Autoimmune encephalitis is a rare neurological disease, which makes it difficult to study and limits the development of tools capable of accurately predicting its course. Currently, prognostic scales are scarce and often overlook clinical and paraclinical variables that have an impact on the condition. To address this, researchers from the Neurodegeneration and Neuroinflammation Research Group at the Girona Biomedical Research Institute (IDIBGI), which is linked to the Neuroimmunology and Multiple Sclerosis Unit (UNIEMTG) of the University Hospital of Girona Dr. Josep Trueta and Santa Caterina Hospital in Salt, have developed a new scale called the ‘Girona Score’ to more precisely identify patients at greater risk of developing disability after the acute phase of the disease.
“Current prognostic scales are not entirely comprehensive, as they do not include clinical and radiological parameters that we commonly use in the diagnosis of autoimmune encephalitis. With our scale, we incorporate new variables to obtain a more accurate prognosis of disease progression,” explains Gary Álvarez, neurologist and current lead of UNIEMTG, IDIBGI researcher, and one of the main developers of the ‘Girona Score’.
Among the variables included in the new scale are those capable of measuring dysautonomia—a term referring to disturbances of the autonomic nervous system, which affect blood pressure, heart rate, and body temperature. Other variables assess changes in the patient’s level of consciousness.
“The first results of the ‘Girona Score’ have shown that dysautonomia and altered mental status are very important factors in predicting poor outcomes at three months,” Álvarez highlights. Based on these findings, the unit has been able to anticipate the need for pharmacological treatment with the goal of reducing the risk of long-term disability or irreversible neurological sequelae, such as residual epilepsy, cognitive decline, or impaired gait stability.
The scale consists of 14 variables, and the first results were obtained from a retrospective analysis of 26 patients diagnosed with autoimmune encephalitis. Each variable is scored between one and three points, and those with higher total scores were found to have worse prognoses.
The next step of the study will be to prospectively include new patients diagnosed with autoimmune encephalitis and analyze their progression based on their ‘Girona Score’ during the acute episode. This phase will involve collaboration with other national and international centers interested in implementing the scale. The objective is to ensure that the results from the initial analysis are also externally reproducible. “If we observe that similar results are obtained from the ‘Girona Score’ in other settings, it would suggest the scale could be used as a standard tool in units from other centers,” Álvarez adds. Contacts have already been established with several institutions to expand the study in the coming months.
Autoimmune encephalitis
The ‘Girona Score’ is the result of research conducted by the UNIEMTG team through the Neurodegeneration and Neuroinflammation Research Group at IDIBGI, with the aim of increasing knowledge of autoimmune encephalitis and actively seeking effective treatments to prevent disability. It is a disease in which the immune system mistakenly attacks the brain, causing inflammation. This can lead to altered mental status, seizures, memory problems, or movement disorders. Its prevalence in developed countries is low. In Spain, according to 2023 data from the Spanish Society of Neurology (SEN), around 1,200 cases are diagnosed each year, although the actual number is likely higher due to underdiagnosis and symptom overlap with other conditions.
Currently, the UNIEMTG at the Martí i Julià Hospital Park in Salt is treating 30 patients with autoimmune encephalitis, providing appropriate treatment and follow-up care. The unit is the referral center in the Girona Health Region for the diagnosis, management, and treatment of patients with central nervous system disorders.
In addition to encephalitis, the unit also treats conditions such as multiple sclerosis—the leading cause of physical and cognitive disability of neurological origin in young adults—and neuromyelitis optica.
Reference article: Álvarez Bravo G, Guglielmini G, Quiroga Varela A, Boix Lago A, Gifreu Fraixinó A, Urso D, Logroscino G, Ramió-Torrentà L. Assessment of clinical prognosis in autoimmune encephalitis: Girona score. Front Psychiatry. 2025 Jan 29;16:1447009. doi: 10.3389/fpsyt.2025.1447009. PMID: 39944213; PMCID: PMC11814168.
Photography credits: Hospital Universitari de Girona Dr. Josep Trueta