Ideggyogyaszati Szemle-Clinical Neuroscience最新文献

Spinal muscular atrophy (SMA) is an autosomal recessive disease leading to progressive muscle weakness and atrophy, in severe cases also affecting the bulbar and respiratory muscles.The clinical spectrum of the disease is extremely variable, in the most severe cases resulting in perinatal death, while at the least severe end of the spectrum causing some motor deficits in old age without the loss of ambulation. Spinal muscular atrophy care has changed dramatically in recent years due to the availability of new therapeutic options. 
The FDA approved nusinersen in 2016, this was followed by the approval of onasemnogene abeparvovec in 2019 and risdiplam in 2020. The EMA approved all three therapies a year later. Two of the threapies work at the pre-mRNA level, one at the DNA level. The clinical studies leading to the approval of the three drugs included patients of different ages and clinical conditions, and utilised partly different motor and functional scales. Therefore, direct comparison of these clinical studies is not possible. However, an increasing amount of real-world data contribute to the better understanding of the efficacy of the different therapies for patients of different ages and clinical conditions, in a real-world setting. Thus, the question may arise “Which is the best SMA therapy?”. This is an impossible question to answer. Indeed the question “Which therapy is the most suitable for a certain patient at a certain time?” is much more realistic. Here, we provide a brief overview of the objectively measurable results of the three therapies to date and an outlook into future therapeutic avenues. 

Background and purpose:

To examine the rehabilitation outcome of patients who have suffered a stroke and subsequently received priority rehabilitation in hospital inpatient care, with a focus on changes in functional status.

Methods:

Retrospective descriptive study. Functional impairment was measured at admission and discharge using the Barthel Index and the Functional Independence Measure scale. The subjects of the study were patients admitted to the Brain Injury Rehabilitation Unit of the National Institute of Medical Rehabilitation for inpatient rehabilitation with a stroke diagnosis between January 1 and December 31, 2018. 

Results:

Eighty-six stroke patients were treated in 2018 at the unit. Data were available for 82 patients (35 women and 47 men). Fifty-nine patients with acute stroke participated at primary rehabilitation and 23 patients with chronic stroke were involved in secondary rehabilitation. Ischaemic stroke was diagnosed in 39 cases and haemorrhagic stroke in 20. Patients were admitted for rehabilitation on the mean of 36th day (range: 8-112) after stroke and length of stay at rehabilitation unit was 84 days (14-232). The mean age of the patients was 56 years (range 22-88). Speech and language therapist treatment was necessary for 26 patients with aphasia, for 11 patients with dysarthria, and for 12 dysphagic patients. Neuropsychologic examination and training was necessary at 31 patients, severe neglect was found in 9 cases, ataxia was found in 14 cases. As a result of rehabilitation Barthel Index changed from 32 to 75, and the FIM scale from 63 to 97. At the end of the rehabilitation the majority (83%) of the stroke patients could be discharged to home, 64% became independent in daily living activities, and 73% of them regained the ability to walk.  

Conclusion:

The rehabilitation of stroke patients transferred from the acute wards who received priority rehabilitation was successful as a result of the rehabilitation activities carried out in the ward as part of a multidisciplinary team approach. The successful rehabilitation of patients with above average functional impairment from the acute ward is attributed to almost 40 years of experience and well-organised multidis­ciplinary teamwork.

Introduction – Methanol is a colorless and highly toxic liquid similar to ethanol in odor and taste. Methanol intake can lead to severe metabolic acidosis, loss of vision, permanent neurological damage, and death.

Case report – We report a case of a 19-year-old female patient who had no known disease history. A large portion of her back was covered in spirits for back pain and kept on for 2 days. Cranial magnetic resonance imaging (MRI) on the 5th day showed hyperintense pathologic signal changes in the T2 sequence without contrast enhancement in the bilateral frontal corticalsubcortical regions and basal ganglia. Following neurological examination of the patient, she was found to have bradymimia, marked resting tremor, bradykinesia, and dystonia on the right upper and lower extremities. Our case is important because it is the only case where parkinsonism developed as a result of acute transdermal methanol intoxication.

Drug therapy with non-opioid, opioid and adjuvant drugs is the mainstay of cancer pain relief. The three step analgesic ladder, published by WHO in 1986 Geneva, is useful for oncologists and general practitioners.
The first step is giving minor analgesics and adjuvant drugs; the second is giving minor analgesics, weak opioid and adjuvant drugs; the third step is giving minor analgesics, strong opioids and adjuvant drugs. For those patients who have severe pain it is a waste of time to prescribe the drugs of the first and second step, we suggest to start immediately with strong opioids!
Analgetic drugs should be given by the clock: the next dose is given before the effect of previous one has fully worn off. In this way it is possible to relieve pain continuously.
In selected cases the analgetic effect of nerve blockade is much better than that of the drug treatment. With successful blockades the patient can stop taking analgetic drugs for a long period of time and the blockades can be performed repeatedly. We briefly summarise the three most effective neuroblockades.

Background and purpose – To analyze the utility of median nerve (MN) to ulnar nerve (UN) comparative parameters on the diagnosis of carpal tunnel syndrome (CTS) in diabetic patients with distal symmetrical sensorimotor polyneuropathy (DSMPNP).

Methods – Patients who were referred to our electroneuromyography laboratory within the last two years were included. We compared the diagnostic accuracy values of traditional MN conduction parameters, and the MN-to-UN comparative tests on electrodiagnosis of CTS between the patients with DSMPNP involving the nerves of upper and lower extremities (UEI-positive group), and the ones without the involvement of upper extremities (UEI-negative group).

Results – There were 64 upper extremities in the UEI-positive group and 70 patients in the UEI-negative group. The most accurate traditional parameter was MN distal motor latency (DML) with a diagnostic accuracy of 70.2% whereas the most accurate comparative technique was the second lumbricalinterosseous DML difference (2L-INT DMLD) with an accuracy of 81.3%. (p=0.03). In addition, when compared diagnostic accuracy values of MN parameters with their corresponding comparative parameters in the UEI-positive group which carries the major diagnostic challenges for detecting co-morbid CTS, MN to UN minimum F wave latency (mFWL) difference, SNAP amplitude ratio on the ring finger (RF), and 2L-INT DMLD had higher accuracy values than MN mFWL, MN SNAP amplitude on RF, and MN DML on lumbrical muscle, respectively (p<0.05 for all comparisons).

Conclusion – MN to UN comparative studies have high accuracy values in electrodiagnosis of CTS in DSMPNP. In particular, 2L-INT DMLD could be helpful to overcome the diagnostic difficulty in the presence of UEI as an additional conduction technique.

Stroke is nowadays one of the most prevalent diseases worldwide causing devastating impairments and negative consequences for survivors. It is a main cause of adult onset disability and it can have a negative impact on psychological health, cognitive function and quality of life. Post-stroke rehabilitation may reduce long-term disability, and in recent years several innovations have emerged to improve recovery. Decades of research suggest that mindfulness-based interventions support a greater capacity to live with chronic medical conditions and contribute to lowering stress levels. Previous works report positive results amoung stroke survivors, improvements in mood, mental fatigue and in some degree in cognitive and physical functioning, plus represent a promising option in secon­dary prevention. Since the early 2000s, numerous clinical studies have investigated the efficacy of mindfulness-based interventions in post-stroke rehabilitation. In this paper the main results of the relevant international research is reviewed and also, the main modalities of the mindfulness-based interventions are presented. Our primary goal is to evaluate the results in order to draw attention to the importance of rehabilitation of patients with stroke and hopefully the theoretical and practical knowledge of the review will contribute to development effective and secure protocols in future research. Mindfulness-based techniques can become clinically valuable complementary therapeutic interventions in post-stroke rehabilitation. More research in this area is warranted: to evaluate these specific practices and their suitability; using randomized, controlled, follow up designs, rigorous methods, and different treatment settings; expanding outcomes to include physiological, health care use, and health-related outcomes; exploring mediating factors; and discerning dose effects and optimal frequency and length of practice. 

Background and purpose:

Despite the decrease in transplant-related mortality, patients who receive hematopoietic stem-cell transplants often suffer from short-and long-term morbidities, poorer quality of life, and psychosocial functioning deficits. Several studies have compared the quality of life and affective symptoms of patients after undergoing autologous and allogeneic hematopoietic stem-cell transplants. Some studies have reported similar or greater quality of life impairments in allogeneic hematopoietic stem-cell recipients, but the findings have been inconsistent. Our purpose was to examine the influence of the type of hematopoietic stem-cell transplantation on the quality of life and affective symptoms of patients.

Methods:

The study sample comprised 121 patients with various hematological diseases who underwent hematopoietic stem-cell transplantation at St. István and St. László Hospitals, Budapest. The study had a cross-sectional design. Quality of life was evaluated using the Hungarian version of the Functional Assessment of Cancer Therapy–Bone Marrow Transplant scale (FACT-BMT). Anxiety and depressive symptoms were assessed using Spielberger’s State and Trait Anxiety Inventory (STAI) and the Beck Dep­ression Inventory (BDI), respectively. Basic sociodemographic and clinical variables were also recorded. Comparisons between autologous and allogeneic recipients were analyzed using a t-test when the variables were normally distributed and a Mann–Whitney U test otherwise. A stepwise multiple linear regression analysis was performed to identify the risk factors that contributed to the quality of life and the affective symptoms in each group.

Results:

Quality of life (p=0.83) and affective symptoms (pBDI=0.24; pSSTAI=0.63) were similar between the autologous and allogeneic transplant groups. The BDI scores of allogeneic transplant patients indicated mild depression, but their STAI scores were similar to those of the general population. Allogeneic transplant patients with symptoms of graft-versus-host disease (GVHD) experienced more severe clinical conditions (p=0.01), poorer functional status (p<0.01) and received more immunosuppressive treatment (p<0.01) than those without graft versus host disease. Patients suffering from graft versus host disease experienced more severe depression (p=0.01), and constant anxiety (p=0.03) than those without graft versus host disease. Quality of life was affected by depressive and anxiety symptoms and psychiatric comorbidity in both the alloge­neic and autologous groups.

Conclusion:

Graft versus host disease-related severe somatic complaints seemed to influence the allogeneic transplant patients’ quality of life by inducing depressive and anxiety symptoms.

Background and purpose – Interdiscipli­ nary researches demonstrate that patients’ fears and anxieties about surgery play a key role in the success of postoperative recovery. Psychoeducation is a professional information transfer method that aims to increase patients’ knowledge about their dis­ ease, and how to cope with it, and to emo­ tionally process the problems associated with the disease. If patients feel competent in their own healing process after surgery, they will experience less pain and become self­sufficient sooner, thereby the number of nursing days spent in the clinic reduces.
Methods – In this study the effect of psycho-education before spinal surgery on the use of postoperative analgetics was investigated. Results – The drug consumption of the study group who had been previously administered patient education is significantly reduced in comparison the control group.
Conclusion – Cooperation of a psychologist in surgical therapy promotes early recovery of patients in physical and mental well­being and reduces the costs of rehabilitation as well.

Background and purpose:

Cortical atrophy and white matter changes are common findings on magnetic resonance imaging among elderly. Several visual scales have been proposed to evaluate these changes using neuroimaging. We have recently proposed a scale (Modified Visual Magnetic Resonance Rating Scale) recently which allows us to evaluate atrophy, white matter hyperintensities, basal ganglia and infratentorial infarcts together. Our aim in this study was to evaluate the interrater reliability of magnetic resonance visual assessment using this scale between two neurologists and a radiologist. 

Methods:

Randomly selected 30 patients in different ages who underwent brain magnetic resonance imaging between January 2014 and March 2015 were included. Axial T1, coronal T2, and axial FLAIR sequences were visually scored by two neurologists and one radiologist separately. Sulcal, ventricular and medial temporal lobe atrophy, periventricular and subcortical white matter hyperintensities, basal ganglia and infratentorial infarcts were graded according to our scale. The interrater reliability and internal consistency analysis were evaluated by using intraclass correlation coefficient and Cronbach’s alpha tests. 

Results:

The interrater agreements vary between good to excellent. The interrater correlations are moderate to excellent. Interrater correlations were excellent between two neurologists, especially on ventricular atrophy, medial temporal atrophy, basal ganglia infarcts, infratentorial infarcts. When assessing ventricular atrophy, interrater correlations between individual raters were higher than sulcal atrophy. We found good correlations between neurologists and radiologist, and excellent correlations between the two neurologists for medial temporal atrophy. We found excellent interrater correlations between neurologists and radiologist for white matter hyperintensities.

Conclusion:

Our scale is a reliable tool assessing both atrophy and white matter hyperintensities with a good interrater reliability. Ventricular atrophy seems to be a more reliable marker than sulcal atrophy when assessing the atrophy on neuroimaging of a patient with memory decline. We think that the total score of the scale will also guide us in clinical practice.

Background and purpose:

Cervical dys­tonia (CD) is the most common form of focal dystonias, where the identification of the involved muscles, the determination of optimal botulinum neurotoxin A (BoNT-A) dose per muscle injection, and precise tar­ge­ting may be challenging. The aim of the current study is to compare local centre data with international data, enabling the iden­tification of population and me­tho­do­­lo­gical factors behind the differences, there­by further improvement of the care of Hun­ga­rian patients with CD.

Methods:

The data of all consecutive CD patients, who were injected with BoNT-A at the botulinum neurotoxin outpatient clinic at the Department of Neurology, University of Szeged between 11 August and 21 Sep­tember 2021, were retrospectively col­lected and analysed in a cross-sectional manner. The frequency of the involved muscles, determined by the application of the collum-caput (COL-CAP) concept, and the parameters for the BoNT-A formulations, injected via ultrasound (US)-guidance, were calculated and compared with available international data.

Results:

In the current study, 58 patients (19 males and 39 females) were involved with mean age of 58.4 (± SD 13.6, range 24-81) years. The most common subtype was torticaput (29.3%). Tremor affected 24.1% of patients. The most injected muscles were trapezius (56.9% of all cases), followed by the levator scapulae (51.7%), splenius capitis (48.3%), sternocleidomastoid (32.8%), and semispinalis capitis (22.4%). The injected mean doses per patient were 117 ± SD 38.5 (range: 50-180) units for onaBoNT-A, 118 ± SD 29.8 (range: 80-180) units for incoBoNT-A, and 405 ± SD 162 (range: 100-750 units) for aboBoNT-A.

Conclusion:

Although there were several similarities between the results of the current and the multicentre studies, all were carried out using the COL-CAP concept and US-guided BoNT-A injections, authors should pay attention to better distinction of torti-forms and the more frequent injection of especially the obliquus capitis inferior, mainly in cases with no-no tremor.