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Autoimmune encephalitis (AIE) is a type of encephalitis, and one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. The clinical manifestations include behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. Autoimmune encephalitis can result from a number of autoimmune diseases including: Rasmussen encephalitis Systemic lupus erythematosus Behçet's disease Hashimoto's encephalopathy Autoimmune limbic encephalitis Sydenham's chorea The severity of the condition can be monitored using the Modified Rankin Scale and the clinical assessment scale in autoimmune encephalitis (CASE) score. Signs and symptoms Patients with AIE may present movement disorders such as ataxia, dystonia, myoclonus, and orofacial dyskinesia. Seizures are the most common symptom and different types of seizures may be seen, including refractory status epilepticus. Autonomic disturbances such as sweating, hypertension, tachycardia and hypoventilation are also frequent. Some patients may develop gastrointestinal manifestations (diarrhea, gastroparesis, and constipation) due to involvement of the myenteric plexus. Sleep disturbances such as insomnia, abnormal sleep movements, sleep apnea, and hypersomnia are also found. Some of these findings are suggestive of certain types of encephalitis and may indicate a specific underlying antibody or tumor. Mechanism Autoimmune encephalitis commonly presents an immune response against neuronal autoantigens with production of antibodies. Anti-neuronal antibodies are classified into antibodies against cell surface antigens (CSAab), antibodies against synaptic antigens (SyAab) and antibodies against intraneuronal antigens (INAab), also known as onconeural antibodies. Diagnosis Diagnostic criteria for possible autoimmune encephalitis (all three of the following criteria met): Subacute onset (rapid progression of less than three months) of working memory deficits (short-term memory loss), altered mental status (decreased level of consciousness, lethargy or personality changes), or psychiatric symptoms At least one of the following: New focal central nervous system findings Seizures not explained by previously-known seizure disorder Cerebrospinal fluid pleocytosis Magnetic resonance imaging suggestive of encephalitis Reasonable exclusion of alternative causes Criteria for autoantibody-negative but probable autoimmune encephalitis (all four criteria met): Subacute onset (rapid progression of less than three months) of working memory deficits (short-term memory loss), altered mental status (decreased level of consciousness, lethargy or personality changes), or psychiatric symptoms Exclusion of well-defined syndromes of autoimmune encephalitis (typical limbic encephalitis, Bickerstaff brainstem encephalitis, acute disseminated encephalomyelitis) Absence of well-characterized autoantibodies in blood serum and cerebrospinal fluid, and at least two of the following criteria: Magnetic resonance imaging suggestive of encephalitis CSF pleocytosis, oligoclonal bands or elevated cerebrospinal fluid IgG index, or both Brain biopsy showing inflammatory infiltrates and excluding other disorders Reasonable exclusion of alternative causes Classification Anti-NMDAR encephalitis Anti-N-methyl-D-aspartate receptor encephalitis is one of the most common causes of AIE and was originally described in 2007 in a cohort of 12 patients, 11 of them with ovarian teratomas. This condition predominantly affects children and young female patients. Underlying malignancies are found mainly in patients between the age of 12–45 years; most of them are ovarian teratomas (94%), followed by extraovarian teratomas (2%), and other tumors (4%). Herpes simplex virus-1 encephalitis appears to be a trigger for anti-NMDAR encephalitis; most AIE cases after herpes zoster are now believed to be anti-NMDAR encephalitis. Anti-AMPAR encephalitis Patients with anti-Α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (anti-AMPAR) encephalitis characteristically present with seizures, memory impairment and psychosis. Some may develop sleep disturbances and movement disorders. Anti-AMPAR encephalitis is paraneoplastic in etiology in 64% of cases, mostly associated with thymoma, ovarian teratoma and lung and breast cancer. Brain MRI shows T2 and FLAIR hyperintensities, particularly in the medial temporal lobe. Lesions in the brain cortex or subcortex, sometimes with demyelination, may also be found. Cerebrospinal fluid (CSF) examination may show pleocytosis and oligoclonal bands. Anti-GABA encephalitis Anti-GABA-AR encephalitis Anti-gamma-aminobutyric acid A receptor (anti-GABA-AR) encephalitis was first reported in 2014 in six patients (two male children, one female teenager and three male adults). They developed a rapidly progressive encephalopathy with early behavioral or cognitive changes that evolved with refractory seizures and multifocal lesions as seen on brain magnetic resonance imaging. In most of these patients, CSF analysis showed lymphocytic pleocytosis. A recent study identified an underlying neoplasia in 27% of these patients, mostly thymomas. Similar to that seen in patients with anti-gamma-aminobutyric acid B receptor (GABA-BR) and anti-AMPAR antibodies, they may also present with coexisting autoimmune disorders such as thyroiditis or myasthenia. Anti-GABA-BR encephalitis Anti-GABA-BR encephalitis is characterized by cognitive symptoms with severe seizures or status epilepticus. Other presentations include ataxia and opsoclonus-myoclonus. In a small series of 20 patients with anti-GABA-BR, about 50% were found to have small-cell lung cancer. Males and females appear to be equally affected. The long-term prognosis in anti-GABA-BR encephalitis is determined by the presence of an underlying malignancy. Anti-LGI1 and anti-CASPR2 encephalitis The first reports of anti-voltage-gated potassium channel-complex antibodies (anti-VGKC) date back to 2001 and described patients with neuromyotonia, Morvan's syndrome and limbic encephalitis. Other rare phenotypes included epilepsy and painful polyneuropathy. Anti-VGKC antibodies, in fact, later turned out to be directed against proteins that form a complex with VGKC called leucine-rich glioma-inactivated 1 (LGI1 and contactin-associated protein-like 2 (CASPR-2). Each of these antibodies lead to specific clinical symptoms. Anti-GAD encephalitis Glutamic acid decarboxylase (GAD) is an enzyme that catalyzes the conversion of glutamic acid to the neurotransmitter GABA. Anti-GAD antibodies have been associated with other autoimmune disorders such as insulin-dependent diabetes mellitus. The main neurological syndromes associated with anti-GAD antibodies include stiff-person syndrome, cerebellar ataxia, epilepsy and limbic encephalitis. An.... Discover the Joan Gelfand popular books. Find the top 100 most popular Joan Gelfand books.