Autoimmune encephalitis

Autoimmune Encephalitis
Classification and external resources
ICD-10 G04.81
ICD-9-CM 323.81

Autoimmune encephalitis (Autoimmune Encephalopathy) is a diverse group of neuro-psychiatric disorders recognized recently, presenting acutely or subacutely with alteration of consciousness, cognitive decline, seizure, abnormal movements. Associated with systemic autoimmune disorders, CNS autoimmune disorders and paraneoplastic syndromes. Pathogenesis is likely to be mediated by antibodies (Abs) to CNS proteins. The Abs are directed against membrane receptors and ion channel-associated proteins that are expressed on the surface of neurons in the CNS, such as N-methyl D-aspartate receptors and leucine-rich, glioma inactivated protein and contactin-associated protein like, that are associated with voltage-gated potassium channels. The diseases are not invariably cancer-related and are therefore different from the classical paraneoplastic neurological diseases that are associated with, but not caused by, Abs to intracellular proteins. Diagnosis of autoimmune encephalopathy is based on the clinical course, serologic evidence of autoimmunity, severe but nonspecific slowing on electroencephalography, and evidence of intrathecal inflammation in the cerebrospinal fluid and neuroimaging by MRI. Treatment include first-line steroids, intravenous immunoglobulins(IVIG), and plasma exchange, and second-line Rituximab and Cyclophosphamide, followed in many cases by steroid-sparing agents in the long-term.[1]

Neurological Syndromes Associated with Autoimmune Antibody (NSAAA)

Sydenham's chorea (SC)

Main article: Sydenham's chorea

Sydenham's chorea epidemic was first documented in 1418 and later in 1686 Thomas Sydenham described the condition.[2] The association of chorea with rheumatism was first recognized in 1802[3] and several cases has been reported by French and English workers during nineteenth century.[4] It was Richard Bright who in 1831 who first made the association between chorea and rheumatic fever. The evidence suggesting that it may be mediated by streptococcal antibodies that cross-react with the brain was published in 1993.[5]

Encephalitis Lethargica (EL)

In 1916, von Economo described encephalitis lethargica (EL), a CNS disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism) and neuropsychiatric sequelae. Since the 1916–1927 epidemic, only sporadic cases have been described. Pathological studies revealed an encephalitis of the midbrain and basal ganglia, with lymphocyte (predominantly plasma cell) infiltration. The EL epidemic occurred during the same time period as the 1918 influenza pandemic, and the two outbreaks have been linked in the medical literature. However, von Economo and other contemporary scientists thought that the 1918 influenza virus was not the cause of EL. Recent examination of archived EL brain material has failed to demonstrate influenza RNA, adding to the evidence that EL was not an invasive influenza encephalitis. By contrast, the findings of intrathecal oligoclonal bands (OCB) and beneficial effects of steroid treatments have provoked the hypothesis that EL may be immune‐mediated.[6][7]

Hashimoto's encephalopathy

In 1966 Brain et al described a 49-year-old man with Hashimoto's thyroiditis who presented with stroke-like episodes and subsequently delirium. The patient did not respond to prednisone, but eventually recovered, leading Brain to suggest that autoimmune mechanisms might be responsible. Since that time, there have been many further cases reported in the literature of Hashimoto's encephalopathy.[8] The term Hashimoto's encephalopathy is used to describe an encephalopathy of presumed autoimmune origin characterized by high titres of antithyroid peroxidase antibodies. Hashimoto's encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features of a severe vasculitis are often absent.[9]

Steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome

Caselli RJ in 1999 reported five patients, age 54 to 80 years, presented between 3 weeks and 18 months after symptomatic onset of progressive cognitive decline, psychosis, and unsteady gait that proved to be due to a steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome.[10]

Limbic Encephalitis (LE)

Main article: Limbic Encephalitis

The term limbic encephalitis (LE) was originally coined by Corsellis in 1968,[11] present with the subacute onset of episodic memory impairment, disorientation and agitation, commonly associated with seizures, hallucinations, sleep disturbance and histological evidence of medial temporal lobe inflammation.[12] Signal changes in the medial temporal lobes or hippocampi are frequently found on MRI.[13] Its association with lung cancer and Hu antibody, Ma2 with testicular tumors and CRMP5/CV2 in patients with thymomas. Anti-NMDA-receptor encephalitis and its association with teratoma was published in 2008 by Josep Dalmau.[14] There may be improvement after treatment of the primary tumor, but in general the prognosis of paraneoplastic limbic encephalitis is poor.[11] A few cases of non‐paraneoplastic limbic encephalitis have been described.[13]

Paraneoplastic Limbic Encephalitis (PLE)

Paraneoplastic limbic encephalitis (PLE) is a disorder characterized by personality changes, irritability, depression, seizures, memory loss and sometimes dementia.The diagnosis of PLE required neuropathological examination or the presence of the four following criteria: (i) a compatible clinical picture; (ii) an interval of <4 years between the development of neurological symptoms and tumour diagnosis; (iii) exclusion of other neuro-oncological complications; and (iv) at least one of the following: CSF with inflammatory changes but negative cytology; MRI demonstrating temporal lobe abnormalities; EEG showing epileptic activity in the temporal lobes.[15]

Table

Paraneoplastic antibodies associated with limbic encephalitis[14][16]

Antibody Syndrome Cancer
Hu Limbic encephalitis, encephalomyelitis SCLC, other
Ma2 Limbic, hypothalamic,a and brainstem encephalitis Testis, lung, other
CV2/CRMP5 Limbic, striatal encephalitis (chorea), cerebellar ataxia, peripheral neuropathy, uveitis SCLC, thymoma
Amphiphysin Limbic encephalitis, stiff-person syndrome Breast, SCLC
NMDA Limbic encephalitis, Teratoma of ovary

Non Paraneoplastic Limbic Encephalitis (NPLE)

Non-paraneoplastic variants of LE (NPLE) are associated with autoimmune diseases and demonstrate antibodies against cell membrane antigens. However, there are cases of NPLE diagnosed on the basis of the clinical picture, MRI findings, absence of tumor, and long-term follow-up results, which do not reveal the aforementioned antibodies, are not preceded by symptoms of acute febrile infection, and present with temporal lobe epilepsy as the prominent symptom. NPLE is a potentially reversible variant of LE.[17]

Mori M in 2002 reported a 21-year-old woman subacutely developed memory loss subsequent to gastroenteritis. Brain MRI with gadolinium enhancement showed symmetric involvement of the amygdala. The CSF was acellular with increased protein level. There was no evidence suggestive of neoplasm or viral infection. Combined treatment with plasmapheresis and immunoglobulin improved her clinical symptoms and lessened abnormalities manifested in the MRI. He suggested the presence of immune-mediated limbic encephalitis without association with neoplasms or infections.[18]

Similar cases of cognitive decline in adults and elderly with anterograde memory loss, hippocampal lesions in MRI and elevated CSF protein with lymphocytic pleocytosis without evidence of neoplasia and steroid responsiveness has been reported .[19][20]

Limbic encephalitis associated with voltage‐gated potassium channel antibodies (VGKC‐Abs)[21] may frequently be non‐paraneoplastic.[22] A recent study of 15 cases of limbic encephalitis found raised VGKC‐Abs associated with non‐paraneoplastic disorders and remission following immunosuppressive treatment.[23] Review of 10 cases of "Potassium channel antibody‐associated encephalopathy Angela Vincent found patients of either sex presenting with subacute onset of disorientation, confusion and memory loss particularly when associated with medial temporal lobe signal change on MRI. Clinically, these cases do not differ substantially from other forms of amnesic encephalopathies, such as the paraneoplastic form of limbic encephalitis associated with small‐cell lung cancer and Hu antibodies, except in the relative absence of cerebellar and brainstem involvement. However, VGKC‐Ab‐associated encephalopathy may have a wider phenotype, and seizures or more florid psychiatric symptoms such as hallucinations, may be the presenting features in some cases.[13] Pediatric non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis and immunotherapy has been reported.[24][25]

Subacute onset partial seizures with recent memory and no evidence of underlying malignancy was recognized as limbic encephalitis.[26][27] Pilomotor seizures were the predominant seizure type in this case of non-paraneoplastic limbic encephalitis. Immunosuppressive therapy may provide recovery including seizure control. However, long-term immunosuppression may be necessary to prevent relapse. Hippocampal sclerosis and chronic epilepsy might evolve as sequelae of limbic encephalitis.[28]

In 2007 the spectrum was further extended by a report on four patients with presumed non-paraneoplastic LE in whom neither VGKC-antibodies nor other antibodies could be found.[25] Despite immunmodulatory treatment all these patients had severe neurological residual symptoms but excellent outcome in some.[29]

Clinical features NMDA Ab related patients with NPLE shows; Early features of higher cognitive dysfunction, confusion, behavioural changes , amnesia , dysphasia. Psychiatric: hallucinations , psychotic , agitation, depressive , anxiety , obsessive. Seizures: generalized, complex partial , simple partial. Late features: Spontaneous reduction in conscious level, Movement disorder: choreoathetoid (orofacial , upper limbs , lower limbs), parkinsonian , rigidity , myoclonus, oculogyric crises, opisthotonus , startle. Dysautonomia : tachy/brady-cardia, hyperhidrosis, persistent pyrexia, central hypoventilation , labile/high blood pressure , hypersalivation , pseudoobstruction , cardiac asystole .[30]

In non-paraneoplastic, non-herpetic acute limbic encephalitis (NHALE), serum antibodies to GluRepsilon2 (NR2B) were detected in approximately 60% of the patients from acute to chronic stages further, these antibodies in the cerebrospinal fluid (CSF) were detected in 50% (acute stage), 40% (recovery stage), and 30% (chronic stage) of the patients.[31]

Non-paraneoplastic limbic encephalitis characterized by frequent mesio-temporal seizures and and amnesia with extratemporal lesions in MRI, and glutamic acid decarboxylase-antibody positive.[32]

Post-partum psychosis followed by seizure and cognitive dysfunction with of limbic encephalitis (LE), and antibodies to voltage-gated potassium channel complex (VGKC) detected.[33]

Non-paraneoplastic autoimmune encephalitis associated with antibodies against membrane antigens was the most common syndrome, especially in the pediatric population. Clinical outcome was better for patients with shorter latency from symptom onset to diagnosis and initiation of immunomodulation.[34][35]

Post Herpes Simplex NMDA Ab Encephalitis

NMDAR Ab Encephalitis Following Herpes Simplex Virus Encephalitis Some children and adults with herpes simplex virus (HSV) encephalitis and subsequent relapses characterized by choreoathetosis in children and behavioral/psychiatric features in adults were found to have NMDAR Abs without HSV reactivation, they improved with immunotherapy.[36][37][38][39]

Morvan's syndrome

Main article: Morvan's syndrome

In 1890, Morvan described a patient with myokymia (muscle twitching) associated with muscle pain, excessive sweating and disordered sleep Morvan's fibrillary chorea (MFC).[40] The course was severe and the patient died 5 weeks after onset. A similar condition characterized by widespread myokymia and cramping, but without overt CNS involvement has since been described and is now referred to as neuromyotonia (NMT) in 1965[41] or Isaacs' syndrome (1961).[42] Acquired NMT is thought to be autoimmune in a high proportion of cases. It can be associated with thymoma,[43] and antibodies to voltage-gated K+ channels (VGKCs) are present in a proportion of patients.[13][44][45] Although anti-VGKC antibody negative cases of Morvan syndrome have been reported in the literature, it is possible that, in these cases, the antigenic components are yet to be formally identified.[46]

Brain MRI was normal in 92% of patients with Morvan syndrome.[47] Limited EEG data have been reported in Morvan syndrome, but in 1 investigation of neurophysiological studies, EEG showed diffuse slow wave abnormalities in 1 patient and was normal in another, with a literature review showing generalized slowing as the principal EEG finding.[48]

Diagnosis of Morvan syndrome should prompt investigation for an underlying antigenic source, particularly thymoma.[46][49]

Immunosuppressive therapies, encompassing corticosteroids, azathioprine, methotrexate and more recently, rituximab, are the mainstay of therapy.[50] Other treatments include PE, IVIG, and thymectomy. Antiepileptics can be used for symptomatic relief of peripheral nerve hyperexcitability. Patients reportedly exhibited a heterogenous response to immunomodulation. Indeed, some patients have exhibited a spontaneous remission of symptoms. Prognosis is inherently clouded; however, current analysis suggests that those associated with thymoma, benign or malignant, show a less favorable prognosis.[46][47]

Autoimmune Antibodies Associated Neurological Syndromes (AAANS)

N-Methyl D-aspartate receptor (NMDAR, NR1, NR2)

N-Methyl D-aspartate receptor (NMDAR) Ab encephalitis is the most common Ab-mediated autoimmune encephalopathy.[51] It was first described in 2005 as a paraneoplastic syndrome associated with ovarian teratomas in young women[52] and the antigenic target was determined in 2007[53] developed prominent psychiatric symptoms, amnesia, seizures, frequent dyskinesias, autonomic dysfunction, and decreased level of consciousness often requiring ventilatory support. All had serum/cerebrospinal fluid antibodies that predominantly immunolabeled the neuropil of hippocampus/forebrain, in particular the cell surface of hippocampal neurons, and reacted with NR2B (and to a lesser extent NR2A) subunits of the NMDAR. NR2B binds glutamate and forms heteromers (NR1/NR2B or NR1/NR2A/NR2B) that are preferentially expressed in the adult hippocampus/forebrain[53] The clinical characteristics of 100 patients with encephalitis and NR1-NR2 antibodies. Median age of patients was 23 years (range 5–76 years); 91 were women. All patients presented with psychiatric symptoms or memory problems; 76 had seizures, 88 unresponsiveness (decreased consciousness), 86 dyskinesias, 69 autonomic instability, and 66 hypoventilation. 58 (59%) of 98 patients for whom results of oncological assessments were available had tumours, most commonly ovarian teratoma. Patients who received early tumour treatment (usually with immunotherapy) had better outcome (p=0.004) and fewer neurological relapses (p=0.009) than the rest of the patients. 75 patients recovered or had mild deficits and 25 had severe deficits or died. Improvement was associated with a decrease of serum antibody titres. The main epitope targeted by the antibodies is in the extracellular N-terminal domain of the NR1 subunit. Patients' antibodiesdecreased the numbers of cell-surface NMDA receptors and NMDA-receptor clusters in postsynaptic dendrites, an effect that could be reversed by antibody removal.[14]

Voltage Gated Potassium-Channels

The voltage-gated potassium channel (VGKC) complex Abs are the second most commonly identified Abs in CNS Ab-mediated diseases, but the clinical spectrum and treatment responses are very different from those of the NMDAR Ab encephalitis. The clinical spectrum includes both central and peripheral nervous system disorders. There are many families and subtypes of VGKCs but the Abs referred to here are those that immunoprecipitate Kv1.1, 1.2, and 1.6 subtypes from mammalian brain tissue extracts. The VGKC complex is composed of Kv1 subunits and other proteins that are tightly complexed with the Kv1 subunits in the nerve membrane. They are widely expressed in the nervous system, particularly at the juxtaparanodes of the nodes of Ranvier, at peripheral motor nerve, and perhaps sensory terminals, and in central synapses. Because opening of the VGKC following each action potential leads to repolarization of the membrane, VGKCs regulate neuronal activity throughout the nervous system.

VGKC complex Abs were first described in association with neuromytonia (NMT) or Isaac’s syndrome. This is characterized by peripheral nerve excitability that manifests with spontaneous muscle contraction, stiffness, sometimes impaired muscle relaxation,[42] and a specific electromyography pattern of burst of random discharges at high frequency in the muscle fibers.[54] It is caused by hyperexcitability of the motor nerves leading to repetitive and spontaneous activity in the muscles. VGKC complex Abs were found in around 40% of patients with NMT and then more frequently and at higher levels in patients with NMT associated with dysautonomia and CNS disturbance including insomnia and limbic dysfunction, which is usually called Morvan’s syndrome (MoS),[47][55] and in a form of nonparaneoplastic limbic encephalitis (LE). Although these Abs were initially identified by immunoprecipitation of radioactive dendrotoxin-labeled VGKCs in digitonin-solubilized mammalian brain homogenates, further evidence indicated that they bind to protein components of the VGKC complex rather than to the VGKC itself; their identification requires Ag-specific cell-based assays.[56][57] The main targets for the Abs are LGI1, typically associated with LE, and a specific focal epilepsy, faciobrachial dystonic seizures (FBDS), and contactin-associated protein like 2 (CASPR2), associated with a broader spectrum of central and peripheral nervous system disorders such as LE, NMT, or a combination of the two (MoS). A third antigen, contactin 2, has been identified, usually in patients with concomitant anti-LGI1 or anti-CASPR2 Abs and with no specific phenotype, suggesting an unclear clinical relevance. In some patients VGKC Abs are detected by radioimmunoassay, in the absence of LGI1, CASPR2, or contactin 2 Ab specificity. In general, high titers (usually > 400 pM) are more frequently associated with defined clinical phenotypes (mainly LE, but also peripheral nerve hyperexcitability (PNH), seizures, or MoS), whereas low titers of Abs can be found in patients with either or both CNS and PNS syndromes but are also detected in patients without a clear immune-mediated phenotype.[58][59][60] It is possible that, in these cases, the Abs bind to intracellular targets on the solubilized VGKC complex.

Potassium channel (KIR4.1): "Dipeptidyl-peptidase-like Protein-6: Dipeptidyl-peptidase-like protein-6 is a protein associated with another VGKC, Kv4.2, that is responsible for regulating firing rates of action potentials in dendrites in the central and peripheral nervous system. Abs to dipeptidyl-peptidase-like protein-6 were initially identified in patients with a form of LE associated with gastrointestinal dysmotility (due to the involvement of the myoenteric plexus), sleep disturbances, cognitive and psychiatric manifestations, and dysautonomic features,[61] and subsequently in a disease presenting with hyperekplexia, trunk rigidity, and cerebellar ataxia.[62] Overall, most patients seem to respond to immunosuppression, irrespective of the treatment strategy chosen. In a recent review of 20 cases, 8 of 12 patients treated with immunotherapy improved, but clinical data were mostly collected retrospectively.[1]

AMPAR (GluR1, GluR2)

AMPAR are a subgroup of ionotropic glutamate receptor mainly present in excitatory synapses of the CNS. Abs against the extracellular domains of AMPA subunits GluR1 and GluR2 were associated originally with a particularly aggressive form of LE, often accompanied by the presence of a tumor.[63] The largest case series reported describes 22 patients and shows that the clinical spectrum can include, in addition to LE, psychosis and multifocal encephalopathy. Administration of first-line treatment (steroids or IVIg) and, when appropriate, tumor removal, often lead to a complete or partial remission of the symptoms. The patients can relapse, but a more aggressive course of treatment involving rituximab and/or cyclophosphamyde seems to be associated with a monophasic disease.[64]

Glycine Receptor

Abs directed against the α1 subunit of the glycine receptor (GlyR) have been described in patients with variants of stiff person syndrome usually identified as progressive encephalomyelitis, rigidity, and myoclonus, which is characterized by the association of rigidity, stimulus-sensitive spasms, myoclonus, hyperkeplexia, autonomic disturbances, and brainstem disorders.[65] In the only prospective case series, patients treated with immunotherapy showed consistent improvement, sometimes with complete resolution of clinical symptoms. Therapeutic approaches were variable but typically involved a combination of IVMP, PLEX, and IVIg followed by oral prednisolone.[66] Nevertheless, 6/45 patients relapsed and required prolonged immunosuppression with MMF, AZA, or cyclophosphamide, which, at the time of publication, were effective in preventing further relapses. Recently, GlyR Abs have also been identified in association with isolated optic neuritis , and they represented the most frequent Ab recognized in patients with focal adult epilepsy of unknown cause . However, in these studies the Abs were also found in a consistent number of patients in the control group, the titers did not correlate clearly with disease presentation or drug administration, and the response to immunosuppression was highly variable. The role of GlyR Abs in such conditions may not be clinically helpful, although it could reflect the presence of autoimmune mechanisms.[67]

Dopamine 2 Receptor

Iinvestigating the role of surface Abs in suspected autoimmune movement disorders Dale et al., identified Abs to the extracellular domain of the dopamine receptor 2 in 12/17 patients with basal ganglia encephalitis compared with 0/67 controls.[68] In addition, dopamine receptor 2 Abs were found in a small number of patients with Tourette’s syndrome and Syndenham’s chorea. Most patients in the study were identified retrospectively and were given no immunotherapy, presenting at the end of follow-up with persistent neurological deficits. Interestingly, patients identified after the study who received immunosuppression showed marked clinical improvement and a reduction in Ab titers (Dale et al., personal communication), suggesting a consistent role of the Ab in disease progression. Further studies are required to confirm these data and help define an optimal treatment course.[1]

GABAB R1 Receptor

GABABR is a protein widely distributed in the brain and located both pre- and postsynaptically. Genetic alterations of the receptor are associated with epilepsy and cognitive impairment, and Abs against the B1 subunit are found in patients with LE and, rarely, ataxia, 50% of whom will have a small-cell lung carcinoma.[69] The administration of immunotherapy, in association with chemotherapy or tumor removal, is accompanied by a prompt improvement and, in a percentage of cases, by a complete recovery.[70] Conversely, patients that did not receive immunotherapy had a bad prognosis, and death occurred in a high percentage of cases within months from the onset of the neurological disease. Some patients have a poor outcome despite sustained immunosuppression, but that is often related to tumor progression or associated with the presence of Abs directed against intracellular Ags such as GAD Abs or amphyphysin Abs, which can reflect the involvement of an additional cytotoxic T-cell mechanism in the progression of the disease.[71] More recently, a novel Ab against GABAA receptor has been described. Pedrol-Petit et al.[72] reported 18 patients with Abs directed against the α1/β3 subunit of the receptor, 6 with high titers in serum and CSF and a definite clinical picture, and 12 with low titers only on serum and variable syndromes, often in association with other Abs. Among the 6 patients with CSF Abs, only 1 had substantial recovery with antiepileptic drugs alone, which had to be maintained for a long time to avoid recurrence. The remaining 5 patients received different immunotherapy regimens (either steroids alone or in combination with IVIg, PLEX, cyclophosphamide or rituximab), with a consistent recovery in 3 of them, whilst 2 died because of septic complications. Another paper described 40 patients with Abs of the IgG and IgM subclasses targeting the α1 and γ2 subunits of the GABAA receptor.[73] Immunosuppressive treatment was administered prospectively only to 1 patient, whose catatonia and frontal dysfunction improved with a fall in Ab titres.

Metabotropic Glutamate Receptor

Subacute cerebellar ataxia associated with metabotropic glutamate receptor type 1 Abs (mGluR1) is a rare clinical entity, so far described in 3 reported patients.[74][75] Paraneoplastic cerebellar degeneration is often associated with Abs directed against cytoplasmic or nuclear Ags that are important for the diagnosis but unlikely to be directly responsible for neuronal damage. By contrast, mGluR1 Abs are directed at a surface Ag on perisynaptic dendritic spines of Purkinje cells and, in one study, shown to be pathogenic [99]. More recently, Abs directed to the mGluR5 Abs were identified in 2 patients with limbic encephalopathy and Hodgkin lymphoma, a combination known as Ophelia syndrome.[76] In both cases prompt tumor treatment (excision or chemotherapy) was performed, and 1 patient also received steroids, resulting in complete regression of the symptoms.

Synaptic proteins: LGI1

In around 20–40 % of patients with LGI1 Abs a specific seizure type can precede the occurrence of full-blown LE .[77] These events have been described as “tonic seizures”,[78] or FBDS,[77] and consist of brief and very frequent involuntary movement with dystonic features involving mainly the arm, the ipsilateral side of the face, and, less frequently, the leg. The response to routinely used anticonvulsant drugs is usually poor, but a dramatic reduction or complete resolution of the FBDS can be obtained with oral steroids.[79] Irani et al.[77] described a prospective cohort of 10 patients with FBDS, where the development of cognitive impairment was only present in patients who did not receive immunotherapy, suggesting that early treatment can result in a better recovery and sometimes prevent progression to encephalopathy.

IgLON5

In 2014, a novel syndrome with sleep disorders (parasomnia and breathing dysfunction), gait instability, and brainstem symptoms was described in 8 patients in association with surface Abs to the neuronal cell adhesion protein IgLON5.[80] Neuropathological investigations in 2 patients identified tau aggregates in the tegmentum of the brainstem and in the hypothalamus that could not be classified within any known tauopathy, suggesting a possible neurodegenerative etiology of the disease. Moreover, despite immunosuppressive treatments including steroids, IVIg, cyclophosphamide, and rituximab, only 1 patient showed some improvement. Whether the Abs are a primary or secondary element in the disease development needs to be clarified.

See also

Sydenham's chorea

Encephalitis lethargica

Limbic Encephalitis

Hashimoto's encephalopathy

Paraneoplastic Encephalitis

Rasmussen encephalitis

Systemic lupus erythematosus

Behcet's disease

Morvan's syndrome

References

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