Update in neurology
The 18th Annual Neurology Update Meeting will take place on Friday, 18 October 2019 in the Hilton Hotel, Lanyon Place, Belfast. This meeting is intended to provide information and insights into new diagnosis and treatment of neurological conditions for clinicians. Consultant physicians, general practitioners, specialist nurses and allied health professionals, in addition to neurologists and doctors in all areas of training, are invited to attend. The meeting is worth 6 CPD credits.
Prof Sarosh R Irani will deliver the guest lecture at the meeting. Prof Irani is head of the Oxford Autoimmune Neurology Group, and Co-director, Oxford Autoimmune Neurology Group, Diagnostic Laboratory. He is a consultant neurologist and clinician-scientist with clinical and laboratory experiences in the field of autoantibody-mediated diseases of the nervous system, in particular the central nervous system. He runs a dedicated clinic for the care of these patients, and runs a research group of 20 clinicians and scientists to learn more about the origins and treatments of these diseases.
The title of Prof Irani’s lecture is ‘Autoimmune Encephalitis’. Autoimmune encephalitis refers to a group of conditions that occur when the body’s immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain. People with autoimmune encephalitis may have various neurologic and/or psychiatric symptoms. Neurologic symptoms may include impaired memory and cognition, abnormal movements, seizures, and/or problems with balance, speech, or vision. Psychiatric symptoms may include psychosis, aggression, inappropriate sexual behaviours, panic attacks, compulsive behaviours, euphoria or fear. Symptoms may fluctuate, but they often progress over days to a few weeks. Symptoms can progress to loss of consciousness or even coma.
It is now well established that a substantial proportion of encephalitides are associated with autoantibodies directed against the extracellular domains of cell-surface proteins, which are critical in the regulation of neuronal excitability. These include LGI1, CASPR2, contactin-2 (VGKC-complex antibodies), and the NMDA, AMPA, and GABAB receptors. The clinical importance of these conditions lies in their frequent immunotherapy-response and, less commonly, their association with distinctive tumours. Studies that have examined cohorts of patients defined by these serum antibodies have identified a number of clinical features that have helped understand the core phenotypes of these conditions. In addition, sensitive antibody assays have allowed the expansion of the phenotypes to include a minority of patients with isolated epilepsies or psychoses. There is also evidence that autoimmune encephalitis may progress to adult-onset hippocampal sclerosis. Clinical, and accumulating scientific, data strongly suggest direct pathogenicity of these autoantibodies. The generation of the autoantibody, in some patients, can be explained by the presence of tumours which express their antigenic target. Serum antibody levels are higher than their levels in CSF in the vast majority of cases. However, the majority of patients do not harbour a tumour. Early treatment is critical, particularly through immunotherapy.
Early immunotherapy for faciobrachial dystonic seizures that precede the onset of limbic encephalitis is highly effective in preventing cognitive impairment and long-term disability, research in which Prof Irani has been involved has found.
The meeting will also cover a range of other neurological conditions. In the opening session, Professor of Human Genetics in the Royal Victoria Hospital (RVH), Belfast, Prof Patrick Morrison, will talk about ‘Neurogenetics — Pitfalls and Benefits of Testing’.
Consultant Neurologist in Altnagelvin Hospital, Derry, Dr Mark McCarron, will deliver a presentation on cerebral amyloid angiopathy. Hereditary cerebral amyloid angiopathy is a condition that can cause a progressive loss of intellectual function (dementia), stroke, and other neurological problems starting in mid-adulthood. Due to neurological decline, this condition is typically fatal to people in their sixties, although there is variation, depending on the severity of the signs and symptoms. There are many different types of hereditary cerebral amyloid angiopathy. The various types of hereditary cerebral amyloid angiopathy are named after the regions where they were first diagnosed.
The Dutch type of hereditary cerebral amyloid angiopathy is the most common form. Stroke is frequently the first sign of the Dutch type and is fatal in about one-third of people who have this condition. Survivors often develop dementia and have recurrent strokes. About half of individuals with the Dutch type who have one or more strokes will have recurrent seizures (epilepsy). Consultant Neurologist in Beaumont Hospital, Dublin, Prof Norman Delanty will end the session with a talk on ‘When to Start and Stop Anticonvulsants’.
Session III of the meeting, which will take place following the guest lecture, will consist of talks on ‘Subarachnoid Haemorrhage — Diagnosis, Investigation and Treatment’ and ‘MS Relapses — Do’s and Don’ts’. The talks will be delivered by Consultant Neurosurgeon in RVH, Belfast, Mr Neil Simms and Consultant Neurologist in RVH, Belfast, Dr Stella Hughes, respectively.
In the final session, Clinical Research Training Fellow at the University of Edinburgh Dr Ingrid Hoeritzauer will talk about functional neurological disorders, while Consultant Neurologist in RVH, Belfast, Dr Michael Kinney, will give a presentation on evidence-based management of status epilepticus.