Abordaje terapéutico de los síntomas no motores de la enfermedad de Parkinson / Therapeutic management of the non-motor symptoms in Parkinson's disease

Introducción. La enfermedad de Parkinson (EP) es una entidad compleja con una sintomatología diversa que presenta, además de los clásicos síntomas motores, un amplio número de síntomas no motores. Estos síntomas son muy prevalentes desde el inicio de la enfermedad e incluso pueden preceder en el tiempo a los síntomas motores (estreñimiento, alteración del olfato, trastorno de conducta del sueño REM) actuando como marcadores precoces de la enfermedad. Causan importante impacto en la calidad de vida de los enfermos con EP. Pueden ser los síntomas más incapacitantes para el paciente. Por todo ello, precisan de un manejo adecuado que mejore el bienestar de nuestros pacientes. Objetivo. Dar una visión actualizada del tratamiento de los síntomas no motores más prevalentes de la EP. Desarrollo. Se describen los síntomas no motores (vegetativos, trastornos del sueño, apatía) más prevalentes y discapacitantes de la enfermedad de Parkinson y se hace una revisión actualizada de su tratamiento. Conclusión: La alteración que la enfermedad produce en otros sistemas distintos al dopaminérgico causa un amplio número de síntomas distintos a los motores. Su mejor conocimiento permitirá diagnosticar y optimizar el tratamiento de estos síntomas, reforzando el bienestar de nuestros pacientes (AU)

Introduction. Parkinson's disease (PD) is a complex condition with a variety of symptoms, including a large number of nonmotor symptoms, in addition to the classic motor symptoms. These symptoms are highly prevalent from the onset of the disease and may even appear earlier than the motor symptoms (constipation, altered sense of smell, REM sleep behaviour disorder) and act as early markers of the disease. They have a significant impact on the quality of life of patients with PD and can be the most disabling symptoms for the patient. As a result, they need adequate management that improves our patients’ welfare. Aims. The objective of this study is to offer an updated view of the most prevalent non-motor symptoms of PD. Development. We describe the most prevalent and disabling non-motor symptoms (vegetative, sleep disorders, apathy) of Parkinson's disease and we also conduct a review of the state-of-the-art in its treatment. Conclusions. The alterations that the illness produces in systems other than the dopaminergic system cause a large number of symptoms in addition to the motor ones. A better understanding of them will make it possible to diagnose and optimise the treatment of these symptoms, thereby boosting our patients' welfare (AU)

Degeneración corticobasal / Corticobasal degeneration

La degeneración corticobasal (DCB) es un proceso neurodegenerativo lentamente progresivo, de inicio en la edad adulta que se presenta típicamente con parkinsonismo asimétrico y disfunción cognitiva. Actualmente, se clasifica como una taupatía. Los hallazgos neuropatológicos muestran una importante pérdida neuronal cortical que hasta fases muy avanzadas de la enfermedad es de predominio asimétrico y multifocal, más a menudo en las regiones frontalesparietales. Es un síndrome de una gran diversidad clínica cuya evolución estará marcada por la asimetría de los principales síntomas. El trastorno cognitivo puede ser una forma habitual de presentación identificándose un espectro de fenotipos clínicos dependiendo de la topografía del área degenerativa. Típicamente, se caracteriza por marcada rigidez e hipocinesia, distonía de predominio en una extremidad, mioclonías corticales reflejas, piramidalismo y temblor grosero postural o de acción. También destacan los déficits apráxicos, el déficit sensitivo cortical y el denominado fenómeno del miembro ajeno que aunque menos habitual cuando aparece es característico. El diagnóstico es fundamentalmente clínico, en base a los criterios diagnósticos del síndrome corticobasal propuestos en el año 2003 y con el apoyo de las pruebas las complementarias. El diagnóstico diferencial debería considerarse con los Parkinson-Plus así como con las demencias degenerativas primarias con predominio de síntomas frontales y/o temporales, en especial, con la PSP y la demencia fronto-temporal .La DCB progresa en 3-15 años hacia la muerte del individuo, normalmente como consecuencia de complicaciones derivadas de la inmovilidad (AU)

Corticobasal degeneration (CBD) is a slowly progressive neurodegenerative process, which begins in adulthood and typically presents with asymmetrical parkinsonism and cognitive dysfunction. It is currently classified as a tauopathy. Neuropathological findings show an important loss of cortical neurons that is predominantly asymmetrical and multifocal until very advanced phases of the disease, more often in the frontal-parietal regions. It is a syndrome with a wide clinical diversity and a progression that will be marked by the asymmetry of the main symptoms. Cognitive disorder may be a common presenting symptom and a range of clinical phenomena have been identified, depending on the topography of the degenerative area. It is typically characterised by a marked rigidity and hypokinesia, dystonia with predominance in one extremity, cortical reflex myoclonus, pyramidal signs and postural or action coarse tremor. Also prominent are apraxic deficits, cortical sensory deficit and the so-called alien limb phenomenon, which, although less frequent, is considered characteristic when it does appear. Diagnosis is fundamentally clinical and based on the diagnostic criteria of corticobasal syndrome put forward in the year 2003 and with the support of complementary tests. Differential diagnosis should be considered with Parkinson-Plus as well as with the primary degenerative dementias with predominance of frontal and/or temporal symptoms, more especially with PSP and fronto-temporal dementia. CBD progresses in 3-15 years towards the death of the individual, normally as a consequence of complications deriving from immobility (AU)

Epicrania fugax: características clínicas de una serie de 18 pacientes / Epicrania fugax: the clinical characteristics of a series of 18 patients

Introducción. La epicrania fugax es una entidad de reciente descripción, consistente en breves paroxismos dolorosos iniciados en regiones cefálicas posteriores, con irradiación hacia ojo, nariz o sien ipsilaterales. Objetivo. Presentar 18 casos de epicrania fugax de una consulta monográfica de cefaleas de un hospital terciario y analizar sus características demográficas y clínicas, así como la indicación y respuesta al tratamiento profiláctico. Pacientes y métodos. Entre marzo de 2008, momento en el que se describe la epicrania fugax y marzo de 2011, 18 pacientes (12 mujeres y 6 hombres), de entre 1.210 atendidos en dicha consulta (1,48%), recibieron dicho diagnóstico. Seis de estos casos se habían publicado con anterioridad. Resultados. Edad media al inicio de 42,5 ± 17,7 años (rango: 23-82 años). Presentaban paroxismos dolorosos iniciados en la región occipital (n = 11; 61,1%), parietal (n = 6; 33,3%) o parietooccipital (n = 1; 5,6%), e irradiados hacia el ojo (n = 12; 66,6%) o la sien (n = 6; 33,3%) ipsilaterales; todo el proceso duraba menos de 15 segundos. La mayoría describía su dolor como lancinante o punzante. En 10 casos (55,5%) persistía un dolor en la zona de origen de los paroxismos, que en 6 (33,3%) estaba circunscrito a una zona circular bien delimitada y reunía criterios de cefalea numular. En 12 casos (66,6%) se utilizó tratamiento profiláctico, sobre todo lamotrigina y gabapentina con respuesta variable. Conclusión. Pretendemos reforzar la propuesta de la epicrania fugax como un nuevo síndrome con un espectro clínico bien caracterizado. No parece una entidad excepcional, y su conocimiento dará lugar probablemente a la descripción de nuevas series. Con frecuencia es necesario tratamiento y, aunque se requiere mayor experiencia, la gabapentina y la lamotrigina tienen un papel prometedor (AU)

Introduction. Epicrania fugax is a recently reported condition consisting in brief painful paroxysms that begin in the posterior regions of the brain and irradiate towards the ipsilateral eye, nose or temple. Aims. To present 18 cases of epicrania fugax from a monographic headache centre in a tertiary hospital and to analyse their demographic and clinical features, as well as the indication and response to prophylactic treatment. Patients and methods. Between March 2008, when epicrania fugax was first reported, and March 2011, of a total of 1210 patients who were attended in that service (1.48%), 18 (12 females and 6 males) were diagnosed as suffering from this condition. Six of these cases had been published earlier. Results. The mean age at onset was 42.5 ± 17.7 years (range: 23-82 years). They presented painful paroxysms that began in the occipital (n = 11; 61.1%), parietal (n = 6; 33.3%) or parieto-occipital (n = 1; 5.6%) regions and irradiated towards the ipsilateral eye (n = 12; 66.6%) or temple (n = 6; 33.3%); the whole process lasted less than 15 seconds. Most of them described the pain as lancinating or stabbing. In 10 cases (55.5%) a pain remained in the area where the paroxysms began, which in 6 cases (33.3%) was limited to a well-defined circular area and met the criteria for classification as nummular headache. In 12 cases (66.6%), prophylactic treatment was used, above all lamotrigine and gabapentin, with varying results. Conclusion. Our aim is to back the proposal of epicrania fugax as a new syndrome with a well-defined clinical spectrum. It does not appear to be an exceptional condition and further knowledge about it will probably give rise to the description of new series. Treatment is often necessary and, although further information and experience are needed, gabapentin and lamotrigine both play a promising role (AU)

[Other non-motor disorders in Parkinson's disease]. / Otros trastornos no motores en la enfermedad de Parkinson.

INTRODUCTION: In addition to the classic triad (tremor, rigidity and akinesia), Parkinson's disease (PD) is also accompanied by several non-motor disorders. AIM: To carry out an updated review of these non-motor symptoms in terms of their pathophysiology, epidemiology, clinical features and treatment. DEVELOPMENT: Autonomic disorders such as seborrhoeic dermatitis and disorders involving sweating, fatigue, weight loss or respiratory problems (dyspnea, inspiratory stridor) are highly prevalent and very disabling symptoms. In addition, they may be the main problem in a particular phase of PD (fatigue, stridor) and condition the quality of life of patients with Parkinson. They are often neglected and remain undetected. Although they may respond to dopaminergic agents, they usually require a different therapeutic approach. Studies are needed to evaluate new therapeutic perspectives that act against the pathophysiological mechanisms of these disorders. CONCLUSIONS: PD affects systems other than the dopaminergic nigrostriatal. A better understanding of this pathology will make it possible to approach and optimise the treatment of the conditioning symptoms, thereby enhancing the welfare of patients with PD.

[Cerebral infarction in an adolescent girl following an overdose of paroxetine and caffedrine combined with theodrenaline]. / Infarto cerebral en una adolescente tras una autointoxicación con paroxetina y una asociación de cafedrina y teodrenalina.

Selective serotonin reuptake inhibitors (SSRI) have demonstrated to be effective, well tolerated and relatively safe drugs in cases of overdosage. However, and related to the potentiation of the serotonergic transmission elicited by them, these drugs have been associated by some authors with the possibility of causing vascular complications. Serotonin is a vasoactive substance with complex actions on vessel wall as a result of its interaction with specific receptors existing at this level. We present the case of an adolescent girl who suffered a cerebral infarction after consuming a toxic dose of paroxetine and two other products, one of them containing caffedrine and theodrenaline, and the other one a phlebotonic agent. In connection with the possible pathophysiological mecanism the implied products as well as the serotonergic vascular receptors are briefly reviewed. Finally, a reference is made to Calls syndrome as a possible entity related to the unfortunate event suffered by the patient. As a conclusion risks of the combined pharmacotherapy, especially in cases of overdosage and in child and adolescent populations, are underlined.

[Horner's syndrome secondary to ophthalmic herpes zoster]. / Síndrome de Horner secundario a herpes zoster oftálmico.

INTRODUCTION: Ophthalmoparesias is a frequent complication of ophthalmic herpes zoster. It occurs in 31% of all cases. However, the presence of Horner's syndrome during viral reactivation is a rarity which has only been previously described on two occasions, and never associated with cranial nerve involvement. CLINICAL CASE: We describe a patient with the first case of Horner's syndrome secondary to ophthalmic herpes zoster, with simultaneous, homolateral lesions of the third and sixth cranial nerves. Clinical evaluation, the course of the disorder, negative magnetic resonance studies and tests with cocaine and foledrin eye drops confirmed the presence of a post-ganglionar sympathetic lesion, probably situated in the ipsilateral cavernous sinus. CONCLUSIONS: Ophthalmoparesias as a complication of ophthalmic herpes zoster may have various origins. Diffusion of viral particles from the Gasserian ganglion and branches of the trigeminal nerve to adjacent structures, muscles, nerves and vessels, is the mechanism often mentioned. Presence of a simultaneous sympathetic lesion is very rare and of unknown pathology. However, it is probable that the origin of the lesion of the vegetative fibres is the same as that of the sensory or motor fibres, and adjacent inflammatory process caused by the virus extending. We analyze the factors involved in the low incidence of this association.