Coexistence of longitudinally extensive transverse myelitis and diffuse midline glioma in the brainstem in an adolescent boy with acute flaccid paralysis.

We present the case of a previously healthy 13-year-old boy who was admitted to the emergency department with acute flaccid paralysis. Magnetic resonance imaging revealed radiological evidence of longitudinally extensive transverse myelitis. Additionally, homogeneous T2 signal increase was observed in the pons and medulla oblongata, initially indicating brainstem encephalitis. Subsequent evaluations confirmed a coexistence of diffuse midline glioma (DMG) in the brain stem alongside acute transverse myelitis (ATM). Children with ATM generally have a more favorable prognosis than adults. However, despite the implementation of advanced treatment methods, the patient's quadriplegia did not improve and resulted in spinal cord sequela atrophy. DMG exhibits an aggressive growth pattern and lacks a known curative treatment. This case represents an exceedingly rare synchronous occurrence of aggressive conditions, underscoring the importance of raising awareness among physicians. Furthermore, we aim to discuss the radiologic differential diagnosis, as this is the first documented instance in the literature.

Neuropathy in multiple sclerosis patients treated with teriflunomide.

OBJECTIVE: Teriflunomide is an oral medication approved for the treatment of patients with multiple sclerosis. The primary effect of teriflunomide is to reduce de novo pyrimidine synthesis by inhibiting mitochondrial dihydroorotate dehydrogenase, thereby causing cell-cycle arrest. We aimed to investigate the occurrence of peripheral neuropathy, a rare side effect of teriflunomide, in patients receiving teriflunomide. METHODS: Multiple sclerosis patients receiving teriflunomide (n=42) or other disease modifying therapies (n=18) and healthy controls (n=25) were enrolled in this cross-sectional study between January 2020 and 2021. The mean duration of teriflunomide treatment was 26 months (ranging from 6 to 54 months). All participants underwent neurological examination and nerve conduction studies of tibial, peroneal, sural, superficial peroneal, median, and ulnar nerves by using surface recording bar and bipolar stimulating electrodes. RESULTS: The mean superficial peroneal nerve distal latency and conduction velocity were significantly slower, and the mean superficial peroneal nerve action potential amplitude was lower in patients using teriflunomide (2.50 ms, p<0.001; 47.35 m/s, p=0.030; and 11.05 µV, p<0.001, respectively). The mean peroneal motor nerve distal latency was significantly longer and amplitude was lower in teriflunomide patients (3.68 ms, p<0.001, and 5.25 mV, p=0.009, respectively). During the study period, treatment switching to another disease-modifying therapy was planned in 10 patients, and all neuropathic complaints were reversed after switching. CONCLUSION: Teriflunomide has the potential to cause peripheral neuropathy. The awareness of peripheral neuropathy, questioning the symptoms, and if suspected, evaluation with electromyography and switching the therapy in patients under teriflunomide treatment are crucial.

Neuropathy in multiple sclerosis patients treated with teriflunomide

SUMMARY OBJECTIVE: Teriflunomide is an oral medication approved for the treatment of patients with multiple sclerosis. The primary effect of teriflunomide is to reduce de novo pyrimidine synthesis by inhibiting mitochondrial dihydroorotate dehydrogenase, thereby causing cell-cycle arrest. We aimed to investigate the occurrence of peripheral neuropathy, a rare side effect of teriflunomide, in patients receiving teriflunomide. METHODS: Multiple sclerosis patients receiving teriflunomide (n=42) or other disease modifying therapies (n=18) and healthy controls (n=25) were enrolled in this cross-sectional study between January 2020 and 2021. The mean duration of teriflunomide treatment was 26 months (ranging from 6 to 54 months). All participants underwent neurological examination and nerve conduction studies of tibial, peroneal, sural, superficial peroneal, median, and ulnar nerves by using surface recording bar and bipolar stimulating electrodes. RESULTS: The mean superficial peroneal nerve distal latency and conduction velocity were significantly slower, and the mean superficial peroneal nerve action potential amplitude was lower in patients using teriflunomide (2.50 ms, p<0.001; 47.35 m/s, p=0.030; and 11.05 μV, p<0.001, respectively). The mean peroneal motor nerve distal latency was significantly longer and amplitude was lower in teriflunomide patients (3.68 ms, p<0.001, and 5.25 mV, p=0.009, respectively). During the study period, treatment switching to another disease-modifying therapy was planned in 10 patients, and all neuropathic complaints were reversed after switching. CONCLUSION: Teriflunomide has the potential to cause peripheral neuropathy. The awareness of peripheral neuropathy, questioning the symptoms, and if suspected, evaluation with electromyography and switching the therapy in patients under teriflunomide treatment are crucial.

The Turkish experience of COVID-19 infection in people with NMOSD and MOGAD: A milder course?

BACKGROUND: COVID-19 is a multisystemic infection with variables consequences depending on individual and comorbid conditions. The course and outcomes of COVID-19 during neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are not clearly known. OBJECTIVE/METHODS: The aim of this study was to examine the features and outcomes of COVID-19 infection in NMOSD and MOGAD patients. The patients' demographic and clinical factors, disease modifying treatment (DMT) used and disease information of COVID-19 infection were recorded. Conditions leading to hospitalization and severe exposure to COVID-19 infection were also analyzed. RESULTS: The study included 63 patients from 25 centers. Thirty-two patients (50.8%) belong to AQP-4 seropositive group, 13 (20.6%) and 18 (28.6%) were in MOG-positive and double-seronegative groups, respectively. Risk factors for severe COVID-19 infection and hospitalization were advanced age, high disability level and the presence of comorbid disease. Disease severity was found to be high in double-seronegative NMOSD and low in MOGAD patients. No statistically significant effect of DMTs on disease severity and hospitalization was found. CONCLUSION: In NMOSD and MOGAD patients, advanced age, high disability and presence of comorbid disease pose risks for severe COVID-19 infection. There was no direct significant effect of DMTs for COVID-19 infection.

The Sole Initial Imaging Finding in Creutzfeldt-Jacob Disease: Focal FDG-PET Hypometabolism.

Laboratory studies such as electroencephalography, cerebrospinal fluid examination and diffusion-weighted magnetic resonance imaging (DWI-MRI) are valuable in the diagnosis of Creutzfeldt-Jacob disease (CJD),. However, these laboratory studies may not show the characteristic findings in the very early stage of the disease. Here, we present a case of CJD who had atypical neurologic presentation initially and had only a focal parietal 2-(18F) fluorodeoxyglucose (FDG) positron emission tomography (PET) hypometabolism as the sole imaging abnormality at the beginning. The patient progressed rapidly, and showed typical neurological findings for CJD. The brain MRI was performed two weeks after the FDG-PET study, finally demonstrated increased signal intensity in DWI in caudat nucleus, putamen, and cerebral cortex, especially on left parietal region. Imaging methods demonstrating functional alterations in the brain should be obtained in the early period in patients with normal MRI suspected having CJD. Repeating DWI could also be an effective diagnostic approach.

Progressive Onset Multiple Sclerosis: Demographic, Clinical and Laboratory Characteristics of Patients With and Without Relapses in the Course.

AMAÇ: Primer progresif multipl skleroz (PPMS) ve progresif relapsing multipl skleroz (PRMS) baslangiçtan beri olan progresyon ile karakterize MS tipleridir. Nadir görülmelerinden dolayi, literatürde diger MS formlarina göre daha az bilgi bulunmaktadir. Bu çalismanin amaci progresif baslangiçli MS (PBMS) hastalarinda klinik ve laboratuvar özelliklerini ortaya koymaktir. YÖNTEM: PBMS hastalari 2010-2014 yillari arasinda degerlendirilip demografik, klinik özellikleri ve beyin omurilik sivisi (BOS) bulgulari belirlendi. BULGULAR: Otuz iki PBMS hastasi ile ilgili veriler degerlendirildi. Hastalik seyri 24 hastada relaps olmadan (PPMS), sekiz hastada ise relapsli progresifti (PRMS). Kadin/erkek orani tüm grupta 1'di. Ortalama baslangiç yasi tüm grup için 40 (23-55) yasti. Gruplar arasinda hastalik baslangiç yasi ortancasi anlamli farkli bulunmadi (p=0,053). En sik prezantasyon belirtisi motor bozukluklardi. Relapslar tüm hastalarda hastaligin ilk 10 yilinda görüldü. BOS analizinde oligoklonal bant pozitifligi ve artmis IgG indeksi açisindan gruplar arasinda fark saptanmadi (p=0,938, p=0,058). Hastalik süresi her iki grupta da benzer oldugu halde, PPMS grubunda degerlendirme sirasinda ortanca EDSS skoru daha yüksek bulundu (p=0,020). SONUÇ: Çalismamiz Türk PBMS hastalarinin klinik seyir ve laboratuvar bulgularina odaklanmis ilk çalismadir. Iki grubun klinik ve laboratuvar bulgularinin karsilastirilmasi benzer sonuçlar göstermistir. Gruplar arasinda hastalik baslangiç yasi ve artmis IgG indeksi açisindan farklilik olup olmadigini netlestirmek için gelecekte daha genis örneklemli çalismalar yapilmasi gerekmektedir.

Optic Neuropathy and Macular Ischemia Associated with Neurosarcoidosis: A Case Report.

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. She also reported complaints of fever, weakness, sweating, arthralgia, and headache for 2 months. She was referred to the pulmonary diseases unit of our hospital due to hilar lymphadenopathy seen in her chest x-ray, and biopsies were taken for diagnostic purposes. Histological analysis of the mediastinal lymph node biopsies revealed chronic, non-caseating, granulomatous inflammation. Furthermore, the patient was referred to a neurologist due to concomitant complaint of intense headaches. She was diagnosed with neurosarcoidosis supported by findings on cranial magnetic resonance imaging and lumbar puncture. She received a 3-day course of high-dose (1 g/day) intravenous steroid treatment (methylprednisolone) followed by a tapering dose of oral prednisone. The patient began receiving oral methotrexate 15 mg/week as a steroid-sparing agent. Significant improvement in neurological and ophthalmological symptoms occurred in the first week of treatment. In this case report, we emphasized that neurosarcoidosis should be included in the differential diagnosis of patients with both bilateral optic neuropathy and macular ischemia. Furthermore, early diagnosis and timely treatment of neurosarcoidosis are important for favorable visual outcomes.

Obesity: a possible risk factor for restless legs syndrome.

AIM/BACKGROUND: Restless legs syndrome (RLS) is a frequent neurological and sleep disorder. Metabolic disorders are known to be related to sleep disorders. We prospectively evaluated whether obesity and its possible cofactors were related to the presence of RLS. MATERIALS AND METHODS: The study included 143 obese and 94 non-obese individuals. Obese patients had a BMI of 30 and over, while non-obese patients had a BMI lower than 30. Patients with arthritis and pregnancy were excluded but not those with diabetes mellitus. Participants who met diagnostic criteria recommended by the International RLS Study Group were diagnosed as having RLS. Depression, anxiety, daytime sleepiness, insomnia and sleep quality were evaluated in detail. RESULTS: The mean age of obese patients was 40.52 years and that of non-obese patients was 39.76 years. The mean body mass index was 36.77 in the obese group and 25.71 in the non-obese group. The occurrence of depression, anxiety, sleep quality, and insomnia scores were significantly higher in obese individuals. The evaluations of daytime sleepiness, sleep efficiency and sleep latency were not significantly different between the groups. DISCUSSION: Although the presence of RLS was correlated with obesity and vascular risk factors at a significant level, it was also shown that depression, anxiety and insomnia were significantly frequent in obese patients (although not daytime sleepiness). Further studies are needed.

Effect of Renal Transplantation in Restless Legs Syndrome.

OBJECTIVES: Restless legs syndrome is a disorder in which patients have irresistible urge to move legs during rest. Restless legs syndrome seems to be common in end-stage renal disease. After a successful renal transplant, symptoms ameliorate with renal function improvement and restless legs syndrome is seen less in this population. Here, we aimed to investigate restless legs syndrome frequency and associated factors in renal transplant patients. MATERIALS AND METHODS: In a cross-sectional study with 193 patients (116 hemodialysis patients, 45 transplant patients, and 32 controls), the presence of restless legs syndrome was assessed using the Restless Legs Syndrome Questionnaire. Medical history, demographic, and laboratory data were collected from the patients' medical records. Patients were questioned about the presence of restless legs syndrome using the Restless Legs Syndrome Questionnaire. Patients were evaluated with Beck Depression Scale for depression and Pittsburgh tests for sleep disturbances. RESULTS: While the rate of restless legs syndrome was similar between transplants and controls, it was significantly greater in hemodialysis patients. Hemodialysis patients and controls had similar depression scores that were higher compared with transplant patients. Pittsburgh score was similar in transplant patients and controls and significantly increased in the hemodialysis patients. The rate of insomnia was significantly higher in the hemodialysis patients compared with the other 2 groups. Logistic regression analysis revealed independent correlates of restless legs syndrome as insomnia, Beck depression score, and being on hemodialysis. Linear regression analysis showed that independent correlates of higher Pittsburgh score were higher depression score, higher age, and presence of restless legs syndrome. CONCLUSIONS: The prevalence of restless legs syndrome is significantly lower in transplant patients than it is in patients on maintenance dialysis. In renal transplant patients, restless legs syndrome frequency was found to be lower because of improved renal functions (normalization of uremia), psychological symptoms, and sleep disturbances.

Promotion of experimental autoimmune encephalomyelitis upon neutrophil granulocytes' stimulation with formyl-methionyl-leucyl-phenylalanine (fMLP) peptide.

It has been acknowledged that neutrophil granulocytes, the common mediators of immune responses against extracellular bacteria, can also intercede autoimmune reactions such as experimental autoimmune encephalomyelitis (EAE). Formyl-methionyl-leucyl-phenylalanine (fMLP) is a microbial peptide that can be well-tolerated when intravenously administered and can directly lead to activation and accumulation of neutrophils into the blood circulation. Here, this antigenic peptide was injected to the mice at the induction of EAE, and the immunological and pathological outcomes were assessed. As a peripheral immune organ, spleen of the animals that received fMLP contained considerably high percentages of Gr-1(hi)Ly7/4(hi) mature neutrophils. In the sera samples, only a slight difference was determined in Th1/Th2/Th17-related cytokine levels. Expression of CXCR1 or CXCR2 chemokine receptors was not significantly modulated in EAE with or without fMLP which might indicate a direct role for this antigenic peptide on neutrophil accumulation. Even though fMLP administration did not propone the clinical (symptomatic) onset of the disease, the animals showed severe body conditions with higher EAE scores. Accordingly, the expression of TNF-α and CXCL1 inflammatory mediators in the brain was increased in fMLP-EAE group. In conclusion, with its potent capacity to mobilize neutrophils and to stimulate innate immune cells, fMLP peptide can be used to aggravate immune reactions in EAE. This observation may indicate that the strength of innate immune responses particularly at the induction phase of EAE might influence the clinical course of the disease.

Malnutrition is associated with dementia severity and geriatric syndromes in patients with Alzheimer disease.

BACKGROUND/AIM: Malnutrition is associated with increased morbidity and mortality in patients with Alzheimer disease (AD). In this study, we aimed to screen for malnutrition and geriatric syndromes and seek their associations in patients with AD. MATERIALS AND METHODS: The Mini Mental State Examination (MMSE), Mini Nutritional Assessment (MNA), Katz Activities of Daily Living (ADL), and Lawton Instrumental Activities of Daily Living (IADL) tests were applied. Mean daily oral fluid intake was assessed according to patients' and relatives' declarations. RESULTS: Seventy-six patients with a mean age of 79 ± 7.4 years were included. Most of the patients had mild or moderate dementia. Malnutrition was associated with increased rates of hospitalization and falls, dysphagia, insomnia, agitation, delusions, hallucinations, immobility, and incontinence. A daily fluid intake of < 1100 mL was associated with malnutrition risk. Multivariate linear regression analysis revealed independent correlations of lower MNA score with lower ADL score, lower daily oral fluid intake, lower MMSE score, and female sex. CONCLUSION: Dependency, inadequate fluid intake, advanced dementia stage, and female sex were independently associated with malnutrition. Malnutrition also seemed to be associated with sleep disturbances, psychological problems, immobility, falls, and increased hospitalization risk in these patients. Daily oral fluid intake may be a practical tool in the screening of malnutrition.

Mass lesions in the brain: tumor or multiple sclerosis? Clinical and imaging characteristics and course from a single reference center.

AIM: In demyelinating disease spectrum, tumor-like (tumefactive) demyelinating lesions (TDL) are rarely seen. Atypical imaging and clinical features of these lesions may cause misdiagnosis of tumor or abscess. MATERIAL AND METHODS: 25 patients with TDL in our center were followed and clinical, magnetic resonance imaging (MRI), magnetic resonance spectroscopy, cerebrospinal fluid (CSF) findings and disease course were retrospectively evaluated. RESULTS: Mean age at symptom onset was 29 years. Motor and sensory deficits were most common symptoms and 18 of them were polysymptomatic. Mostly frontal and parietal regions were affected. 10/25 patients were initially misdiagnosed clinically as brain abscess, primary central nervous system tumor metastasis. T2-hypointense rim, incomplete ring enhancement of the lesions on post-gadolinium T1- weighted imaging on brain MRI enabled accurate diagnosis of TDLs. 13 of 21 patients with first-TDL presentation sustained a monophasic course, remaining 8 patients converted to multiple sclerosis (MS) at a mean 38.4 months follow-up. Clinical isolated syndrome (CIS) patients were older than patients who developed MS and Expanded Disability Status Scale was lower (0.96 vs 3.7). CONCLUSION: Although MRI, CSF and pathologic examination help in differential diagnosis of the mass lesions, close follow-up is still crucial for the definite diagnosis. A higher MS conversion rate was found in patients with a younger TDL onset age.

Evaluation of Th17-related cytokines and receptors in multiple sclerosis patients under interferon ß-1 therapy.

Th17-related cytokines (IL-17, IL-23, and IL-26) and receptors (IL-17R and IL-23R) were evaluated in MS patients under immunomodulatory IFN-ß1 therapy during a 2year follow-up. Before the initiation of treatment, no significant difference was found in cytokine or receptor expression between controls and MS patients. Of the three cytokines evaluated, IL-26 was the highest in the patients' sera. The amount of IL-17 and CD13(+)IL-17R(+) cells was steadily decreased whereas IL-23 and IL-26 levels were gradually increased with IFN-ß1 therapy. The patients in progressive phase had very high levels of IL-17. Th17-associated parameters should be considered in the immunomodulatory IFN-ß1 therapy of MS.