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Clinical Neuroscience

MARCH 30, 2021


[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

Clinical Neuroscience

MARCH 05, 1951

[The issue of myelitis necroticans]


[The authors have reviewed two recent cases of myelitis necroticans, addressing questions of pathogenesis and aetiology and whether it is a distinct disease. It is concluded that the clinical presentation is variable and that the newer cases rarely agree completely with the original description. Histopathologically, necrosis of certain sections of the spinal cord is present in all cases, but, unlike in Foix and Alajouanine, it does not extend to the central part of the spinal cord and may also start higher up outside the lumbar sections. In many cases, the extra- and intraspinal vascular flow originally described is absent or modified and less marked. The vascular flow cannot be considered to play a primary role in the pathogenesis, but is a phenomenon parallel to the destruction of the spinal cord parenchyma. In the pathology, the infectious-inflammatory nature, given the absence of true tissue inflammation even in acute cases, cannot be confirmed and the spinal cord involvement is probably the result of various toxic agents. Thus, from a pathological point of view, it is not possible to speak of a single disease and the name "necrotising myelopathy" is justified instead of "myelitis necroticans".]

Clinical Neuroscience

MARCH 30, 2019

[Prognostic factors of surgically treated spinal meningeomas and long-term surgical outcomes ]


[Introduction, the aim of article - The spinal meningeomas are one of the most common types of spinal tumors. In the treatment of spinal meningeomas, the surgical removal is the gold standard method. There are many factors that have impacts on surgical outcomes such as age, preoperative neurological condition, the extent of resection and histological grade. The aim of our article is to analyze surgical experiences, prognostic features and long-term surgical outcomes of spinal meningeomas. Patients and methods - Retrospective database of surgically treated patients with spinal meningeomas between 2008 and 2016 was made in the National Institute of Clinical Neuroscience, Budapest, Hungary. Demographic data, preoperative neurological symptoms, radiological discrepancies, pathological results (histology, grade), types of treatments and postoperative results were examined. All of our patients were followed clinically and radiologically in the postoperative courses. Results - All of the 153 patients were surgically treated. We have examined 112 women and 41 men. The average age of the patients was 65.5 years. In 98.7% of the cases, the postoperative control examinations (postoperative 6th week) showed a significant improvement in sensorial and motorial functions. The neurological improvements were evident right after the surgeries. In 2 cases (1.30%), no changes were observed in the preoperative symptoms. Recurrence was noticed in 4 cases (2.61%). Conclusion - According to our results, the spinal meningeomas can be sufficiently treated with early diagnosis and total surgical removal. Most of the patients become asymptomatic and the rate of recurrence is quite low.]

Clinical Neuroscience

MARCH 11, 1954

[Experimental data on the mechanism of the tetanus inhibitory reflex]


[1. Our various tetanus interventions: During max. flexion, extension, trunk flexion, and slight compression of the chest, one or more muscle groups are always stretched. Prolongation of muscle or tendon is an adequate stimulus of proprioceptive receptors. Proprioceptive stimuli trigger the tetanus reflex. 2. Tetanus inhibition lasts only as long as the muscle is kept stretched. 3. During inhibition, the animal is capable of appropriate reflex movement. 4. The anti-tetanus reflex, when properly affected, also develops in the spinal cord of an incised animal during transection. 5. Clonus spasms caused by Cardiazol cannot be reduced by effects based on muscle or muscle elongation. 6. If the spinal cord is cut at the height of one of the dorsal segments and then the animal is poisoned with strychnine, the known effect on the pharmacy develops weaker and later during the incision.]

Clinical Neuroscience

JUNE 09, 1950

[A case of multiple myeloma with spinal cord compression and complete rib disappearance]


[A case report of multiple myeloma with spinal cord compression confirmed by clinical, biopsy, histological, radiological and chemical examination. Interesting features of the case are complete disappearance of the VI rib, metastasis of the contact fly segment, remission with surgery and RTG irradiation, no neurological symptoms for one and a half years from the onset of symptoms and one year from surgery.]

Lege Artis Medicinae

MAY 01, 2000

[The cervical spine in rheumatoid arthritis]

KORDA Judit, VERES Róbert

[The neck is the third most common site of involvement after the hands and feet in rheumatoid arthritis (RA). Deformities of the cervical spine can appear in the early stage of the disease, but most often they are seen in patients with rheumatoid arthritis with more than ten years of duration. Progression of the deformity is unpredictable, but follow up for five to ten years has shown the worsening of the instability in 16-80% of the patients. Many patients with rheumatoid disease of the cervical spine remain asymptomatic for years, but they are at risk of a range of neurological complications and even sudden death from me dullary compression. Neurological abnormalities may be subtle and difficult to establish in the presence of deforming arthritis. Once myelopathy develops, it is usually rapidly progressive. The primary goal in the management of the cervical spine is to prevent the onset of irreversible neurological deficit. Patients should have regular physical examinations to avoid the masking of subtle changes of myelopathy by severe peripheral joint disease. Use of the different measurements, especially posterior atlantodental interval and subaxial canal diameter measured on plain lateral cervical radiograph is a reliable screening tool to identify high risk patients who require further evaluation. The primary technical objective of surgery is stabilization of the diseased spinal segments and relief of spinal cord compression via reduction of subluxation or decompression. Complications are not uncommon, but tend to occur less frequently, and neurological recovery is most favorable when severe cord compression is not present preoperatively. ]

Clinical Neuroscience

JANUARY 30, 2020

The effects of the level of spinal cord injury on life satisfaction and disability

GULSAH Karatas, NESLIHAN Metli, ELIF Yalcin, RAMAZAN Gündüz, FATIH Karatas, MÜFIT Akyuz

Introduction - Spinal Cord Injury (SCI) may often lead to significant disability in affected individuals and reduce life satisfaction. Herein we aimed to investigate the effects of the level of injury on disability and life satisfaction as well as the relation between life satisfaction and disability. Methods - Patients with at least one-year history of SCI were included. Demographic-clinical data of patients were recorded. The Craig Handicap Assessment and Reporting Technique-Short Form (CHART-SF) was used for quantifying the degree of patients’ disability. Life satisfaction was assessed by the Satisfaction with Life Scale (SWLS). Results - Of the 76 patients, 21 (27.6%) were tetraplegic and 55 (72.4%) were paraplegic. SWLS was found to be similar in tetraplegic vs. paraplegic patients (P=0.59), whereas CHART parameters such as physical independence, mobility, occupation, and total CHART value were significantly higher in paraplegic patients (P=0.04, P=0.04, P=0.001 and P=0.01, respectively). Social integration was found similarly high in both groups. There was a positive correlation between elapsed time after the injury and CHART physical independence, occupation and the level of economic sufficiency (P<0.01, P<0.01, P=0.01). Excluding the economic sufficiency (P=0.02), there was not any other association between the rest of CHART parameters and SWLS. Conclusions - According to our findings, although the level of injury seems to be influential on disability, it seems to have no significant effect on life satisfaction. Since the only thing that positively affects life satisfaction is economic sufficiency, more emphasis should be placed on regulations that increase the return to work in patients.

Clinical Neuroscience

JANUARY 30, 2020

[Current questions of multiple sclerosis: the secunder progressive form of the disease]


[Recent data suggest that long-term worsening is common in relapsing-remitting multiple sclerosis patients and is largely independent of relapses or new lesion formation on brain MRI. The current definition of secunder progressive multiple sclerosis is worsening of disability independent of relapses over at least 6-month interval. Early focal inflammatory disease activity and spinal cord lesion are predictors of very-long term disease outcomes in relapse - onset multiple sclerosis. The potential of PET imaging to visualize hidden inflammation in MS brain in vivo is an important contribution for better understanding the progression of the disease. Therefore, PET imaging is a promising tool in detecting the conversion from relapsing remitting multiple sclerosis to secunder progressive form of multiple sclerosis. Furthermore, neuro-axonal damage is the pathological substrate of permanent disability in different neurological disorders including multiple sclerosis. The neurofilament proteins have promise in this context because their levels rise upon neuro-axonal damage not only in the cerebrospinal fluid but also in blood. Patients with increased serum levels of neurofilament at baseline, independent of other clinical and MRI variables, experience significantly more brain and spinal cord volume loss over 2 years and 5 years of follow-up. The kynurenine-pathway abnormalities may be associated with the swich from early-mild stage multiple sclerosis to debilitating progressive forms of the disease. Analysis of these metabolites in serum may have application as multiple sclerosis disease biomarkers. Free radical action has been suggested as a causal factor in the illness. Increased free radical production and consumption of the scavenger molecules were found during the active phase of the disease. Based on the clinical findings (EXPAND Study) and pathomechanism of the disease siponimod is approved by the US Food and Drug Administration for the treatment of relapsing remitting forms of multiple sclerosis, to include secunder progressive multiple sclerosis with active disease, relapsing-remitting multiple sclerosis and clinically isolated syndrome.]

Clinical Neuroscience

MAY 30, 2021

Comparison of direct costs of percutaneous full-endoscopic interlaminar lumbar discectomy and microdiscectomy: Results from Turkey

ÜNSAL Ünlü Ülkün, ŞENTÜRK Salim

Microdiscectomy (MD) is a stan­dard technique for the surgical treatment of lumbar disc herniation (LDH). Uniportal percutaneous full-endoscopic in­terlaminar lumbar discectomy (PELD) is another surgical op­tion that has become popular owing to reports of shorter hos­pitalization and earlier functional recovery. There are very few articles analyzing the total costs of these two techniques. The purpose of this study was to compare total hospital costs among microdiscectomy (MD) and uniportal percutaneous full-endoscopic interlaminar lumbar discectomy (PELD). Forty patients aged between 22-70 years who underwent PELD or MD with different anesthesia techniques were divided into four groups: (i) PELD-local anesthesia (PELD-Local) (n=10), (ii) PELD-general anesthesia (PELD-General) (n=10), (iii) MD-spinal anesthesia (MD-Spinal) (n=10), (iv) MD-general anesthesia (MD-General) (n=10). Health care costs were defined as the sum of direct costs. Data were then analyzed based on anesthetic modality to produce a direct cost evaluation. Direct costs were compared statistically between MD and PELD groups. The sum of total costs was $1,249.50 in the PELD-Local group, $1,741.50 in the PELD-General group, $2,015.60 in the MD-Spinal group, and $2,348.70 in the MD-General group. The sum of total costs was higher in the MD-Spinal and MD-General groups than in the PELD-Local and PELD-General groups. The costs of surgical operation, surgical equipment, anesthesia (anesthetist’s costs), hospital stay, anesthetic drugs and materials, laboratory wor­kup, nur­sing care, and two main groups (PELD-MD) me­dication diffe­red significantly among the two main groups (PELD-MD) (p<0.01). This study demonstrated that PELD is less costly than MD.

Clinical Neuroscience

SEPTEMBER 30, 2018

A multidisciplinary clinical approach to facioscapulohumeral muscular dystrophy

CAKMAK Öztop Özgür, EREN Ilker, ASLANGER Ayca, GÜNERBÜYÜK Caner, KAYSERILI Hülya, OFLAZER Piraye, SAR Cüneyt, DEMIRHAN Mehmet, ÖZDEMIR Gürsoy Yasemin

Background - Impaired shoulder function is the most disabling problem for daily life of Fascioscapulohumeral muscular dystrophy (FSHD) patients. Scapulothoracic arthrodesis can give a high impact to the functionality of patients. Here we report our experience with scapulothoracic arthrodesis and spinal stenosis surgery in FSHD patients. Patients and methods - 32 FSHD patients were collected between 2015-2016. Demographical and clinical features were documented. All the patients were neurologically examined. The Medical Research Council (MRC) and the FSHD evaluation scale was used to assess muscle involvement1. Scapulothoracic arthrodesis and spinal stenosis surgeries were performed in eligible patients. Results - There were 16 male and 16 female (mean age 34.4 years; range 12-73) patients. 6 shoulders of 4 patients aged between 2132 years underwent scapulothoracic arthrodesis (two bilateral, one left and one right sided). Only one 63 years old female patient with severe hyperlordosis had spinal fusion surgery. All of the patients undergoing these corrective surgeries have better functionality in daily life, as well as superior shoulder elevation. Conclusion - Until the emergence and clinical use of novel therapeutics, surgical interventions are indicated in carefully selected patients with FSHD to improve arm movements, the posture and the quality of life of patients in general. Scapulothorosic arthrodesis is a management with good clinical results and patient satisfaction. In selected cases other corrective orthopedic surgeries like spinal fusion may also be considered.