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Clinical Neuroscience

JULY 30, 2020

[The complex intensive care and rehabilitation of a quadriplegic patient using a diaphragm pacemaker]

FODOR Gábor, GARTNER Béla, KECSKÉS Gabriella

[A 21 year female polytraumatized patient was admitted to our unit after a serious motorbike accident. We carried out CT imaging, which confirmed the fracture of the C-II vertebra and compression of spinal cord. Futhermore, the diagnostic investigations detected the compound and comminuted fracture of the left humerus and femur; the sacrum and the pubic bones were broken as well. After the stabilization of the cervical vertebra, a tracheotomy and the fixation of her limbs were performed. She spent 1.5 years in our unit. Meanwhile we tried to fix all the medical problems related to tetraplegia and respiratory insufficiency. As part of this process she underwent an electrophysiological examination in Uppsala (Sweden) and a diaphragm pacemaker was implanted. Our main goal was to reach the fully available quality of life. It is worth making this case familiar in a wider range of public as it could be an excellent example for the close collaboration of medical and non-medical fields.]

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

JULY 30, 2018

Atypical type of Hirayama disease: Onset of proximal upper extremity

AYAS Özözen Zeynep, ASIL Kıyasettin

Hirayama disease is a rare, benign motor neuron disease. It has been proposed that the dura mater’s posterior wall lacks sufficient elasticity in the lower cervical region and this causes the tense dura part to displace anteriorly upon flexion. The disease is described as involving unilateral upper extremity with a distal-onset. We reported weakness and atrophy of the proximal part of an extremity in a 45-year-old man who is diagnosed with Hirayama disease. Proximal onset is a rare type of Hirayama Disease. Clinicians must be alert of proximal involvement and the diagnosis should be confirmed with electrophysiological and flexion MRI studies.

Clinical Neuroscience

JANUARY 30, 2018

Insights into the structure and function of the hippocampal formation: Relevance to Parkinson’s disease

GYÖRFI Orsolya, NAGY Helga, BOKOR Magdolna, KÉRI Szabolcs

The link between the hippocampus and declarative memory dysfunctions following the removal of the medial temporal lobe opened unexplored fields in neuroscience. In the first part of our review, we summarized current theoretical frameworks discussing the role of hippocampus in learning and memory. Several theories are highlighted suggesting that the hippocampus is responsible for assembling stimulus elements into a unitary representation that later can be utilized to simulate future events. The hippocampal formation has been implicated in a growing number of disorders, from neurodegenerative diseases to atypical cognitive ageing and depression. Recent neuroimaging studies provided new opportunities to study in detail the hippocampal formation’s role in higher levels of the nervous system. We will present data regarding the regional specialization of the hippocampus in experimental models developed for healthy and neurological conditions with a special focus on Parkinson’s disease. Combined evidence from neuroimaging studies suggested that hippocampal volume is reduced in non-demented, newly diagnosed patients with Parkinson’s disease, which is associated with impaired memory performance. These findings proposed that, beyond the well-known striatal dopamine loss, impaired hippocampal synaptic plasticity may contribute to cognitive and affective impairments in early Parkinson’s disease.

Clinical Oncology

DECEMBER 05, 2014

[Bone metastases - Current treatment strategy]

BOÉR Katalin, NÉMETH Zsuzsanna

[Bone is the most common site of metastatic disease in many solid tumours, mainly in breast, prostate and lung cancer. Patients with bone metastases are at risk for skeletal-related events such as bone pain, pathological fractures requiring surgery and/or radiation to bone lesions, hypercalcemia, and spinal cord compression. Skeletal-related events are major source of morbidity for cancer patients and may be associated with negative impact on quality of life and survival. Bisphosphonates inhibit osteoclast function and are widely used in the treatment of malignant bone disease, as preventive therapy against skeletal-related events. Recently, the NF-κappa B-ligand (RANKL)-mediated osteoclast activity and this pathway in bone metabolism became a prime target for the treatment of bone metastases. The fi rst drug targeting the RANK-RANKL pathway is denosumab, a fully monoclonal human antibody which binds to RANKL and inhibits osteoclast activity. Nowadays optimal treatment of bone metastases requires multidisciplinary management of patients including the administration of bone-modifying agents such bisphosphonates or denosumab. The use of bone-targeted agents is a valuable additional treatment in the fi ght against bone metastases and multiple, randomised trials have demonstrated the effectivity of these drugs in reducing skeletal morbidity caused by advanced cancer.]

LAM KID

FEBRUARY 20, 2014

[Bone metabolism and the 10-year probability of hip fracture and a major osteoporotic fracture using the country specific FRAX algorithm in men with type 2 diabetes mellitus]

PETHŐ Zsófia, KULCSÁR-JAKAB Éva, UGO Onyeka, MOLNÁR Zsuzsanna, KALINA Edit, BALOGH Ádám, PARAGH György, ANTAL-SZALMÁS Péter, KÁPLÁR Miklós, BHATTOA HARJIT Pál

[Objectives: Was to evaluate 10-year probability of hip fracture and a major osteoporotic fracture using the FRAX algorithm, vitamin D status, bone mineral density (BMD) and biochemical markers of bone turnover in men over 50 years of age with type 2 diabetes (T2DM). We compared FRAX-predicted 10-year fracture probability, levels of 25-hydroxyvitamin D (25-OH-D), markers of bone turnover and bone mineral density at L1-L4 (LS) and femur neck (FN) in 68 men with T2DM with an age- and gender-matched group (n=68). The mean (range) age of the T2DM group was 61.4 (51-78) years. The prevalence of hypovitaminosis D (25-OH-D <75 nmol/L) was 59%. The mean (range) FRAX hip fracture and FRAX major osteoporotic fracture was 0.7 (0-2.8)% and 3.2 (0-8.5)%, respectively. BMD at the FN (0.974 gm/cm2 vs. 0.915 gm/cm2; p = 0.008) and LS (1.221 gm/cm2 vs. 1.068 gm/cm2; p < 0.001) was significantly higher in the T2DM cohort as compared to the healthy age matched males. 25-OH-vitamin D (67.7 nmol/L vs.79.8 nmol/L; p < 0.001), crosslaps (0.19 μg/L vs. 0.24 μg/L; p = 0.004) and osteocalcin (13.3 μg/L vs. 15.7 μg/L; p = 0.004) were significantly lower in the T2DM group. There was no difference in FRAX-related fracture probability between the two groups. The increased BMD in T2DM and the lack of inclusion of T2DM as a risk factor in the FRAX algorithm are probable explanations for the discordance between literature-observed and FRAX-related fracture probabilities.]

Clinical Neuroscience

JANUARY 30, 2015

[Treatment of osteoporotic vertebral compression fracture with PMMA augmented pedicle screw fixation]

PADÁNYI Csaba, MISIK Ferenc, PAPP Zoltán, VITANOVICS Dusan, BALOGH Attila, VERES Róbert, LIPÓTH László, BANCZEROWSKI Péter

[Background - Over the last few decades many innovative operation technique were developed due to the increase of porotic vertebral fractures. These new techniques aim to reach the required stability of the vertebral column. In case of significant instability, spinal canal stenosis or neural compression, decompressive intervention may be necessary, which results in further weakening of the column of the spine, the minimal invasive percutan vertebroplasty is not an adequate method to reach the required stability, that is why insertion of complementary pedicular screws is needed. Considering the limited screw-fixing ability of the porotic bone structure, with this new technique we are able to reach the appropriate stability of cement-augmented pedicle screws by dosing cement carefully through the screws into the vertebral body. We used this technique in our Institute in case of 12 patients and followed up the required stability and the severity of complications. Methods - Fifteen vertebral compression fractures of 12 patients were treated in our Institute. Using the classification proposed by Genant et al. we found that the severity of the vertebral compression was grade 3 in case of 13, while grade 2 in case of two fractures. The average follow up time of the patients was 22 months (12-39), during this period X-ray, CT and clinical control examinations were taken. During the surgery the involved segments were localised by using X-ray and after the exploration the canulated screws were put through the pedicles of the spine and the vertebral body was filled through the transpedicular screws with bone cement. Depending on the grade of the spinal canal stenosis, we made the decompression, vertebroplasty or corpectomy of the fractured vertebral body, and the replacement of the body. Finally the concerned segments were fixed by titanium rods. Results - In all cases the stenosis of spinal canal was resolved and the bone cement injected into the corpus resulted in adequated stability of the spine. In case of six patients we observed cement extravasation without any clinical signs, and by one patient - as a serious complication - pulmonary embolism. Neurological progression or screw loosening were not detected during the follow up period. Part of the patients had residual disability after the surgery due to their older ages and the problem of their rehabilitation process. Conclusion - After the right consideration of indications, age, general health condition and the chance of successful rehabilitation, the technique appears to be safe for the patients. With the use of this surgical method, the stability of the spine can be improved compared to the preoperative condition, the spinal canal stenosis can be solved and the neural structures can be decompressed. The severity of complications can be reduced by a precise surgical technique and the careful use of the injected cement. The indication of the surgical method needs to be considered in the light of the expected outcome and the rehabilitation.]

LAM KID

SEPTEMBER 19, 2014

[Forgotten agent: raloxifene]

BENKŐ Ágota

[The largest group of the patients with osteoporosis is postmenopausal women characterized by a state of menopausal hormone deficiency which is results in accelerated bone loss. This increased bone resorption significantly elevates the risk of bone fractures including the most common type, i.e. vertebral fractures. In addition to the increased risk of fractures, estrogen deficiency affects other organs, thus, increasing the incidence of cardiovascular diseases, cancers, mood disorders and the symptoms of menopausal syndrome in women after menopause. In postmenopausal osteoporosis, the primary objective is to maintain the existing bone mass, a priority for the prevention and treatment of bone fractures. Hormone deficiency may be prevented by the administration of estrogen but the treatment may have adverse effects such as increased risk of endometrial cancer. An etiological therapy is desirable where the compound used for treatment exerts effects similar to that of estrogen to prevent postmenopausal bone loss as well as reduces the risk of cardiovascular disease without the stimulation of reproductive tissues.]