Impulsivity in Parkinson's disease patients treated with subthalamic nucleus deep brain stimulation-An exploratory study.

BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is a recognized treatment in Parkinson's disease (PD). Knowledge is still limited regarding the possible impact of STN-DBS on personality traits and the personality characteristics of PD patients who undergo surgery. METHODS: To assess personality traits in relation to STN-DBS we did an ancillary protocol as part of a prospective randomized study that compared two surgical strategies. Patients were assessed with the Temperament and Character Inventory (TCI), the Urgency, Premeditation, Perseverance and Sensation Seeking impulse behavior scale, the Eysenck Personality Questionnaire (EPQ) and the Toronto Alexithymia Scale preoperatively and after one year of STN-DBS. EPQ and TCI baseline scores were compared with mean scores of healthy reference populations. RESULTS: After 12-months of STN-DBS, there was a significant decline in Persistence compared to baseline. Preoperatively, the STN-DBS patients had significantly lower Persistence and Self-Transcendence scores, and significantly higher scores on Novelty-Seeking, Self-Directedness, Cooperativeness and on Social Conformity than referenced populations. No difference was found in Neuroticism or Harm-Avoidance scores. The baseline prevalence of alexithymia was low and at 1-year follow-up there was no significant change in alexithymia scores. CONCLUSIONS: We found a higher baseline level of impulsivity in PD patients who underwent STN-DBS. After one year of STN-DBS, our results indicated that the treatment may affect the patients' personality by increasing certain aspects of impulsivity. There was no effect on alexithymia. The preoperative personality profile of PD patients might influence the outcome of STN-DBS.

Dystonia--new advances in classification, genetics, pathophysiology and treatment.

Dystonia is a heterogeneous movement disorder and has been defined as 'a syndrome of sustained muscle contractions, frequently causing twisted and repetitive movements, or abnormal postures'. The classification of dystonia has developed along with increasing knowledge, and different schemes have been suggested, including age at onset, body distribution, and etiology as the main differentiating factors. A revised definition and a new classification of dystonia have now been proposed by a group of leading dystonia experts and will be referred here. The discovery of the first two gene mutations causing primary generalized dystonia (DYT1-TOR1A and DYT6-THAP1) has facilitated studies on pathogenesis and pathophysiology of primary dystonias, by comparing neurophysiology between manifesting and non-manifesting carriers, and by studying the molecular biology of the mutant gene products. During recent years, several other gene mutations causing primary dystonia, dystonia-plus, and paroxysmal dystonia disorders have been discovered. Only during the last year, by the use of whole-exome sequencing techniques, mutations in three different genes in families with predominantly cervical dystonia were found, which may lead to improved insight into the pathogenesis also of the more frequent focal dystonias. Botulinum neurotoxin (BoNT) and deep brain stimulation (DBS) have revolutionized the symptomatic treatment for dystonia during the last two decades and continue to be refined to improve efficacy and expand their indications. Unfortunately, no progress has been made in the oral medication of dystonia. Current and future new insights into pathogenetic and pathophysiological mechanisms of dystonia will hopefully lead to improvement also in this area soon.

Good long-term efficacy of pallidal stimulation in cervical dystonia: a prospective, observer-blinded study.

BACKGROUND AND PURPOSE: Deep brain stimulation of the internal globus pallidus (GPi-DBS) is established as an effective treatment of primary generalised dystonia in controlled studies. In cervical dystonia (CD), only one previous study has reported observer-blinded outcome assessment of long-term GPi-DBS, with 1-year follow-up. METHODS: In this prospective, single-centre study, eight patients with CD (7 women:1 man, 4 focal:4 segmental) treated with bilateral GPi-DBS for median (range) 30 (12-48) months, were evaluated by the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS; Severity, Disability and Pain scores), the Short-Form Health Survey-36 (SF-36), and the Becks Depression Index in an open design. In addition, a blinded rater assessed the TWSTRS Severity score from videos obtained preoperatively and at the last follow-up. RESULTS: In the blinded evaluation, median (range) TWSTRS Severity score improved from 25 (19-30) to 8 (4-23) (P = 0.028), thus a 70% (23-82) score reduction. In the open evaluation, median Severity score improvement at the last follow-up was 73%, representing a significant further improvement from 50% at 6 months. The Disability and Pain scores improved by median 91% and 92%, respectively, and the SF-36 subdomain scores improved significantly. A reversible right hemiparesis and aphasia occured in one patient 4 days postoperatively, because of reversible oedema around the left electrode. No other serious adverse effects and no permanent morbidity were observed. CONCLUSIONS: This single-blinded study shows good long-term efficacy of GPi-DBS in CD patients and supports using this treatment in those who have insufficient response to medical treatment.

Pallidal deep brain stimulation is effective, and improves quality of life in primary segmental and generalized dystonia.

BACKGROUND: Dystonia is one of the most prevalent movement disorders, and may lead to abnormal postures, pain, significant disability and social isolation if not well treated. In widespread segmental or generalized dystonia efficient treatment options were lacking until the recent introduction of deep brain stimulation of the internal globus pallidus (pallidal DBS). METHODS: The first case series and single-blinded controlled study showed promising results regarding the effect of pallidal DBS on dystonic movements, pain and disability, but the impact of this treatment on health-related quality of life (HRQoL) remained uncertain. Then, as part of the first randomized, and sham stimulation-controlled trial performed by the DBS for Dystonia Study Group, HRQoL was evaluated using the Short Form-36 Health Survey (SF-36) at baseline, after the 3 months sham-controlled phase, and after 6 months of continuous pallidal DBS, in 40 patients with severe primary segmental or generalized dystonia. RESULTS: The 3-month sham-stimulation controlled phase resulted in significant improvement of dystonic movements, pain and disability in the active stimulation group, and four of the eight SF-36 domain scores also improved significantly compared with the sham-stimulated group. After 6 months of active stimulation in all patients, significant improvement in all eight SF-36 domains was observed, with comparable improvement in the segmental and generalized dystonia groups. CONCLUSION: Convincing evidence has been obtained that pallidal DBS effectively reduces dystonia-related symptoms, and markedly improves HRQoL in patients suffering from otherwise intractable, primary segmental or generalized dystonia, thus providing new opportunities for this patient group.

Determinants and status of quality of life after long-term botulinum toxin therapy for cervical dystonia.

The aim of this study was to assess health-related quality of life (HRQoL), using the Short Form Health Survey-36 (SF-36), in 70 cervical dystonia (CD) patients after long-term botulinum toxin (BTX) treatment (median 5.5 years), and to identify factors determining reduced HRQoL. We used combined patient-and physician-based measures to assess both CD severity [Toronto Western Spasmodic Torticollis Rating Scale, (TWSTRS)] and effect of long-term BTX treatment, and the Hospital Anxiety and Depression Scale (HAD) and General Health Questionnaire-30 to assess psychological distress. Mean SF-36 domain scores of the CD patients were reduced by <1 SD compared with age- and gender-matched population samples. High TWSTRS total scores and high HAD-depression (HAD-D) scores were the main factors associated with reduced scores in the physical and mental SF-36 domains, respectively. Patients evaluated to have a 'good effect' of long-term BTX treatment (n = 47), had significantly lower median TWSTRS total score, and a 3x lower frequency of high HAD-D scores, than those evaluated to an 'unsatisfactory effect' (n = 23). In conclusion, most CD patients enjoy a good HRQoL after long-term BTX therapy. Reduced HRQoL was associated with more severe disease and/or depressive symptoms.

The course of cervical dystonia and patient satisfaction with long-term botulinum toxin A treatment.

In 78 patients with idiopathic cervical dystonia (CD), we studied the course of the disease and the patients' satisfaction with long-term botulinum toxin A (BTX) treatment (median 5.5 years, range 1.5-10). On a seven-point scale ranging from excellent to worsening, the effect of treatment was scored as excellent or good by 52% of patients and moderate by 33%. The independent scores of the treating neurologists were excellent or good in 65% and moderate in 27%, respectively, and correlated well with the patients' scores. The 'Global Burden of Disease', as expressed on Visual Analog Scales (VAS, 0-10) before and at evaluation of treatment, was reduced by a median of 4 in individual patients. By combining these outcome measures, 67% of the patients were characterized as having a good effect, and 33% an unsatisfactory effect. This outcome (good or unsatisfactory effect) was independent of the severity of head deviation or complexity pattern of CD prior to treatment, the delay from onset to start of BTX treatment, or the number of treatments. The complexity pattern remained stable during treatment in 64% of the patients, became less complex in 19%, whereas 17% of the patients developed more complex patterns.

[Usefulness of SPECT and MRI in the diagnosis of atypical parkinsonism]. / Diagnostisk nytte av SPECT- og MR-undersøkelse ved atypisk parkinsonisme.

BACKGROUND: Clinico-pathological studies have shown that only three out of four patients with parkinsonism have idiopathic Parkinson's disease. In patients with so-called Parkinson plus syndrome, the degeneration in the brain is more widespread and the variety of neurological signs greater than in Parkinson's disease. The differentiation of these syndromes from Parkinson's disease can be difficult. Single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI) can be of value in the differential diagnosis of parkinsonism. MATERIAL AND METHODS: We present three patients with atypical parkinsonism in whom MRI and SPECT with beta-CIT and epidepride was performed in addition to the clinical evaluation. RESULTS: The three patients all had a rapidly developing symmetric akinetic-rigid syndrome that responded poorly to levodopa. MRI showed findings regarded as typical for multiple system atrophy in two patients, but only nonspecific findings in the third patient. SPECT with beta-CIT showed a pronounced bilateral and relatively symmetric reduction in the striatal dopaminergic activity in all patients. SPECT with epidepride showed a clearly reduced striatal D2-receptor binding bilaterally in only one of the patients. INTERPRETATION: In patients with atypical parkinsonism, MRI and SPECT with beta-CIT and epidepride can give valuable support to the clinical diagnosis of a Parkinson plus syndrome.

[Intracranial venous thrombosis]. / Intrakranial venetrombose.

Cerebral venous thrombosis has traditionally been considered to be a severe and rare condition. Our knowledge of this condition has increased, however, along with the development of better radiological methods for visualization of the brain and its vasculature. Cerebral angiography, computed tomography (CT) and, in particular, magnetic resonance imaging (MRI) have made it easier to diagnose this condition earlier. It has thus been discovered that cerebral venous thrombosis occurs more frequently than previously supposed and shows a wide variation of the clinical spectrum. We describe a series of eight patients with cerebral venous thrombosis who were admitted to the Neurosurgical Department, Haukeland Hospital, over a period of three years. The presentation shows the great variation in the clinical and radiological picture of this disease and the consequent diagnostic and therapeutic challenges. We conclude that this diagnosis should be considered in patients with cerebral symptoms of uncertain cause, and that MRI is an important tool in diagnosis.

Increased serum creatine kinase BB and neuron specific enolase following head injury indicates brain damage.

The aim of this study was to examine whether an increase in the serum concentrations of the two brain enzymes creatine kinase BB (CK-BB) and neuron specific enolase (NSE) can be demonstrated in patients with acute head injury and whether such an increase reflects release from damaged brain tissue. In 60 patients who had suffered minor to severe head injury, serial blood samples were drawn during the first hours after impact, and CK-BB and NSE were measured by radioimmunoassay. Computed tomography (CT) was also performed shortly after admission to hospital, and was repeated 1-3 days later in selected patients. Increased serum concentrations of both CK-BB and NSE were found in 88% of the patients with moderate to severe head injury (group 1, n = 18) and in 23% of the patients with minor head injury (group 2, n = 42), whereas CT showed contusion in only 41% and 2% of the group 1 and 2 patients, respectively. The following findings suggest that the enzymes had been released from brain tissue: 1) The maximum concentrations of CK-BB and NSE correlated with the severity of injury as assessed clinically and with the volume of contusion as estimated from CT (r = 0.79 with CK-BB, r = 0.72 with NSE). 2) The maximum concentrations of CK-BB and NSE were closely correlated (r = 0.87).(ABSTRACT TRUNCATED AT 250 WORDS)