Brain and neurology
Neurology-related clinical indications
Many important neurological diseases are accompanied with an autoimmune pathophysiology, so making it difficult to treat using conventional approaches. Therapeutic apheresis has shown to be a valuable and important treatment option in neurological diseases such as Guillain-Barré syndrome, myasthenia gravis and other peripheral conditions. There has also been great interest in progress made using extracorporeal techniques to treat idiopathic encephalitis and a subset of patients with multiple sclerosis. Furthermore, a new field of research has begun evaluating the scope for therapeutic apheresis to improve the cognitive functions of patients with vascular dementia.
Guillain-Barré syndrome (GBS) is an acute-onset, usually monophasic immune-mediated disorder of the peripheral nervous system. Often, an unremarkable infection – such as an upper respiratory infection – predates the onset of GBS by 14 days. Typical initial symptoms are changes in sensation or pain along with muscle weakness. These begin in the feet and hands. Reported incidence rates for GBS are 1–2 per 100,000 individuals. To decrease the high plasma levels of IgG that are characteristic of GBS, the patient can be treated either with plasma exchange, which is a rather unselective method, or through the use of immunoadsorption techniques that selectively reduce IgG. LIGASORB® is a selective single-use, multiple-pass IgG adsorber for intervention in the acute exacerbation of autoimmune diseases.1
Myasthenia gravis (MG) is a long-term neuromuscular disease that leads to varying degrees of muscle weakness. Acquired MG is a disorder of neuromuscular transmission, resulting from the binding of autoantibodies to components of the neuromuscular junction, most commonly the acetylcholine receptor (AChR). This prevents nerve impulses from triggering muscle contractions. The incidence of MG ranges from 0.3 to 2.8 per 100,000 and the disease is estimated to affect more than 700,000 people worldwide. Current treatment options for long-term therapy are cholinesterase inhibitors, immunosuppression and thymectomy. In acute crises, the rapid reduction of elevated IgG levels is the main goal. This can be achieved using either plasma exchange, which is a rather unselective method, or immunoadsorption techniques. LIGASORB® is a selective single-use, multiple-pass IgG adsorber for intervention in the acute exacerbation of autoimmune diseases.1
Autoimmune encephalitis was only recently recognised and is increasingly diagnosed as a significant brain illness that is related to a malfunctioning immune system. Symptoms are the alteration of consciousness, cognitive decline, seizure, abnormal movements and more serious neuronal disorders. Autoantibodies are directed against structural proteins that are expressed on the surface of neurons in the CNS. Immunosuppressive treatment with drugs, plasma exchange and IVIG may prove insufficient. The use of immunoadsorption in addition to immunosuppression therapy in patients with surface antibodies may accelerate recovery.2
Multiple sclerosis (MS) is the most common disease of the central nervous system in young adults. It involves an immune-mediated process directed against the myelin cells of the central nervous system (CNS). Symptoms are unpredictable, but it is often a disabling disease. The use of therapeutic plasma exchange or immunoadsorption to remove antibodies is indicated in patients who do not respond, or only weakly respond, to immunosuppressive therapy.3,4
Besides the well-known hypothesis concerning the deposition of amyloid beta plaques, vascular dementia also seems to play an important role in the development and progression of cognitive impairment. New insights shed light on agonistic autoantibodies directed against the adrenergic receptors of vessels (predominantly alpha-1), causing a malperfusion of cerebral structures. This gives rise to the thesis that the use of semi-selective immunoadsorption to eliminate these autoantibodies might benefit patients with dementia. Recent studies have shown that patients diagnosed with Alzheimer’s dementia suffer from IgG antibodies in approximately 40% of cases.5 An applied immunoadsorption with Immunosorba® is known to have put a stop to cognitive deterioration.6 Further studies are currently running to confirm the validity of this novel treatment approach.
In addition to the aforementioned indications, therapeutic apheresis can also be applied to:
- Chronic inflammatory demyelinising polyneuropathy (CIDP) 7, 8
- Lambert-Eaton syndrome 9
- Morvan’s syndrome 10
1 Instruction for use (IFU) LIGASORB®, Art. Nr. F00001564, 35840318 / Mar.–May 2016
2 Dogan et al, Immunoadsorption therapy in autoimmune encephalitides. Neurology 26 Feb. 2016; 3(2): 207. Retreived from http://nn.neurology.org
3 Dorst et al, (2016) Immunoadsorption with regenerating columns in treatment of steroid-refractory relapse in multiple sclerosis and optic neuritis. Multiple Sclerosis Journal (Foster City) 3: 178.
4 Schneidewind et al, Immunoadsorption: A new therapeutic possibility for multiple sclerosis? Transfusion Science Mar. 1998; 19 Suppl: 59–63.
5 Karczewski et al, Agonistic autoantibodies to the α(1) -adrenergic receptor and the β(2) -adrenergic receptor in Alzheimer's and vascular dementia. Scandinavian Journal of Immunology May 2012; 75(5): 524–30.
6 Hempel et al, Immunoadsorption of agonistic autoantibodies against α1-adrenergic receptors in patients with mild to moderate dementia. Therapeutic Apheresis and Dialysis Oct. 2016; 20(5): 523–29.
7 Zinman et al, A pilot study to compare the use of the Excorim staphylococcal protein immunoadsorption system and IVIG in chronic inflammatory demyelinating polyneuropathy. Transfusion and Apheresis Science Nov. 2005; 33(3): 317-24.
8 Rech et al, Remission of demyelinating polyneuropathy with immunoadsorption, low dose corticosteroids and anti-CD20 monoclonal antibody. Therapeutic Apheresis and Dialysis June 2008; 12(3): 205–8.
9 Baggi et al, Effect of IgG immunoadsorption on serum cytokines in MG and LEMS patients. Journal of Neuroimmunology 15 September 2008; 201/202: 104–10.
10 Schwartz et al, Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: The sixth special issue. Journal of Clinical Apheresis 28 July 2013; 28(3): 145–284.