Some cases of isaacs syndrome are associated with autoantibodies to caspr2 or other, often undefined, members of the voltagegated potassium channel vgkc complex. We describe the first case of acquired motor neuron disease associated with voltagegated potassium channel antibodies. Vgkc voltage gated potassium channel complex l imbic encephalitis, typically characterized clinically by the subacute onset of partial and secondary generalized seizures, memory loss, and neuropsychiatric changes can be of either infectious or autoimmune etiology. Case report high grade glioma mimicking voltage gated potassium channel complex associated antibody limbic encephalitis dilanathauda, 1 r. His seizures and cognitive deficits improved and stabilized. Clinical spectrum of voltagegated potassium channel autoimmunity. Most patients improve if properly diagnosed and treated expediently with immunotherapy. Limbic encephalitis that is associated with antivoltagegated potassium channel complex vgkcc antibodies vgkcc syndrome is an autoimmune, usually nonparaneoplastic form of encephalitis, and most patients with this condition improve after early initiation of steroids or other immunemodulatory therapies. Diseases associated with antibodies to voltagegated. Voltage gated potassium channel vgkc antibodyassociated encephalitis is an autoimmune limbic encephalitis caused by binding of antibodies to voltagegated potassium channel associated proteins.
Background and purpose autoantibodies abs to glutamic acid decarboxylase gad and to voltage. Voltagegated potassium channel vgkc antibodyassociated encephalitis is a neuropsychiatric condition that is potentially fully reversible with early diagnosis and treatment with immunotherapy. Autoimmune limbic encephalitis le associated with voltage gated potassium channel antibodies vgkcabs in children is more common than previously thought and is not always paraneoplastic. Diagnosis voltagegated potassium channel antibodyrelated limbic encephalitis. Voltage gated, potassium channel vgkc antibodyassociated limbic encephalitis is a neurological condition characteristically associated with confusion, memory disturbance, epilepsy, and altered behavior. A case study of voltagegated potassium channel antibody. Encephalitis due to antibodies to voltage gated potassium channel. Voltagegated potassium channel antibodyassociated limbic.
Treatment with immunomodulatory therapy resulted in almost complete recovery. Voltage gated potassium channel vgkc antibody encephalitis is an autoimmune encephalitis with antibodies against the voltage gated potassium channel. Infectious and autoantibodyassociated encephalitis. By sarosh irani, associate professor and consultant neurologist, university of oxford and john radcliffe hospital, oxford, uk.
Postmalaria neurologic syndromeautoimmune encephalitis. A 58yearold female and with a known case of auto immune encephalitis voltage gated potassium channel and steroid induced diabetes mellitus presented with progressive worsening of vertigo, recurrent myoclonic jerks and post ictal confusion for last 7 days. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from morvan syndrome and hashimoto encephalopathy. Apr 18, 2017 the discovery, in 2010, of autoantibodies against the extracellular proteins lgi1 and caspr2 facilitated a change of view regarding the clinical importance of voltage gated potassium channel vgkc. Objective to describe patients thought initially to have cjd but found subsequently to have immunotherapyresponsive vgkc autoimmunity. Presence of voltagegated potassium channel complex antibody. The main ab groups associated with encephalitis in children are those against nmethyldaspartate receptor nmdar, voltage gated potassium channel complex vgkc, and glutamic acid decarboxylase gad. But when they had conducted the voltage gated potassium channel antibody test, the report came to be positive. Presence of voltagegated potassium channel complex. Limbic encephalitis associated with voltagegated potassium channelcomplex antibodies. Suspected limbic encephalitis and seizure in cats associated.
He was found to have high titre of voltagegated potassium channel antibodies in his serum. It is one of the most common forms of autoimmune limbic encephalitis in the absence of primary extracns tumors. Voltage gated potassium channel vgkc encephalitis is a recently recognized autoimmune condition with antibodies against components of the vgkc protein complex. Neurological diseases associated with autoantibodies. Hyponatremia and voltage gated potassium channel antibody.
A paraneoplastic panel was positive for voltagegated potassium channel antibodies. Background voltage gated potassium channel vgkccomplex antibodies can be associated with a range of immunotherapyresponsive clinical presentations including limbic encephalitis, morvans syndrome and acquired neuromyotonia. Voltage gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. Neurological diseases associated with autoantibodies targeting the voltagegated potassium channel complex. Voltagegated potassium channel antibodies associated limbic. Treatment of vgkc complex antibody associated limbic. Jun, 2006 antibodies reactive with neuronal voltagegated potassium channels vgkcs have been reported in patients with limbic encephalitis, which affects the medial temporal lobes and is characterized by subacute onset of temporal lobe seizures, memory impairment, and personality changes. Voltagegated, potassiumchannel antibodyassociated limbic. Limbic encephalitis associated with voltagegated potassium. Psychiatric symptoms have not been emphasised in the literature. Since autoimmune mediated encephalitis was recognised 15 years ago, several autoantibodies have been identified. The value of lgi1, caspr2 and voltagegated potassium channel.
Benign neonatal febrile convulsions1 and hereditary deafness syndromes2 are associated with mutations in the. Clinical relevance of positive voltagegated potassium channel. The diagnosis and treatment of autoimmune encephalitis. Vgkc voltagegated potassium channel complex l imbic encephalitis, typically characterized clinically by the subacute onset of partial and secondary generalized seizures, memory loss, and neuropsychiatric changes can be of either infectious or autoimmune. Neurological diseases associated with autoantibodies targeting the. A recent study of 15 cases of limbic encephalitis found raised vgkcabs associated with nonparaneoplastic disorders and remission following immunosuppressive treatment. He was found to have high titre of voltage gated potassium channel antibodies in his serum. Voltagegated potassium channel autoimmunity mimicking. Lgi1caspr2antibody encephalitis the encephalitis society. Voltage gated potassium channel vgkc antibodyassociated encephalitis is an autoimmune limbic encephalitis caused by binding of antibodies to voltage gated potassium channel associated proteins. Jul 04, 2017 some cases have been associated with cancer, and others occur without neoplasia. We describe a case of year girl female with encephalitis due to antibodies to vgkc with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. In the paraneoplastic form of the neuromyotonia, treatment of.
Voltagegated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. Voltagegated potassium channel vgkc complex antibodymediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of creutzfeldtjakob disease cjd. Voltagegated potassium channel antibodyassociated encephalitis with basal ganglia lesions article pdf available in neurology 6611. Mri findings in autoimmune voltagegated potassium channel. Lgi1 is the autoantigen associated with limbic encephalitis previously attributed to voltagegated potassium channels. Diseases associated with antibodies to voltagegated potassium channels p otassium channelopathies are caused either by autoantibodies directed against the potassium channels or by mutations in their encoding genes. Research into autoimmune encephalitides have significantly advanced recently. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and. R journal of clinical case reports kummer et al, j clin. Autoantibodies against the voltage gated kv1 potassium channel vgkc complex detected by radio immunoprecipitation assay ria in the sera of patients with autoimmune encephalitis do not bind to vgkccomplex channel how to cite this paper. Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak. However, the presence of these antibodies has not been previously associated with motor neuropathy.
Limbic encephalitis morvans syndrome neuromyotonia voltagegated potassium channel a 31yearold righthanded man presented with a 2month history of brief episodes of daydreaming, visual and olfactory hallucinations, memory loss, poor attention, confusion, and sleep disturbances. The prompt recognition and treatment could mitigate the morbidity associated with this entity, but the broadspectrum of neurological manifestations often makes the diagnosis a challenge. Clinical phenotype can vary, but patients typically present with a limbic encephalitis involving amnesia, seizures, psychiatric disturbance and occasionally faciobrachial dystonic. Postmalaria neurologic syndromeautoimmune encephalitis with. Three groups of voltagegated potassium channel vgkc antibodypositive patients should be distinguished.
Some cases have been associated with cancer, and others occur without neoplasia. R journal of clinical case reports kummer et al, j clin case. Causes of encephalitis and differences in their clinical. Vgkc antibodies were first discovered by shillito et al. Case report confusion, faciobrachial dystonic seizures. The term limbic encephalitis associated with antibodies against voltagegated potassium channels should be changed to limbic encephalitis associated with lgi1 antibodies, and this disorder should be classed as an autoimmune synaptic encephalopathy. Pdf voltagegated potassium channel antibodyassociated. Psychiatric presentation of voltagegated potassium. Autoimmune voltage gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. We describe a case of year girl female with encephalitis due to antibodies to. Management antiepileptic drugs, immunomodulatory therapy, oral steroids, plasma exchange. We are emphasising the importance of considering a rare diagnosis, voltagegated potassium channel antibodyassociated limbic encephalitis, in an 80yearold gentleman who presented with memory impairment, seizure and hyponatraemia. Autoimmune encephalitis is an antibodymediated, subacute inflammatory process with neuropsychiatric features.
Voltagegated potassium channel antibodymediated syndromes. Psychiatric presentation of voltagegated potassium channel. Objective to evaluate the clinical significance associated. The main ab groups associated with encephalitis in children are those against nmethyldaspartate receptor nmdar, voltagegated potassium channel complex vgkc, and glutamic acid decarboxylase gad. Pdf voltagegated potassium channelassociated limbic. The searches were restricted to studies on patients age 18 or above, published in english.
Voltage gated potassium channel vgkc antibody encephalitis. Antivoltagegated potassium channel limbic encephalitis with. However, there are patients with positive levels in whom the significance is uncertain. These antibodies were present in three of six patients with neuromyotonia, a. Voltage gated limbic encephalitis question encephalitis. The discovery, in 2010, of autoantibodies against the extracellular proteins lgi1 and caspr2 facilitated a change of view regarding the clinical. Response to treatment is assessed by serially measuring. Voltage gated potassium channel vgkc complex antibodymediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of creutzfeldtjakob disease cjd. Antibodies to voltage gated potassium channel, antivgkc. Background voltagegated potassium channel vgkccomplex antibodies can be associated with a range of immunotherapyresponsive clinical presentations including limbic encephalitis, morvans syndrome and acquired neuromyotonia. Clinical relevance of positive voltagegated potassium. Early diagnosis is crucial because, unlike other neurodegenerative conditions, it is potentially fully reversible with. Jan 01, 2015 neurological diseases associated with autoantibodies targeting the voltage gated potassium channel complex. Limbic encephalitis associated with antivoltagegated.
Although it is often characterized by amnesia, disorientation, and seizures, it is still very poorly characterized, and a myriad of presentations have been described. Encephalitis due to antibodies to voltage gated potassium. Stroke preceding autoimmune encephalitis with neuronal. About 3 months after the onset of his symptoms, the patient was found to be positive for voltagegated potassium channel antibodies at 2000 pm normal treatment, immunotherapy and supportive care are important components of treatment of pns. Patients often spend some weeks in hospital receiving specialist treatment and assessment. Our study aimed to describe the frequency, clinical spectrum, and longterm outcomes in patients presenting with acute psychosis who have antinmethyldaspartate nmda receptor or anti voltage gated potassium channel vgkc encephalitis.
By using consensus definitions and a retrospective singlecenter cohort of 164 australian children, we performed clinical and radiologic phenotyping blinded to etiology and outcomes, and we tested archived acute sera for autoantibodies to nmethyldaspartate receptor, voltagegated potassium channel complex, and other neuronal antigens. The value of lgi1, caspr2 and voltagegated potassium. Pdf the syndrome of limbic encephalitis le associated with antibodies against voltagegated potassium channels vgkcle has recently been. Antibodies reactive with neuronal voltagegated potassium channels vgkcs have been reported in patients with limbic encephalitis, which affects the medial temporal lobes and is characterized by subacute onset of temporal lobe seizures, memory impairment, and personality changes. Encephalitis due to antibodies to voltage gated potassium channel vgkc typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. If the antibodies are found in a patient with the recognised clinical features, lgi1caspr2 antibody encephalitis can be treated by dampening down the immune reaction that is causing the inflammation. The features had some similarity to those described in humans with limbic encephalitis and voltage. Jul 04, 20 he had a relapse and on june 22, 20 and was rushed to the hospital where a pet scan was conducted. Voltagegated potassium channels vgkcs represent a group of. Guidelines for the treatment of autoimmune neuromuscular transmission disorders. Hi my name is ella, i have a 74 year old father that been diagnosed since feb 2014 with voltagegated potassium channel antibodyrelated limbic encephalitis. Antivgkccomplex encephalitis are caused by antibodies against the voltage gated potassium channel complex vgkccomplex and are implicated in several autoimmune conditions including limbic encephalitis, epilepsy and neuromyotonia i.
Voltagegated potassium channel antibody encephalopathy, a rare cause of limbic encephalopathy, typically presents with memory impairment and seizures. Immunotherapy is very effective in pns associated with cell membraneassociated antibodies like voltage gated potassium channel complex, nmda receptor antibodies and voltage gated calcium channel antibodies. A 61 yo male presented with cognitive decline and frequent right arm stiffening, which is recognised as faciobrachial dystonic seizure. Her lumbar puncture was clear, although there were some abnormalities with her eeg. We present 3 patients with positive vgkc antibodies to emphasise the spectrum of clinic presentation and value of antibody testing. Pdf on oct 2, 2014, akiyuki hiraga and others published voltagegated potassium channel antibodyassociated encephalitis with claustrum lesions find, read and cite all the research you need on. Voltagegated potassium channel vgkc autoantibodies were reported initially in acquired neuromyotonia 10 and subsequently in morvan syndrome, 11 limbic encephalitis, 12 other subacute encephalopathies, and limited manifestations of autoimmune dysautonomia. Nonneoplastic, autoimmune le associated with vgkcabs has been described recently. Background rapidly progressive dementia has a variety of causes, including creutzfeldtjakob disease cjd and neuronal voltagegated potassium channel vgkc autoantibodyassociated encephalopathy. Case report high grade glioma mimicking voltage gated. Serum voltagegated potassium channel vgkc antibodies were. A case of voltagegated potassium channel antibodyrelated.
Autoimmune voltagegated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Each alpha subunit is composed of six transmembrane domains with the pore occurring between the fifth and sixth domains, and the fourth domain serving as the voltage sensor due to its high positive charge. Voltage gated potassium channel antibodies positive autoimmune encephalopathy in a child. Aug 02, 2015 however, as a matter of course more tests were done and the test for voltage gated potassium channel antibodies has been consistently raised. Voltage gated potassium channel antibody encephalopathy, a rare cause of limbic encephalopathy, typically presents with memory impairment and seizures. Autoantibodies against the voltagegated potassium channel vgkc. Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion. No tumours or lung cancer was detected except for a slightly elevated nse. He was found to have antibodies to voltagegated potassium channels. Early treatment may prevent mortality associated with intractable seizures. Voltage gated potassium channel antibodies positive. A 58yearold female, a known case of auto immune encephalitis voltage gated potassium channel and steroid induced diabetes mellitus presented with progressive worsening of vertigo, recurrent myoclonic jerks and post ictal confusion for last 7 days. Treatment with intravenous immunoglobulin and pulse methylprednisolone led to complete resolution of symptoms, weight gain, and normalization of vital signs and plasma metanephrines. Postmalaria neurologic syndrome pmns is a neurologic or psychiatric disorder that occurs within 2 months of treatment and resolution.
Patient with voltagegated potassium channel encephalitis julian yaxley department of internal medicine, redcliffe hospital, redcliffe, queensland, australia autoimmune limbic encephalitis is a rare cause of encephalitic disease. Autoimmune encephalitis related to voltagegated potassium channel vgkc antibodies can occur as a complication of cancer but, more frequently, as a nonparaneoplastic disorder. Antibodies to voltagegated potassium channels have been implicated in causing a host of peripheral and central nervous system disorders. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. Autoimmune vgkc encephalitis can be diagnosed by serologic testing and is.
Postmalaria neurologic syndrome pmns is a neurologic or psychiatric disorder that occurs within 2 months of treatment and resolution of acute cerebral malaria 3, 4. Limbic encephalitis associated with voltagegated potassium channel antibodies vgkcabs may frequently be nonparaneoplastic. The term limbic encephalitis le describes the condition when limbic areas of the brain are inflamed swollen and. The patient was diagnosed with voltagegated potassium channel antibodyrelated limbic encephalitis based on clinical presentation, imaging findings, and antibody testing. Adem, antibodyassociated encephalitis voltagegated potassium channel and nmda receptor, mycobacterium tuberculosis, other bacterial encephalitis, herpes simplex virus. Limbic encephalitis morvans syndrome neuromyotonia voltagegated potassium channel a 31yearold righthanded man presented with a 2month history of brief episodes of daydreaming, visual and olfactory hallucinations, memory loss, poor attention, confusion, and. We are emphasising the importance of considering a rare diagnosis, voltage gated potassium channel antibodyassociated limbic encephalitis, in an 80yearold gentleman who presented with memory impairment, seizure and hyponatraemia. Our study aimed to describe the frequency, clinical spectrum, and longterm outcomes in patients presenting with acute psychosis who have antinmethyldaspartate nmda receptor or antivoltagegated potassium channel vgkc encephalitis. Limbic encephalitis associated with voltage gated potassium channel complex antibodies. Clinical presentation of antinmethyldaspartate receptor. It is associated with various target antigens and is difficult to diagnose, and experience with its treatment is. Lgi1caspr2 antibody encephalitis is a serious disease. Treatment included the addition of a second antiepileptic agent and oral steroids.
1403 1523 1176 113 746 1245 258 696 183 452 1249 1448 1139 1622 68 1675 320 568 573 333 58 1336 231 732 559 90 1427 923 1317 1272 974 887 1200 79 851 919 509 310