Search

Background Patients with multiple sclerosis (MS) often receive disease-modifying therapies (DMTs) that can expose them to reactivation of potential occult hepatitis B virus (HBV) infection (pOBI). We aimed to evaluate the MS Centers behavior regarding HBV screening and prophylaxis in a large cohort of MS patients receiving anti-CD20 or cladribine. Methods Retrospective, multicentric study recruiting Italian MS patients treated with rituximab, ocrelizumab and cladribine. Results We included 931 MS patients from 15 centers. All but 38 patients performed a complete HBV screening. Patients’ age > 50 years was significantly associated with no history of vaccination and HBsAb titres p p = 0.5), pre-or post-therapy vaccination (p = 0.2) and number of previous DMTs (p = 0.2). Among pOBI patients (n = 53), 21 received antiviral prophylaxis, while only 13 had HBV DNA monitoring and 19 patients neither monitored HBV DNA nor received prophylaxis. Conclusions Baseline HBV screening in patients receiving anti-CD20 and cladribine is a consolidated practice. Nonetheless, HBV vaccination coverage is still lacking in such population and age is a significant factor associated with low HBV protection. Rituximab, ocrelizumab and cladribine did not impair HBV vaccine response. Almost 35% of pOBI patients fail to receive HBVr prevention. Management of HBV prophylaxis could be improved in MS patients and further prospective studies are needed to assess the effectiveness of prophylactic strategies in such patients.

Anti-myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are new emerging diseases with heterogeneous course, treatment, response, and prognosis. We herein present 2 cases with antibodies to MOG, one with a cerebellar/brainstem monophasic syndrome which partially improved after treatment, and the other with an optic neuritis onset then relapsed with cortical encephalitis and presented a subsequent complete recovery. We further discuss elements possibly associated with disease heterogeneity and influencing treatment choices. MOGAD is an extremely variable disease which can relapse and accumulate disability over time. An early diagnosis and correct timely treatment is fundamental to improve clinical outcome.

Introduction: The approval of 9-δ-tetrahydocannabinol (THC)+cannabidiol (CBD) oromucosal spray (Sativex®) in Italy as an add-on medication for the management of moderate to severe spasticity in multiple sclerosis (MS) has provided a new opportunity for MS patients with drug-resistant spasticity. We aimed to investigate the improvement of MS spasticity-related symptoms in a large cohort of patients with moderate to severe spasticity in daily clinical practice. Materials and methods: MS patients with drug-resistant spasticity were recruited from 30 Italian MS centers. All patients were eligible for THC:CBD treatment according to the approved label: ≥ 18 years of age, at least moderate spasticity (MS spasticity numerical rating scale [NRS] score ≥ 4) and not responding to the common antispastic drugs. Patients were evaluated at baseline (T0) and after 4 weeks of treatment (T1) with the spasticity NRS scale and were also asked about meaningful improvements in 6 key spasticity-related symptoms. Results: Out of 1615 enrolled patients, 1432 reached the end of the first month trial period (T1). Of these, 1010 patients (70.5%) reached a ≥ 20% NRS score reduction compared with baseline (initial responders; IR). We found that 627 (43.8% of 1432) patients showed an improvement in at least one spasticity-related symptom (SRSr group), 543 (86.6%) of them belonging to the IR group and 84 (13.4%) to the spasticity NRS non-responders group. Conclusion: Our study confirmed that the therapeutic benefit of cannabinoids may extend beyond spasticity, improving spasticity-related symptoms even in non-NRS responder patients. © 2020, Fondazione Società Italiana di Neurologia.

Anti Yo antibodies (AYA) are the antibodies most frequently identified with Paraneoplastic Cerebellar Degeneration (PCD). They are detected above all in PCD associated with breast and gynecologic malignancies. Here, we report a third case of PCD with cerebellar contrast enhancement findings at MRI. In fact, AYA cross anti cerebellar cortex immune reaction leads mainly to hyperintensity signals in T2-weighted-MRI sequences, followed by cerebellar atrophy. As such, meningeal contrast enhancement could lead to a diagnostic pitfall as it can be considered a carcinomatosis - above all in serum antibody negative cases as well as when a metastatic tumour is found. Our patient had no tumoral cells in the cerebrospinal fluid and her neurological syndrome remained stable after immunoglobulin treatment. AYA serum finding was diriment for PCD diagnosis.

Neurological disorders are considered rare complications of immune-checkpoint inhibitor. We report a 63-year-old man with recurrence of melanoma who presented epilepsy, limbic encephalitis, cerebellar ataxia, and stiff person syndrome soon after treatment with nivolumab, an immune-checkpoint inhibitor. On autoimmune screening, serum and CSF GAD65 were detected. Significant response to steroids and intravenous immunoglobulins were observed, but cancer recurred after nivolumab discontinuation in parallel with epileptic seizure and worsening of cognitive impairment and the patient died. This case expands the spectrum of GAD65-associated conditions induced by immune-checkpoint inhibitor and underlines treatment complexity when both neurological complications and tumour recurrence occur.

Tonic pupil, or Adie’s pupil, is an autonomic disorder due to a loss of function of parasympathetic ciliary ganglion cells. Upon examination, the pupil appears tonically dilated and shrinks poorly to light, while reacting better to accommodation stimulus. The denervated iris sphincter is very sensitive to topical parasympathomimetic pilocarpine drops. Indeed, if Adie is present, the dilated pupil will constrict more than the normal pupil. Most cases are idiopathic or due to a viral infection and have a benign course. They usually occur in women between 20 and 40 years of age. Other reported causes of tonic pupils can be vascular, traumatic, inflammatory, paraneoplastic, and neoplastic. Breast cancer generally metastasizes to the brain in advanced stages and orbital localizations have sometimes been reported. In our case, a meningeal and retro-orbit cancer invasion caused a dysfunction of the parasympathetic fibers which lead to the pupil through the optical foramen. Indeed, first ophthalmic symptoms were only an enlarged pupil poorly responsive to light. This unusual clinical sign led to the discovery of an unknown breast cancer in an advanced stage. In young adult women, unilateral tonic pupils are considered a benign parasympathetic ocular disorder that is rarely observed in clinical practice. However, it can be found in numerous systemic diseases; thus instrumental and laboratory findings are mandatory. Tumors can also represent etiologic agents and secondary localizations from breast cancer to extra parenchymal CNS structures should be considered in differential diagnosis hypothesis.

This cross-sectional study has investigated the diagnostic and therapeutic management of patients suffering from multiple sclerosis (MS) in the Campania Region (Italy). A survey involving all the reference centers for MS in Campania Region was conducted from March to August 2011. Centers responded to a web-administered questionnaire on management and clinical characteristics of MS patients. In the study period, 3263 patients (mean age 37 years, 66 % females) accessed the centers. Patients received a first diagnosis of MS in 161 cases (4.9 %). About 37 % of the subjects without a previous diagnosis came to the centers on their own initiative. All patients underwent a complete neurological examination and expanded disability status scale. The other most common investigations were magnetic resonance imaging (44.0 %) and evoked potentials (22.1 %). The number of treated patients was 2797 (87.1 %). The most used drugs were interferon β and glatiramer acetate. The time between diagnosis and initiation of therapy exceeded 6 months in 32 % of cases. Second-line drugs were under-used: 16 % of patients who might benefit from them show high clinical and radiological disease activity despite treatment with immunomodulant drugs. The MS care management of the surveyed centers showed consistent margins for improvement in 2011. Even though these data do not represent the current situation, they can be used to monitor improvements in MS care.

Multiple sclerosis is an important chronic disorders of central nervous system that can lead to severe disability because of the presence of multiple demyelinating lesion. We report a 20-year-old woman with relapsing-remitting MS, with pediatric onset of MS, who developed aggressive disease with visual acuity lost (1/60 in both eyes). Frequent magnetic resonance imaging (MRI) detected persisting inflammatory activity and increase of lesion burden so she was submitted to natalizumab treatment with stability of MRI. After about 10 administrations of natalizumab, she improved her visual acuity in left eye (visual acuity 1/10); visual evocate potential show mild improvement of latency in left eye.