Neuromyelitis optica spectrum disorder is a severe autoimmune disease of the central nervous system characterized by recurrent inflammatory events primarily involving the optic nerves and spinal ...cord. Recently, a triggering role of infectious events in the development of NMOSD has been suggested. Varicella zoster virus is the most common viral infection involved, though the linkage with anti-aquaporin-4 antibodies is so far unknown. We report, to the best of our knowledge, the first pediatric case report about NMOSD relapse triggered by herpes zoster infection. The strict temporal relationship between VZV infection and NMO attacks seems to be more than simply due to chance; however, further reports are needed to be confirmed.
Herpes simplex virus encephalitis (HSE) is the most common cause of letal encephalitis and its prevalence appears higher among oncologic patients who undergo brain radiotherapy (RT). We describe a ...case of 76-year-old woman with glioblastoma multiforme (GBM) who developed HSE shortly after brain RT. Cerebrospinal fluid analysis (CSF) was normal and the diagnosis was driven by brain MRI and EEG. Prompt introduction of antiviral therapy improved the clinical picture. We highlight the importance of EEG and brain MRI for the diagnosis and suggest the possibility of antiviral profilaxys in oncologic patients who undergo brain RT.
Neuromyelitis optica spectrum disorder (NMOSD) is a severe, inflammatory, immune-mediated astrocytopathy of the central nervous system, characterized by recurrent inflammatory events primarily ...involving optic nerves and the spinal cord. Recently, a triggering role of infectious events in the development of NMOSD has been suggested. Varicella zoster virus (VZV) is the agent most involved, although the linkage with anti-aquaporin-4 antibodies is so far unknown. A review of the literature on the association between NMOSD and VZV infection was carried out by searching PUBMED and EMBASE from 1975 to July 2020. A total of 13 articles concerning Herpes zoster preceding NMOSD were identified. All patients were female and the median age at NMOSD presentation was 28.5 (range 5–63) years. Four NMOSD cases occurred after chicken pox while the remaining ten after HZ. Full recovery occurred in 5/14 patients. From the review of the literature, we can infer that VZV seems to trigger LETM attacks and not the disease itself. The strict temporal relationship between VZV infection and NMOSD seems to exceed the pure chance and represents an unusual clinical scenario posing several diagnostic and management challenges.
Objectives/Background
Tension‐type headache and migraine without aura are the most common primary headaches occurring in people with demyelinating diseases, whereas cluster headache (CH) can be ...considered exceptional. The location of demyelinating lesions could be strategic in these cases, involving areas interacting with the trigeminovascular system.
Methods and Results
We report a case of a 54‐year‐old woman with right‐sided CH as the initial manifestation of multiple sclerosis and showing a left dorsal brainstem lesion on magnetic resonance imaging, in the region of the dorsal longitudinal fasciculus (DLF).
Conclusion
Our case seems to suggest a possible role of the DLF in the process that leads to CH attacks. Because neuroimaging clearly showed a lesion contralateral to CH pain, we hypothesize that some fibers from periaqueductal gray matter project to the contralateral side, besides the known ipsilateral connections.
Objective
This study was undertaken to assess the impact of immunosuppressive and immunomodulatory therapies on the severity of coronavirus disease 2019 (COVID‐19) in people with multiple sclerosis ...(PwMS).
Methods
We retrospectively collected data of PwMS with suspected or confirmed COVID‐19. All the patients had complete follow‐up to death or recovery. Severe COVID‐19 was defined by a 3‐level variable: mild disease not requiring hospitalization versus pneumonia or hospitalization versus intensive care unit (ICU) admission or death. We evaluated baseline characteristics and MS therapies associated with severe COVID‐19 by multivariate and propensity score (PS)‐weighted ordinal logistic models. Sensitivity analyses were run to confirm the results.
Results
Of 844 PwMS with suspected (n = 565) or confirmed (n = 279) COVID‐19, 13 (1.54%) died; 11 of them were in a progressive MS phase, and 8 were without any therapy. Thirty‐eight (4.5%) were admitted to an ICU; 99 (11.7%) had radiologically documented pneumonia; 96 (11.4%) were hospitalized.
After adjusting for region, age, sex, progressive MS course, Expanded Disability Status Scale, disease duration, body mass index, comorbidities, and recent methylprednisolone use, therapy with an anti‐CD20 agent (ocrelizumab or rituximab) was significantly associated (odds ratio OR = 2.37, 95% confidence interval CI = 1.18–4.74, p = 0.015) with increased risk of severe COVID‐19. Recent use (<1 month) of methylprednisolone was also associated with a worse outcome (OR = 5.24, 95% CI = 2.20–12.53, p = 0.001). Results were confirmed by the PS‐weighted analysis and by all the sensitivity analyses.
Interpretation
This study showed an acceptable level of safety of therapies with a broad array of mechanisms of action. However, some specific elements of risk emerged. These will need to be considered while the COVID‐19 pandemic persists. ANN NEUROL 2021;89:780–789
Background
Spinal cord (SC) involvement correlates with poor prognosis in patients with multiple sclerosis (MS). Nevertheless, there is no consensus on the use of SC-MRI at follow-up, mainly because ...of the belief that SC lesions are nearly always symptomatic.
Objectives
The aim of the present study was to investigate the frequency of asymptomatic SC combined unique activity (CUA, new/enlarging T2 or gadolinium-positive Gd+ lesions) on MRI in a cohort of patients diagnosed with clinically isolated syndrome (CIS) or relapsing–remitting MS (RRMS).
Methods
We retrospectively investigated all scans showing SC-CUA in patients with CIS or RRMS referred to a single Italian MS centre. We determined whether they were symptomatic and whether they had associated brain radiological activity.
Results
In 340 SC-MRI scans with SC-CUA (230 patients), SC-CUA was asymptomatic in 31.2%; 12.1% of SC-CUA had neither clinical activity nor brain radiological activity (44.5% and 25.4%, respectively, considering only follow-up SC-CUA). At multivariate analysis asymptomatic SC-CUAs were associated with older age at onset (34.0 ± 10.37 vs 31.0 ± 9.99 years,
p
= 0.006), non-spinal onset (76.4 vs 47.4%,
p
< 0.001), lower EDSS score at MRI (1.8 ± 0.93 vs 2.4 ± 1.28,
p
= 0.001) and lower number of Gd+ SC lesions (0.1 ± 0.33 vs 0.3 ± 0.54,
p
= 0.04), compared to symptomatic SC-CUAs.
Conclusions
A substantial proportion of our patients had SC-CUA without clinical symptoms and/or without concomitant brain MRI activity. In these patients, SC-CUA was the only sign of disease activity, suggesting that regular SC-MRI follow-up is required for reliable assessment of radiological activity and may improve the management of patients with MS.
The concept of ‘no evidence of disease activity’ (NEDA) has been proposed as a surrogate marker for treatment response in relapsing-remittent multiple sclerosis (MS). However, there is no agreement ...regarding its prognostic value, nor about the starting time for evaluation of drug effectiveness. Aim of this study was to investigate if the status preservation of two-year NEDA, ‘minimal evidence of disease activity’ (MEDA) and six-month delayed NEDA (6md-NEDA, with a “rebaseline” six months after the treatment start) predicts the achievement of long-term disability outcomes (EDSS score ≥ 4.0 or 6.0, 3-month confirmed disability progression (CDP) or conversion to secondary progressive MS) after five and seven years of disease. A total of 271 treatment courses (TCs) were analyzed in this retrospective study, involving all TCs started with any disease-modifying treatments (DMT). Overall, 72 (27%), 77 (28%) and 92 (34%) TCs maintained NEDA, MEDA and 6md-NEDA status after a two-year treatment. NEDA, MEDA and 6md-NEDA TCs had a lower risk of attaining all disability outcomes, compared to ‘evidence of disease activity’ (EDA) TCs. NEDA status determined a lower risk of CDP after five (OR 0.18, 95% CI 0.07–0.45, p < .0001) and seven years of disease (OR 0.15, 95% CI 0.05–0.44, p < .0001), with high positive (90%) and low negative (42%) predictive value, good specificity and low sensitivity. NEDA TCs had a lower risk of CDP compared to MEDA TCs after seven years (OR 0.30, 95% CI 0.10–0.91, p = .04). 6md-NEDA had a small impact on the improvement of NEDA prognostic value.
•NEDA concept has been proposed as surrogate marker for treatment response in RRMS.•NEDA prognostic value and the starting time to evaluate drug efficacy are not clear.•2 year-NEDA status during DMT protects against 5- and 7-year disability accrual.•MEDA status heralds worse disability outcomes, compared to NEDA.•A fixed six month-rebaseline has small impact in improving NEDA prognostic value.
Objectives
Gray matter (GM) damage is well known as a fundamental aspect of multiple sclerosis (MS). Above all, cortical lesions (CLs) burden, detectable at MRI with double inversion recovery (DIR) ...sequences, has been demonstrated to correlate with cognitive impairment (CI). The aim of this study was to investigate the role of CLs number in predicting CI in a cohort of patients with MS in a clinical practice setting.
Materials and methods
Thirty consecutive patients with MS presenting CLs (CL+) at high‐field (3.0 T) MRI 3D‐DIR sequences and an even group of MS patients without CLs (CL‐) as a control, were investigated with the Rao Brief Repeatable Battery of Neuropsychological Tests (BRB), Version A. Total and lobar CLs number were computed in CL+ patients.
Results
Among the sixty patients with MS enrolled, forty‐seven (78.3%) had a relapsing‐remitting course, while thirteen (21.7%) a progressive one, eleven secondary progressive, and two primary progressive. Compared to CL−, CL+ patients had a greater proportion of progressive forms (p = .03). The most affected region was the frontal lobe (73.3% of patients), followed by temporal and parietal ones (both 60.0%). Multivariate (logistic regression) analysis revealed a significant correlation between total CLs number and the presence of mild cognitive impairment defined as pathologic score in at least one BRB test (p = .04); it was also correlated with deficit at PASAT 3 (p = .05) and Stroop Test (p = .02).
Conclusions
We confirmed CLs number, evaluated with a technique quite commonly available in clinical practice, as a predictive factor of CI in patients with MS, in order to improve the diagnosis and management of CI and monitor potential neuroprotective effects of therapies.
Gray matter disease is a fundamental aspect of multiple sclerosis (MS), and cognitive impairment (CI) is an important component of MS‐related disability, even though both of them, often, are not properly investigated in clinical practice.
In this study, conducted in a clinical practice setting in a cohort of relapsing–remitting and progressive patients with MS investigated with the Rao Brief Repeatable Battery of Neuropsychological Tests (BRB), Version A and with high‐field (3.0) MRI and double inversion recovery (3D‐DIR) sequences, we found a significant correlation between the cognitive domains most affected in MS and cortical lesions number, a parameter easily achievable also outside research setting and useful for the clinical management of MS‐related CI.
The recent improvements in multiple sclerosis therapy have lead to consequent improvements in its prognosis: however, it still remains a chronic and unpredictable disease. The moment of the diagnosis ...is the starting point of a durable relationship between the physician and the patient, but, most of all, it is often referred to as the most traumatic experience in patients’ life. Patients’ compliance to prescribed therapies, so important in the course of every chronic condition, particularly hangs on the psychological approach used by the doctor in communicating and explaining the diagnosis for the first time, in addition to the patient’s personality. A brief overview on the main types of physicians’ and patients’ behaviours and communications styles is provided in this article.
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