Clinical Neuroscience

[Treatment and new evidences in neuromyelitis optica spectrum disorder ]

ILLÉS Zsolt1,2

SEPTEMBER 30, 2021

Clinical Neuroscience - 2021;74(09-10)

DOI: https://doi.org/10.18071/isz.74.0309

Review

[Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9–10):309–321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.]

AFFILIATIONS

  1. Pécsi Tudományegyetem, Neurológiai Klinika, Pécs
  2. Department of Neurology, Odense University Hospital, University of Southern Denmark, Odense, Dánia

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

SANZ-MENGIBAR Jose Manuel , MENENDEZ-PARDIÑAS Monica , SANTONJA-MEDINA Fernando

[Vojta therapy has been reported as clinically beneficial for strength, movement and gross motor activities in individual cases and is being included within the second of three levels of evidence in interventions for cerebral palsy. The goal of this study is to understand the effect of Vojta therapy on the gross motor function. Our clinical trial followed a one group, pre-post design to quantify rates of changes in GMFM-88 after a two-months period undergoing Vojta therapy. A total of 16 patients were recruited. Post-intervention acceleration rates of GMFM-88-items acquisition (0.005; p<0.001) and Locomotor Stages (1.063; p<0.0001) increased significatively following Vojta the­rapy intervention. In this study, Vojta therapy has shown to accelerate the acquisition of GMFM-88-items and Loco­motor Stages in children with cerebral palsy younger than 18 months. Because functional training was not utilised, and other non-Vojta therapy intervention did not influence the outcome, Vojta therapy seems to activate the postural control required to achieve uncompleted GMFM-88-items. ]

Clinical Neuroscience

[Mentalizing deficit among patients with traumatic brain injury ]

BÜKI András, HEROLD Róbert, TAMÁS Viktória

[Mentalization or theory of mind as an aspect of our social cognition, is our ability to infer mental states of others (intentions, desires, thoughts, emotions) and to predict their behavior accordingly. This function significantly affects our participation and orientation in the social world and plays an important role in conversational situations, social interactions, social integ­ration and adaptation. The brain regions that serve as the basis for mind-reading function can be damaged as a consequence of traumatic brain injury, which frequently occurs among the younger population. Traumatic brain injury can cause focal or diffuse cerebral injuries, often leading to theory of mind deficit. In this topic such publications were researched that compared theory of mind ability between traumatic brain injury patients and control subjects (comparative case-control studies). We searched for the studies in the following internet based/online databases: PubMed, Web of Science, ScienceDirect, Google Scholar, APA PsycNET (PsycARTICLES) and EBSCO Host. The search was performed using the following key word combinations: theory of mind or mentalizing or social cognition AND traumatic brain injury or head/brain injury or diffuse axonal injury. Based on the results of the included and processed studies (21 pc), traumatic brain injury often leads to mentalization deficit with different severity. With this present review we aim to draw attention to the fact that the appearance and severity of mind reading dysfunction can considerably affect the outcome of the disease, the length of rehabilitation time and the prognosis of traumatic brain injury patients. Besides this, with this review, we aim to take sides in whether theory of mind ability is domain-specific or domian-general based on studies including traumatic brain injury patients.]

Clinical Neuroscience

Medication overuse headache: The effectivity of iv lidocaine – magnesium

TEPE Nermin, TERTEMIZ Faysal Oktay

The detoxification process in medication overuse headache is the most difficult process for the patient. We aimed to investigate the effectiveness of the combination of low dose IV lidocaine and magnesium (100 mg lidocaine and 1.25 mg magnesium) in patients with medication overuse headache during the detoxification process. A total of 30 patients were included in the study; 15 received 24 hours of IV hydration, 15 received 1-hour lidocaine-magnesium infusion at the onset of pain in addition to the 24 hours of IV hydration. Headache severity (numeric rating scale, NRS), attack durations, onset of headache, monthly analgesic/triptan intakes, numbers of monthly headache days data were documented. We evaluated the severity of headache before and after daily treatment of two groups for one week. When both groups were compared, there was no significant difference in the pre-treatment NRS values, whe­reas, in the group receiving IV lidocaine-magnesium combination, there was a statistically significant decrease in the post-treatment NRS values in the first five days (p <0.05). An 1-hour combined infusion of lidocaine-magnesium may be considered as an alternative option for the patient to have a more quality detoxification process during the hospital stay, so that in parallel to the reduction in the use of multiple treatments (such as neuroleptics, benzodiazepines, antiemetics and opioids) and duration length of stay, the economic costs can also be reduced. The administration of combination will bring fewer side effects compared to their administration separately.

Clinical Neuroscience

[The examination of burnout among healthcare workers]

FEJES Éva, MÁK Kornél, POHL Marietta, BANK Gyula, FEHÉR Gergely, TIBOLD Antal

[Health reforms in recent decades have been largely based on economic considerations and have led to a significant problem in the sector today, with the issue of human resources being pushed back, which is exacerbated by burnout syndrome. The aim of this questionnaire-based study was to examine the complex background of burnout among health care workers in the cities of Komló, Pécs and Kecskemét. Baseline demographic data were recorded. Burnout was assessed by the Maslach Burnout Inventory (MBI), and the intensity of dysfunctional attitudes were also studied. Depression was detected by the Beck Scale and social supports, and effort-reward dysbalance were also examined. Overall 411 employees participated in our study. Age group distribution was middle aged access, vast majority of the workers was between 36 and 55 years. Mean burnout scale was 58.6 (SD = 16.3), 63 workers had mild (14.2%), 356 had moderate (80.7%) and 22 had severe (5.1%) burnout. In a multivariate analysis the type of work (OR = 1.018), age (OR = 2.514), marital status (OR = 1.148), job type (OR = 1.246) the lack of social support (OR = 1.189) and allowance (OR = 9.719) were independently associated with burnout (p < 0.05 in all cases). There was a significant association among burnout, depression and dysfunctional attitudes. The vast majority of our social workers suffered from moderate and a small, but significant proportion suffered from severe burnout. Our work draws attention to the modifiable and unmodifiable risk factors of burnout in this population, which may help in the development of preventive strategies.]

Clinical Neuroscience

Chronic form of Pisa syndrome after prolonged exposure to low-dose amisulpride treatment

ERDEM Şimşek Nazan, ÖZKAYNAK Sibel Sehur

Pisa syndrome is a movement problem defined by tonic, sustained lateral flexion with a slight posterior rotation of the trunk. It seems to be a side effect of antipsychotic medicine in most cases. The clinical duration of Pisa syndrome can be acute, chronic, or recurrent. As far as we know, no reports are available in the literature on the chronic form of Pisa syndrome caused by low-dose amisulpride. A case of refractory tardive dystonia form of Pisa syndrome during treatment with stable low-dose amisulpride is presented in this report. Long-term, low-dosage amisulpride therapy may induce tardive dystonia even in patients with no other risk factors for dystonia.

All articles in the issue

Related contents

Clinical Neuroscience

CANOMAD syndrome with respiratory failure

VÉCSEI László, RAJDA Cecília, KLIVÉNYI Péter, HORTOBÁGYI Tibor, DÉZSI Lívia, SALAMON András, RADICS Bence, VARGA Tímea Edina, TÖMÖSVÁRI Adrienn

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

[Rituximab therapy in rheumatoid arthritis]

SÜTŐ Gábor

[Rheumatoid arthritis is a chronic, lifelong disease that causes severe joint deformity, reduces quality of life, and, if not treated appopriately, leads to disability and substantial premature mortality. Its treatment is a multistep procedure, where different grades of treatment options follow each other. Besides traditional, diseasemodifying antirheumatic drugs (DMARDs) and biological therapies inhibiting TNF, a new therapautic option is the use of a chimeric antibody, rituximab, which inhibits B lymphocyte function. This drug is an effective and safe choice for those patients who have received various anti- TNF therapies or do not tolerate TNF inhibition.]

Clinical Neuroscience

[Devic syndrome - case report, diagnostic and therapeutic principles]

BERECZKI Dániel, BARSI Péter, VÁRALLYAY György, RUDAS Gábor, SIMÓ Magdolna, ILJICSOV Anna, TÁTRAI Erika, SOMFAI Gábor Márk

[Neuromyelitis optica (NMO, Devic-syndrome) is a rare, relapsing autoimmun disease of the central nervous system, which is distinguished from other demyelinating disorders by a recently identified, specific autoantibody. By demonstrating the anti-aquaporin-4 IgG in the serum, a heterogenous group of syndromes can be defined, called NMO-spectrum. In the future, optical coherence tomography may support this diagnosis besides the clinical features, imaging examinations and presence of serum antibody. Early recognition and treatment can improve clinical outcome even in serious condition. Long-term immunosuppressive therapy is advised to prevent further relapses and to stabilize or improve clinical status. Hereby, we report a case of a 51-year-old woman, under treatment for 1.5 years. We summarize the up-to-date knowledge about the pathomechanism, diagnostic strategy and therapy of neuromyelitis optica. We review recent findings and the diagnostic value of a new, non-invasive ophtalmological examination, the optical coherence tomography. According to the first results, this method may be helpful in the early differential diagnosis of optic neuritis.]

Lege Artis Medicinae

[Rituximab therapy in idiopathic inflammatory myopathies]

GRIGER Zoltán, DANKÓ Katalin, HORTOBÁGYI Tibor, BODOKI Levente, VINCZE Melinda, CSONKA Tamás, CSERI Karolina

[Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by symmetrical weakness of proximal limb muscles. In the everyday practice it is not easy to treat idiopathic inflammatory myopathies. First-line therapy is based on corticosteroids. If there is no improvement in the symptoms and complaints in two months, a new immunosuppressant therapy has to be initiated. The aim of this summary is to present the biological agent rituximab in the therapy of this condition.]

Hypertension and nephrology

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 1]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy, both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]