Autoimmune encephalitis
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Autoimmune encephalitis | |
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Rasmussen's encephalitis . |
Autoimmune encephalitis (AIE) is a type of
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[2]
- 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.[3][4]
Signs and symptoms
Patients with AIE may present movement disorders such as
Some of these findings are suggestive of certain types of encephalitis and may indicate a specific underlying antibody or tumor.[1]
Mechanism
Autoimmune encephalitis commonly presents an
Diagnosis
Diagnostic criteria for possible autoimmune encephalitis (all three of the following criteria met):[1]
- 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 psychiatricsymptoms
- 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 infiltratesand excluding other disorders
- Reasonable exclusion of alternative causes
Classification
Anti-NMDAR encephalitis
Anti-
Anti-AMPAR encephalitis
Patients with anti-
Anti-GABA encephalitis
Anti-GABA-AR encephalitis
Anti-
Anti-GABA-BR encephalitis
Anti-GABA-BR encephalitis is characterized by cognitive symptoms with severe seizures or status epilepticus.[15] 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.[16] 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.[17][1]
Anti-LGI1 and anti-CASPR2 encephalitis
The first reports of anti-
Anti-GAD encephalitis
Anti-GlyR encephalitis
Anti-DPPX encephalitis
Dipeptidyl peptidase-like protein 6 (DPPX) is a subunit of Kv4.2
Encephalopathy associated with anti-IgLON5 antibodies
The IgLON family member 5 (IgLON5) is a neuronal cell adhesion molecule of the immunoglobulin superfamily. Patients with anti-IgLON5 antibodies present with a unique non-REM (rapid eye movement) and REM parasomnia with obstructive sleep apnea, stridor, episodic central hypoventilation, dementia, gait instability, chorea, dysarthria, dysphagia, dysautonomia and supranuclear gaze palsy resembling that seen in classic tauopathy.[28][27] All published cases reported the presence of the alleles HLA-DQB1*0501 and HLA-DRB1*1001 suggesting genetic susceptibility to this disease. Neuropathological postmortem studies have shown a novel tauopathy with extensive neuronal deposits of hyperphosphorylated tau mainly involving the tegmentum of the brainstem and hypothalamus. This novel encephalopathy provides an intriguing link between neurodegeneration and cell-surface autoimmunity. A recent study has shown that anti-IgLON5 antibodies recognize Ig-like domain 2 as an immunogenic region and causes irreversible internalization of IgLON5 from the neuronal membrane. These findings support a potential pathogenic role of anti-IgLON5 antibodies in the associated encephalopathy.[29][1]
Anti-mGluR1 and anti-mGluR5 encephalitis
Metabotropic glutamate receptor 1 (
All patients with anti-mGluR1 antibodies develop cerebellar ataxia of subacute onset, and some may present with additional symptoms such as paranoia, dysgeusia, diplopia and cognitive deficits. Common tumors found to be associated with anti-mGluR1 antibodies are hematologic malignancies and prostate adenocarcinoma.[30][1]
Patients with anti-mGluR5-abs present with a form of encephalitis named "Ophelia syndrome", a clinical syndrome that includes memory loss and psychosis in association with Hodgkin's lymphoma.[31] The outcome of reported cases is generally good after treatment of the lymphoma and immunotherapy.[31][1]
Seronegative autoimmune encephalitis
Autoimmune encephalitis might occur without the identification of any pathogenic antibody, in which case it is called seronegative autoimmune encephalitis.[4]
It can be further categorized in three subtypes: antibody-negative probable autoimmune encephalitis, autoimmune limbic encephalitis and acute disseminated encephalomyelitis.[4]
One therapeutic approach to seronegative autoimmune encephalitis is using as a first-line treatment
A study in a south-korean hospital with 142 patients identified 5 factors that should be considered when evaluating the disease:[4]
- Presence of refractory status epilepticus
- Advanced age of onset (over or equal to 60 years)
- Having the subtype of probable AE (ANPRA)
- Infra-tentorium involvement in brain magnetic resonance imaging
- Delay of immunotherapy of more than 1 month
The less of those factors are present, the better the chance of good recovery in a 2-year period.[4]
See also
References
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