Jordi Rumià

Bilateral subthalamic nucleus stimulation and quality of life in advanced Parkinson's disease

We examined the impact of the subthalamic nuclei (STN) deep brain stimulation (DBS) on the health‐related quality of life (QoL) of patients with advanced Parkinsons disease (PD). Seventeen consecutive patients with refractory motor fluctuations and dyskinesia were included in the study (mean age, 60.9 ± 7.7 years [range, 43–74 years]; disease duration, 16.4 ± 8.5 years [range, 7–38 years]; mean off‐medication Hoehn and Yahr stage, 4.23 ± 0.66 [range, 2.5–5]). Each patients assessment was carried out using common rating scales, following the Core Assessment Program for Intracerebral Transplantation (CAPIT) protocol. Dyskinesia and emotional state were evaluated through the Abnormal Involuntary Movement Scale (AIMS) and the Hospital Anxiety and Depression Scale (HAD). QoL was assessed by means of the Parkinsons Disease Questionnaire Spanish version (PDQ‐39). Significant benefit was obtained in the motor manifestations and complications of disease, as well as in the functional state and mood (P < 0.001). Some QoL dimensions (mobility and activities of daily living) and the PDQ‐39 Summary Index (PDQ‐39SI) showed a significant improvement (P < 0.001). Benefit was modest (P < 0.05) for three other domains (emotional well‐being, stigma, bodily discomfort) and nil for the rest. There was no correlation between the change obtained in the QoL (PDQ‐39SI) and in the other variables. As measured by the PDQ‐39, STN‐DBS significantly improves important aspects of QoL in patients with advanced PD.

Sleep symptoms and polysomnographic architecture in advanced Parkinson's disease after chronic bilateral subthalamic stimulation

Objective: To evaluate the sleep symptoms and polysomnographic architecture in advanced Parkinsons disease after chronic bilateral subthalamic stimulation (STN-DBS). Methods: Sleep was studied in 11 patients (six women and five men; mean (SD) age 63.6 (7.8) years) who underwent STN-DBS. Subjective sleep evaluation was assessed by clinical sleep interview and the Pittsburgh sleep quality index (PSQI) questionnaire, and sleep architecture by polysomnography with audiovisual recording. Nocturnal mobility was evaluated. Results: Before surgery, eight patients rated their sleep quality as unsatisfactory; seven of these had a marked improvement after surgery, and the PSQI questionnaire showed significantly improved sleep quality. After surgery, polysomnography showed an increase in the longest period of uninterrupted sleep and a decrease in the arousal index. There was an increase in nocturnal mobility after surgery, but no change in REM sleep behaviour disorder. Conclusions: In advanced Parkinsons disease, chronic STN-DBS is associated with subjective improvement in sleep quality, probably through increased nocturnal mobility and reduction of sleep fragmentation.

Bilateral subthalamic stimulation monotherapy in advanced Parkinson's disease: Long-term follow-up of patients

Bilateral subthalamic nucleus stimulation (STN‐DBS) is used to improve parkinsonian symptoms and attenuate levodopa‐induced motor complications. In some patients, such clinical improvement allows antiparkinsonian medication (ApMed) withdrawal. We show the clinical outcome at the long‐term follow‐up of patients with advanced Parkinsons disease (PD) in which STN‐DBS was used in monotherapy, and compare the clinical results of patients without medication with those obtained in parkinsonian patients in which ApMed were reduced but could not be totally displaced after surgery. We analyzed clinical outcome of ten patients with PD in which all ApMed was withdrawn after bilateral subthalamic stimulation and 16 parkinsonian patients still taking antiparkinsonian medication after surgery. After 1.5 years, STN‐DBS monotherapy produced UPDRS motor scores similar to those observed in the on‐drug condition before surgery without the inconvenience of motor fluctuations and dyskinesias. No significant differences were seen in most of clinical outcome measures when comparing patients still taking ApMed with patients in STN‐DBS monotherapy but a few patients still taking ApMed presented mild dyskinesias and motor fluctuations and patients with STN‐DBS monotherapy did not. STN‐DBS is useful in the treatment of advanced PD and in some patients it is possible to maintain this therapy alone in the long term. The therapeutic effect of STN‐DBS on motor signs can be equipotent to that of levodopa with the additional benefit of avoiding motor fluctuations and dyskinesias.

Neuroendoscopic management of pineal region tumours

SummaryThe management of pineal tumours remains controversial. During 1994 we treated four consecutive adults (16–44 yrs) harbouring a pineal tumour with a neuroendoscopic procedure. All of them presented with hydrocephalus. Pre-operative workup included cranial computerized tomography (CT), craniospinal magnetic resonance imaging (MRI) and serum levels of biological tumour markers. The endoscopic procedure consisted of a third ventriculostomy followed by biopsy with a flexible, steerable neuroendoscope. Histological diagnosis was achieved in three patients who no longer required a shunt device. Recorded complications were: bleeding during ventriculostomy that prevented us from obtaining a good sample for biopsy, short-term memory loss that cleared over a twoweek period, and transient increase of pre-operative hemiparesis.Complications and morbidity are emphasized so as to be avoided with further technical experience. Neuroendoscopy affords a minimally invasive way of reaching three objectives by one-step surgery in the management of pineal region lesions: 1) CSF sample for analysis of tumour markers. 2) Treatment of hydrocephalus by third ventriculostomy. 3) Several biopsy specimens can be obtained identifying tumours which will require further open surgery or adjuvant radiation and/or chemotherapy.

Prospective comparative study on cost‐effectiveness of subthalamic stimulation and best medical treatment in advanced Parkinson's disease

This is an open, prospective, longitudinal study designed to compare two cohorts of patients with advanced Parkinsons disease during 1 year, one undergoing bilateral subthalamic stimulation (STN‐DBS) and the other receiving the best medical treatment (BMT), with respect to the clinical effects observed and the medical expenses produced. Assessments were done by using clinical measures and a generic health related quality of life scale. A questionnaire was used to collect direct healthcare resources. As a measure of cost‐effectiveness, we calculated life years gained adjusted by health‐related quality of life (QALY) and the incremental cost‐effectiveness ratio (ICER). Clinical and demographic variables of both groups were comparable at baseline. Total UPDRS scores improved from 50.5 ± 3.6 to 28.5 ± 3.8 in STN‐DBS patients and worsened from 44.3 ± 3.3 to 54.2 ± 4 in the control group. Pharmacological costs in the operated patients were 3,799 ± 940€, while in the BMT group the costs were 13,208 ± 4,966€. Other medical costs were 1,280 ± 720€ in the STN‐DBS group and 4,017 ± 2,962€ in BMT patients. Nondirect medical costs were 4,079 ± 1,289 in operated patients and 2,787 ± 1,209€ in the BMT group. Mean QALYs were 0.7611 ± 0.03 in STN‐DBS and 0.5401 ± 0.06 in BMT patients. In STN‐DBS patients, the ICER needed to obtain an improvement of one point in the total UPDRS score was of 239.8€ and the ICER/QALY was of 34,389€. Cost‐effectiveness parameters were mostly related to the degree of clinical improvement and the reduction of pharmacological costs after STN‐DBS. An ICER of 34,389€/QALY is within appropriate limits to consider subthalamic stimulation as an efficient therapy.

Comparative cognitive effects of bilateral subthalamic stimulation and subcutaneous continuous infusion of apomorphine in Parkinson's disease

Bilateral subthalamic deep brain stimulation (STN‐DBS) and continuous subcutaneous infusion of apomorphine (APM‐csi) can provide a comparable improvement on motor function in patients with advanced Parkinsons disease (PD), but the mechanisms by which both therapies exert their effects are different. We analyzed the cognitive effects of APM‐csi. We also compared neuropsychological effects induced by STN‐DBS and APM‐csi in advanced PD to ascertain the neuropsychological aspects relevant in determining the therapeutic procedure that is the most appropriate in a particular patient. We studied 9 patients treated with STN‐DBS and 7 patients with APM‐csi. Neuropsychological measures included Reys Auditory‐Verbal Learning, Stroop, Trail Making, phonetic verbal fluency, and Judgment of Line Orientation tests. In the APM‐csi group, significant changes were not observed in the neuropsychological tests performance. By contrast, in the STN‐DBS group, moderate worsening was found in phonetic verbal fluency and Stroop Naming scores that was partially reversible at long‐term follow‐up and did not have consequences on regular activities. Consequently, these findings could be interpreted as being not relevant in deciding the most suitable treatment in a given patient.

Cortical laminar necrosis related to prolonged focal status epilepticus

Cortical laminar necrosis (CLN) is radiologically defined as high intensity cortical lesions on T1 weighted MRI images following a gyral distribution. Histopathologically, CLN is characterised by pannecrosis of the cortex involving neurones, glial cells, and blood vessels. It has been reported to be associated with hypoxia, metabolic disturbances, drugs, and infections. We present two patients who developed CLN and permanent neurological deficits after prolonged and repeated focal status epilepticus. The possible mechanisms leading to CLN in these patients are discussed, together with the implications of prompt and aggressive treatment in similar cases.

Pallidotomy and quality of life in patients with parkinson's disease: An early study

The purpose of this study was to analyze the effect of stereotactic neurophysiologically guided pallidotomy on health‐related quality of life (QoL) of patients with Parkinsons disease (PD). Eleven patients with PD (seven men, four women; mean age, 57.2 years; mean duration of disease, 14 years) with motor complications refractory to medical therapy underwent unilateral pallidotomy. Clinical assessment was carried out a week before surgery and 4 months after the surgical procedure and was based on the Core Assessment Program for Intracerebral Transplantations protocol. QoL was measured by means of the PDQ‐39. A set of rating scales (Hoehn & Yahr, Unified Parkinsons Disease Rating Scale, Schwab and England, Northwestern University Disability Scale of Walking, Abnormal Involuntary Movement Scale), timed tests, and self‐evaluations of motor function and mood were applied. Improvement was found in dyskinesias (74%) and off‐period disability (42%). Cardinal motor signs improved significantly (30%–59%). Four dimensions of the PDQ‐39 (Mobility, ADL, Emotions, Bodily Pain) showed a significant improvement (p <0.01–0.001). The global effect on QoL, measured through the PDQ‐39 Summary Index (35.3%; 95% confidence interval: 15.60–54.97), was also significant (p <0.01) but unrelated to major clinical changes. Pallidotomy significantly improves QoL in patients with advanced PD. QoL measurement provides relevant information that is probably not attainable by clinical assessment.

Efficacy and safety of pallidal stimulation in primary dystonia: results of the Spanish multicentric study

Background: Dystonia is a complex clinical syndrome originated by a wide range of aetiologies. The diagnosis of dystonia is made after the evaluation of aetiological, phenomenological and genetic factors.1 Medications, except in patients with dopa-responsive dystonia, are of limited efficacy. Botulinum toxin injections are not applicable to patients with generalised dystonia, since many muscular groups contribute to disability.2 Clinical studies in children and adults with primary generalised dystonia (PGD) have reported beneficial effects of bilateral GPi deep brain stimulation (DBS) in both motor symptoms and disability produced by dystonia3 4 5 as well as a favourable impact of DBS in the health-related quality of life (HRQoL).6 Some clinical aspects of GPi stimulation in primary dystonia still remain controversial such as the influence of disease duration or age at onset in determining the postoperative clinical outcome. Results: The authors report the results of a multicentric study designed to assess the tolerability and clinical effects of bilateral pallidal DBS on motor impairment, functional disability, quality of life, pain and mood in patients with medically refractory primary generalised or segmental dystonia.

Validation of FDG‐PET/MRI coregistration in nonlesional refractory childhood epilepsy

Purpose:  To validate the use of 18F‐fluorodeoxyglucose–positron emission tomography/magnetic resonance imaging (FDG‐PET/MRI) coregistration for epileptogenic zone detection in children with MRI nonlesional refractory epilepsy and to assess its ability to guide a second interpretation of the MRI studies.