Extensive validation study of the Parkinson's Disease Composite Scale.

BACKGROUND AND PURPOSE: A composite instrument able to rapidly and reliably assess the most relevant motor and non-motor afflictions suffered by Parkinson's disease (PD) patients in a real world clinic setting is an unmet need. The recently validated PD Composite Scale (PDCS) was designed to fulfil this gap as a quick, comprehensive PD assessment. The objective of this study was extensive evaluation of the PDCS's clinimetric properties using a large international sample. METHODS: This was a cross-sectional study in which the PDCS, the Movement Disorder Society Unified Parkinson's Disease Rating Scale and the Clinical Impression of Severity Index for PD were applied. Basic clinimetric attributes of the PDCS were analysed. RESULTS: In total, 776 PD patients were included. The PDCS total score showed negligible floor and ceiling effects. Three factors (54.5% of the variance) were identified: factor 1 included motor impairment, fluctuations and disability; factor 2, non-motor symptoms; and factor 3, tremor and complications of therapy. Cronbach's alpha was from 0.66 to 0.79. Inter-rater reliability showed weighted kappa values from 0.79 to 0.98 for items and intraclass correlation coefficient values from 0.95 (Disability) to 0.99 (Motor and total score). The Bland-Altmann method, however, showed irregular concordance. PDCS standard error of measurement and convergent validity with equivalent constructs of other measures were satisfactory (≥0.70). PDCS scores significantly differed by Hoehn and Yahr stage. CONCLUSION: Overall, in line with previous findings, the PDCS is a feasible, acceptable, valid, reliable and precise instrument for quickly and comprehensively assessing PD patients.

Can early clinical parameters predict post-traumatic pituitary dysfunction in severe traumatic brain injury?

BACKGROUND: Post-traumatic hypopituitarism is a major complication after severe head trauma. The aim of our study was to evaluate the possible role of early clinical parameters in the development of endocrine deficits. METHODS: Data on endocrine function, on-admission clinical-, laboratory-, and ICU-monitored parameters were available in 63 patients of the surviving 86 severe head injury patients (post-resuscitation GCS under 8) treated at one neurosurgical center during a 10-year period. RESULTS: Hypopituitarism was diagnosed in 68.3 % of the patients. The most frequently affected pituitary axis was the growth hormone (GH): GH deficiency or insufficiency was present in 50.8 %. Central hypogonadism affected 23.8 % of male patients; hypothyroidism and secondary adrenal failure were found in 22.2 and 9.5 % of the investigated population, respectively. Early onset (within 1 year of brain injury) hypopituitarism was found in 24 patients. No connection was found between the development of hypopituitarism and any of the clinical parameters assessed on-admission or at ICU. Significant correlations were found between early endocrine dysfunctions and surgical intervention (OR: 4.64) and the diagnosis of subdural hematoma (OR: 12). In our population, after road traffic accidents, the development of late-onset hypopituitarism was less prevalent (OR: 0.22). CONCLUSIONS: Since our results do not indicate any reliable predictive parameter for the development of endocrine dysfunction in a cohort of patients with severe traumatic brain injury, regular endocrine screening of this specific patient population seems obligatory.

Transradial and transulnar access for iliac artery interventions using sheathless guiding systems: A feasibility study.

PURPOSE: Our aim was to evaluate the acute success and complication rates of the transradial and transulnar access for iliac artery stenting using sheathless guiding systems. METHODS: Clinical and angiographic data from 156 consecutive patients with symptomatic iliac artery stenosis who were treated with transradial or transulnar access were evaluated. All patients underwent Duplex ultrasound before and after the intervention. The primary endpoints were the procedural success rate, major adverse events, and access site complication rates. The secondary endpoints were the angiographic result of the iliac artery intervention, fluoroscopy time, X-ray dose, procedure length, crossover rate to another puncture site and hospitalization duration. The impact of the learning curve was also investigated, along with right or left radial access. RESULTS: The indication for the intervention was intermittent claudication in 109 patients (69.9%), critical limb ischemia in 44 (28.2%) subjects and acute limb ischemia in three individuals (1.9%). Technical success was achieved in 155 patients (99.4%), with a crossover rate of 3.8%. Radial and ulnar artery access was used in 151 (96.8%) and 7 (4.5%) patients, respectively. The Ankle-brachial index increased from 0.69 [0.65-0.72] to 0.91 [0.88-0.95] as a result of the procedures (P < 0.001). The cumulative incidence of major adverse events was 3.8% at the 2-month follow-up (0% in patients with intermittent claudication and 13.8% in patients with critical limb ischemia). Radial artery access site complications were encountered in eight patients (5.1%). We documented decreased X-ray doses (1742.0 [783.9-2701] vs. 1435 [991.1-1879] vs. 692.8 [275.3-1110] Gy cm-2 P < 0.05) over time; however, the fluoroscopy time, procedure time, and contrast consumption were not significantly different. Left hand access was not associated with significantly better results than right radial artery access. CONCLUSIONS: Iliac artery stenting can be safely and effectively performed using radial or ulnar artery access and sheathless guiding catheters, with acceptable complication rates and high levels of technical success. The physician learning curve plays an important role in decreasing the X-ray dose. © 2016 The Authors. Catheterization and Cardiovascular Interventions Published by Wiley Periodicals, Inc.

'Wind-up' in Parkinson's disease: A functional magnetic resonance imaging study.

BACKGROUND: Parkinson's disease (PD) is a neurodegenerative disorder mainly marked by selective degeneration of dopaminergic neurons that leads to disabling motor and cognitive impairment. This condition is less widely appreciated as a disease associated with a substantial variety of pain syndromes, although the prevalence of pain is relatively high. Repeated painful stimulation of peripheral nerves can cause pain 'wind-up' if the frequency of the stimulation is adequate and specifically stimulates the afferent C-fibres. We presumed that in case of PD, pain or pain severeness might be frequently caused by the aggravation of the 'wind-up' phenomenon due to any central or peripheral lesions or functional alterations. METHODS: To test for this hypothesis, we compared three groups (patients with left- and right-dominant PD and control subjects) using functional magnetic resonance imaging and thermally induced pain. RESULTS: Patient showed higher average 'wind-up' scores, compared to the healthy subjects, with lower values on the more affected sides compared to the less affected ones. In group level comparisons, patients had higher activation during 'wind-up' compared to control subjects in two main areas; these were the posterior division of cingulate gyrus and the precuneus cortex. In case of patients, further analyses showed that applied heat pain on the less affected side elicited higher activation in the supramarginal and postcentral gyri. CONCLUSIONS: These differences may arise from the deficiency in the efferent information, as well as the alterations in the central processing. It is highly likely that both processes contribute to this phenomenon simultaneously.

Cortical involvement during myotonia in myotonic dystrophy: an fMRI study.

OBJECTIVE: Myotonic dystrophy type 1 (DM1) is a common adulthood muscular dystrophy, characterized by muscle wasting, myotonia, and multisystemic manifestations. The phenomenon of involuntary muscle contraction during myotonia offers a unique possibility of investigating brain motor functions. This study explores cortical involvement during grip myotonia in DM1. MATERIALS AND METHODS: Sixteen DM1 patients were enrolled in the study. Eight patients had apparent grip myotonia, while eight patients did not (control subjects). All patients underwent functional MRI grip task examination twice: prior a warm-up procedure (myotonia was elicited in patients with apparent grip myotonia) and after a warm-up procedure (myotonia was attenuated in patients with apparent grip myotonia). No myotonia was elicited during either examination in patients without apparent grip myotonia. Cerebral blood oxygen level-dependent (BOLD) signals were compared both between groups with and without apparent myotonia, and between pre- and post-warm-up sessions. RESULTS: Significantly higher BOLD signal was found during myotonia phase in patients with apparent grip myotonia compared to corresponding non-myotonia phase of patients without apparent grip myotonia in the supplementary motor area and in the dorsal anterior cingulate cortex. Significant differences in BOLD signal levels of very similar pattern were detected between prewarm-up session myotonia phase and post-warm-up session myotonia absent phase in the group of patients with apparent grip myotonia. CONCLUSION: We showed that myotonia is related to cortical function in high-order motor control areas. This cortical involvement is most likely to represent action of inhibitory circuits intending motor termination.

Valproate treatment normalizes EEG functional connectivity in successfully treated idiopathic generalized epilepsy patients.

AIM: To investigate the effect of chronic VPA treatment of EEG functional connectivity in successfully treated idiopathic generalized epilepsy (IGE) patients. PATIENTS AND METHODS: 19-channel waking, resting-state EEG records of 26 IGE patients were analyzed before treatment (IGE) and after the 90th day of treatment (VPA), in seizure-free condition. Three minutes of artifact-free EEG background activity (without epileptiform potentials) was analyzed for each patient in both conditions. A group of 26 age-matched healthy normative control persons (NC) was analyzed in the same way. All the EEG samples were processed to LORETA (Low Resolution Electromagnetic Tomography) to localize multiple distributed sources of EEG activity. Current source density time series were generated for 33 regions of interest (ROI) in each hemisphere for four frequency bands. Pearson correlation coefficients (R) were computed between all ROIs in each hemisphere, for four bands across the investigated samples. R values corresponded to intrahemispheric, cortico-cortical functional EEG connectivity (EEGfC). Group and condition differences were analyzed by statistical parametric network method. MAIN RESULTS: p<0.05, corrected for multiple comparisons: (1) The untreated IGE group showed increased EEGfC in the delta and theta bands, and decreased EEGfC in the alpha band (as compared to the NC group); (2) VPA treatment normalized EEGfC in the delta, theta and alpha bands; and (3) degree of normalization depended on frequency band and cortical region. CONCLUSIONS: VPA treatment normalizes EEGfC in IGE patients.

Sensitivity and specificity of Addenbrooke's Cognitive Examination, Mattis Dementia Rating Scale, Frontal Assessment Battery and Mini Mental State Examination for diagnosing dementia in Parkinson's disease.

INTRODUCTION: Among the non-motor features of Parkinson's disease (PD), cognitive impairment is one of the most troublesome problems. Highly sensitive and specific screening instruments for detecting dementia in PD (PDD) are required in the clinical practice. METHODS: In our study we evaluated the sensitivity and specificity of different neuropsychological tests (Addenbrooke's Cognitive Examination, ACE; Frontal Assessment Battery, FAB and Mattis Dementia Rating Scale, MDRS) in 73 Parkinson's disease patients without depression. By receiver operating characteristic curve analysis, these screening instruments were tested against the recently established clinical diagnostic criteria of PDD. RESULTS: Best cut-off score for ACE to identify PDD was 80 points (sensitivity = 74.0%, specificity = 78.1%). For FAB the most optimal cut-off value was 12 points (sensitivity = 66.3%, specificity = 72.2%); whereas for MDRS it was 125 points (sensitivity = 89.8%, specificity = 98.3%). Among the examined test batteries, MDRS had the best clinicometric profile for detecting PDD. CONCLUSION: Although the types of applied screening instruments might differ from movement disorder clinic to clinic within a country, determination of the most specific and sensitive test for the given population remains to be an important task. Our results demonstrated that the specificity and sensitivity of MDRS was better than those of ACE, FAB and MMSE in Hungary. However, further studies with larger sample size and more uniform criteria for participation are required to determine the most suitable screening instrument for cognitive impairment.

Lateralisation of non-metric rhythm.

There are contradictory results on lateralisation and localisation of rhythm processing. Our aim was to test whether there is a hemispheric dissociation of metric and non-metric rhythm processing. We created a non-metric rhythm stimulus without a sense of metre and we measured brain activities during passive rhythm perception. A total of 11 healthy, right-handed, native female Hungarian speakers aged 21.3 ± 1.1 were investigated by functional magnetic resonance imaging (fMRI) using a 3T MR scanner. The experimental acoustic stimulus consisted of comprehensive sentences transformed to Morse code, which represent a non-metric rhythm with irregular perceptual accent structure. Activations were found in the right hemisphere, in the posterior parts of the right-sided superior and middle temporal gyri and temporal pole as well as in the orbital part of the right inferior frontal gyrus. Additional activation appeared in the left-sided superior temporal region. Our study suggests that non-metric rhythm with irregular perceptual accents structure is confined to the right hemisphere. Furthermore, a right-lateralised fronto-temporal network extracts the continuously altering temporal structure of the non-metric rhythm.

[Validation of intensive medicine degree in the United Kingdom]. / Convalidación del título de Medicina Intensiva en el Reino Unido.

The specialty of Intensive Care Medicine was established over twenty years ago in Spain as part of the MIR (Resident training) system. The European Union allows for free circulation of its workers and the multilateral recognition of their university degrees as well as their postgraduate training. Unfortunately, our specialty is excluded from such a privilege. This limits our European rights and hinders the mobility of the intensivists trained in our country. The main objective of this article is to provide a practical guide on how to obtain recognition of the Spanish Certificate of Training (MIR system) in Intensive Care Medicine in the United Kingdom (UK). This review has been done by several Spanish intensivists with vast professional experience in the UK.

Periictal heart rate variability analysis suggests long-term postictal autonomic disturbance in epilepsy.

BACKGROUND: One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure. METHODS: We included 31 periictal video-EEG-ECG recordings of 31 patients with epilepsy who had consecutively undergone pre-surgical evaluation. Nine generalized tonic-clonic (GTCS), 15 complex partial, and seven simple motor seizures were included. HRV was evaluated by analyzing 5-min-long ECG epochs, sampling from baseline, direct preictal, early-postictal (<15 min after the seizure), and late-postictal (5-6 h after the seizure) periods. RESULTS: The heart rate was elevated immediately after the seizures, but 5-6 h postictally returned to the baseline level. Time-domain components of HRV decreased after the seizure and this decrease lasted for 5-6 h. Low-frequency power decreased in the early-postictal phase and high-frequency power of HRV dropped in the late-postictal phase. GTCS had an impact on short-term but not on long-term postictal HRV decrease. CONCLUSIONS: We found decreased HRV immediately after the seizures, which lasted at least 5-6 h postictally, indicating a long-term postictal disturbance of the autonomous nervous system. GTCS were accompanied by a more decreased HRV than other seizures. Our results may have relevance in explaining pathomechanism of SUDEP.

Methylprednisolone therapy for short-term prevention of SUNCT syndrome.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache period. Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention. We report rapidly suppressed headache attacks with orally administered methylprednisolone in eight headache periods of three patients with idiopathic, episodic SUNCT syndrome. The remission was maintained until the period was over in all cases. Although the mechanism of methylprednisolone action is unclear, it is probably based on the anti-inflammatory effects of the drug.

Optical platelet aggregometry does not appear useful as a means of assessing the risk of recurrent vascular events in aspirin-treated patients.

OBJECTIVES: To investigate whether the results of optical platelet aggregometry indicate the risk of recurrent ischemic events. MATERIALS AND METHODS: Cerebro- and cardiovascular patients taking aspirin for at least 30 days were studied retrospectively. Ischemic vascular events occurring prior to testing and the presence of vascular risk factors were recorded. RESULTS: 241 subjects were included. Among the 78 patients (32.4%) who displayed recurrent vascular episodes, the age (62.5 +/- 10.6 vs. 58.4 +/- 11.6, P = 0.009) and the proportion of hypertensives (80.8% vs. 68.1%, P = 0.040) were significantly higher when compared with the participants who exhibited single events. The degree of platelet aggregation did not differ significantly between the patients with and those without recurrent episodes. Logistic regression analysis identified only age (OR 1.033, 95% CI 1.008-1.058, P = 0.010), and not aggregation values, as a risk condition for recurrent vascular episodes. CONCLUSIONS: Results of optical platelet aggregometry were not indicative of the risk of recurrent vascular events. The role of conventional risk factors appeared to be more important.

1H-MRS experiences after bilateral DBS of the STN in Parkinson's disease.

UNLABELLED: The objective of this study was to evaluate the changes in the concentrations of certain brain metabolites in 13 patients with Parkinson's disease before and after bilateral subthalamic nucleus (STN DBS). The N-acetylaspartate (NAA)/choline (Chol), NAA/creatine (Cr), Chol/Cr ratios were determined by single voxel Proton magnetic resonance spectroscopy ((1)H-MRS) studies on 1.0T unit using short TE stimulated echo acquisition mode (STEAM) sequence. Spectra were obtained from the right and left globus pallidus, and left fronto-basal cortex. The patients were also assessed according to the UPDRS part III, in the "medication-on and off" conditions. CONCLUSIONS: after STN DBS cortical NAA/Cho, NAA/Cr ratios increased significantly, which were highly correlated with the significant improvements of the UPDRS scores.

Peri-ictal vegetative symptoms in temporal lobe epilepsy.

We investigated peri-ictal vegetative symptoms (PIVS) in 141 patients with adult temporal lobe epilepsy (TLE) and assessed frequency, gender effect, and lateralizing value of peri-ictal autonomic signs. We recorded abdominal auras in 62%, goosebumps in 3%, hypersalivation in 12%, spitting in 1%, cold shivering in 3%, urinary urge in 3%, water drinking in 7%, postictal nose wiping (PNW) in 44%, and postictal coughing in 16%. At least one vegetative sign appeared in 86% of the patients. The presence of PIVS did not have a significant lateralizing value. PNW occurred in 52% of women and in 33% of men, whereas any PIVS was present in 93% of women and 77% of men. In summary, contradictory to previous studies, the presence of PIVS has no lateralizing value, which may be linked to a low frequency of occurrence of PIVS. PIVS, especially PNW, occurred more frequently in women, supporting the gender differences in epilepsy.

Patients' ability to react before complex partial seizures.

OBJECTIVE: The purpose of the study described here was to investigate the pathophysiology of patients' ability to react during the conscious (aura) phase of complex partial seizures (CPS) originating from the temporal lobe. METHODS: We reviewed video recordings of CPS experienced by 130 adult patients who had undergone epilepsy surgery for intractable medial temporal lobe epilepsy. All patients were instructed to push the alarm button when they felt an aura. We defined the preictal reactivity as the ability to push the alarm button before the complex partial (unconscious) phase of seizures. RESULTS: Seventy-seven patients (59%) pushed the alarm button before seizures. Patients with preictal reactivity were significantly younger, more often had lateralized EEG seizure patterns, and had a better postoperative outcome. Patients who did not push the alarm button had secondarily generalized seizures more often. CONCLUSIONS: Ability to react before CPS is associated with a circumscribed region involved at seizure onset and spread, and with a seizure-free postoperative outcome.

Unilateral hand automatisms in temporal lobe epilepsy.

OBJECTIVES: To analyse the lateralising value of unilateral manual automatism (UMA), its relation to contralateral dystonia and the hand by which the UMA was performed. METHODS: In this retrospective study, we reviewed video recordings of 141 patients (mean age 34.1+/-10) who had consecutively undergone presurgical evaluations with ictal video-EEG recordings and high-resolution MRI, had had epilepsy surgery due to intractable medial temporal lobe epilepsy with complex partial seizures due to unilateral medial temporal lobe lesions. The video recordings were prospectively reviewed by one of the authors blinded to patient's clinical data except the diagnosis of medial temporal lobe epilepsy. Altogether 310 archived seizures were analysed. RESULTS: Hand automatisms occurred in 86.5% of patients. UMA occurred in 53% of patients. If UMA was accompanied by contralateral hand dystonia, it had a high lateralising value to the ipsilateral epileptic focus (EF), it was ipsilateral in 85% of patients. Conversely, if UMA occurred without contralateral dystonia, it had only a limited lateralising value because it was ipsilateral to the EF in only 63% of patients. However, we found that left-sided UMA without dystonia had a high lateralising value to the left hemisphere (ipsilateral to the EF in 82%), while right-sided UMA without dystonia has practically no lateralising value. CONCLUSIONS: UMA with contralateral dystonia has a high lateralising value to the ipsilateral hemisphere. Left-sided UMA without contralateral dystonia has a lateralising value to the left hemisphere. Right-sided UMA without contralateral dystonia has no lateralising value.

Fatal case of community-acquired pneumonia caused by Legionella longbeachae in a patient with systemic lupus erythematosus.

Reported here is a rare case of atypical pneumonia due to a non- pneumophila Legionella sp. that occurred in a young patient with systemic lupus erythematosus. In spite of aggressive treatment, the patient died 24 h following admission to the intensive care unit. Legionella longbeachae was cultured from respiratory tract specimens and identified to the genus level by PCR and to the species level by an immunofluorescence test. Since most current laboratory tests for Legionella spp., including urinary antigen and serology, cannot detect infections caused by non- pneumophila Legionella spp., culture on legionella-selective media should be strongly considered when diagnosing immunosuppressed patients with pneumonia.