October NEWSLETTER
Selected Highlighted News in the field of Autoimmune
Encephalitis October 2018 2nd edition
In this issue: Autoimmune Neurology Patient Day Nov-3rd Salt Lake City, Utah, faciobrachial dystonic seizures (FBDS) predict cognitive decline in anti LGI1 limbic encephalitis, FBDS video, MRI investigation on LGI1 (FBDS). Award winning study of EEG predictors of outcome in NMDAR antibody encephalitis, Sure to be ‘go to’ paper on Movement Disorders in AE
The importance of early immunotherapy in patients with faciobrachial dystonic seizures (LGI1 Limbic Encephalitis)
If you have LGI1 antibody, which is the 2nd most common type of Autoimmune Encephalitis, you may also have Faciobrachial dystonic seizures. This is a type of seizure specific to LGI1 where the patient has a face twitching. This paper talks about something significant: faciobrachial dystonic seizures predict cognitive decline in anti LGI1 limbic encephalitis.
This study, the largest available series of FBDS, describes the short- and long-term impacts of delay to treatment. It demonstrated the importance of rapid clinical recognition and prompt treatment of FBDS in the prevention of cognitive impairment.
Noteworthy: Despite the sensitivity of FBDS to immunotherapy, and the successful prevention of ensuing cognitive impairment, patients with cognitive impairment showed significant residual disability at 4 years. These observations suggest that although seizure recognition and termination improve long-term outcomes, an unmet medical need frequently persists. Given the observed IgG4-subclass predominance suggests early rituximab may be effective but has, to date, only been trialed relatively late in the course of patients with LGI1-antibodies.
For AE patients who are positive for the LGI1 antibody and Fasciobrachial dystonic seizures (FBDS).
This is the first functional MRI investigation on autoantibody-associated Fasciobrachial dystonic seizures (FBDS). faciobrachial dystonic seizures (FBDS) are characterized by very frequent, short-lived seizures that typically affect the arm and face and these seizures have been identified as an early sign of certain forms of autoimmune encephalitis (LGI1 Limbic encephalitis).
Conclusion:
Functional characteristics of the medial temporal lobe (MTL) in patients treated for autoimmune encephalitis with FBDS may describe clinical features and parameters beyond that of structural damage, though the influence of subclinical seizure activity on these metrics cannot be ruled out. These functional imaging features may also serve as optimal biomarkers for treatment trials targeting seizures and chronic memory impairments.
IAES Congratulates Clinical Research Fellow Dr. Graham
Blackman who was awarded the Adrian Prize by the British
Society of Clinical Neurophysiologists for his study of EEG
predictors of outcome in NMDAR antibody encephalitis!
Fantastic work!”
Results: The commonest presenting symptoms were confusion, followed by psychosis. This is the first study to demonstrate that EEG may be a potentially useful prognostic tool in patients with anti-NMDAR encephalitis. In particular, early findings suggest patients with anti-NMDAR encephalitis with greater slow wave activity on their admission EEG have worse long term outcome using either qualitative or quantitative techniques.
If you have an antibody that has a movement disorder, this
is THE PAPER for you!
Open Access paper from the Oxford Group~ The Clinical Features, Underlying Immunology, and Treatment of Autoantibody‐mediated Movement Disorders. Includes a GREAT table of all the antibodies with movement disorders and sections on each one. It is going to be the ‘go to’ paper to answer your questions about this as well as treatment.
Dr. Irani said about this paper: “Our review in Movement Disorders aims to tie in clinical observations with the likely underlying immunology. Still lots to prove but disease models are definitely coming together.”
International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.
Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles. Raising awareness of Autoimmune Encephalitis across the many specialties of medicine that come in contact with these disorders and with the general public to ensure a timely and accurate diagnosis resulting in an aggressive treatment plan for best outcomes.
“Empowering victims of Autoimmune Encephalitis and their caregivers through Support and Education”.
