Ideggyogyaszati Szemle-Clinical Neuroscience最新文献

Isaacs' syndrome is an immune-mediated primary peripheral nerve hyperexcitability syndrome characterized by muscle twitches, cramps, and muscle stiffness. In 30-50% of cases, antibodies against voltage-gated potassium channel (VGKC) complex proteins are present. Symptomatic treatment, intravenous immunoglobulin (IVIg), plasma exchange (PE), and intravenous methylprednisolone (IVMP) are generally effective in controlling symptoms. However, some cases remain treatment-refractory. We present a 52-year-old female patient with walking difficulty, fasciculations, and severe back and hip pain. The patient was positive for anti-VGKC antibodies with a history of thymoma. Symptomatic treatment, highdose IVMP, IVIg and PE treatments improved the patient's gait partially but failed to control the pain and fasciculations. Eventually, we treated the patient with rituximab (RTX), because of its effect on antibody-mediated autoimmunity, resulting in complete symptomatic relief without side effects. RTX is a safe and effective option for controlling symptoms in treatment-refractory cases with Isaacs' syndrome.

Overlap myositis (OM) is an idiopathic inflammatory myopathy characterized by proximal muscle weakness. However, atypical patterns of muscle involvement may also occur. We present a challenging case of OM mimicking bilateral posterior interosseous nerve (PIN) palsy. The patient presented with a gradual onset of bilateral finger drop over two years. Despite initial clinical suspicion of PIN palsy, electromyography revealed myopathic features. Laboratory assessments showed anti-ribonucleoprotein antibody positivity, with subsequent muscle biopsies supporting OM associated with mixed connective tissue disease. This case highlights the significance of considering atypical presentations of OM. Furthermore, it emphasizes the pivotal role of targeted muscle biopsies when initial biopsy results are inconclusive.

Background and purpose: Our aim was to use visual evoked potentials (VEP) to evaluate changes in brain bioelectrical activity in Hashimoto thyroiditis patients with normal neurological examination and imaging findings and to correlate the evoked potential parameters with clinical data.

Methods: Our aim was to use visual evoked potentials (VEP) to evaluate changes in brain bioelectrical activity in Hashimoto thyroiditis patients with normal neurological examination and imaging findings and to correlate the evoked potential parameters with clinical data.

Results: Of the Hashimoto thyroiditis patients included in the study, 92% (n=69) were female, the mean age was 40.3±12.2 years, and the mean disease duration was 5.7±7.5 years (range: 0-40 years, median: 4 years). Euthyroid patients showed significantly longer P100 latency difference and higher N75/P100 amplitude than the control group (p=0.014 and 0.007, respectively). Elevated thyroid-stimulating hormone level was associated with longer N75 and P100 latencies.

Conclusion: Our results demonstrated the presence of abnormal brain bioelectric activity in Hashimoto thyroiditis patients without active neurological findings. These effects increase with disease severity and duration and necessitate the development of early treatment strategies to halt the progressive autoimmune process.

Background and purpose: There is few naturalistic evaluation on the spontaneous plasticity in the long-term follow-up of pharmacoresistant epilepsies. The author aims to answer the following questions: What are the trends and characteristics in spontaneous clinical development (amelioration or worsening) of these patients? Which external circumstances can influence the outcome - with special concern to seizure precipitating/ provoking factors?

Methods: The author summarizes the history of 4 patients from the 14 ones with pharmacoresistant epilepsy presented consecutively between 1th January to 30th June 2024 at his out-patient clinic in whom he observed meaningful 'spontaneous' improvement in the follow-up without any modification of pharmacotherapy, and that of further 3 patients in whom unwanted seizure occurred after a long lasting seizure freedom. In their short case-reports he pointed the presence of some actual life events of physical, mental or emotional nature which possibly acted on the outcome.

Results: During the long-term follow-up of pharmacoresistant epilepsies the author found that i./ The clinical course can show 'spontaneous' improvement concerning both in the frequency and intensity of seizures. ii./ In the majority of patients it shows close relation to the changes in life circumstances and to the presence of transient life events. iii./ The increased risk for relapse has been still remaining after a seizure-free life period lasting longer than that of established for the cured state.

Conclusion: Occurrence of habitual seizures can be in closer connection with precipitating factors even is pharmacoresistant epilepsies. So, decrease or lack of some of them can lead to spontaneous recovery. The applied pills did not play significant role. On the other hand, the pharmacoresistant epilepsies can temporarily fulfill the conditions of 'cured epilepsy' but the risk of recurrence still remains higher. Based on these observations, a more detailed evaluation of precipitating and provoking factors of seizure is recommended in pharmacoresistant patients for achieving their optimal care. This may also include some new concepts in the definition of pharmacoresistant epilepsy.

Introduction - Cardiac myxomas are the most common cardiac tumors that can present with a spectrum of clinical manifestations. They are an uncommon cause of cerebral stroke, and simultaneous involvement of the aorto-iliac vessels is extremely rare with 8 cases reported in the literature over the past 10 years. We provide a pictorial illustration of the radiological, surgical, and histopathological findings, which differentiates our case from others in the literature. Case presentation - A 41-year-old female was admitted with right side weakness and aphasia (NIHSS score of 15). CT and CT-angiography revealed hyperacute brain ischemia with occlusion of left M1-segment of MiddleCerebral-Artery. The patient was shifted to a thrombectomy-capable center, where the left femoral pulse was found to be absence and the right femoral artery had weak pulsations. Mechanical thrombectomy of left MCA was done. Angiography showed occlusions of the distal abdominal aorta, the bilateral common iliac arteries, the left external iliac artery and the left common femoral artery. Surgical embolectomy of peripheral vessels was done. Pathohistology of clots taken out from the cerebral as well as peripheral vessels revealed cardiac myxoma. Patient had complete recovery of the stroke as well as the lower limb ischemia. Conclusion - Atrial myxoma is a rare cause of cerebral stroke requiring a thorough investigation to reach a diagnosis. Mechanical thrombectomy is a safe and effective method for treatment of such strokes. Important to emphasize the need for a multi-disciplinary team in the management of distal embolization in these cases. Data in the literature with regards to its treatment is limited.

Background and purpose: Allgrove syndrome or triple A (3A) syndrome is a multisystem disorder classically defined as the triad of esophageal achalasia, alacrimia and adrenal insufficiency due to adrenocorticotropic hormone insensitivity. Approximately one third of patients experience neurological dysfunction, including peripheral and autonomic nervous system dysfunction, leading some authors to use the term 4A syndrome (achalasia, alacrimia, adrenal insufficiency and autonomic abnormalities). Since its first description in 1978, knowledge of its clinical and genetic features has increased; however, the current literature is limited to case reports and case reviews.

Methods: A 20-year-old male patient was admitted to the clinic with the following complaints: difficulty in walking, tingling sensation in the feet and weakness of 1.5 years' duration. He had undergone endoscopy and balloon dilatation surgery 2 years previously.A 20-year-old male patient was admitted to the clinic with the following complaints: difficulty in walking, tingling sensation in the feet and weakness of 1.5 years' duration. He had undergone endoscopy and balloon dilatation surgery 2 years previously.

Results: - We performed whole exome analysis on the patient and detected the c464G>A p.(Arg155His) variant in the AAAS gene in homozygous form. It was interpreted as 'pathogenic' according to the ACMG 2015 criteria: homozygous pathogenic variants in this gene correspond to the phenotype 'AchalasiaAddisonism-Alacrimia' (OMIM:231550).

Conclusion: We present this case to draw attention to the fact that patients may present with late-onset neurological findings without the classic Allgrove syndrome triadWe present this case to draw attention to the fact that patients may present with late-onset neurological findings without the classic Allgrove syndrome triad.

Background and purpose: Stroke is one of the leading causes of death and disability worldwide. Mental health problems occur in approximately one-third of stroke patients and have a negative impact on morbidity and mortality. With this study, we aimed to examine the effects of increasing knowledge about stroke risk factors, symptoms, and treatment on post-stroke anxiety and prognosis.

Methods: The research was conducted as a single-center, prospective study. Among 871 patients, 181 patients who were met the criteria were included in the study. Demographic data and etiological factors were examined. Patients were scored according to the Beck Anxiety Index at one-month follow-up, and statistical analyses were performed by determining modified Rankin scores at three-month follow-up.

Results: The mean age of the individuals participating in the study was determined to be 66.60± 13.26 years. A weak level of statistically significant negative correlation was observed between age and Beck Anxiety Index Score. No significant relationship was observed between anxiety and scale infarct localization. Significant differences were found in the responses to questions evaluating stroke awareness according to age. A significant difference was found in the comparison of educational status based on known stroke risk factors. As the number of known stroke risk factors increased, the mean age decreased. It was shown that those who were aware of stroke treatment sought medical help earlier. Individuals who were aware of stroke symptoms had higher 3rd-month modified Rankin Scale scores. Significant difference was found in the responses to the question "What symptoms occur in stroke survivors?" based on Beck Anxiety Index scores.

Conclusion: This study investigated the relationship between post-stroke anxiety and demographic variables, infarct localization, stroke awareness, factors influencing stroke awareness, patients' sources of information, factors affecting early hospital admission, and the impact of stroke awareness and post-stroke anxiety on prognosis. Studies aimed at increasing the knowledge level of patients with lower education levels and the elderly regarding stroke may contribute to their early hospital admission and improvement in prognosis if they experience a stroke. However, careful attention should be paid to anxiety in follow-ups of patients, especially in younger individuals with better knowledge about stroke.

The loss of cerebral blood flow autoregulation is experienced after severe neurological injuries. Its impairment leads to a cascade of secondary neurological injuries that often follow the primary neurological insult. However, although its maintenance is important, cerebral autoregulation monitoring remains and does not follow consistent clinical standards. The traditional neurocritical monitoring method is invasive intracranial pressure monitoring. Several methods have also been tested for noninvasive neuromonitoring. Rheoencephalography is a form of non-invasive monitoring based on bioimpedance measurement; however, it is not used in routine clinical practice today. This review presents selected results of in-vitro and in-vivo studies evaluating the rheoencephalogram pulse waveform quantification during cerebral blood flow alterations, correlations with the intracranial pressure and other cerebral blood flow measuring modalities, and calculation of the rheoencephalogram-based autoregulation index. A total number of 1027 human and 638 animal measurements were involved. An in-vitro study documented the correlation between Doppler ultrasound flow and bioimpedance. In-vivo animal and human studies established a correlation with other modalities such as laser Doppler flow, carotid flow, near-infrared spectroscopy, and intracranial pressure as well as invasive and noninvasive cerebral blood flow autoregulation indexes. Studies documented that rheoencephalogram reflects cerebral volume change, cerebrovascular reactivity, intracranial pressure, and cerebral blood flow autoregulation. Human measurements documented that rheoencephalogram pulse wave morphology changes (peak 2) and that cerebral blood flow autoregulation's active/ passive status and peak 2 increases during the Trendelenburg position. These studies support the idea that rheoencephalography can be considered as a future non-invasive neuromonitoring modality.

Background and purpose: High titers of anti-SARS-CoV-2 immunoglobulin G (IgG) have been reported in cerebrospinal fluid (CSF) samples of patients with COVID-19, but the data are limited. Our purpose is to report the clinical features and CSF findings of patients with neurologic symptoms in the pandemic era, and to discuss the origin and importance of anti-SARS-CoV-2 IgG in CSF.

Methods: The data of 100 patients hospitalized in neurology inpatient clinics and tested for anti-SARS-CoV-2 IgG antibodies in CSF samples, between May 2021 and March 2022, were evaluated in a retrospective manner. Demographic features, presence of coronavirus disease 2019 (COVID-19) and/or COVID-19 history, vaccination, vaccine type, and neurologic diagnoses were noted. CSF and serum anti-SARS-CoV-2 antibody ratio (AR) were assessed using a Euroimmun ELISA assay. CSF cytology, CSF protein level, oligoclonal band, IgG index, and albumin quotient (QAlb) were analyzed and compared between CSF AR positive and negative groups.

Results: QAlb and CSF protein levels were higher in the CSF anti-SARS-CoV-2 AR-positive group compared with CSF anti-SARS-CoV-2 AR-negative group (p.

Conclusion: Anti-SARS-CoV-2 antibodies in CSF seem to be related to a past immunization or a pre-existing cross reactive immunity and passive diffusion through a disrupted blood-brain-barrier, but not to an intrathecal antibody synthesis.

Background and purpose: Flunarizine is a specific calcium antagonist and is frequently used in adults for the prophylactic treatment of migraine. The use of flunarizine may lead to somnolence and weight gain, depression, and rarely extrapyramidal symptoms in adults. However, studies detecting the efficacy and safety of flunarizine use in adolescents are limited. In the current study, the effectiveness of flunarizine for the management of migraine without aura in Turkish adolescents was evaluated.

Methods: Forty-six patients with migraine without aura, receiving flunarizine 5mg per day were included. In this retrospective study, the medical records of the cases were examined. Visual Analog Scale (VAS) and MIDAS scores were compared to assess the efficacy of the 3 months treatment.

Results: The mean age was 14.37±1.83 years. There was a significant improvement in the VAS and MIDAS scores of the patients at the end of the third month (p.

Conclusion: Although the advancement of migraine research and treatment is inevitable, our findings support that flunarizine should still be considered as an effective and tolerable treatment option in adolescent migraineurs.