EFNS/MDS-ES/ENS [corrected] recommendations for the diagnosis of Parkinson's disease.

BACKGROUND: A Task Force was convened by the EFNS/MDS-ES Scientist Panel on Parkinson's disease (PD) and other movement disorders to systemically review relevant publications on the diagnosis of PD. METHODS: Following the EFNS instruction for the preparation of neurological diagnostic guidelines, recommendation levels have been generated for diagnostic criteria and investigations. RESULTS: For the clinical diagnosis, we recommend the use of the Queen Square Brain Bank criteria (Level B). Genetic testing for specific mutations is recommended on an individual basis (Level B), taking into account specific features (i.e. family history and age of onset). We recommend olfactory testing to differentiate PD from other parkinsonian disorders including recessive forms (Level A). Screening for pre-motor PD with olfactory testing requires additional tests due to limited specificity. Drug challenge tests are not recommended for the diagnosis in de novo parkinsonian patients. There is an insufficient evidence to support their role in the differential diagnosis between PD and other parkinsonian syndromes. We recommend an assessment of cognition and a screening for REM sleep behaviour disorder, psychotic manifestations and severe depression in the initial evaluation of suspected PD cases (Level A). Transcranial sonography is recommended for the differentiation of PD from atypical and secondary parkinsonian disorders (Level A), for the early diagnosis of PD and in the detection of subjects at risk for PD (Level A), although the technique is so far not universally used and requires some expertise. Because specificity of TCS for the development of PD is limited, TCS should be used in conjunction with other screening tests. Conventional magnetic resonance imaging and diffusion-weighted imaging at 1.5 T are recommended as neuroimaging tools that can support a diagnosis of multiple system atrophy (MSA) or progressive supranuclear palsy versus PD on the basis of regional atrophy and signal change as well as diffusivity patterns (Level A). DaTscan SPECT is registered in Europe and the United States for the differential diagnosis between degenerative parkinsonisms and essential tremor (Level A). More specifically, DaTscan is indicated in the presence of significant diagnostic uncertainty such as parkinsonism associated with neuroleptic exposure and atypical tremor manifestations such as isolated unilateral postural tremor. Studies of [(123) I]MIBG/SPECT cardiac uptake may be used to identify patients with PD versus controls and MSA patients (Level A). All other SPECT imaging studies do not fulfil registration standards and cannot be recommended for routine clinical use. At the moment, no conclusion can be drawn as to diagnostic efficacy of autonomic function tests, neurophysiological tests and positron emission tomography imaging in PD. CONCLUSIONS: The diagnosis of PD is still largely based on the correct identification of its clinical features. Selected investigations (genetic, olfactory, and neuroimaging studies) have an ancillary role in confirming the diagnosis, and some of them could be possibly used in the near future to identify subjects in a pre-symptomatic phase of the disease.

Microvascular decompression for trigeminal neuralgia: A retrospective analysis of long-term outcomes and prognostic factors.

INTRODUCTION: Microvascular decompression is considered to be the most effective and only etiological surgical treatment for classical trigeminal neuralgia, relieving the neurovascular compression found in up to 95% of cases. This study aims to report the long-term outcomes and to identify prognostic factors in a series of patients with trigeminal neuralgia treated by microvascular decompression. METHODS: A retrospective observational study of 152 consecutive patients operated by microvascular decompression with at least six months of follow-up. The surgical results, including pain relief according to the Barrow Neurological Institute pain scale, complications and the medical treatment during the follow-up period were reviewed. Binary regression analysis was performed to identify factors associated with a good long-term outcome. RESULTS: A total of 152 patients with a mean age of 60 years and a mean follow-up of 43 months were included. At the final follow-up visit, 83% of the patients had achieved significant relief of the pain and 63% could reduce the absolute drug doses by 50% or more. The most frequent complications were wound infection (4.5%) and CSF fistula (7%). Being over 70 years of age and having paroxysmal pain were associated with a long-term pain relief. CONCLUSIONS: Our results support the notion that microvascular decompression is an effective and safe therapy in patients with trigeminal neuralgia. A multidisciplinary approach with an early referral to a neurosurgical unit many be beneficial in patients who are refractory to pharmacological treatment.

Traumatic brain injury in the elderly: a significant phenomenon.

INTRODUCTION: Traumatic brain injury (TBI) in the elderly is becoming an increasingly frequent phenomenon. Studies have mainly analyzed the influence of age as a continuous variable and have not specifically looked at geriatric patients as a group. The aim of this study is to map the magnitude and characteristics of geriatric TBI and to identify factors contributing to their poorer outcome. MATERIAL AND METHODS: Based on the ICD-9 register of the University Hospitals Leuven demographic and clinical variables of TBI were analyzed (2002-2008). The influence of older age on physiological variables was assessed using the Brain-IT database. RESULTS: The elderly (aged ≥65 years) accounted for 38.2% of non-concussion TBI and 32.6% of ICU admissions, representing the largest age group. The elderly had a significantly lower ICP (median 10.06 mmHg versus median 14.52 mmHg; p = 0.048), but no difference in their measure of autoregulation (daily mABP/ICP correlation coefficient) compared with 20-35 year-olds. TBI was caused by a fall in 78.9% of elderly patients and 42.3% suffered a mass lesion. 72.1% had cardiovascular comorbidity. Complications did not differ from their younger counterparts. DISCUSSION: Geriatric TBI is a significant phenomenon. Poorer outcomes are not yet sufficiently explained by physiological monitoring data, but reduced vascular versatility is likely to contribute. More research is needed in order to develop specific management protocols.

Microvascular decompression for trigeminal neuralgia: A retrospective analysis of long-term outcomes and prognostic factors.

INTRODUCTION: Microvascular decompression is considered to be the most effective and only etiological surgical treatment for classical trigeminal neuralgia, relieving the neurovascular compression found in up to 95% of cases. This study aims to report the long-term outcomes and to identify prognostic factors in a series of patients with trigeminal neuralgia treated by microvascular decompression. METHODS: A retrospective observational study of 152 consecutive patients operated by microvascular decompression with at least six months of follow-up. The surgical results, including pain relief according to the Barrow Neurological Institute pain scale, complications and the medical treatment during the follow-up period were reviewed. Binary regression analysis was performed to identify factors associated with a good long-term outcome. RESULTS: A total of 152 patients with a mean age of 60 years and a mean follow-up of 43 months were included. At the final follow-up visit, 83% of the patients had achieved significant relief of the pain and 63% could reduce the absolute drug doses by 50% or more. The most frequent complications were wound infection (4.5%) and CSF fistula (7%). Being over 70 years of age and having paroxysmal pain were associated with a long-term pain relief. CONCLUSIONS: Our results support the notion that microvascular decompression is an effective and safe therapy in patients with trigeminal neuralgia. A multidisciplinary approach with an early referral to a neurosurgical unit many be beneficial in patients who are refractory to pharmacological treatment.

Continuous intestinal infusion of levodopa-carbidopa in patients with advanced Parkinson's disease in Spain: Subanalysis by autonomous community. / Uso de la infusión intestinal continua de levodopa-carbidopa en pacientes con enfermedad de Parkinson avanzada en España. Subanálisis por comunidades autónomas.

OBJECTIVES: To compare the characteristics of patients undergoing treatment with continuous intestinal infusion of levodopa-carbidopa (CIILC) for advanced Parkinson's disease and the data on the effectiveness and safety of CIILC in the different autonomous communities (AC) of Spain. METHODS: A retrospective, longitudinal, observational study was carried out into 177 patients from 11 CAs who underwent CIILC between January 2006 and December 2011. We analysed data on patients' clinical and demographic characteristics, variables related to effectiveness (changes in off time/on time with or without disabling dyskinesia; changes in Hoehn and Yahr scale and Unified Parkinson's Disease Rating Scale scores; non-motor symptoms; and Clinical Global Impression scale scores) and safety (adverse events), and the rate of CIILC discontinuation. RESULTS: Significant differences were observed between CAs for several baseline variables: duration of disease progression prior to CIILC onset, off time (34.9-59.7%) and on time (2.6-48.0%; with or without disabling dyskinesia), Hoehn and Yahr score during on time, Unified Parkinson's Disease Rating Scale-III score during both on and off time, presence of≥ 4 motor symptoms, and CIILC dose. Significant differences were observed during follow-up (> 24 months in 9 of the 11 CAs studied) for the percentage of off time and on time without disabling dyskinesia, adverse events frequency, and Clinical Global Impression scores. The rate of CIILC discontinuation was between 20-40% in 9 CAs (78 and 80% in remaining 2 CAs). CONCLUSIONS: This study reveals a marked variability between CAs in terms of patient selection and CIILC safety and effectiveness. These results may have been influenced by patients' baseline characteristics, the availability of multidisciplinary teams, and clinical experience.

Spanish expert consensus on the use of safinamide in Parkinson's disease.

Safinamide is a new add-on drug to levodopa for the treatment of Parkinson's disease (PD) with motor fluctuations. Due to the recent incorporation of safinamide into routine clinical practice, no post-authorisation phase IV studies on the safety of safinamide have been conducted to date. This study provides clinical management guidelines for safinamide based on the opinion of a group of experts in movement disorders. This project was developed in 2 phases: 16 local meetings in phase 1 and a national meeting in phase 2. The meetings followed a pre-established agenda. The present clinical practice guidelines are based on the main conclusions reached during the national meeting. The group concluded that safinamide is effective in reducing motor and non-motor fluctuations. PD patients with mild-to-moderate fluctuations benefit most from treatment, although the drug may also improve the clinical status of patients with advanced PD. The dose of other dopaminergic drugs may be reduced after introducing safinamide, which would contribute to reducing such adverse reactions as impulse control disorder. At doses higher than those usually prescribed, safinamide may also improve dyskinesia. The experts agreed that safinamide is well tolerated and causes few adverse reactions when compared with placebo.

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. 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. 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 dystonia as well as a favourable impact of DBS in the health-related quality of life (HRQoL). 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.

Acquired hepatocerebral degeneration: clinical characteristics and MRI findings.

OBJECTIVE: To determine the prevalence of acquired hepatocerebral degeneration (AHD), its clinical and neuroimaging characteristics and response to treatments. BACKGROUND: Acquired hepatocerebral degeneration is a chronic encephalopathy with predominant motor signs in the context of severe liver disease. Its clinical picture is not well defined, and its prevalence and risk factors are not well known. METHODS: Review of a database of 1000 patients with cirrhosis to identify cases of AHD. Clinical and neuroimaging data, follow-up and response to treatments, including liver transplantation, were recorded. RESULTS: Eight patients with AHD were identified. Its prevalence was 0.8% of patients with cirrhosis. The main risk factor for AHD was the presence of portosystemic shunts. Movement disorders, especially a combination of parkinsonism and cerebellar signs were observed in all patients. All AHD cases showed on MRI T1-weighted images hyperintensities in the globus pallidus, and 75% had extrapallidal involvement as well. Antiparkisonian drugs and treatments to prevent acute encephalopathies were ineffective. Three patients who underwent liver transplantation did not experience neurological improvement. Persistence of portosystemic shunts was demonstrated in two cases. CONCLUSIONS: Acquired hepatocerebral degeneration is a chronic encephalopathy which occurs in ∼1% of patients with liver cirrhosis and seems related to portosystemic shunts. Its is characterized by a combination of parkinsonism and cerebellar signs. MRI pallidal and extrapallidal lesions are seen in most patients, probably reflecting intracerebral deposits of manganese. Liver transplant did not improve the neurological signs in our patients, perhaps because of the persistence of portosystemic shunts.