Palliative care management in patients with Parkinson's disease and other movement disorders in Spain. National survey of neurologists.

INTRODUCTION: Palliative care in neurodegenerative diseases is useful but underused. The objective of this study is to know how palliative care (PC) is applied in Spain in order to identify limitations and unmet needs. MATERIALS AND METHODS: It is a descriptive, observational, cross-sectional study, anonymous survey type of 20 questions, directed and answered by neurologists dedicated to movement disorders (MD) in Spain. RESULTS: 58 responses were obtained from neurologists from 15 autonomous communities. 69% answered that they did not have a specialised MD nursing facility but did have a PC team in their centre (81%). No specific protocol for PC in MD was identified. All except one neurologist stated that they lacked sufficient training in PC, the main training need being the "advance directives explanation". Only 1 in 4 neurologists answered routinely explaining advance healthcare planning to their patients, recognising up to 84.5% of neurologists not knowing how to assess the patient's competence. 60.3% of those surveyed answered that between 10% and 30% of their patients would be candidates for PC, although 1 in 3 said they were not clear when to refer the patient to PC. 100% of neurologists affirmed the priority need to implement PC protocols in MD. CONCLUSIONS: Our study shows a formative deficit in PC in this area and in the care of the patient with movement disorders and their environment, and should serve as a starting point to develop consensual care protocols.

Versión española del test perfil de síntomas autonómicos / Spanish version of the autonomic symptom profile test

Resumen. Introducción. El perfil de síntomas autonómicos (PSA) es un cuestionario autorrellenable, que ha sido validado en lengua inglesa para valorar los síntomas autonómicos y diferenciar entre pacientes y sujetos sanos según dicha sintomatología. Nuestro grupo ha estudiado su aplicación en lengua castellana. Sujetos y métodos. Después de traducir al castellano la versión inglesa y retrotraducirla, se evaluó la fiabilidad y la validez de criterio de la versión española del test PSA en una muestra de 50 sujetos (27 controles y 23 enfermos). A todos los pacientes con síntomas de disfunción autonómica se les estudió mediante la batería de Ewing y Clarke. Resultados. La fiabilidad de la versión española del PSA fue alta, y se obtuvo un coeficiente de correlación intraclase de 0,93. Con una puntuación en el test de 47 o superior, se obtiene una sensibilidad del 73,9% y una especificidad del 100% para detectar disfunción autonómica. Además, la puntuación de la versión española del test PSA mostró diferencias estadísticamente significativas entre el grupo de pacientes y el grupo control. Conclusiones. La versión española del PSA es fiable y permite distinguir entre pacientes con disfunción autonómica y sujetos control. En nuestro conocimiento, se trata del primer test que valora la sintomatología del sistema nervioso autónomo de forma global y en castellano (AU)

Summary. Introduction. The Autonomic Symptom Profile (ASP) is a self-administered questionnaire that has been validated in English to assess autonomic symptoms and to differentiate between patients and healthy subjects according to said symptoms. Our group has studied its application in Spanish. Subjects and methods. After translating the English version into Spanish and then back-translating it, the reliability and validity of criteria in the Spanish version of the ASP test were evaluated in a sample of 50 subjects (27 controls and 23 patients). All the patients with symptoms of autonomic dysfunction were studied by means of the Ewing and Clarke battery. Results. The reliability of the Spanish version of the ASP was high and an interclass correlation coefficient of 0.93 was obtained. A score of 47 or over in the test yields a sensitivity of 73.9% and a specificity of 100% for the detection of autonomic dysfunction. Furthermore, the score in the Spanish version of the ASP test showed statistically significant differences between the group of patients and the control group. Conclusions. The Spanish version of the ASP is reliable and makes it possible to distinguish between patients with autonomic dysfunction and control subjects. As far as we are aware, this is the first test to evaluate the symptoms of the autonomic nervous system in a global manner and in Spanish (AU)

[Spanish version of the Autonomic Symptom Profile test]. / Versión española del test perfil de síntomas autonómicos.

INTRODUCTION: The Autonomic Symptom Profile (ASP) is a self-administered questionnaire that has been validated in English to assess autonomic symptoms and to differentiate between patients and healthy subjects according to said symptoms. Our group has studied its application in Spanish. SUBJECTS AND METHODS: After translating the English version into Spanish and then back-translating it, the reliability and validity of criteria in the Spanish version of the ASP test were evaluated in a sample of 50 subjects (27 controls and 23 patients). All the patients with symptoms of autonomic dysfunction were studied by means of the Ewing and Clarke battery. RESULTS: The reliability of the Spanish version of the ASP was high and an interclass correlation coefficient of 0.93 was obtained. A score of 47 or over in the test yields a sensitivity of 73.9% and a specificity of 100% for the detection of autonomic dysfunction. Furthermore, the score in the Spanish version of the ASP test showed statistically significant differences between the group of patients and the control group. CONCLUSIONS: The Spanish version of the ASP is reliable and makes it possible to distinguish between patients with autonomic dysfunction and control subjects. As far as we are aware, this is the first test to evaluate the symptoms of the autonomic nervous system in a global manner and in Spanish.

[Two years' experience in a specific autonomic nervous system service]. / Experiencia de dos años en una consulta específica de sistema nervioso autónomo.

INTRODUCTION: Despite the high incidence and prevalence of pathologies affecting the autonomic nervous system (ANS), this part of neurology has received very little specific attention in clinical care in our country. AIM: To present the experience we have gained over a two-year period in an ANS-specific service. PATIENTS AND METHODS: Our patients were referred to the ANS service by other colleagues, most of whom were neurologists, between April 2006 and April 2008, after proposing a set of eligibility and exclusion criteria. Clinical history, examination and general analysis were performed for all patients. The following tests were also carried out on an individualised basis: Ewing-Clarke test, the Spanish version of the autonomic symptom profile test, tilt table test, holter heart monitor, urodynamic study and reflex sympathetic test, among other complementary studies. RESULTS: Thirty-four first visits and 62 successive visits were registered. The most frequent diagnoses were neurologically mediated syncopes and diabetic autonomic neuropathies, but other less prevalent conditions were also diagnosed. The most cost-effective complementary tests were the Ewing-Clarke test and the autonomic symptom profile test. Apart from benzodiazepines, the most commonly prescribed pharmacological treatments were paroxetine and pyridostigmine. CONCLUSIONS: As expected, neurologically mediated syncopes and diabetic neuropathies with an autonomic component are the most frequent pathologies in an ANS service. Nevertheless, their diagnosis and individualised treatment, together with that of other less prevalent autonomic pathologies, may require specific attention. To our knowledge, this is the first service of its kind in our country.

Experiencia de dos años en una consulta específica de sistema nervioso autónomo / Two years'experience in a specific autonomic nervous system service

Introducción. A pesar de la alta incidencia y prevalencia de la patología del sistema nervioso autónomo (SNA), esta parte de la neurología apenas ha merecido una atención específica en la asistencia clínica de nuestro país. Objetivo. Presentar nuestra experiencia de dos años en una consulta específica de SNA. Pacientes y métodos. Pacientes enviados a la consulta de SNA por otros compañeros, en su mayoría neurólogos, entre abril de 2006 y abril de 2008, tras proponer unos criterios de inclusión y exclusión. A todos los pacientes se les realizó anamnesis, exploración y analítica general. De forma individualizada, se realizaron test de Ewing-Clarke, versión española del test perfil de síntomas autonómicos, test de la mesa basculante, holter cardíaco, estudio urodinámico y test simpático reflejo, entre otros estudios complementarios. Resultados. Se realizaron 34 primeras consultas y 62 sucesivas. Los diagnósticos más frecuentes fueron los síncopes neurológicamente mediados y las neuropatías diabéticas autonómicas, pero se han diagnosticado otras entidades menos prevalentes. Las pruebas complementarias más rentables fueron el test de Ewing-Clarke y el test perfil de síntomas autonómicos. Los tratamientos farmacológicos más prescritos han sido, aparte de las benzodiacepinas, la paroxetina y la piridostigmina. Conclusiones. Como cabía esperar, los síncopes neurológicamente mediados y las neuropatías diabéticas con componente autonómico son las patologías más frecuentes en una consulta de SNA. Sin embargo, su diagnóstico y tratamiento individualizado, así como el de otras patologías autonómicas menos prevalentes, pueden requerir una atención específica. En nuestro conocimiento, se trata de la primera consulta de estas características en nuestro país (AU)

Introduction. Despite the high incidence and prevalence of pathologies affecting the autonomic nervous system (ANS), this part of neurology has received very little specific attention in clinical care in our country. Aim. To present the experience we have gained over a two-year period in an ANS-specific service. Patients and methods. Our patients were referred to the ANS service by other colleagues, most of whom were neurologists, between April 2006 and April 2008, after proposing a set of eligibility and exclusion criteria. Clinical history, examination and general analysis were performed for all patients. The following tests were also carried out on an individualised basis: Ewing-Clarke test, the Spanish version of the autonomic symptom profile test, tilt table test, holter heart monitor, urodynamic study and reflex sympathetic test, among other complementary studies. Results. Thirty-four first visits and 62 successive visits were registered. The most frequent diagnoses were neurologically mediated syncopes and diabetic autonomic neuropathies, but other less prevalent conditions were also diagnosed. The most cost-effective complementary tests were the Ewing-Clarke test and the autonomic symptom profile test. Apart from benzodiazepines, the most commonly prescribed pharmacological treatments were paroxetine and pyridostigmine. Conclusions. As expected, neurologically mediated syncopes and diabetic neuropathies with an autonomic component are the most frequent pathologies in an ANS service. Nevertheless, their diagnosis and individualised treatment, together with that of other less prevalent autonomic pathologies, may require specific attention. To our knowledge, this is the first service of its kind in our country (AU)

Papilitis unilateral como presentación de neurosífilis / Unilateral papillitis as the presenting symptom of neurosyphilis

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Edema de papila bilateral como manifestación inicial de un síndrome de POEMS / Bilateral papilloedema as the initial symptom of POEMS syndrome

Introducción. El edema de papila suele ser secundario a hipertensión intracraneal, pero en ocasiones puede ser la manifestación inicial de enfermedades sistémicas como síndrome de Guillain-Barré, neurosarcoidosis, enfermedad de Leber, enfermedad de Lyme, síndrome de POEMS y otros. Presentamos un caso ocurrido en el Hospital General Universitario de Alicante, en el que el edema de papila fue la manifestación inicial de un síndrome de POEMS. Caso clínico. Mujer de 36 años, sin antecedentes de interés, que consulta por cefalea opresiva frontal izquierda, de cinco días de evolución, de inicio brusco, sin náusea ni vómitos acompañantes, sin exacerbación con las maniobras de Valsalva y sin otra sintomatología acompañante. En la exploración destaca borramiento nasal del ojo derecho y papiledema en el ojo izquierdo, hiperpigmentación cutánea en cara y escote que respetaba pliegues, hipoestesia táctil dolorosa distal en miembros inferiores y arreflexia aquílea e hiporreflexia rotuliana bilateral. Tras realizar distintas pruebas complementarias se descartó lesión ocupante de espacio intracraneal e hipertensión intracraneal, y se llegó al diagnóstico de polineuropatía crónica sensitivomotora de tipo mixto en miembros superiores e inferiores con afectación predominantemente proximal, edema de papila bilateral y fotosensibilidad cutánea a estudio. Tras dos años, la paciente presentó hepatomegalia, hipotiroidismo subclínico y alteraciones cutáneas esclerodermiformes, con lo que se diagnosticó como probable síndrome de POEMS, confirmado por la presencia de componente monoclonal en sangre y orina. Conclusión. El edema de papila como manifestación inicial de un síndrome de POEMS es infrecuente, pero debe estar presente en el diagnóstico diferencial de esta patología por las implicaciones diagnósticas y terapéuticas que conlleva

INTRODUCTION. Oedema of the papilla, or papilloedema, is usually due to intracranial hypertension, but can sometimes be the initial symptom of systemic diseases like Guillain-Barre syndrome, neurosarcoidosis, Leber’s disease, Lyme disease or POEMS syndrome, among others. We report on a case that occurred at the Hospital General Universitario in Alicante, in which papilloedema was the initial symptom of POEMS syndrome. CASE REPORT. A 36-year-old female, with no relevant past history, who visited after suffering a left-side frontal oppressive headache for five days; onset was sudden, there was no concomitant nausea or vomiting, it did not get worse when Valsalva manoeuvres were performed and there were no other accompanying symptoms. The examination revealed nasal blurring in the visual field of the right eye and papilloedema in the left eye, hyperpigmentation of the skin on the face and upper chest area that respected creases in the skin, painful distal tactile hypaesthesia in the lower limbs and bilateral areflexia of Achilles’ tendons and patellar hyperreflexia. After conducting a number of different complementary tests intracranial space-occupying lesions and intracranial hypotension were both ruled out and the patient was diagnosed with mixed-type sensory-motor chronic polyneuropathy in the upper limbs, and in the lower extremities with predominantly distal involvement, bilateral oedema of the papilla and skin photosensitivity. Two years later the patient had hepatomegaly, subclinical hypothyroidism and sclerodermiform alterations of the skin, which suggested a probable diagnosis of POEMS syndrome that was later confirmed by the presence of monoclonal component in blood and urine. CONCLUSIONS. Papilloedema is rarely seen as the initial symptom of POEMS syndrome, but it must be included in the differential diagnosis of this pathology due to the diagnostic and therapeutic implications it entails

[Bilateral papilloedema as the initial symptom of POEMS syndrome]. / Edema de papila bilateral como manifestación inicial de un síndrome de POEMS.

INTRODUCTION: Oedema of the papilla, or papilloedema, is usually due to intracranial hypertension, but can sometimes be the initial symptom of systemic diseases like Guillain-Barre syndrome, neurosarcoidosis, Leber's disease, Lyme disease or POEMS syndrome, among others. We report on a case that occurred at the Hospital General Universitario in Alicante, in which papilloedema was the initial symptom of POEMS syndrome. CASE REPORT: A 36-year-old female, with no relevant past history, who visited after suffering a left-side frontal oppressive headache for five days; onset was sudden, there was no concomitant nausea or vomiting, it did not get worse when Valsalva manoeuvres were performed and there were no other accompanying symptoms. The examination revealed nasal blurring in the visual field of the right eye and papilloedema in the left eye, hyperpigmentation of the skin on the face and upper chest area that respected creases in the skin, painful distal tactile hypaesthesia in the lower limbs and bilateral areflexia of Achilles' tendons and patellar hyperreflexia. After conducting a number of different complementary tests intracranial space-occupying lesions and intracranial hypotension were both ruled out and the patient was diagnosed with mixed-type sensory-motor chronic polyneuropathy in the upper limbs, and in the lower extremities with predominantly distal involvement, bilateral oedema of the papilla and skin photosensitivity. Two years later the patient had hepatomegaly, subclinical hypothyroidism and sclerodermiform alterations of the skin, which suggested a probable diagnosis of POEMS syndrome that was later confirmed by the presence of monoclonal component in blood and urine. CONCLUSIONS: Papilloedema is rarely seen as the initial symptom of POEMS syndrome, but it must be included in the differential diagnosis of this pathology due to the diagnostic and therapeutic implications it entails.

Meningitis por Escherichia coli como complicación tardía de bomba intratecal de baclofeno / Meningitis due to Escherichia coli as a delayed complication of intrathecal de baclofeno

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[Binocular diplopy as the presentation of a haematoma in the rectus inferior]. / Diplopía binocular como presentación de un hematoma del recto inferior.

INTRODUCTION: The presence of quick onset binocular diplopy makes it essential to carry out a comprehensive differential diagnosis. The most frequent causes in adults include vascular, post-traumatic, tumorous and myopathic pathologies. Yet, to perform a differential diagnosis we also have to take into account less common aetiologies such as demyelinating disease, carotid-cavernous fistulas and Tolosa Hunt syndrome, among others. We report the case of a patient who was admitted to our hospital because she presented an acute onset diplopy secondary to a spontaneous haematoma of the left rectus inferior. CASE REPORT: We describe the case of a 54-year-old female with a history of hypercholesterolemia, who was a smoker and allergic to iodine contrasts and who visited because of an acute onset diplopy accompanied by instability and pain in the left eye. The neurological examination revealed paresis of the extraocular muscles dependent on the left oculomotor nerve with left palpebral ptosis, paresis of the rectus inferior, with no involvement of the pupils. Other results of the exploration were within normal limits. A computerised axial tomography scan of the head showed a haemorrhage in the rectus inferior. Magnetic resonance images of the head revealed an increase in the volume of the left-side rectus inferior with signs of bleeding, and the rest of the complementary explorations were normal. The patient progressed favourably and the diplopy gradually disappeared. CONCLUSIONS: Spontaneous bleeding of the extraocular muscles, although infrequent, must be taken into account in the differential diagnosis of painful ophthalmoplegia with diplopy in patients with no underlying pathology, especially in the absence of other neurological disorders.

Diplopía binocular como presentación de un hematoma del recto inferior / Binocular diplopy as the presentation of a haematoma in the rectus inferior

Introducción. La presencia de una diplopía binocular de instauración rápida obliga a un amplio diagnóstico diferencial. Entre las causas más frecuentes en personas adultas encontramos la patología vascular, postraumática, tumoral o miopática. Sin embargo, para realizar el diagnóstico diferencial debemos tener en cuenta etiologías menos frecuentes, como enfermedad desmielinizante, fístulas carotidocavernosas y síndrome de Tolosa-Hunt, entre otras. Presentamos el caso de un paciente que ingresó en nuestro servicio por presentar diplopía de instauración aguda secundaria a un hematoma espontáneo del recto inferior izquierdo. Caso clínico. Mujer de 54 años con antecedentes de hipercolesterolemia, fumadora y alérgica a contrastes yodados, que consulta por diplopía de instauración aguda acompañada de dolor ocular izquierdo e inestabilidad. A la exploración neurológica destaca paresia de la musculatura extraocular dependiente del III par craneal izquierdo con ptosis palpebral izquierda y paresia del recto inferior, sin afectación pupilar. El resto de la exploración estuvo dentro de la normalidad. En la tomografía axial computarizada craneal se observa una hemorragia en el recto inferior. En la resonancia magnética craneal se observó un aumento de volumen del recto inferior izquierdo con signos de sangrado, y el resto de las exploraciones complementarias fueron normales. La paciente evolucionó favorablemente y desapareció progresivamente la diplopía. Conclusión. La hemorragia espontánea de los músculos extraoculares, aunque infrecuente, debe considerarse en el diagnóstico diferencial de la oftalmoplejía dolorosa con diplopía en pacientes sin patología de base, especialmente en ausencia de otras alteraciones neurológicas (AU)

Introduction. The presence of quick onset binocular diplopy makes it essential to carry out a comprehensive differential diagnosis. The most frequent causes in adults include vascular, post-traumatic, tumorous and myopathic pathologies. Yet, to perform a differential diagnosis we also have to take into account less common aetiologies such as demyelinating disease, carotid-cavernous fistulas and Tolosa-Hunt syndrome, among others. We report the case of a patient who was admitted to our hospital because she presented an acute onset diplopy secondary to a spontaneous haematoma of the left rectus inferior. Case report. We describe the case of a 54-year-old female with a history of hypercholesterolemia, who was a smoker and allergic to iodine contrasts and who visited because of an acute onset diplopy accompanied by instability and pain in the left eye. The neurological examination revealed paresis of the extraocular muscles dependent on the left oculomotor nerve with left palpebral ptosis, paresis of the rectus inferior, with no involvement of the pupils. Other results of the exploration were within normal limits. A computerised axial tomography scan of the head showed a haemorrhage in the rectus inferior. Magnetic resonance images of the head revealed an increase in the volume of the left-side rectus inferior with signs of bleeding, and the rest of the complementary explorations were normal. The patient progressed favourably and the diplopy gradually disappeared. Conclusions. Spontaneous bleeding of the extraocular muscles, although infrequent, must be taken into account in the differential diagnosis of painful ophthalmoplegia with diplopy in patients with no underlying pathology, especially in the absence of other neurological disorders (AU)