonald. El ámbito de aplicación es el territorio de Cataluña, a través una red de hospitales de referencia especializados en el manejo de EM, que notifican la información mediante un aplicativo informático conectado a internet. Conclusiones. Los estudios epidemiológicos de la EM de las últimas décadas han descrito un incremento de su incidencia. Para dimensionar este fenómeno en nuestro ámbito, creemos pertinente la puesta en marcha de un registro poblacional de la enfermedad en Cataluña (AU)

Introduction. The first epidemiological studies on multiple sclerosis (MS) around the world pictured a north to south latitudinal gradient that led to the first genetic and environmental pathogenic hypothesis. MS incidence seems to be increasing during the past 20 years based on recent data from prospective studies performed in Europe, America and Asia. This phenomenon could be explained by a better case ascertainment as well as a change in causal factors. The few prospective studies in our area together with the increase in the disease in other regions, justifies an epidemiological MS project in order to describe the incidence and temporal trends of MS. Development. A prospective multicenter MS registry has been established according to the actual requirements of an epidemiological surveillance system. Case definition is based on the fulfillment of the McDonald diagnostic criteria. The registry setting is the geographical area of Cataluña (northeastern Spain), using a wide network of hospitals specialized in MS management. Conclusion. Recent epidemiological studies have described an increase in MS incidence. In order to contrast this finding in our area, we consider appropriate to set up a population based registry (AU)

NARP-MILS syndrome caused by 8993 T>G mitochondrial DNA mutation: a clinical, genetic and neuropathological study.

The 8993 T>G mutation in mitochondrial DNA has been associated with variable syndromes of differing severity ranging from maternally inherited Leigh's syndrome (MILS) to neuropathy, ataxia, retinitis pigmentosa (NARP), depending on the mutation loads in affected patients. We report a kindred with several members in the same generation suffering NARP or Leigh's syndrome due to a 8993 T>G mutation. Post-mortem studies of the brain in one affected member clinically presenting with a neurological disorder intermediate between adult Leigh's syndrome and NARP showed symmetrical lesions of the basal ganglia and brainstem closely resembling those usually described in typical Leigh's syndrome. Analysis of mtDNA in different tissues showed a high proportion of mutant genome in brainstem, cerebral cortex, putamen, cerebellum and thalamus. These observations illustrate the continuum of clinical and neuropathological manifestations associated with the 8993 T>G mutation of the mtDNA.

[Survey on the current situation of the young neurologists in Spain: analysis of their current working stability and degree of social protection]. / Encuesta sobre la situación actual de los neurólogos jóvenes en España: análisis de la estabilidad laboral y de la protección social.

INTRODUCTION: The aims of this study were to evaluate the working stability and degree of social protection of the Spanish young neurologists, and to know their opinion about their own situation. METHODS: The 343 neurologists that became specialists in Spain between 2000 and 2004 were asked to participate in two consecutive surveys. The first, conducted online, included questions about the availability to change the place of work and the opinion about the situation of young neurologists, and obtained 66 answers. The second was a telephonic and online survey, answered by 217 neurologists, whose questions referred to: places of neurological education and work, type of working contract, and degree of social protection (estimated by the percentage of worked time during which they paid Social Security contributions). RESULTS: Sixty-three per cent (136/217) of the Spanish young neurologists had an unstable job. The most frequent unstable working contracts were: eventual (n=65; 31%), on-call contracts (n=54; 25%) and grants (n=53; 24%). Forty-eight per cent of the neurologists who ended their specialization in 2000 still remained working-unstable. The mean percentage of worked time with full social protection was 71.01+/-36.74%. Less than a half (n=101; 46%) had social protection during the entire worked time, 60 (28.6%) were socially protected during <50% of the worked time, and 23 (11%) never had social protection. A direct relationship was observed 68 between working instability and lower social protection (p=0.0002). The working situation of the Spanish young neurologists was seen as problematic by 97% of the 66 participants in the first survey. CONCLUSIONS: The current situation of the Spanish young neurologists, attending their working stability and degree of social protection, seems precarious and problematic. Urgent actions should be taken by the Administration to improve it.

Encuesta sobre la situación actual de los neurólogos jóvenes en España: análisis de la estabilidad laboral y de la protección social / Survey on the current situation of the young neurologists in Spain: analysis of their current working stability and degree of social protection

Introducción. No existen estudios que hayan analizado la problemática laboral de los neurólogos jóvenes. Este trabajo tuvo como objetivos evaluar la estabilidad laboral y grado de protección social de los neurólogos jóvenes en España y conocer la visión que éstos tienen sobre su propia situación. Métodos. Sobre una muestra total de 343 neurólogos que acabaron su formación especializada en España entre 2000 y 2004 se realizaron dos encuestas consecutivas (electrónica y electrónica más telefónica], obteniéndose datos de 66 y de 217 neurólogos, respectivamente. En la primera se preguntó por la disponibilidad de desplazamiento en caso de contrato inestable y por la impresión sobre la situación de la neurología joven. La segunda versó sobre los lugares de formación y trabajo actual, la estabilidad laboral y la protección social (porcentaje del tiempo trabajado cotizado a la Seguridad Social). Resultados. El 63 % (136/217) de los neurólogos encuestados se encontraba en situación laboral inestable. Los tipos de contrato inestable más frecuentes fueron: contratos eventuales (n = 65; 31 %), contratos de guardias (n = 54; 25%) Y becas (n=53; 24%). Un 48% de los neurólogos titulados en 2000 permanecía en situación laboral inestable. El porcentaje medio de tiempo cotizado a la Seguridad Social fue de 71,01 :t36,74%. Menos de la mitad (46%) de los encuesta dos cotizó el 100% del tiempo trabajado, un 28,6% cotizó durante menos del 50% y un 11 % no cotizó nunca. Se observó una relación directa entre inestabilidad laboral y mayor desprotección social (p=0,0002). En la otra encuesta, el 97% de los 66 encuestados consideró que la situación actual de la neurología joven se podía calificar de problemática. Conclusiones. La situación actual de los neurólogos jóvenes en España, en lo que se refiere a estabilidad laboral y protección social, es muy precaria y problemática. Dada la creciente demanda social de la neurología es necesaria una planificación adecuada de las necesidades y los recursos por parte de la Administración para mejorar la situación laboral de los neurólogos jóvenes

Introduction. The aims of this study were to evaluate the working stability and degree of social protection of the Spanish young neurologists, and to know their opinion about their own situation. Methods. The 343 neurologists that became specialists in Spain between 2000 and 2004 were asked to participate in two consecutive surveys. The first, conducted online, included questions about the availability to change the place of work and the opinion about the situation of young neurologists, and obtained 66 answers. The second was a telephonic and online survey, answered by 217 neurologists, whose questions referred to: places of neurological education and work, type of working contract, and degree of social protection (estimated by the percentage of worked time during which they paid Social Security contributions). Results. Sixty-three per cent (136/217) of the Spanish young neurologists had an unstable job. The most frequent unstable working contracts were: eventual (n=65; 31 %), on-call contracts (n=54; 25%) and grants (n = 53; 24 %). Forty-eight per cent of the neurologists who ended their specialization in 2000 still remained working-unstable. The mean percentage of worked time with full social protection was 71.01 :t36.74%. Less than a half (n = 10 1; 46 %) had social protection during the entire worked time, 60 (28,6 %) were socially protected during < 50% of the worked time, and 23 (11 %) never had social protection. A direct relationship was observed between working instability and lower social protection (p = 0.0002). The working situation of the Spanish young neurologists was seen as problematic by 97 % of the 66 participants in the first survey. Conclusions. The current situation of the Spanish young neurologists, attending their working stability and degree of social protection, seems precarious and problematic. Urgent actions should be taken by the Administration to improve it

Spinocerebellar ataxia type 2 (SCA2) with white matter involvement.

Two sisters presented with olivopontocerebellar atrophy, neuronal loss in the substantia nigra, intranuclear ubiquitin-, ataxin-2-positive inclusions in neurons, and severe demyelination and axon loss of the cerebral white matter with no accompanying inflammatory pathology. The genetic study demonstrated a 22/36 CAG triplet expansion in the SCA2 gene in one of the sisters; SCA1, SCA3, SCA6, SCA7, SCA8, SCA12, SCA17 and DRPL were ruled out in this patient. The present report shows that severe cerebral white matter pathology may occur in the context of SCA2.

Corticosteroids, ibuprofen, and acetaminophen for IFNbeta-1a flu symptoms in MS: a randomized trial.

OBJECTIVE: To compare the efficacy of acetaminophen, ibuprofen, and prednisone in the treatment of interferon beta-1a (IFNbeta-1a) flu-like syndrome (FLS). METHODS: Patients with relapsing-remitting multiple sclerosis initiating treatment with IM IFNbeta-1a were randomized in a multicenter, randomized, double-blind, controlled trial to receive acetaminophen 500 mg before and 6 and 12 hours after each injection, ibuprofen 400 mg before and 6 and 12 hours after each injection, or prednisone 60 mg daily for 1 week, plus tapering. Patients were instructed to keep a daily diary of fever severity, myalgia, chills, headache, and asthenia for 27 days. The sum of the scores of individual symptoms was used to obtain a daily FLS index. The primary outcome was the FLS index area under the curve (AUC) corrected by the number of measurement days. RESULTS: Eighty-four patients were randomized at 11 hospitals: acetaminophen (n = 28), ibuprofen (n = 28), and corticosteroids (n = 28). No differences were detected between treatments in the mean AUC of the FLS index. With limitation of the analysis to the days of IM IFNbeta-1a injection, differences favoring ibuprofen were observed in the mean FLS index (p = 0.0007). CONCLUSIONS: No prophylactic treatment for flu-like syndrome seems to be superior to another in terms of overall well-being during the first month of IM IFNbeta-1a therapy. However, ibuprofen confers better control of symptoms immediately following IM IFNbeta-1a injection.

Neuroradiological and clinical findings in rhombencephalosynapsis.

Rhombencephalosynapsis is an unusual disorder characterised by maldevelopment of the rhombencephalon, sometimes with supratentorial midline anomalies. We report MRI findings in a 39-year-old woman, the oldest in the literature. MRI demonstrated hypoplasia of the cerebellar vermis, with fusion of the cerebellar hemispheres and abnormally oriented folia. Supratentorial anomalies were also seen.

[Voluntary changes of visual evoked potentials in cases with hysteria and/or simulation]. / Alteración voluntaria de los potenciales evocados visuales (VEP) en casos de histeria y/o simulación.

We report a 25-year-old woman with gradual loss of visual acuteness, during puerperium and blindness in fifteen days. Two months latter she remain steady. Visual evoked potentials using monocular checkerboard pattern-reversal, were abnormal. Clinical suspicion of voluntary alteration of VEP was considered. A second VEP exploration with binocular stimulation and maneuvers of distraction were carried out. Normal VEP were recorded. Deliberate alteration of the visual evoked potential should be considered in patients suspicious of hysteria or malingering.

[Antiaggregant treatment for cerebral ischemia: ticlopidine versus aspirin]. / Tratamiento antiagregante en la isquemia cerebral: ticlopidina frente aspirina.

INTRODUCTION: There at present exists marked controversy as to the possible greater efficacy of ticlopidine as opposed to aspirin in brain ischaemia secondary prophylaxis. In our study we compare the efficacy and safety of antiaggregant treatment with ticlopidine as opposed to aspirin in a group of 310 patients admitted to the 'Hospital Mutua de Terrassa' between the years 1990 and 1994. RESULTS: In the group of patients treated with ticlopidine we found a larger number of new cerebrovascular incidents (p = 0.02) and peripheral vascular incidents (p = 0.01). New cerebrovascular incidents were more frequent in males (p = 0.03), in those patients with substantiated infarct (p = 0.02) and in patients with ischaemia in the carotidal region (p = 0.04). On the other hand, the group of patients treated with ticlopidine presented more secondary effects than the group treated with aspirin at the digestive (p < 0.001) and haematologic (p < 0.001) levels. The most frequent digestive secondary effects were diarrhoea (p < 0.001) and hepatopathy (p = 0.02); abnormalities in the leucocyte count were more frequent in patients treated with ticlopidine (p < 0.001), neutropenia being found in 0.8% of cases. CONCLUSION: In the patients we studied ticlopidine was less efficacious than aspirin in the secondary prophylaxis of new vascular incidents (both cerebrovascular and peripheral vascular) and also presented a greater incidence of secondary (digestive and haematological) effects.

Indolent anti-Hu-associated paraneoplastic sensory neuropathy.

Paraneoplastic sensory neuropathy (PSN) usually runs a subacute progressive course, leaving the patient with severe sensory dysfunction in weeks to months. We describe five patients with PSN, high titers of anti-Hu antibodies (type 1 antineuronal nuclear autoantibodies), and an indolent clinical course. The patients had a median age of 55 years (range, 41 to 72). Four had small-cell (3) or undifferentiated large-cell (1) lung cancer. Patients presented with mild, asymmetric sensory symptoms; in two, the neuropathy was predominant in the arms. Two patients also had a visceral neuropathy causing gastrointestinal dysfunction. The PSN was stable or progressed very slowly without treatment for a median of 18 months (range, 5 to 32) and remained so after treatment with immunoglobulins (1 patient), chemotherapy (3), or both therapies (1). All patients were ambulatory, leading an independent life up until the time of the last visit or until death from the tumor (2 patients). The median follow-up was 36 months (range, 22 to 52). A paraneoplastic origin should be considered in patients with mild, very slowly progressive sensory neuropathies.

[Congenital muscular dystrophy: report on 10 cases]. / Distrofia muscular congénita: presentación de una serie de diez casos.

Clinical features in 10 cases (3 males, 7 females) with congenital muscular dystrophy (CMD) are reported. Six patients suffered from pure forms of CMD. Two patients had diffuse cerebral white matter involvement of both hemispheres, demonstrated by computerized tomography and magnetic resonance imaging. Two patients were diagnosed as having Walker-Warburg syndrome on the basis of clinical and neuroradiological findings. The present series of patients further illustrates the heterogeneity of the various entities included under the term CMD even when central nervous system involvement is not suspected.

[Risk factors in cerebrovascular disease. Do transient ischemic attacks lead to changes in lifestyle?]. / Factores de riesgo en la enfermedad cerebrovascular: sensibiliza un ictus a cambiar de hábitos?

A minor stroke does not cause sufficient alarm in patients to change their lifestyles. 46 patients who suffered strokes in the previous 2 years were contacted by telephone, recording rehabilitation programs, medical therapy and modification of dietary habits. It was discovered that only 50% of those requiring motor rehabilitation actually received such treatment; and only one of twenty carried on speech exercises. Subsequent ischemic events were reported by 22 patients: 12 of whom had strokes, 8 ischemic myocardial attacks and two patients suffered both conditions. The majority of patients followed the prescribed medical treatment and advice badly. Of the 46 patients reviewed, 31 were hypertensive and only 16 follow recommended diets. Out of 15 smokers only, 6 gave up, and from 21 with dislypemia only 10 modified their diets. Of all patients only the most severely affected complied satisfactorily to medical treatments; a possible explanation for this would be that the contact between patients and physician (neurologist and family doctors) was discontinued immediately after the patients discharge from hospital. It is recommended therefore that steps be taken to insure better communication between neurologist, general practitioner and patient; thereby improving prognosis.

[Pancoast's syndrome: difficulties in its diagnosis]. / Tumor de Pancoast: dificultades para su diagnóstico.

We present four patients with pancoast tumor diagnosed between 6 to 13 months after the appearance of symptoms. In three of the patients, the presence of old pulmonary apical fibrous lesions complicated diagnosis by simple radiology. Clinical suspicion of the tumor provided the basis for further complementary explorations which confirmed the apical lesion: TC in two patients and RM in the third. Three patients histological diagnosis was obtained by aspirative transthoracic punction. The late diagnosis resulted in the treatment being purely palliative in three cases. It should be stressed that when presented with persistent shoulder pain, clinicoradiological assessment need to be exhaustive in order to make an early diagnosis.