Introduction
While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to ...be developed.
Objective
To survey the current global clinical practice of clinicians treating MOGAD.
Method
Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February–April 2019).
Results
Fifty-two responses were received (response rate 60.5%) from 86 invited experts, comprising adult (78.8%, 41/52) and paediatric (21.2%, 11/52) neurologists in 22 countries. All treat acute attacks with high dose corticosteroids. If recovery is incomplete, 71.2% (37/52) proceed next to plasma exchange (PE). 45.5% (5/11) of paediatric neurologists use IV immunoglobulin (IVIg) in preference to PE. Following an acute attack, 55.8% (29/52) of respondents typically continue corticosteroids for ≥ 3 months; though less commonly when treating children. After an index event, 60% (31/51) usually start steroid-sparing maintenance therapy (MT); after ≥ 2 attacks 92.3% (48/52) would start MT. Repeat MOG antibody status is used by 52.9% (27/51) to help decide on MT initiation. Commonly used first line MTs in adults are azathioprine (30.8%, 16/52), mycophenolate mofetil (25.0%, 13/52) and rituximab (17.3%, 9/52). In children, IVIg is the preferred first line MT (54.5%; 6/11). Treatment response is monitored by MRI (53.8%; 28/52), optical coherence tomography (23.1%; 12/52) and MOG antibody titres (36.5%; 19/52). Regardless of monitoring results, 25.0% (13/52) would not stop MT.
Conclusion
Current treatment of MOGAD is highly variable, indicating a need for consensus-based treatment guidelines, while awaiting definitive clinical trials.
Atypical imaging features of multiple sclerosis lesions include size >2 cm, mass effect, oedema and/or ring enhancement. This constellation is often referred to as ‘tumefactive multiple sclerosis’. ...Previous series emphasize their unifocal and clinically isolated nature, however, evolution of these lesions is not well defined. Biopsy may be required for diagnosis. We describe clinical and radiographic features in 168 patients with biopsy confirmed CNS inflammatory demyelinating disease (IDD). Lesions were analysed on pre- and post-biopsy magnetic resonance imaging (MRI) for location, size, mass effect/oedema, enhancement, multifocality and fulfilment of Barkhof criteria. Clinical data were correlated to MRI. Female to male ratio was 1.2 : 1, median age at onset, 37 years, duration between symptom onset and biopsy, 7.1 weeks and total disease duration, 3.9 years. Clinical course prior to biopsy was a first neurological event in 61%, relapsing–remitting in 29% and progressive in 4%. Presentations were typically polysymptomatic, with motor, cognitive and sensory symptoms predominating. Aphasia, agnosia, seizures and visual field defects were observed. At follow-up, 70% developed definite multiple sclerosis, and 14% had an isolated demyelinating syndrome. Median time to second attack was 4.8 years, and median EDSS at follow-up was 3.0. Multiple lesions were present in 70% on pre-biopsy MRI, and in 83% by last MRI, with Barkhof criteria fulfilled in 46% prior to biopsy and 55% by follow-up. Only 17% of cases remained unifocal. Median largest lesion size on T2-weighted images was 4 cm (range 0.5–12), with a discernible size of 2.1 cm (range 0.5–7.5). Biopsied lesions demonstrated mass effect in 45% and oedema in 77%. A strong association was found between lesion size, and presence of mass effect and/or oedema (P < 0.001). Ring enhancement was frequent. Most tumefactive features did not correlate with gender, course or diagnosis. Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better (EDSS 1.5) compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001). Given the retrospective nature of the study, the precise reason for biopsy could not always be determined. This study underscores the diagnostically challenging nature of CNS IDDs that present with atypical clinical or radiographic features. Most have multifocal disease at onset, and develop RRMS by follow-up. Although increased awareness of this broad spectrum may obviate need for biopsy in many circumstances, an important role for diagnostic brain biopsy may be required in some cases.
Background: Demyelinating lesions over 20 mm in size, referred to as tumefactive demyelinating lesions, can be misdiagnosed as being either a tumor or an abscess. Although some radiological ...characteristics can help make a differential diagnosis easier, a cerebral biopsy may still be necessary.
Objective: Our objective was to assess the clinical characteristics of tumefactive lesions, with or without a diagnosis of multiple sclerosis (MS), and present follow-up data for 54 patients with tumefactive lesions.
Methods: Demographic, clinical, radiological and laboratory data were gathered and treatment responses were evaluated in a total of 54 patients from five medical centers.
Result: Twenty-nine patients were diagnosed with tumefactive lesions at the onset, whereas 25 patients were diagnosed with tumefactive lesions after a diagnosis of MS. Median follow-up was 38.12 months. At final examination, 19 of the patients with a tumefactive lesion diagnosis at the onset eventually developed relapsing–remitting MS, while 10 remained with the condition as a clinically isolated syndrome. The tumefactive lesions studied were mostly focal, with closed-ring enhancement. We found that oligoclonal band positivity was less frequent in the patients with tumefactive onset.
Conclusion: Although our demographic data were similar to formerly collected Turkish MS data, we found that the distribution of the patients’ clinical course differed if there was an absence of primary progressive MS and that there was a lower frequency of secondary progressive MS cases in our group of patients. We believe that less frequent oligoclonal band positivity and the difference we witnessed in the clinical course of disease in our study groups suggest that there is a need for further studies to compare all the biological and immunological differences between MS and tumefactive lesion cases, in order to reveal whether there are different pathogenetic mechanisms involved.
Splint immobilization of the forearm is often performed in clinical practice. Previous studies investigated the effect of immobilization on bone, cartilage, muscle, and tendon, however, the acute ...effects on human skin microcirculation and histomorphology remains elusive.
In 12 healthy, nonsmoking individuals (aged 29.7 ± 9.1 years) a randomly selected forearm was immobilized by splinting for 72 h, whereas the other forearm served as control. In vivo Reflectance-Mode Confocal-Microscopy (RMCM) was performed prior (baseline value) and postimmobilization to evaluate: quantitative blood cell flow; density of functional dermal capillaries; epidermal thickness; and granular cell size.
At 72h forearm immobilization, quantitative blood cell flow was significantly reduced (42.86 ± 3.68 cells/min) compared to the control blood flow (53.11 ± 3.68 cells/min, P < 0.05) and dermal capillaries indicates less functional density (5.73 ± 0.63 capillaries/mm2) compared to the controls (7.04 ± 0.81 capillaries/mm2, P < 0.05). Histometric assessment reveals significantly thinner epidermis following immobilization compared to the control site (40.02 ± 2.91 vs. 46.64±3.09 µm, P < 0.05). Granular cell size was significantly altered at 72 h splinting (730.1 ± 42.53 µm2) compared to the control cell size at 770.2 ± 38.21 µm2. Comparison of baseline values of both forearms indicate statistically insignificance (P > 0.05) for each parameter.
At 72 h splint immobilization, for the first time, significant adaptive mechanisms were evaluated on human skin microcirculation and histomorphology using in vivo RMCM. These adaptations may be considered as an incipient atrophy of the human skin. Long-term effects of immobilization including the regenerative potential should be evaluated in further RMCM studies.
Background and objective:
We explored which clinical and biochemical variables predict conversion from clinically isolated syndrome (CIS) to clinically definite multiple sclerosis (CDMS) in a large ...international cohort.
Methods:
Thirty-three centres provided serum samples from 1047 CIS cases with at least two years’ follow-up. Age, sex, clinical presentation, T2-hyperintense lesions, cerebrospinal fluid (CSF) oligoclonal bands (OCBs), CSF IgG index, CSF cell count, serum 25-hydroxyvitamin D3 (25-OH-D), cotinine and IgG titres against Epstein-Barr nuclear antigen 1 (EBNA-1) and cytomegalovirus were tested for association with risk of CDMS.
Results:
At median follow-up of 4.31 years, 623 CIS cases converted to CDMS. Predictors of conversion in multivariable analyses were OCB (HR = 2.18, 95% CI = 1.71–2.77, p < 0.001), number of T2 lesions (two to nine lesions vs 0/1 lesions: HR = 1.97, 95% CI = 1.52–2.55, p < 0.001; >9 lesions vs 0/1 lesions: HR = 2.74, 95% CI = 2.04–3.68, p < 0.001) and age at CIS (HR per year inversely increase = 0.98, 95% CI = 0.98–0.99, p < 0.001). Lower 25-OH-D levels were associated with CDMS in univariable analysis, but this was attenuated in the multivariable model. OCB positivity was associated with higher EBNA-1 IgG titres.
Conclusions:
We validated MRI lesion load, OCB and age at CIS as the strongest independent predictors of conversion to CDMS in this multicentre setting. A role for vitamin D is suggested but requires further investigation.
In Turkey, cyst hydatid disease (CHD) or cystic echinococcosis (CE) is publicly known as “dog cyst”, a fatal and serious disease not only affects livestock husbandry and human health but also brings ...about economic loss to our country. According to the data of the Ministry of Health; number of annual cases was 408 in 2008, and this number reached 1,867 by the end of 2019. Cystic echinococcosis is especially taken up during childhood and emerged at an older age. They become exposed to the eggs of the tapeworm after close contact with an infected dog or its contaminated environment. The infected dogs also pass in their feces
eggs that adhere to the dogs’ hairs, and pass on to the children who are in the course of playful and intimate contact with the infected dogs. This study was to create the awareness of risk factors of CE among 10 different districts of Izmir province. Awareness raising seminars are essential component of this study because local people living in CE endemic areas, are crucial to continue and sustain the long-time effort that is needed to tackle this disease. In each district, 3 awareness raising seminars were held to the target groups: (a) in schools for students, teachers, administrators, (b) for general public, (c) for healthcare professionals. 4090 students attended to the trainings, 242 administrators and teachers who attended to the presentations together with the students, 524 people were attended to the trainings and 327 health workers attended to the trainings from different institutions. This study helped improving this situation by organising educational events for the rural populations for preventing transmission of the disease. This is the first educational intervention study regarding creating awareness on CE in Izmir Province which includes 10 districts between January 2019 to January 2020.
Reflectance-mode confocal microscopy (RCM) enables in vivo assessment of the human skin. Impact of overweight on both human skin microcirculation and histomorphology has not been investigated in ...vivo. The purpose of this study is to evaluate both microcirculation and histomorphology in vivo in overweight. In 10 normotensive overweight nondiabetic individuals (OW-group, BMI 29.1±2.7 kg/m2) and 10 age- and sex-matched healthy lean controls (CO-group, BMI 20.4±1.9 kg/m2) the following parameters were evaluated using RCM: dermal blood cell flow (DBCF), density of dermal capillaries (DDC), epidermal thickness (ET), and epidermal cell size (ECS). DBCF was counted at 63.11±4.14 cells/min in OW-group and at 51.06±3.84 cells/min in CO-group (P<0.05). DDC was reduced in OW-group (4.91±0.39 capillaries/mm2) compared to the controls (6.02±0.64 capillaries/mm2, P<0.05). Histometric evaluation of ET reveals thickening in OW-group compared to the CO-group (54.79±4.25 μm versus 44.03±3.11 μm, P<0.05). ECS differed significantly (P<0.05) in OW-group (821.3±42.02 μm2) compared to the controls (772.6±34.79 μm2). Inverse correlation of dermal capillary density and overweight point to reduced total tissue perfusion while positive related blood cell flow reveals vasodilatation. Increase of both ET and cell size indicates remodeling of cutaneous histomorphology, maybe as an early stage of adiposity-related skin condition.
Purpose Radial nerve damage results in substantial functional limitations of the upper extremity. No detailed data exist regarding long-term results, patient satisfaction, and professional and social ...reintegration after tendon transfer for irreparable damage to the radial nerve. In this retrospective study, we investigated these data through the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire. Methods Between 1995 and 2006, 77 patients underwent a modified Brooks and d'Aubigne surgical technique for radial nerve palsy in our department. In 19 cases, the flexor carpi radialis muscle was used as a donor instead of the flexor carpi ulnaris muscle. The mean follow-up period was 60 months (range, 24–150 months); motion of the wrist and finger joints and pinch-grip power were compared with the healthy side. We assessed the limitation in pursuing daily activities using the DASH score. Results Wrist extension averaged 73% of the contralateral side, whereas the value for movement of digital extension was 32% and for thumb abduction in the palmar direction it was 80%. The power grip was reduced to 49% and the pinch grip was reduced to 28%. The mean DASH score was 15 ± 9, the symptom score mean was 15 ± 7, and the working score mean was 12 ± 10. The mean total DASH score was 16 ± 10. The proportion of patients who remained employed after surgical treatment was 89%. Conclusions Functional results, adequate patient satisfaction, and sufficient professional and social reintegration can be achieved after modified Brooks and d'Aubigne tendon transfer. Accordingly, the tendon transfer offers an important alternative—possibly the procedure of choice—to microsurgical nerve reconstruction, particularly when early professional and social reintegration is important. Type of study/level of evidence Therapeutic IV.
Abstract Background Local cold therapy for burns is generally recommended to relief pain and limit tissue damage, however, there is limited data of its physiological benefit. This study aimed to ...evaluate pathophysiological effects of cold therapy in superficial burn on microcirculation, edema formation, and histomorphology. Methods In 12 volunteers (8f, 4m; aged 30.4 ± 14.1 years) circumscribed superficial burn was induced on both hand back and either left untreated as control (control-group) or treated by local-cold-application (cold-treatment-group). Prior to burn (t0), immediately (t1), 15 min (t2), and 30 min (t3) following cold therapy, following parameter was evaluated using intravital-microscopy; epidermal-thickness (ET), granular-cell-size (GCS), individual-blood-cell-flow (IBCF), and functional-capillary-density (FCD). Results Both ET and GCS increased significantly more in control-group and slightly in cold-treatment-group in t1, while turns to insignificant t2 onwards. IBCF and FCD raised up in control-group compared to dramatically decrease in cold-treatment-group in t1. In t2 both parameter remains in control-group and increased in cold-treatment-group. Comparison of both groups for IBCF and FCD indicates significant difference in t1 and t2, however, insignificant in t0 and t3. Conclusions Microcirculation, edema formation, and histomorphology of superficial burn has been significantly influenced through immediate cold therapy, however, this alterations are transient and turns to ineffective after 30 min.
Abstract Objective The current determination of burn depth is based both on a visual and clinical assessment. Confocal-laser-scanning microscopy (CLSM) enables in vivo histomorphological images. We ...hypothesized that CLSM can differentiate superficial-partial vs. deep-partial thickness burns on a histomorphological level. Methods Thirty-eight burn wounds in 14 patients were clinically divided in three groups from superficial (group 1), superficial-partial (group 2) to deep-partial (group 3) thickness burns. CLSM was performed with the Vivascope1500 (Lucid Inc., Rochester, NY, USA) 24 h after burn. The following parameters were assessed: cell size of the granular-layer, thickness of the basal-layer, minimal thickness of the epidermis and number of perfused dermal papillae. Results Superficial burns resulted in a significant increase of the cell size of the granular-layer and a higher increase of the minimal thickness of the epidermis as in superficial-partial thickness burns. The granular-layer in partial thickness burns was destroyed. Superficial burns had an increased thickness of the basal-layer; in superficial-partial thickness burns the basal-layer was partly destroyed with complete destruction in deep-partial thickness burns. In superficial burns the perfused dermal papillae were increased significantly, while decreased in superficial-partial thickness, and completely destroyed in deep-partial thickness burns up to a depth of 350 μm. Conclusions In vivo confocal-laser-scanning microscopy can differentiate superficial-partial vs. deep-partial thickness burns on a histomorphological level.
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