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utcome.
Clinical Trials Gov NCT03185234, registered 14 June 2017 ; Deutsches Register für Klinische Studien DRKS00012292, registered 01 June 2017.
Participant enrollment began on 22 June 2017. The trial is expected to be completed on 30 June 2022.
Participant enrollment began on 22 June 2017. The trial is expected to be completed on 30 June 2022.
Over the past decade increasing scientific progress in the field of autoantibody-mediated neurological diseases was achieved. Movement disorders are a frequent and often prominent feature in such diseases which are potentially treatable.
Antibody-mediated movement disorders encompass a large clinical spectrum of diverse neurologic disorders occurring either in isolation or accompanying more complex autoimmune encephalopathic diseases. Since autoimmune movement disorders can easily be misdiagnosed as neurodegenerative or metabolic conditions, appropriate immunotherapy can be delayed or even missed. Recognition of typical clinical patterns is important to reach the correct diagnosis.
There is a growing number of newly discovered antibodies which can cause movement disorders. Several antibodies can cause distinctive phenotypes of movement disorders which are important to be aware of. Early diagnosis is important because immunotherapy can result in major improvement.In this review article we summarize the c
Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. compound library inhibitor The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed.
Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not.
With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.Management of primary orthostatic tremor (POT) remains challenging, and medication is often ineffective. We report the case of a 53-year-old female with orthostatic tremor for 6 years who was refractory to gabapentin, clonazepam, primidone and propranolol. After treatment with 4 mg/day perampanel, she reported almost complete resolution of tremor. The diagnosis of POT was confirmed by tremor analysis using surface electromyography. Our report shows the potential use of the novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist perampanel for the treatment of POT. To date, only two similar patients, one refractory to treatment and the other previously treated with clonazepam only, have been reported. We would like to note that our patient was refractory to all previous therapy and responded to a low dose of perampanel without side effects. The striking clinical improvement suggests a putative role of glutamate in the pathophysiology of orthostatic tremor.We present the case of an 18 year old Caucasian with known celiac disease, who suffered a severe first attack of acute intermittent porphyria (AIP) with neuropsychiatric symptoms, severe tetraparesis and respiratory insufficiency. Treatment with heme arginate and high-dose intravenous glucose and rigorous rehabilitation resulted in a slow but almost complete recovery of her motor symptoms. link2 To our knowledge this is the first case of acute intermittent porphyria triggered by malnutrition in the context of celiac disease. It is remarkable that the patient showed a favourable outcome despite the severity of her initial symptoms. This case shows the importance of early and systematic symptomatic treatment in patients with severe neurologic manifestation of AIP.
Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AE-specific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare.
This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses.
Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided within an accompanying text and a table describing the most important autoantibodies and their characteristics.
The initial steps of the AE flow chart are based on clinical symptoms and the patient's history. Assignment to paraneoplastic or antibody-mediated AE is sometimes clinically possible. Diagnostics should include MRI, EEG and CSF analysis with antibody panel diagnostic. Definite AE can be diagnosed if the underlying antibody is compatible with the clinical presentation. Classification of probable AE may be possible even with negative anti-neuronal autoantibodies if the clinical presentation and laboratory abnormalities are highly suggestive of AE. The confirmed AE diagnosis requires immediate initiation of immunotherapy.
The SOP facilitates the recognition of patients with AE and presents the necessary diagnostic and therapeutic steps.
The SOP facilitates the recognition of patients with AE and presents the necessary diagnostic and therapeutic steps.
Many stroke survivors suffer recurrent stroke because paroxysmal atrial fibrillation (AF) was missed and no preventive anticoagulation initiated. This prospective cohort study determined the added diagnostic yield of second-look 24-h electrocardiographic recording (ECG) in a population at high risk for AF patients who suffered a stroke of such severity that they require inpatient neurorehabilitation.
We enrolled 508 patients with ischemic stroke admitted to post-acute inpatient neurorehabilitation and determined whether AF was detected during acute care at the referring hospital. Second-look baseline and 24-h Holter ECG were then conducted during neurorehabilitation. Primary outcome was number of newly detected AF with duration of > 30 s; secondary outcomes were number of newly detected absolute arrhythmia of 10-30 s and < 10 s duration. For comparison, we further enrolled 100 patients with hemorrhagic stroke without history of AF (age = 72+11 years, 51% female).
In 206 of the 508 ischemic stroke patients, AF had been detected during acute phase work-up (age = 78+10 years, 55% female). For the remaining 302 ischemic stroke patients, no AF was detected during acute phase work-up (age = 74+9 years; 47% female). Second-look 24-h ECG showed previously missed AF of > 30 s in 20 of these patients, i.e. link3 6.6% of the sample, and shorter absolute arrhythmia in 50 patients (i.e. 16.5%).
Second-look 24-Hour ECG performed during post-acute inpatient neurorehabilitation has a high diagnostic yield and should become a standard component of recurrent stroke prevention.
Second-look 24-Hour ECG performed during post-acute inpatient neurorehabilitation has a high diagnostic yield and should become a standard component of recurrent stroke prevention.
Multiple sclerosis is one of the most prevalent neurological diseases in young adults affecting over 2 million people worldwide. Alemtuzumab is a highly effective therapy in relapsing remitting MS. Alemtuzumab is a monoclonal CD52 antibody that proved its efficacy against an active comparator (interferon [IFN]-β1a) in a phase II trial and two phase III trials regarding clinical and MRI outcomes. Nevertheless, the exact mode of action is still unknown. Alemtuzumab is commonly associated with secondary autoimmune disorders significantly affecting the risk-benefit ratio. Therefore, new biomarkers predicting treatment response and adverse events are urgently needed. This study aims to further elucidate the mechanism of action of the neuroprotective potential of alemtuzumab in relapsing-remitting multiple sclerosis (RRMS).
This is a 3-year multicentre, explorative study including overall 150 patients comprising three different groups (i) de novo patients prior and after alemtuzumab treatment initiation, (ii) plying immunological changes in a longitudinal analysis of alemtuzumab treated RRMS patients. By combining clinical, radiological and functional immune-phenotype data, we will be able to identify biomarkers and/or immune signatures predicting treatment response and adverse events. Thereby, the understanding of the mechanisms of action of alemtuzumab will improve its efficacy and safety for present and future patients.
Comprehensive administrative data on TIA and stroke cases and treatment modalities are fundamental for improving structural conditions and adjusting future strategies of stroke care.
The nationwide administrative database (German federal statistical office) was used to extract all adult inpatient TIA and stroke cases and corresponding procedural codes for the period 2011-2017. Numbers were specified according to age, sex, stroke unit (SU) and critical care treatment (ICU), early transfer, and in-hospital mortality.
Inpatient adult TIA/stroke cases increased from annually 102,406 / 250,199 (2011) to 106,245 / 264,208 (2017). 84% of strokes were ischemic (AIS) also having the highest relative increase most likely due to more accurate coding within the time period, 68.2% of AIS were treated on SUs. 78% of hemorrhagic strokes were intracerebral hematomas (ICH; rather than subarachnoid hemorrhages [SAH]). Hemorrhagic strokes were increasingly treated on SUs (32.6% [2011], 37.8% [2017]). 68.8% of SAH were treated on ICUs (ICH36.3%, AIS10.3%). Early transfer in AIS increased (2.0 to 3.1%). Hemorrhagic strokes were associated with higher in-hospital mortality (SAH19.6%, ICH28.2%, AIS7.3%).
The absolute increase of strokes presumably reflects the aging society and more awareness for cerebrovascular disease. The relative increase of AIS may be attributable to an increased neurological expertise. The increasing amount of early transfers in AIS reflects new specialized treatment options. Our findings reflect the need for structural adjustments in inpatient stroke care.
The absolute increase of strokes presumably reflects the aging society and more awareness for cerebrovascular disease. The relative increase of AIS may be attributable to an increased neurological expertise. The increasing amount of early transfers in AIS reflects new specialized treatment options. Our findings reflect the need for structural adjustments in inpatient stroke care.
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