Autoimmune encephalitis

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Autoimmune encephalitis
CT scan Rasmussen's encephalitis.png
Brain CT scan without contrast enhancement of a patient, female, 8 years old, with Rasmussen's encephalitis.

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. [1]

Contents

Autoimmune encephalitis can result from a number of autoimmune diseases including:

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 ataxia, dystonia, myoclonus, and orofacial dyskinesia. Seizures are the most common symptom and different types of seizures may be seen, including refractory status epilepticus. [5] 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. [6] [1]

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 immune response against neuronal autoantigens with production of antibodies. [7] 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. [7] [1]

Diagnosis

Diagnostic criteria for possible autoimmune encephalitis (all three of the following criteria met): [1]

  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 psychiatric symptoms
  2. At least one of the following:
  3. Reasonable exclusion of alternative causes

Criteria for autoantibody-negative but probable autoimmune encephalitis (all four 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 psychiatric symptoms
  2. Exclusion of well-defined syndromes of autoimmune encephalitis (typical limbic encephalitis, Bickerstaff brainstem encephalitis, acute disseminated encephalomyelitis)
  3. Absence of well-characterized autoantibodies in blood serum and cerebrospinal fluid, and at least two of the following criteria:
  4. 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. [8] This condition predominantly affects children and young female patients. [9] 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. [10] [1]

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. [11] [1]

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. [12] In most of these patients, CSF analysis showed lymphocytic pleocytosis. A recent study identified an underlying neoplasia in 27% of these patients, mostly thymomas. [13] 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. [14] [1]

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-voltage-gated potassium channel-complex antibodies (anti-VGKC) date back to 2001 and described patients with neuromyotonia, Morvan's syndrome and limbic encephalitis. [18] 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). [19] [20] Each of these antibodies lead to specific clinical symptoms. [1]

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. [21] [1]

Anti-GlyR encephalitis

Glycine receptors (GlyR) are chloride channels that facilitate inhibitory neurotransmission in the brain and spinal cord. Anti-GlyR antibodies were first described in patients with progressive encephalomyelitis with rigidity and myoclonus and later in patients with stiff-person syndrome. [22] [23] Recently, anti-GlyR antibodies have also been reported in patients with cerebellar ataxia and anti-GAD antibodies and patients with demyelinating diseases including optic neuritis and multiple sclerosis, but their clinical significance remains unclear. [24] [25] Anti-GlyR antibodies are usually not associated with tumors, although there have been reports of patients with underlying thymoma, small-cell lung cancer, breast cancer and chronic lymphocytic leukemia. [1]

Anti-DPPX encephalitis

Dipeptidyl peptidase-like protein 6 (DPPX) is a subunit of Kv4.2 potassium channels expressed in the hippocampus, cerebellum, striatum, and myenteric plexus. Patients with anti-DPPX antibodies show neuropsychiatric symptoms (agitation and confusion), myoclonus, tremor, startle reflex, seizures, stiff-person syndrome and prodromal diarrhea of unknown etiology. In addition, they may have symptoms of dysautonomia including arrhythmias, thermodysregulation, diaphoresis, urinary symptoms and sleep disorders. [26] [27] [1]

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 (mGluR1) and metabotropic glutamate receptor 5 (mGluR5) are both G-protein-coupled receptors that share an 85% amino acid sequence homology. Both receptors are involved in modulating synaptic functions including the electrical change in neuronal response called long-term depression (a term not related to the mood-changing disorder major depression). While mGluR1 facilitates long-term depression at parallel fiber to Purkinje cell synapses, which are critical for cerebellar motor learning, mGluR5 is more relevant for long-term depression in the hippocampus. [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 corticosteroids and intravenous immunoglobulin. [4] Other options include the use of rituximab (second-line) and tocilizumab or cyclophosphamide (next-line). [4]

A study in a South Korean hospital with 142 patients identified 5 factors that should be considered when evaluating the disease: [4]

The less of those factors are present, the better the chance of good recovery in a 2-year period. [4]

See also

Related Research Articles

Morvan's syndrome is a rare, life-threatening autoimmune disease named after the nineteenth century French physician Augustin Marie Morvan. "La chorée fibrillaire" was first coined by Morvan in 1890 when describing patients with multiple, irregular contractions of the long muscles, cramping, weakness, pruritus, hyperhidrosis, insomnia and delirium. It normally presents with a slow insidious onset over months to years. Approximately 90% of cases spontaneously go into remission, while the other 10% of cases lead to death.

Encephalomyelitis is inflammation of the brain and spinal cord. Various types of encephalomyelitis include:

Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of autoimmune diseases characterized by acute inflammation of the optic nerve and the spinal cord (myelitis). Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients.

Rasmussen syndrome or Rasmussen's encephalitis is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motor skills and speech, hemiparesis, encephalitis, and dementia. The illness affects a single cerebral hemisphere and generally occurs in children under the age of 15.

<span class="mw-page-title-main">Meningoencephalitis</span> Medical condition

Meningoencephalitis, also known as herpes meningoencephalitis, is a medical condition that simultaneously resembles both meningitis, which is an infection or inflammation of the meninges, and encephalitis, which is an infection or inflammation of the brain tissue.

<span class="mw-page-title-main">Chronic inflammatory demyelinating polyneuropathy</span> Medical condition

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy. CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic syndrome associated with a broad variety of tumors including lung cancer, ovarian cancer, breast cancer, Hodgkin’s lymphoma and others. PCD is a rare condition that occurs in less than 1% of cancer patients.

Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process. IDDs share characteristics with and are often grouped together under Multiple Sclerosis. They are sometimes considered different diseases from Multiple Sclerosis, but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course.

<span class="mw-page-title-main">Limbic encephalitis</span> Inflammation involving the limbic system in the brain

Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain. Limbic encephalitis is caused by autoimmunity: an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not. Although the disease is known as "limbic" encephalitis, it is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain. The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968 and confirmed by later investigators.

A paraneoplastic syndrome is a syndrome that is the consequence of a tumor in the body. It is specifically due to the production of chemical signaling molecules by tumor cells or by an immune response against the tumor. Unlike a mass effect, it is not due to the local presence of cancer cells.

Anti-glutamate receptor antibodies are autoantibodies detected in serum and/or cerebrospinal fluid samples of a variety of disorders such as encephalitis, epilepsy and ataxia. Clinical and experimental studies starting around the year 2000 suggest that these antibodies are not simply epiphenomena and are involved in autoimmune disease pathogenesis.

Sleep-related hypermotor epilepsy (SHE), previously known as nocturnal frontal lobe epilepsy, is a form of focal epilepsy characterized by seizures which arise during sleep. The seizures are most typically characterized by complex motor behaviors. It is a relatively uncommon form of epilepsy that constitutes approximately 9-13% of cases. This disorder is associated with cognitive impairment in at least half of patients as well as excessive daytime sleepiness due to poor sleep quality. This disorder is sometimes misdiagnosed as a non-epileptic sleep disorder. There are many potential causes of SHE including genetic, acquired injuries and structural abnormalities.

<span class="mw-page-title-main">Anti-NMDA receptor encephalitis</span> Rare disease which results in brain inflammation

Anti-NMDA receptor encephalitis is a type of brain inflammation caused by antibodies. Early symptoms may include fever, headache, and feeling tired. This is then typically followed by psychosis which presents with false beliefs (delusions) and seeing or hearing things that others do not see or hear (hallucinations). People are also often agitated or confused. Over time, seizures, decreased breathing, and blood pressure and heart rate variability typically occur. In some cases, patients may develop catatonia.

Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by Edwin Bickerstaff in 1951. It may also affect the peripheral nervous system, and has features in common with both Miller Fisher syndrome and Guillain–Barré syndrome.

<span class="mw-page-title-main">Autoimmune autonomic ganglionopathy</span> Medical condition

Autoimmune autonomic ganglionopathy is a type of immune-mediated autonomic failure that is associated with antibodies against the ganglionic nicotinic acetylcholine receptor present in sympathetic, parasympathetic, and enteric ganglia. Typical symptoms include gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils. Many cases have a sudden onset, but others worsen over time, resembling degenerative forms of autonomic dysfunction. For milder cases, supportive treatment is used to manage symptoms. Plasma exchange, intravenous immunoglobulin, corticosteroids, or immunosuppression have been used successfully to treat more severe cases.

Anti-VGKC-complex encephalitis are caused by antibodies against the voltage gated potassium channel-complex (VGKC-complex) and are implicated in several autoimmune conditions including limbic encephalitis, epilepsy and neuromyotonia.

MOG antibody disease (MOGAD) or MOG antibody-associated encephalomyelitis (MOG-EM) is an inflammatory demyelinating disease of the central nervous system. Serum anti-myelin oligodendrocyte glycoprotein antibodies are present in up to half of patients with an acquired demyelinating syndrome and have been described in association with a range of phenotypic presentations, including acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, and neuromyelitis optica.

Anti-Hu associated encephalitis, also known as Anti-ANNA1 associated encephalitis, is an uncommon form of brain inflammation that is associated with an underlying cancer. It can cause psychiatric symptoms such as depression, anxiety, and hallucinations. It can also produce neurological symptoms such as confusion, memory loss, weakness, sensory loss, pain, seizures, and problems coordinating the movement of the body.

Anti-IgLON5 disease is an uncommon neurological autoimmune condition linked to autoantibodies directed against the IgLON5 protein. Sleep disturbance, bulbar symptoms, and abnormal gait make up the majority of the clinical presentation, which is then followed by cognitive dysfunction. The diagnosis of anti-IgLON5 disease is primarily based on clinical signs and the identification of IgLON5 antibodies in patient serum and/or cerebrospinal fluid.

Michael D. Geschwind is a professor of neurology at the UCSF Memory and Aging Center (MAC), specializing in neurodegenerative disorders.

References

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  25. ↑ Martinez-Hernandez, Eugenia; Sepulveda, Maria; Rostásy, Kevin; Höftberger, Romana; Graus, Francesc; Harvey, Robert J.; Saiz, Albert; Dalmau, Josep (February 2015). "Antibodies to Aquaporin 4, Myelin-Oligodendrocyte Glycoprotein, and the Glycine Receptor α1 Subunit in Patients With Isolated Optic Neuritis". JAMA Neurology. 72 (2): 187–193. doi:10.1001/jamaneurol.2014.3602. PMC   4836943 . PMID   25506781 . Retrieved 9 December 2022.
  26. ↑ Piepgras, Johannes; Höltje, Markus; Michel, Klaus; Li, Qin; Otto, Carolin; Drenckhahn, Christoph; Probst, Christian; Schemann, Michael; Jarius, Sven; Stöcker, Winfried; Balint, Bettina; Meinck, Hans-Michael; Buchert, Ralph; Dalmau, Josep; Ahnert-Hilger, Gudrun (8 September 2015). "Anti-DPPX encephalitis: Pathogenic effects of antibodies on gut and brain neurons". Neurology. 85 (10): 890–897. doi:10.1212/WNL.0000000000001907. ISSN   0028-3878. PMC   4560062 . PMID   26291285.
  27. 1 2 Boronat, Anna; Gelfand, Jeffrey M.; Gresa-Arribas, Nuria; Jeong, Hyo-Young; Walsh, Michael; Roberts, Kirk; Martinez-Hernandez, Eugenia; Rosenfeld, Myrna R.; Balice-Gordon, Rita; Graus, Francesc; Rudy, Bernardo; Dalmau, Josep (January 2013). "Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6, a subunit of Kv4.2 potassium channels". Annals of Neurology. 73 (1): 120–128. doi:10.1002/ana.23756. PMC   3563722 . PMID   23225603.
  28. ↑ Sabater, Lidia; Gaig, Carles; Gelpi, Ellen; Bataller, Luis; Lewerenz, Jan; Torres-Vega, Estefanía; Contreras, Angeles; Giometto, Bruno; Compta, Yaroslau; Embid, Cristina; Vilaseca, Isabel; Iranzo, Alex; Santamaría, Joan; Dalmau, Josep; Graus, Francesc (1 June 2014). "A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study". The Lancet Neurology. 13 (6): 575–586. doi:10.1016/S1474-4422(14)70051-1. ISSN   1474-4422. PMC   4104022 . PMID   24703753.
  29. ↑ Gaig, Carles; Graus, Francesc; Compta, Yarko; Högl, Birgit; Bataller, Luis; Brüggemann, Norbert; Giordana, Caroline; Heidbreder, Anna; Kotschet, Katya; Lewerenz, Jan; Macher, Stefan; Martí, Maria J.; Montojo, Teresa; Pérez-Pérez, Jesus; Puertas, Inmaculada (2 May 2017). "Clinical manifestations of the anti-IgLON5 disease". Neurology. 88 (18): 1736–1743. doi:10.1212/WNL.0000000000003887. ISSN   0028-3878. PMC   5409845 . PMID   28381508.
  30. ↑ Lopez-Chiriboga, A. Sebastian; Komorowski, Lars; Kümpfel, Tania; Probst, Christian; Hinson, Shannon R.; Pittock, Sean J.; McKeon, Andrew (15 March 2016). "Metabotropic glutamate receptor type 1 autoimmunity: Clinical features and treatment outcomes". Neurology. 86 (11): 1009–1013. doi:10.1212/WNL.0000000000002476. ISSN   0028-3878. PMC   4799712 . PMID   26888994.
  31. 1 2 Lancaster, E.; Martinez-Hernandez, E.; Titulaer, M. J.; Boulos, M.; Weaver, S.; Antoine, J.-C.; Liebers, E.; Kornblum, C.; Bien, C. G.; Honnorat, J.; Wong, S.; Xu, J.; Contractor, A.; Balice-Gordon, R.; Dalmau, J. (1 November 2011). "Antibodies to metabotropic glutamate receptor 5 in the Ophelia syndrome". Neurology. 77 (18): 1698–1701. doi:10.1212/WNL.0b013e3182364a44. ISSN   0028-3878. PMC   3208954 . PMID   22013185.
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