Are 5-2-1 Delphi criteria and MANAGE-PD useful screening tools for general neurologists for qualification to device-aided therapies in advanced Parkinson's Disease?

AIM OF STUDY: We sought to compare MANAGE-PD and 5-2-1 Delphi criteria which are two commonly used and approved screening tools in Parkinson's Disease, in order to highlight their strengths and limitations. CLINICAL RATIONALE FOR STUDY: Timely intervention with device-aided therapies is vital as it enables improving motor symptoms, lowering the dosage and side-effects of dopaminergic treatment, and improving patients' and caregivers' quality of life. Various screening tools have been created to help clinicians find the best candidates for device-aided therapies (DAT) for advanced Parkinson's Disease. In this study, we aimed to compare the 5-2-1 Delphi criteria to MANAGE-PD to determine how they could be used specifically to maximise their potential. MATERIAL AND METHODS: All of the patients (260) included in this study were DAT-naive, > 18 years of age, diagnosed with Parkinson's Disease, and had been referred to the Department of Neurology for qualification for advanced therapies over a 4-year period (2019-2022). They were subjected to both 5-2-1 Delphi criteria and MANAGE-PD tools and divided into subgroups based on the results of the screening. The data of patients was then statistically analysed. RESULTS: In the study group, 51 patients (19.5%) met all three of the 5-2-1 criteria, and 123 (47.1%) patients were categorised as '3' in MANAGE-PD, meaning that they may benefit from DAT. Finally, at the local centre level, 64 (24.5%) patients were qualified for DAT. 22 (34.4%) patients who were qualified for DAT by a clinician did not meet the 5-2-1 criteria. CONCLUSIONS: The 5-2-1 scheme based on the data from this study was characterised by a 92.5% specificity level and 65.1% sensitivity level compared to 69.5% specificity and 98.4% sensitivity level of MANAGE-PD. CLINICAL IMPLICATIONS: We found that MANAGE-PD has a better screening potential of DAT admission than 5-2-1 criteria. While both tools are reliable and valuable in daily practice, our study suggests that some patients may be omitted when using only less complicated tools such as 5-2-1 during the assessment.

Reflexive and voluntary saccadic eye movements as biomarker of Huntington's Disease.

INTRODUCTION: Subtle abnormalities in the preclinical stage of Huntington's Disease (HD) can be detected using saccadic eye movement assessment reflecting disease progression. This study was aimed to evaluate abnormalities in saccade parameters in asymptomatic carriers and symptomatic HD patients at various stages of HD. MATERIAL AND METHODS: The study enrolled 104 participants, including 14 asymptomatic carriers of HTT mutations, 44 symptomatic HD patients, and 46 control subjects. HD severity was measured using the Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) and Total Functional Capacity Scale (TFC). The evaluation of rapid eye movements (reflexive saccades, anti-saccades, memory-guided saccades) was carried out using 'Saccadometer Research'. RESULTS: Measures of reflexive and volitional saccades did not differ between the asymptomatic carriers and controls. Significant latency prolongation and increased physiological variability of latency times, as well as higher error rates among HD patients, were found in all saccade tasks (p < 0.001) compared to the controls. Abnormalities in saccade parameters were more pronounced in the advanced stages of the disease. Latency of saccades and error rate of volitional saccades correlated with the UHDRS-TMS and TFC scores. CONCLUSIONS: The saccade parameters in asymptomatic HD carriers with a long time to disease development were similar to those in the control group. Saccade abnormalities appeared in symptomatic patients at the beginning of the disease, and correlated with HD severity.

Does Ultrasound Guidance Improve the Effectiveness of Neurotoxin Injections in Patients with Cervical Dystonia? (A Prospective, Partially-Blinded, Clinical Study).

AIM: The aim of this study was to evaluate the efficacy of ultrasound guidance (US) in the treatment of cervical dystonia (CD) with botulinum neurotoxin type A (BoNT-A) injections in comparison to anatomical landmarks (AL). To date, US is routinely used in many centers, but others deny its usefulness. MATERIALS AND METHODS: Thirty-five patients (12 males, 23 females) with a clinical diagnosis of CD were included in the study. Intramuscular administration of BoNT-A was performed using either US guidance, or with AL, in two separate therapeutic sessions. The efficacy of BoNT-A administration was assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Tsui modified scale, Craniocervical Dystonia Questionnaire (CDQ-24) and Clinical Global Impression-Improvement scale (CGI-I). Additionally, patients at therapeutic sessions were digitally recorded and evaluated by two blinded and independent raters. RESULTS: A significant decrease in total TWSTRS, severity subscale TWSTRS, Tsui score, and CDQ-24 was found in both the AL and US group; however, in the TWSTRS disability and pain subscales, a significant decrease was found only in the US group. Moreover, US guided treatment also resulted in a greater decrease in TWSTRS, Tsui score and CDQ-24 compared to anatomical landmarks use only. CONCLUSIONS: US guidance might be helpful in improving the results of BoNT-A injections in cervical dystonia, reducing associated pain and disability; however, more studies are needed to evaluate its clinical efficacy.

Efficacy and safety of therapeutic plasma exchange in stiff person syndrome.

The stiff person syndrome (SPS) is an extremely rare neurological disorder with primarily immune-mediated etiology. The cardinal symptoms are progressive, fluctuating axial/proximal limb muscle stiffness and spasms. The diagnosis is based on the clinical picture, electromyography examination and detection of antibodies to glutamic acid decarboxylase (anti-GAD). Adverse effects of medications might preclude its use or increase in dosing, therefore symptomatic and/or immunomodulatory medical therapy might be ineffective in acute exacerbation of the disease. We present a case of a 49-year-old female with exacerbation of SPS, in whom some standard pharmacotherapy could not be introduced (clonazepam, baclofen used in the past) and doses of existing standard medications could not be increased (diazepam, gabapentin, and levetiracetam) due to adverse effects. Moreover, a newly introduced medication (methylprednisolone) also led to a serious adverse effect (severe hyperglycemia). The patient underwent therapeutic plasma exchange (TPE) with good effect and no complications. We review the literature regarding the efficacy and safety profile of TPE in exacerbation of SPS unresponsive to medical therapy. The procedure seems to have a good safety profile as an adjunct therapy for exacerbation of SPS not responding to standard medical therapy in this patient population.

Fatal intracranial haemorrhage in a hypertensive patient with atrial fibrillation and coronavirus disease 2019.

We present a case of a fatal cerebral haemorrhage in an 82-year-old male patient with coronavirus disease 2019 (COVID-19), who was taking prophylactic oral anticoagulation because of atrial fibrillation (rivaroxaban 20 mg q.d. for two years). On admission, the patient was deeply comatose, mechanically ventilated, with tachycardia up to 150 bpm, high blood pressure >210/120 mmHg and a body temperature >39°C. A computed tomography scan of the head showed a large intracerebral haemorrhage located in the deep structures of the right hemisphere, with a mass effect and bleeding to the ventricles. Rivaroxaban was discontinued at admission. The patient tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), but he did not have typical symptoms of pneumonia. In the following days, the patient's neurological condition did not improve, and a fever of up to 40°C and abnormal coagulation parameters remained resistant to pharmacotherapy. The patient developed multi-system organ failure and died on day 8. Here, we review the recent literature and discuss the possible association of SARS-CoV-2-mediated endothelial injury and cardiovascular disorders with cerebrovascular complications. We postulate that anti-inflammatory treatment in COVID-19 and the stabilisation of endothelium functions can be particularly important in patients with pre-existing cardiovascular conditions.

Adequate Knowledge of Stroke Symptoms, Risk Factors, and Necessary Actions in the General Population of Southern Poland.

BACKGROUND AND OBJECTIVES: Stroke is the third most common cause of mortality in developed countries and the primary cause of neurological disability in adults. Recombinant tissue plasminogen activator administered intravenously within 4.5 h from the onset of symptoms constitutes a gold standard in the treatment of acute ischemic stroke. Prompt hospital admission is the prerequisite of effective thrombolysis. Therefore, stroke awareness in the general population is the key factor in timely recognition of the acute stroke victims and determines proper actions. Therefore, the purpose of this study was to determine the awareness of stroke in the general population of the Silesian voivodeship, the most populated region of Poland. We assessed also the "adequate knowledge of stroke", a combined measure of the optimal level of stroke awareness, as a prerequisite for effective stroke management, and aimed to identify most impacting factor for adequate stoke knowledge, to help shaping education strategies. MATERIALS AND METHODS: A proprietary anonymous questionnaire consisting of 15 items related to stroke was used in this research. A total of 1134 individuals were surveyed. Additionally to the knowledge of individual aspects of stroke, we assessed "adequate knowledge of stroke", which was combined measure of risk factors, symptoms, and actions in the case of acute stroke. RESULTS: The accurate definition of stroke was selected by 834 participants (73.5%). The vast majority of them indicated that a stroke is an emergency (92.8%) and medical assistance is required (97.5%). However, 42.4% of respondents did not know any specific symptom of stroke and only 38.6% participants were able to list two or more risk factors, which resulted in only 36.3% of individuals with adequate knowledge of stroke. Education duration, previous occurrence of stroke in relatives or friends, gender and place of residence were identified as independent predictors of adequate knowledge of stroke. CONCLUSIONS: 1. Knowledge of stroke in the population of southern Poland is low and may be considered insufficient to address the needs of timely management in the action chain. 2. Previous occurrence of stroke in the relatives or friends is the most impacting factor for adequate knowledge of stroke.