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Lege Artis Medicinae

JULY 01, 2020

[Sarcopenia – muscle loss – pathomechanism, clinical presentation and metabolic comorbidities]

VERECKEI Edit, HODINKA László

[Sarcopenia, or the age-related involution of muscle strength and muscle mass, is a serious public health concern, due to the growing number of elderly population caused by nowadays demographic changes i.e. prolonged life expectancy. By ageing, the muscle tissue is shrinking gradually, leading to the loss of muscle strength and masses. This condition is called sarcopenia. Sar­co­penia is the simultaneous decrease of muscle mass, muscle strength and functional independence. In parallel the physical performance deteriorates (weakness, slowness and poor physical balancing). Fatigue, el­derly behaviour and weight loss are the consequences of these accumulating deficits, which associate with cognitive decline and result in increasing social isolation. The primary form of sarcopenia is the decrease of the energy production of muscle cells and then the death of muscle cells. Se­con­dary, endocrine dysfunctions, diseases of the nervous system, decreased physical activity, malnutrition or malabsorption, chronic infection accelerate the process and aggravate the patient’s condition. Complex genetic, biochemical and endocrine mechanisms take part in the development of sarcopenia. This involution is due to the impaired balance of restoring and depleting processes of muscles. A questionnaire and algorithm have been developed to recognize, screen and diagnose the risks of sarcopenic condition; these separate the sarcopenic and non-sarcopenic patients with specific cut-off values. Sar­co­penia can be diagnosed based on walking speed, decreased handgrip strength and measured or calculated muscle mass in persons over 65. Sarcopenia can be considered as a phenomenon of “physiological” aging, however, it becomes a disease when diagnostic cut-offs are exceeded and the patient experiences functional disability and declining quality of life. Prevention and treatment of sarcopenia and reducing the risk of falling are based on regular active resistance and coordination exercises. Options for pharmaceutical treatments are limited since despite of identified molecular targets there are no convincingly effective innovative therapy on the horizon. Nevertheless, there are some weak evidence for efficacy of the application of amino acids stimulating muscle cell differentiation, such as leucine or the analogue of beta-hydoxy-methylbutyrate beside exercise therapy.]

Lege Artis Medicinae

SEPTEMBER 30, 2020

[Case report about the choice of care at Olajág Elderly Home in Budapest during the COVID-19 epidemic in spring 2020]

PETRÓ Kata, PETKE Zsolt, RADNAI Zoltán, SZIRMAI Viktor

[During the COVID-19 pandemic caused by the SARS-CoV-2 virus, elderly people in long-term nursing homes were espe­cially risks exposed. In this study, the au­thors analyse the causes of local outbreak at the Olajág Elderly Home Vezér street facility in Budapest and the means and ways to curb the spreading of infection. They summarise the measures, which proved to be effective in isolation and treat­ing the patients. The number of infected residents as well as the course of the disease are demonstrated by statistical data. The authors consider as pre-eminently important to identify asymptomatic but in­fec­ted residents, further the rapid introduc­tion of screening tests, strict compliance with protocols, patient path regulation and the use of enhanced, supportive communication.]

Lege Artis Medicinae

JULY 01, 2020

[Minority students in Hungarian medical training]

SZÉL Zsuzsanna

[General health of minority people is usually worse than that of their majority peers and they often expe rience discrimination in the healthcare system. According to international literature, physicians belonging to any minority group are more likely to care for other mi nority people, therefore they may play a key role in reducing healthcare inequities. Anonymous, online questionnaire was distributed to medical students of the four Hungarian universities with medical schools (response rate was 8.86%). In this paper, we analyze our collected data about perceived discrimination with descriptive statistical methods. Results of confirmative statistical analyses (statistical tests) were considered exploratory in nature. 29.6% of respondents self-identified as a member of any minority. 63.0% of minority students and 53.8% of women indicated that they realized discriminated or were harassed in the last 12 months, meanwhile, 37.8% of non-minority students and 31.9% of males have experienced discrimination. Dis­cri­mi­na­tion related to ethnic origin, sexual orientation and disability are regarded as the most widespread forms of discrimination according to our respondents. Students are most likely to say that there is no age related discrimination on the grounds of age – both being under 30 years old (12.0%) and being over 55 (8.6%). Being the member of any minority group seems to have no effect on student’s/ one’s opinion how widespread the forms of dis crimination are. Minority students are more comfortable to work with a member of another minority. However male students feel more uncomfortable to work with a member of sexual or gender minorities compared to female counterparts. Mi­no­rity students tend to be more critical to the universities’ efforts to enhance diversity. Minority students and females may play a key role in reducing discrimination in medical training and in the healthcare system and in providing high-quality care for individuals who belong to any minority. Although there are more females than males in medical training they still report higher occurrence of perceived discrimination. However, it is important to emphasize the low response rate in our study, which does not allow us to draw any general conclusions.]

Lege Artis Medicinae

JULY 01, 2020

[The significance of the identification of SARS-CoV-2 virus and the possible errors of the sampling method]

NOSZEK László

[The COVID-19 pandemic caused by SARS-CoV-2 virus while emerging suddenly and spreading throughout the globe challenged seriously also the modern medicine. Diag­nostic methods recognising viral infections of the upper airways developed essentially in the last 20 years, and it was specifically progressing during the SARS and MERS epidemics thus facilitating the recognition and identifica­tion of infections by influenza, RS- and adenoviruses as well. Nevertheless the present novel coronavirus (SARS-CoV-2) also de­mon­strated that the relatively simple procedures of naso- and oropharyngeal sampling are fallible too. Thus they may have a relatively high risk of false-negative outcomes. However the correct sampling prior the RT-PCR tests provides reliable diagnosis by high sensitivity and specificity. Thus improving the quality of sampling and avoiding failures by correct training and education of the personnel make more reliable the detection of viral infection or indicate recovery after the infection. Finally, this is a key issue while overcoming the present pan­demic.]

Clinical Neuroscience

JULY 30, 2020

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

MAY 30, 2020

[The long-term follow-up of enzyme replacement treatment in late onset Pompe disease]

MOLNÁR Mária Judit, BORSOS Beáta, VÁRDI Visy Katalin, GROSZ Zoltán, SEBÕK Ágnes, DÉZSI Lívia, ALMÁSSY Zsuzsanna, KERÉNYI Levente, JOBBÁGY Zita, JÁVOR László, BIDLÓ Judit

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]

Clinical Neuroscience

MARCH 30, 2016

The electrophysiological changes after 1 hz RTMS in ALS patients. A pilot study

MAJOR Zsigmond Zoltán, VACARAS Vitalie, MARIS Emilia, CRISAN Ioana, FLOREA Bogdan, MAJOR Andrea Kinga, MURESANU Fior Dafin

Motor neuron diseases are disabling poor prognostic conditions, with no successful treatment. Repetitive transcranial magnetic stimulation might offer a temporary functional improvement. Objective - We intended to evaluate the extent of the functional improvement using electrophysiological and clinical tests. Methods - Patients with motor neuron disease (amyotrophic lateral sclerosis) were included. Muscle strength and respiratory function assessment represents the clinical approach, and central motor conduction time, motor unit number estimation, blink reflex and H-reflex stands for electrophysiology. Two tests were performed using the whole battery prior and after low frequency repetitive transcranial magnetic stimulation, using 1 Hz stimulation frequency for five consecutive days, 20 minutes daily, at 80% of the individual resting motor threshold. Results - Central motor conduction time, muscle strength and pulmonary function showed no statistically significant differences, but a tendency towards improvement. Motor unit number estimation, blink reflex and H-reflex showed a significantly better outcome after the five day repetitive transcranial magnetic stimulation treatment. Conclusion - Low frequency repetitive transcranial magnetic stimulation influences beneficially electrophysiological parameters in amyotrophic lateral sclerosis, but with little clinical impact; further studies are needed to validate the extent of the effect.

Clinical Neuroscience

MARCH 30, 2020

To handle the HaNDL syndrome through a case: The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis

ÇOBAN Eda, TEKER Ruken Serap, SERİNDAĞ Helin, SAKALLI Nazan, SOYSAL Aysun

The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related to migrainous headaches. It is a benign, self-limited disorder, which is characterized by fluctuating neurological symptoms and cerebrospinal fluid lymphocytosis. We describe a case of a 47 years old man with acute onset of headache and aphasia. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis (25 cells/μl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with temporoparietal predominance, and without epileptiform activity. His blood tests as well as magnetic resonance imaging (MRI) results were normal. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged with full recovery.

Clinical Neuroscience

MARCH 30, 2020

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

MARCH 10, 2020

[Nutritional status, realizing sarcopenia and the importance of prehabilitation in surgical departments]

CSIBA Borbála, NAGY Ákos, LUKOVICH Péter, BAROK Bianka

[INTRODUCTION - Malnutrition can significantly influence the surgery’s outcomes. Currently, patients risk grouping is based on the body mass index (BMI), and the preparation for surgery is concerned only as nut­rients administration. PATIENTS AND METHODS - The Nutrition Support Team established in our Department is assessing first the patients’ nutritional status (BMI, MUST), sarcopenia (skinfold measure, handgrip strength) and fitness status (6 min step test, sit to stand test). Risk group patients were suggested nutrients and physiotherapy prior to the surgical operation. In order to follow up our patients we created an online interface and repeated the tests immediately before the operation. RESULTS - 135 cancer patients (76 male and 59 female) were operated. Their average age was 69.6 years. 33 patients had weight loss before the first consultation (average=8.7 kg). Their average BMI was 26.3. 21 patients had gained weight in the last 6 months (average=7.8 kg). Patients with left descending colon, liver - and also pancreatic tumors had overweight BMI values while the rest of pa­tients ill with right colon and stomach neo­plasm had normal weight. Those patients who we enrolled to the online system had better results at the second assessment. CONCLUSIONS - According to our survey, most of the patients had overweight BMI values but had sarcopenia based on anthropometric tests. Therefore, the importance of in time recognized and preoperatively started nutritional therapy must be coupled with the parallel applied physiotherapy. ]