[Apomorphine and frontal dysfunction in Parkinson's disease]. / Apomorfina y disfunción frontal en la enfermedad de Parkinson.

INTRODUCTION: Dysexecutive disorder and apathy are characteristic symptoms of frontal dysfunction linked to Parkinson's disease. The effect of continuous subcutaneous apomorphine infusion is not known in detail. DEVELOPMENT: A search for the most relevant studies published to date in this field was carried out, along with their analysis. Apomorphine achieves improvements in tests that measure tasks such as planning, attention, verbal fluency and apathy. CONCLUSIONS: Due to its distinctive pharmacological profile, with enhanced activity on D1-type dopaminergic receptors, apomorphine may have beneficial effects on the frontal dysfunction produced by the disease.

TITLE: Apomorfina y disfunción frontal en la enfermedad de Parkinson.Introducción. El trastorno disejecutivo y la apatía son síntomas característicos de la disfunción frontal ligada a la enfermedad de Parkinson. El efecto de la infusión continua subcutánea de apomorfina en la disfunción frontal no se conoce con detalle. Desarrollo. Se ha realizado una búsqueda y análisis de los trabajos publicados más relevantes en este campo. La apomorfina logra mejorías en las pruebas que miden tareas como la planificación, la atención, la fluencia verbal y la apatía. Conclusiones. Debido a su perfil farmacológico distintivo, con mayor actividad sobre los receptores dopaminérgicos de tipo D1, la apomorfina puede resultar beneficiosa en la disfunción frontal de la enfermedad.

Apomorfina y disfunción frontal en la enfermedad de Parkinson / Apomorphine and frontal dysfunction in Parkinson’s disease

Introducción: El trastorno disejecutivo y la apatía son síntomas característicos de la disfunción frontal ligada a la enfermedad de Parkinson. El efecto de la infusión continua subcutánea de apomorfina en la disfunción frontal no se conoce con detalle. Desarrollo: Se ha realizado una búsqueda y análisis de los trabajos publicados más relevantes en este campo. La apomorfina logra mejorías en las pruebas que miden tareas como la planificación, la atención, la fluencia verbal y la apatía. Conclusiones: Debido a su perfil farmacológico distintivo, con mayor actividad sobre los receptores dopaminérgicos de tipo D1, la apomorfina puede resultar beneficiosa en la disfunción frontal de la enfermedad.(AU)

Introduction: Dysexecutive disorder and apathy are characteristic symptoms of frontal dysfunction linked to Parkinson’s disease. The effect of continuous subcutaneous apomorphine infusion is not known in detail. Development: A search for the most relevant studies published to date in this field was carried out, along with their analysis. Apomorphine achieves improvements in tests that measure tasks such as planning, attention, verbal fluency and apathy. Conclusions: Due to its distinctive pharmacological profile, with enhanced activity on D1-type dopaminergic receptors, apomorphine may have beneficial effects on the frontal dysfunction produced by the diseas.(AU)

[Psychiatric and cognitive complications arising from subthalamic stimulation in Parkinson's disease]. / Complicaciones psiquiátricas y cognitivas de la estimulación subtalámica en la enfermedad de Parkinson.

INTRODUCTION AND DEVELOPMENT: Subthalamic stimulation is a therapeutic option that can be used to treat advanced cases of Parkinson's disease. However, psychiatric or cognitive disorders have been reported in some patients treated using this technique. Age and a long disease history are two important risk factors for the appearance of these problems. The complications that have been reported include cases of depression, apathy, manias and psychosis. Surgery can also exacerbate the syndrome of addiction to levodopa that is sometimes observed. In contrast, sleep disorders usually improve with this technique. As far as the cognitive sphere is concerned, verbal fluency has been seen to deteriorate and the executive functions become impaired in patients over 69 years of age. These disorders are usually due to a number of different causes and have been attributed to the action of stimulating areas close to the subthalamic nucleus, to the presence of previously existing cognitive or psychiatric problems, to unrealistic expectations about this technique or to the individual's inability to adapt to the functional situation after surgery. CONCLUSIONS: Although generally speaking these disorders are not usually serious, they must be borne in mind so that adequate treatment can be indicated.

[Psychiatric complications of Parkinson's disease: their symptoms and treatment]. / Complicaciones psiquiátricas de la enfermedad de Parkinson: clínica y tratamiento.

INTRODUCTION AND DEVELOPMENT: Psychiatric symptoms in Parkinson's disease are relatively frequent, with a variation between 10 and 50%, according to different publications. They generally occur after several years of treatment. They may be symptoms derived from the disease itself or linked to the treatment used. These complications vary and include anxiety, affective and psychotic (hallucinations, delirium) disorders, as well as other alterations such as sexual, sleep and cognitive problems. CONCLUSIONS: Treatment must always be tailor-made to suit each individual and in many cases we have to resort to lowering or withdrawing the dopaminergic medication while, in others, treatment with benzodiazepines, antidepressants and atypical neuroleptic drugs is indicated.

[Rapidly progressing dementia as the presenting symptom of Waldenstrom's macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing Neel syndrome]. / Demencia rapidamente progresiva como presentacion de macroglobulinemia de Waldenstrom: hallazgos de la resonancia magnetica cerebral en el sindrome de Bing Neel.

CASE REPORT: A 68 year old male with no relevant clinical history was admitted to hospital because of symptoms of cognitive impairment (attentional deficit, short term memory disorders and behavioural disorders), accompanied by apraxia of gait and rectal and urinary incontinence. Results of a general clinical exploration were normal. In the MRI study of the brain numerous areas of hypersignal were observed in different arterial territories, which stood out in the T2, FLAIR and, above all, in diffusion weighted sequences. Analytical studies showed hypergammaglobulinemia with monoclonal IgM k and a bone marrow biopsy revealed infiltration by plasmatic cells. Bing Neel syndrome was diagnosed. CONCLUSIONS: We underline this exceptional form of presentation of Waldenstrom's macroglobulinemia and highlight the findings of diffusion weighted MRI, which suggested multiple infarcts for which a neoplastic vascular obstruction mechanism similar to that involved in malignant angioendotheliomatosis is posited.

[Headache and fainting as initial symptoms of syringomyelia associated to Arnold-Chiari and facial angiomatous nevus]. / Cefalea y síncope como manifestaciones iniciales de siringomielia asociada a Arnold-Chiari y nevo angiomatoso facial.

INTRODUCTION: Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. CASE REPORT: 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. CONCLUSION: This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions.

[Kleine-Levin syndrome: contribution of brain SPECT in diagnosis]. / Sindrome de Kleine-Levin: aportación diagnóstica de la SPECT cerebral.

INTRODUCTION: Kleine Levin syndrome is an infrequent disorder of uncertain aetiopathogenesis that usually affects adolescent males, producing drowsiness and alterations in behaviour, appetite and sexuality. We report a clinically typical case in which the brain SPECT showed right frontal hypoperfusion. CASE REPORT: Male aged 17, right handed, who presented drowsiness, apathy, alterations in his sexual behaviour (masturbations in public and attempted assault of women) and hyperphagia, which coincided with his undergoing an emotionally stressful period in his life. Brain SPECT revealed reduced flow in the right frontal lobe, although MRI and CSF study were normal. The polysomnographic study revealed a destructured pattern of sleep, with a reduction in phases III and IV and in REM sleep. He was treated with lithium and evolved favourably without any relapses during the 18 month follow up. CONCLUSIONS: Cases of Kleine Levin syndrome with structural lesions have been reported, but most of them are idiopathic. A disorder in the hypothalamus and the limbic system is suggested. In our case, the findings from the brain SPECT confirmed a non dominant hypoperfusion of the frontal lobe, which could result from a phenomenon of diaschisis brought about by a diencephalic dysfunction.

Epilepsia como forma de presentación de un aneurisma intracraneal no roto / Epilepsy as the presenting symptom of an unruptured intracranial aneurysm

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Atrofia muscular peroneal secundaria a hernia discal dorsal / Peroneal muscular atrophy secondary to a herniated dorsal disc

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Complicaciones psiquiátricas y cognitivas de la estimulación subtalámica en la enfermedad de Parkinson / Psychiatric and cognitive complications arising from subthalamic stimulation in parkinson’s disease

Introducción y desarrollo. La estimulación subtalámica esuna opción terapéutica para los casos de enfermedad de Parkinsonavanzada. En algunos pacientes tratados con esta técnica se handescrito alteraciones psiquiátricas o cognitivas. La edad y la largaevolución de la enfermedad son dos factores de riesgo importantespara la aparición de estos problemas. Así, se han descrito casos dedepresión, apatía, manía y psicosis. La cirugía puede empeorar elsíndrome de adicción a la levodopa que se ve en ocasiones. Por elcontrario, esta técnica suele mejorar los trastornos del sueño. Sobrela esfera cognitiva, se ha constatado un empeoramiento de la fluenciaverbal, y en los pacientes mayores de 69 años, un empeoramientode las funciones ejecutivas. Las causas de estos trastornos suelenser variadas y se han atribuido a la acción de la estimulación sobreáreas próximas al núcleo subtalámico, a la existencia de problemascognitivos o psiquiátricos previos, a expectativas poco realistas sobreesta técnica o a la incapacidad de adaptarse a la situación funcionaltras cirugía. Conclusión. Aunque estas alteraciones en generalno suelen ser graves, se deben tener en cuenta para poder indicarun tratamiento adecuado

Introduction and development. Subthalamic stimulation is a therapeutic option that can be used to treat advancedcases of Parkinson’s disease. However, psychiatric or cognitive disorders have been reported in some patients treated usingthis technique. Age and a long disease history are two important risk factors for the appearance of these problems. Thecomplications that have been reported include cases of depression, apathy, manias and psychosis. Surgery can also exacerbatethe syndrome of addiction to levodopa that is sometimes observed. In contrast, sleep disorders usually improve with thistechnique. As far as the cognitive sphere is concerned, verbal fluency has been seen to deteriorate and the executive functionsbecome impaired in patients over 69 years of age. These disorders are usually due to a number of different causes and havebeen attributed to the action of stimulating areas close to the subthalamic nucleus, to the presence of previously existingcognitive or psychiatric problems, to unrealistic expectations about this technique or to the individual’s inability to adapt tothe functional situation after surgery. Conclusions. Although generally speaking these disorders are not usually serious, theymust be borne in mind so that adequate treatment can be indicated

Síndrome de Kleine-Levin:aportación diagnóstica de la SPECT cerebral / Kleine-Levin syndrome: contribution of brain SPECT in diagnosis

Introducción. El síndrome de Kleine-Levin es un trastorno infrecuente, de etiopatogenia no aclarada, que suele afectar a adolescentes varones, produciendo somnolencia y alteraciones del comportamiento, del apetito y de la sexualidad. Se presenta un caso con curso clínico típico, en el que la tomografía estandarizada por emisión de fotón único (SPECT) cerebral mostró una hipoperfusión frontal derecha. Caso clínico. Un varón de 17 años, diestro, que coincidía con una situación de estrés emocional, presentó somnolencia, apatía, alteración del comportamiento sexual (masturbaciones públicas e intentos de agresión a personal femenino) e hiperfagia. El estudio de SPECT cerebral mostró una disminución del flujo en el lóbulo frontal derecho, mientras que la resonancia magnética (RM) y el estudio del líquido cefalorraquídeo resultaron normales. El estudio polisomnográfico mostró una desestructuración del sueño, con una reducción de las fases III y IV y del sueño REM. Se trató con litio y evolucionó favorablemente, sin recidiva en 18 meses de seguimiento. Conclusiones. Se han descrito casos de síndrome de Kleine-Levin con lesiones estructurales encefálicas, pero la mayoría son idiopáticos. Se postula una disfunción hipotalámica y del sistema límbico. Los hallazgos del SPECT cerebral de nuestro caso confirmaron una hipoperfusión del lóbulo frontal no dominante, que podría traducir un fenómeno de diasquisis, propiciado por una disfunción diencefálica (AU)

Cefalea y síncope como manifestaciones iniciales de siringomielia asociada a Arnold-Chiari y nevo angiomatoso facial / Headache and fainting as initial symptoms of syringomyelia associated to arnold-chiari and facial angiomatous nevus

Introducción. La siringomielia y la malformación de Arnold-Chiari son dos malformaciones disráficas que frecuentemente se presentan asociadas; sus manifestaciones clínicas son muy variadas y algunos pacientes permanecen asintomáticos durante años. El síncope, atribuido a una disfunción de los baroceptores bulbares y del asta intermediolateral medular es una manifestación paroxística poco habitual, especialmente como forma de inicio. Caso clínico. Una mujer de 34 años, portadora de un nevo angiomatoso facial, consultó por presentar dos episodios de cefalea y síncope. La exploración neurológica resultó normal. El estudio de resonancia magnética demostró una moderada hidrocefalia supratentorial, malformación de Arnold-Chiari y siringomielia cervical, sin observarse alteraciones vasculares. Conclusión. Este caso, que comparte algunas características del síndrome de Sturge-Weber y el síndrome PHACE, es un ejemplo de la importancia de investigar alteraciones del sistema nervioso central en pacientes con síntomas paroxísticos y alteraciones cutáneas (AU)

Introduction. Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. Case report. 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. Conclusion. This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions (AU)