[Diagnostic value of cardiac 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy in Lewy body disorders]. / Valor diagnóstico de la gammagrafía cardíaca con 123I-metaiodobenzilguanidina en las enfermedades con cuerpos de Lewy.

INTRODUCTION: Lewy body disorders such as Parkinson's disease (PD) and Lewy body dementia (LBD) are associated with cardiac sympathetic denervation, which can be visualized on 123I-MIBG scintigraphy. Our objectives were to study the diagnostic value of this technique in Lewy body disorders and its relationship with PD clinical variables. PATIENTS AND METHODS: We studied 90 patients: 51 with PD, 19 with LBD, 9 with multiple system atrophy (MSA) and 11 controls. Scintigraphy images were qualitatively evaluated and early and delayed heart-to-mediastinum ratios (HMR) were calculated. The main confounding factors (ischemic heart disease, diabetes, hypertension and drugs) were controlled by multivariate linear regression analysis. We investigated correlations between scintigraphy variables and PD variables. RESULTS: The delayed HMR, which showed better discriminative ability was 2.03 +/- 0.32 in controls, 1.37 +/- 0.30 in PD (p<0.001 vs controls), 1.47+/-0.45 in LBD (p=0.001 vs controls) and 1.69+/-0.28 in MSA (p=0.02 vs controls; p=0.004 vs PD). This ratio was influenced by PD/LBD diagnosis (beta= -0.638; p<0.001) and to a lesser degree, by ischemic heart disease (beta= -0.244; p=0.028). Optimal cut-off value between PD/LBD and controls was 1.71 (83% sensitivity and 82% specificity). Within the PD group, those with a family history of PD/LB showed higher delayed HMR values (1.65+/-0.34 vs 1.30+/-0.24 without history; p<0.001) and proportion with normal scintigraphy (56% vs 5%; p=0.001). CONCLUSIONS: Cardiac 123I-MIBG scintigraphy is useful in the diagnosis of Lewy body disorders, although its value in PD is conditioned by having a family history of PD.

[Clinical utility of deep brain stimulation in an advanced Parkinson's disease]. / Utilidad clínica de la estimulación cerebral profunda en la enfermedad de Parkinson avanzada.

INTRODUCTION: Bilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) or globus pallidus internus (GPi) have demonstrated efficacy in advanced Parkinson's disease (PD). We aimed to assess the clinical utility of these procedures in terms of the quality of life, and to determine the pre and postsurgical characteristics related to the outcome. METHOD: A prospective study was conducted on a cohort of 20 patients with advanced PD who underwent bilateral DBS: 14 in STN and 6 in GPi. They were assessed according to the CAPSIT-PD protocol before and after surgery, with a mean follow-up of 9 and 11 months, respectively. The main outcome variables were change in the UPDRS III score in off efficacy and the PDQ-39 quality of life questionnaire score (clinical utility). RESULTS: The STN group improved their UPDRS III in off by a mean of 35% (p = 0.001) and their PDQ-39 by 21% (p = 0.026). The GPi group improved their UPDRS III in off by 21% (p = 0.028) and their PDQ-39 by 37% (p = 0.116). The presurgical levodopa-equivalent dose was a positive predictor of the efficacy and clinical utility of STN DBS and a negative predictor of the efficacy of GPi DBS. In both groups, the clinical utility was determined by improvement in functional disability in off scales. CONCLUSIONS: Bilateral DBS demonstrated middle-term efficacy and clinical utility in the treatment of advanced PD. The presurgical levodopa-equivalent dose was a predictor of the efficacy and clinical utility of DBS.

[Callosotomy in the treatment of drug-resistant epilepsy]. / La callosotomía en el tratamiento de la epilepsia farmacorresistente.

At the present time corpus callosotomy is a valuable option in the management of some patients with drug-resistant epilepsy who are not candidates for resective procedures. The records of six patients who underwent callosotomy at 'Hospital Virgen de las Nieves' (Granada, Spain) in the past four years were retrospectively analyzed. The patients all had intractable primary or secondarily generalized seizures, were severely handicapped by its frequency and nature (especially with drop attacks and multiple injuries) and were not suitable for other surgical procedure. The results of surgery (five anterior callosotomies and one subtotal section) are described after an average follow-up period of 2.5 years. Overall, four patients achieved significant improvement (at least 50% reduction in seizure frequency, severity, or both, affecting quality of life), with a marked reduction (> 75%) in two of them. There was no clinical deterioration, significant surgical complication nor relevant additional long-term neuro-psychological deficit in any case. Previous studies have been reviewed mainly to find those prognostic factors associated with a better seizure outcome or with the occurrence of complications. The best results are obtained in those patients with drop attacks (including atonic seizures) as the most frequent and disabling seizure type. According to the type of epilepsy, patients with localization-related epilepsy (especially when symptomatic of a focal brain damage) and those with the Lennox-Gastaut syndrome are the most likely to benefit from the procedure. It is suggested that, in the first place, a two-thirds anterior callosotomy should be performed particularly with atonic seizure are the most frequent seizure type. We may proceed with completion of callosal division as a second stage in those patients in whom a significant improvement has not been observed. In severely retarded patients with multiple seizure types, one-stage complete section may be performed. The procedure is relatively safe, with a low incidence of morbidity and clinically significant long-term neuro-psychological deficits. Further larger clinical studies are necessary to elucidate many aspects which are still unresolved. More uniformity would be desirable in the evaluation of patients, surgical technique, follow-up and presentation of results.

[Non-giant cell temporal arteritis as a manifestation of Churg-Strauss syndrome. Presentation of a case and review of literature]. / Arteritis no de células gigantes de la temporal como manifestación clínica del síndrome de Churg-Strauss. Presentación de un caso y revisión de la literatura.

Most cases of temporal arteritis are of the giant cell variety, with cases involving other histologic patterns occurring rarely. There are only 4 descriptions in the literature of non giant cell temporal arteritis as a manifestation of Churg-Strauss syndrome. We report the case of a 74-year-old man with a history of bronchial asthma who presented with systemic symptoms and right temporal cephalea with diplopia, diffuse muscle pain and transient skin lesions on the extremities. The right temporal artery was enlarged and painful but pulsatile. Tests showed a high erythrocyte sedimentation rate and leukocytosis with relative and absolute eosinophilia. Biopsy of the temporal artery revealed polymorphic inflammatory infiltration throughout the vas, with numerous eosinophils and non giant cells, confirming a diagnosis of Churg-Strauss syndrome with extension to the temporal artery. Temporal arteritis should be considered a syndrome with variable substrate pathology; the possibility that it is a rare manifestation of systemic necrotizing vasculitis should not be ruled out.

[Complete third nerve paralysis as clinical presentation of multiple sclerosis]. / Parálisis completa del tercer par como forma de presentación de esclerosis múltiple.

An acute attack of cephalea and third nerve palsy with pupillary involvement may be caused by a variety of entities, but aneurysm of the posterior communicating artery must certainly be ruled out. We describe a 22 year old patient in whom this clinical profile was an unusual first sign of multiple sclerosis.

[Clinical iconographic dissociation in a case of monophasic demyelinating disease]. / Disociación clinicoiconográfica en un caso de enfermedad desmielinizante monofásica.

We report a patient with monophasic inflammatory demyelinizing disease whose initial symptoms and imaging studies led to the undertaking of a cerebral biopsy for suspicion of an expansive process. The evolution of the both the CT and MR imaging studies with contrast and overall the surprising size of the lesions in MR when the patient was clinically asymptomatic support the hypothesis of residual dysfunction in the hemato-encephalic barrier as a cause of the persistence of MR images. This explanation appears more acceptable than its attribution to a gliosis secondary to previous inflammation.