Percutaneous endoscopic gastrostomy in atypical parkinsonian syndromes: survival and aspiration outcomes from a retrospective international cohort.

Introduction Dysphagia frequently occurs in movement disorders, leading to malnutrition and aspiration. Percutaneous endoscopic gastrostomy (PEG) provides nutrition directly into the stomach, bypassing the dysfunctional swallow. However, PEG insertion is a complex decision, both clinically and ethically. Although PEG outcomes have been reported in other neurological disorders, there is limited research in atypical Parkinsonian syndromes such as Multiple Systems Atrophy (MSA), Progressive Supranuclear Palsy (PSP), and Corticobasal Degeneration (CBD). Insertion rates for these disorders remain variable, reflecting the paucity of research and lack of consistent guidelines. Basic mortality and morbidity data would help inform practice. To our knowledge, this is the first international study to assess whether PEG insertion improves survival and reduces aspiration pneumonia in atypical Parkinsonism. Method International retrospective study of 72 patients with MSA, PSP or CBD. Survival was recorded from reported onset of dysphagia to death. Secondary outcomes included hospital admission rate for aspiration pneumonia. Results Mean survival for the PEG group (n = 12) was 45.5 months (95% CI 34.6–56.4), compared to 20.8 months (95% CI 16.8–25.1) in the non-PEG group (n = 60). From the onset of dysphagia, the mean hospital admission rate for aspiration pneumonia was almost identical (PEG: 0.064/month, non-PEG: 0.062/month). However, within the PEG group, admissions for aspiration pneumonia increased following PEG insertion (0.054 to 0.145/month). Conclusion PEG insertion may improve survival in atypical Parkinsonism, though we found no evidence of reduced aspiration risk. Given the rarity of these conditions, international registries may help to determine the safety and efficacy of PEG use.

Discriminating among degenerative parkinsonisms using advanced (123)I-ioflupane SPECT analyses.

Atypical parkinsonism or atypical parkinsonian syndromes (APS) refer to a group of neurodegenerative disorders which mimic typical Parkinson's disease but poorly respond to levodopa treatment and deteriorate faster. APS are very rare and among them, progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD) are the three relatively better characterized entities. The prevalence estimates of PSP, MSA, or CBD are mostly <10/105, and the incidence estimates are around 1/105 person-year; both estimates remain stable over the past few decades. The age at onset is relatively young for MSA at late 50s, followed by CBD at early 60s, and then PSP at late 60s. The gender difference is not significant in APS, although slight female predominance in CBD has been reported in literature. Little is known about genetic and environmental risk factors for PSP, MSA, and CBD; although the COQ2 mutation has been identified as a genetic risk for MSA, familial cases are extremely rare. Survival after symptom onset is generally within 10 years, but cases with longer disease duration do exist. Respiratory infection remains the major cause of death for APS, but cardiac arrest should be particularly considered in MSA. In addition to disease rarity, the phenotype–pathology discrepancy in APS makes the epidemiological studies even more challenging. Including biomarkers in future diagnostic criteria and establishing disease registry for collecting sufficient number of APS cases may increase the likelihood of finding modifiable risk factors for prevention and intervention.

Comparison of dystonia between Parkinson's disease and atypical parkinsonism: The clinical usefulness of dystonia distribution and characteristics in the differential diagnosis of parkinsonism

Outcome of percutaneous endoscopic gastrostomy (PEG): comparison of two policies in a 4-year experience

Outcome of percutaneous endoscopic gastrostomy (PEG): comparison of two policies in a 4-year experience

Brain imaging with positron emission tomography using [(18) F]fluorodeoxyglucose (FDG-PET) and transcranial B-mode sonography (TCS) improves the differential diagnosis of parkinsonism. The diagnostic merits of these approaches in identifying and differentiating atypical parkinsonian syndromes (APS) are compared. Data were included from 36 patients with clinically suspected APS who underwent PET and TCS. FDG-PET scans were analyzed by visual assessment (including voxel-based statistical maps) of a priori defined disease-specific metabolic patterns. Sonographers achieved diagnoses according to pre-defined criteria for echogenicities of the substantia nigra and lenticular nucleus, and third ventricle diameter. Patients with APS were identified and allocated to the subgroups multiple system atrophy (MSA), progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD). After a median follow-up period of 9months, the final clinical diagnoses (reference standard) were Parkinson's disease, n=15; MSA, n=9; PSP, n=7; and CBD, n=5 (n=21 APS in total). Six patients (4 APS) showed an insufficient bone window for TCS. In the remaining 30 patients, sensitivity/specificity for diagnosing APS were 82%/100% and 82%/85% for FDG-PET and TCS, respectively. Diagnostic accuracies did not differ between FDG-PET (90%) and TCS (83%; P=0.69). Likewise, overall accuracy of subgroup classification (non-APS, MSA, PSP and CBD) did not differ between modalities (FDG-PET 87% and TCS 83%; P=1.00). FDG-PET and TCS show comparable accuracies for differential diagnosis of neurodegenerative parkinsonism. This preliminary study supports the use of TCS and warrants further prospective validation.

IntroductionPatients referred for percutaneous Endoscopic Gastrostomy (PEG) insertion often have multiple co-morbidities which do not improve with PEG feeding and lead to significant post-procedure complications. Pre-assessment of patients for PEG...

Parkinsonian disorders consist mostly of Parkinson’s disease (PD); other forms are relatively rare, although dementia with Lewy bodies (DLB) seems to be, after Alzheimer’s disease, one of the most common causes of dementia. Atypical parkinsonisms (other than idiopathic PD) include progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD) and DLB. In addition to parkinsonism symptoms the most characteristic features of these disorders, sometimes called parkinsonism-plus syndromes, are cerebellar ataxia in MSA, apraxia in CBD, gaze palsy in PSP or early in the course of dementia in DLB, and minimal, sometimes moderate (in MSA or PSP) or complete lack (in CBD) of response to L-dopa therapy. When appraising the condition, it is vital to separate it from those conditions that mimic the appearance of PD, but that are caused by another underlying pathology. Because of the lack of biological markers, differential diagnosis has to be based mostly on clinical findings. Nevertheless, neuroimaging studies, utilizing MRI, SPECT and PET examinations, seem to be helpful in establishing diagnosis. Nuclear medicine uses different tracers, specific for the dopamine transporting system or dopamine receptor ligands, which makes it possible to differentiate presynaptic dopaminergic system involvement (seen in PD) from pre- and postsynaptic involvement in MSA or PSP. Highly asymmetric tracer uptake reflects the asymmetry of cortical-subcortical atrophy and is characteristic for CBD. All of these conditions are accompanied by different psychiatric problems. In PD, depression is the most common, followed by dementia and psychosis at late stages. In PSP, subcortical dementia is typical with frontal behavior. Dementia is less frequent and late in CBD, but usually is the first or early symptom in DLB, followed by parkinsonian features. Functional neuroimaging may be useful for assessment of the preclinical period of PD (with potential early neuroprotective therapy), the rate of progression (evaluation of the influence of drugs and stereotaxic surgery on disease progression), the role of different structures in late complications (dyskinesias, fluctuations), and differential diagnosis of PD with essential tremor and atypical parkinsonian disorders. These techniques may also be helpful for investigation of pathogenesis and pathology underlying the non-motor symptoms of PD and atypical parkinsonisms as depression, dementia and psychosis. This review summarizes recent applications of SPECT, PET, but also MRI in the study of parkinsonian disorders, in terms of differential diagnosis and understanding concomitant neuropsychiatric phenomena.

Nutrition is a key part of the management of chronic liver disease and supplemental feeds are often required in young children with significant cholestasis. Whilst the insertion and use of...

Introduction The BSG guidelines 1 recommend that every endoscopy unit in an acute hospital setting should provide a basic percutaneous endoscopic gastrostomy (PEG) service, which is a part of the nutritional support team. The service should provide a framework for patient selection, pre-assessment and post-procedural care as well as working closely with the community-based services. Our trust recently appointed an accredited therapeutic endoscopist and gastroenterology nurse practitioner to run this service. Methods Retrospective analysis of all PEG insertions performed from Jan 2014 to Nov 2015 over a 23 month period. We looked at early-term (four weeks) and late term (eight weeks) mortality after PEG insertion. Results All patients were referred via a revised pathway proforma and examined by the team before the procedure to assess suitability. Further help and advice is offered to the community team upon discharge. 71 patients were referred for PEG insertion during the period of study. 29 (41%) were male, with a mean age 68 (range 29–87 years), 42 (59%) were female, with a mean age 69 (range 18–93 years). Indications for referrals included: 37 (52%) stroke related dysphagia, 15 (21%) head and neck cancers, 6 (8.5%) Huntington’s disease, 4 (5.6%) traumatic head injury, 3 (4.2%) learning disability, 2 (2.8%) cerebral palsy, 2 (2.8%) multiple sclerosis, 1 (1.4%) supranuclear palsy, 1 (1.4%) mitochondrial myopathy, 1 (1.4%) syringomyelia, 1 (1.4%) parkinsonism, 1 (1.4%) Korsakoff’s psychosis, and 1 (1.4%) myoclonic epilepsy with ragged-red fibres (MERRF) syrdrome. Patients with a formal diagnosis of dementia were not selected to undergo PEG insertion during this period. No short-term complications were reported post-insertion. Early-term mortality was 12.7% and late-term rose to 22.5%. Previous departmental audit in 2014 revealed early-term mortality of 20% and late-term mortality of 28%. Conclusion Meta-analysis has reported a 19% 30 day mortality following PEG insertion . 3 We have shown that in our centre, both early and late-term mortality has improved due to careful patient selection and a dedicated PEG service. Adherence to the BSG guidelines on PEG service has had a direct impact on improving mortality and clinical outcome. References 1 Westaby D, Young A, O’Toole P, Smith G, Sanders DS. The provision of a percutaneously placed enteral tube feeding service. Gut . 2010; 59 :1592–1605. doi:10.1136/gut.2009.204982 2 Johnston S, Tham T, Mason M. Death after PEG: results of the national confidential enquiry into patient outcome and death. Gastrointestinal Endoscopy 2008; 68 :223–7. 3 Mitchell SL, Tetroe JM. Survival after percutaneous endoscopic gastrostomy placement in older persons. J Gerontol A Biol Sci Med Sci 2000; 55 :M735–9. Disclosure of Interest None Declared

Corticobasal degeneration is an uncommon parkinsonian variant condition that is diagnosed mainly on clinical examination. To facilitate the differential diagnosis of this disorder, we used metabolic brain imaging to characterize a specific network that can be used to discriminate corticobasal degeneration from other atypical parkinsonian syndromes. Ten non-demented patients (eight females/two males; age 73.9 ± 5.7 years) underwent metabolic brain imaging with (18)F-fluorodeoxyglucose positron emission tomography for atypical parkinsonism. These individuals were diagnosed clinically with probable corticobasal degeneration. This diagnosis was confirmed in the three subjects who additionally underwent post-mortem examination. Ten age-matched healthy subjects (five females/five males; age 71.7 ± 6.7 years) served as controls for the imaging studies. Spatial covariance analysis was applied to scan data from the combined group to identify a significant corticobasal degeneration-related metabolic pattern that discriminated (P < 0.001) the patients from the healthy control group. This pattern was characterized by bilateral, asymmetric metabolic reductions involving frontal and parietal cortex, thalamus, and caudate nucleus. These pattern-related changes were greater in magnitude in the cerebral hemisphere opposite the more clinically affected body side. The presence of this corticobasal degeneration-related metabolic topography was confirmed in two independent testing sets of patient and control scans, with elevated pattern expression (P < 0.001) in both disease groups relative to corresponding normal values. We next determined whether prospectively computed expression values for this pattern accurately discriminated corticobasal degeneration from multiple system atrophy and progressive supranuclear palsy (the two most common atypical parkinsonian syndromes) on a single case basis. Based upon this measure, corticobasal degeneration was successfully distinguished from multiple system atrophy (P < 0.001) but not progressive supranuclear palsy, presumably because of the overlap (∼ 24%) that existed between the corticobasal degeneration- and the progressive supranuclear palsy-related metabolic topographies. Nonetheless, excellent discrimination between these disease entities was achieved by computing hemispheric asymmetry scores for the corticobasal degeneration-related pattern on a prospective single scan basis. Indeed, a logistic algorithm based on the asymmetry scores combined with separately computed expression values for a previously validated progressive supranuclear palsy-related pattern provided excellent specificity (corticobasal degeneration: 92.7%; progressive supranuclear palsy: 94.1%) in classifying 58 testing subjects. In conclusion, corticobasal degeneration is associated with a reproducible disease-related metabolic covariance pattern that may help to distinguish this disorder from other atypical parkinsonian syndromes.

Percutaneous endoscopic gastrostomy (PEG) can facilitate feeding and medication administration in dysphagic patients with Parkinson's disease and related disorders. Information on survival, institutionalization, and complications post PEG might inform feeding decisions. A total of 93 patients with Parkinson's disease and related disorders were identified by review of PEG registers and by searching the administrative databases in 2 large UK university hospitals (2005-2017); 83 case notes were available for retrospective review. Care processes and outcomes were assessed. The following were the diagnoses: 58 (70%) had Parkinson's disease, 10 (12%) had progressive supranuclear palsy, 5 (6%) had multiple system atrophy, 3 (4%) had dementia with Lewy bodies, and 7 (8%) had vascular parkinsonism. The median age was 78 years (interquartile range 72-82); 29 (35%) were women. Care processes included a future care plan in place prior to admission for 18 patients (22%), and PEG was placed during emergency admission in 68 patients (82%). The outcomes included median survival at 422 days; 30-day mortality rate was 6% (5 patients); and of 56 patients admitted from home, 18 (32%) were discharged to institutions (nursing or care homes). The most common complication was aspiration pneumonia for 18 (22%) of patients. Age, sex, diagnosis, admission type, comorbidities, and place of residence did not predict survival. Discharge to own home and follow-up by the home enteral feeding team were associated with longer survival. We recommend markers of advanced disease should prompt advanced care planning. Discussions about PEG feeding should include information about post-PEG survival, complications, and risk of institutionalization. Further research is needed on quality-of-life post PEG and ways to reduce aspiration pneumonia. All PEG patients should have nutrition team follow-up.

Although increasingly recognized, atypical parkinsonian syndromes remain challenging to diagnose and are underrecognized due to overlap with other parkinsonisms. This article provides a diagnostic approach to atypical parkinsonian syndromes, including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and dementia with Lewy bodies. The goal of this review is to aid the clinician in recognizing key clinical and pathologic features and to raise awareness of recent advances in diagnostics and treatment. Diagnostic criteria for atypical parkinsonian syndromes are evolving to encompass increasingly recognized heterogeneity in the presentation of these disorders and information gleamed from clinicopathologic correlations. PSP and CBD in particular now share similar pathologic clinical features and include a number of phenotypic variants. Pathologic diagnoses are increasingly used in clinical practice, and there is frequent reference now by clinicians to tauopathies, including PSP and CBD, and the synucleinopathies, which include MSA and dementia with Lewy bodies (as well as Parkinson disease). Research into biomarkers, including both tissue and imaging modalities and genetics, has the potential to increase disease recognition and make earlier diagnosis and treatment possible. Although novel therapeutics are being studied for atypical parkinsonian syndromes such as PSP, no new breakthrough interventions have emerged for the treatment of PSP, CBD, and MSA. Current therapeutic management for these disorders frequently uses a multidisciplinary team approach. The approach to atypical parkinsonian syndromes requires recognition of a constellation of overlapping but distinct clinical features that help with identifying and distinguishing them from Parkinson disease and other similar disorders.

Aim: We aimed to present our experience and findings in patients which in we applied percutaneous endoscopic gastrostomy (PEG) tube insertion because of the oral nutritional deficiency. Material andMethods: The data of 41 patients who had PEG tube insertion between 2014 and 2018 years in the General Surgery Clinic of Medicine Faculty of Ordu University were evaluated retrospectively. The indications, complications, mortality and short-term outcomes of the patients were analyzed. Results: 43 patients underwent gastroscopy due to insertion of PEG. In 41(95.3%) patients, PEG insertion was successful. In 2(4.7%) patients, peg insertion failed due to obesity. 16(39%) of the PEG patients were males and 25 (61%) were females. The mean age was 77.68 ± 13.9 (20-94) years. PEG indications were chronic neurological disease in 22 (53.6%) patients, cerebrovascular disease in 15 (36.6%) patients and malignancy in 4 (9.8%) patients. Minor complications in 11(26.8%) patients and major complications in 2 (4.9%) patients were observed. 10 (24.4%) of the complications were in the early period and 3 (7.3%) were in the late period. During the follow up, the PEG tube in 3 (7.3%) patients was pull out. No mortality due to PEG insertion was observed. During the mean follow-up period of 9.37 ± 7.8 months, 14 (34.1%) of the PEG-treated patients died due to their primary disease. Conclusions: PEG tube insertion is an easy method with the low rates of the complication and mortality in the patients with poor oral intake who have a functional gastrointestinal system. PEG is the first choice for long-term enteral nutrition in appropriate patients.