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Limbic encephalopathy

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https://www.readbyqxmd.com/read/28628018/autoimmune-limbic-encephalitis-case-reports
#1
K Kobaidze, A Harrison, Y Burklin, V Patidar, M Riccardi
Limbic encephalitis (LE) is an autoimmune or paraneoplastic disease that affects the medial temporal lobes. The patient will usually present with cognitive impairment, psychiatric changes, and seizures. Autoimmune limbic encephalitis (LE) is a challenging diagnosis as it is not always included in the typical paraneoplastic/autoimmune panels. Anti-GAD antibodies are associated with various disease including type I diabetes mellitus, various autoimmune processes, some neoplastic and infectious diseases. Thus, it is not as specific as some of the antibodies causing LE...
May 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28545782/autoimmune-encephalopathy-for-psychiatrists-when-to-suspect-autoimmunity-and-what-to-do-next
#2
REVIEW
Mark Oldham
OBJECTIVE: To provide a critical review of autoimmune encephalopathy-broadly defined as neuropsychiatric features directly related to an autoimmune process-relevant for psychiatric practice. METHODS: We consulted rheumatology textbooks to define the scope of autoimmune conditions and identified recent reviews of rheumatic conditions, autoimmune vasculitis, and autoimmune encephalitis. We integrated these with primary reports to provide a clinically relevant overview of autoimmune encephalopathy...
May 2017: Psychosomatics
https://www.readbyqxmd.com/read/28360986/confusion-faciobrachial-dystonic-seizures-and-critical-hyponatremia-in-a-patient-with-voltage-gated-potassium-channel-encephalitis
#3
Julian Yaxley
Autoimmune limbic encephalitis is a rare cause of encephalitic disease. It is associated with various target antigens and is difficult to diagnose, and experience with its treatment is limited. This case report describes a 69-year-old man, who presented with life-threatening hyponatremia and confusion, following several months of gradually worsening faciobrachial dystonic seizures. Faciobrachial dystonic seizures are a well-described feature classically observed in voltage-gated potassium channel autoimmune encephalitis...
March 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28272206/limbic-encephalitis-associated-with-anti-nh2-terminal-of-%C3%AE-enolase-antibodies-a-clinical-subtype-of-hashimoto-encephalopathy
#4
Toru Kishitani, Akiko Matsunaga, Masamichi Ikawa, Kouji Hayashi, Osamu Yamamura, Tadanori Hamano, Osamu Watanabe, Keiko Tanaka, Yasunari Nakamoto, Makoto Yoneda
Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagnosed with HE and respond to immunotherapy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28183838/autoimmune-encephalitis-pathophysiology-and-imaging-review-of-an-overlooked-diagnosis
#5
REVIEW
B P Kelley, S C Patel, H L Marin, J J Corrigan, P D Mitsias, B Griffith
Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteristic findings within limbic structures suggestive of autoimmune encephalitis can be a key step in alerting clinicians to the potential diagnosis and ensuring a prompt and appropriate clinical work-up...
June 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28121634/fluorodeoxyglucose-positron-emission-tomography-fdg-pet-correlation-of-histopathology-and-mri-in-prion-disease
#6
Karin P Mente, James K O'Donnell, Stephen E Jones, Mark L Cohen, Nicolas R Thompson, Alberto Bizzi, Pierluigi Gambetti, Jiri G Safar, Brian S Appleby
Creutzfeldt-Jakob disease (CJD) and other prion diseases are rapidly progressive spongiform encephalopathies that are invariably fatal. Clinical features and magnetic resonance imaging, electroencephalogram, and cerebrospinal fluid abnormalities may suggest prion disease, but a definitive diagnosis can only be made by means of neuropathologic examination. Fluorodeoxyglucose positron emission tomography (FDG-PET) is not routinely used to evaluate patients with suspected prion disease. This study includes 11 cases of definite prion disease in which FDG-PET scans were obtained...
January 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/27940600/the-prion-like-behavior-of-assembled-tau-in-transgenic-mice
#7
Florence Clavaguera, Markus Tolnay, Michel Goedert
Tauopathies constitute neurodegenerative diseases that are characterized by the intracellular deposition of filaments made of hyperphosphorylated tau protein. The pattern of tau deposition in Alzheimer's disease follows a stereotypical progression, with the first lesions appearing in the locus coeruleus and entorhinal cortex, from where they appear to spread to the hippocampus and neocortex. Propagation of pathological tau is also characteristic of argyrophilic grain disease, where the lesions seem to spread through distinct regions of the limbic system...
December 9, 2016: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/27688922/delirious-mania-associated-with-autoimmune-gastrothyroidal-syndrome-of-a-mid-life-female-the-role-of-hashimoto-encephalopathy-and-a-3-year-follow-up-including-serum-autoantibody-levels
#8
Udo Bonnet, Claudia Selle, Ralf Kuhlmann
We report the case study of a 57-year-old Caucasian female with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), commonly termed Hashimoto encephalopathy (HE). This presentation includes one of the longest lasting follow-up studies of HE considering the neuropsychiatric symptoms (here delirium, mania, and EEG-slowing) and their relation to serum autoantibody levels. Antithyroid-peroxidase autoantibodies, the hallmark of autoimmune thyroiditis, were found in the serum and also in the cerebrospinal fluid...
2016: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/27667487/-voltage-gated-potassium-channel-complex-antibodies-associated-encephalopathy-and-related-diseases
#9
Osamu Watanabe
Voltage-gated potassium channel (VGKC) complex antibodies are auto-antibodies, initially identified in acquired neuromyotonia (aNMT; Isaacs' syndrome), which cause muscle cramps and difficulty in opening the palm of the hands. Subsequently, these antibodies were found in patients presenting with aNMT along with psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) patient with prominent amnesia and frequent seizures. Typical LE cases have a distinctive adult-onset, frequent, brief dystonic seizure semiology that predominantly affects the arms and ipsilateral face...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27538995/roseolovirus-associated-encephalitis-in-immunocompetent-and-immunocompromised-individuals
#10
REVIEW
Joseph Ongrádi, Dharam V Ablashi, Tetsushi Yoshikawa, Balázs Stercz, Masao Ogata
The roseoloviruses, human herpesvirus (HHV)-6A, HHV-6B, and HHV-7, can cause severe encephalitis or encephalopathy. In immunocompetent children, primary HHV-6B infection is occasionally accompanied by diverse clinical forms of encephalitis. Roseolovirus coinfections with heterologous viruses and delayed primary HHV-7 infection in immunocompetent adults result in very severe neurological and generalized symptoms. Recovery from neurological sequelae is slow and sometimes incomplete. In immunocompromised patients with underlying hematological malignancies and transplantation, frequent single or simultaneous reactivation of roseoloviruses elicit severe, lethal organ dysfunctions, including damages in the limbic system, brain stem, and hippocampus...
February 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/27466467/anti-lgi1-associated-cognitive-impairment-presentation-and-long-term-outcome
#11
Helena Ariño, Thais Armangué, Mar Petit-Pedrol, Lidia Sabater, Eugenia Martinez-Hernandez, Makoto Hara, Eric Lancaster, Albert Saiz, Josep Dalmau, Francesc Graus
OBJECTIVE: We investigated a series of patients with LGI1 antibody (Ab)-related cognitive deterioration to determine the clinical presentation, long-term outcome, and LGI1 Ab evolution. METHODS: We retrospectively analyzed the clinical information of 76 patients with LGI1 Ab-related cognitive deterioration. Presenting syndromes were classified as limbic encephalitis (LE), non-LE, or encephalopathy (normal MRI and no CSF pleocytosis). Frequency of relapses and clinical outcome were assessed in 48 patients with prolonged follow-up (median 39 months, range 18-200)...
August 23, 2016: Neurology
https://www.readbyqxmd.com/read/27465029/rapidly-progressive-neurological-deterioration-in-anti-ampa-receptor-encephalitis-with-additional-crmp5-antibodies
#12
Shuangshuang Yang, Jie Qin, Jinghong Li, Yuan Gao, Lu Zhao, Jun Wu, Bo Song, Yuming Xu, Shilei Sun
Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis positive for additional onconeural antibodies is rarely reported. Here we report the clinical features of a patient who developed limbic encephalitis with both glutamate receptor 2 (GluR2) and collapsin response mediator protein 5 (CRMP5) antibodies. Brain magnetic resonance imaging revealed multifocal encephalopathy. Chest computed tomography showed a highly suspicious malignant thymoma. He experienced rapid neurological deterioration during hospitalization...
November 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27460184/anti-ma-and-anti-ma2-associated-paraneoplastic-neurological-syndromes
#13
G Ortega Suero, N Sola-Valls, D Escudero, A Saiz, F Graus
OBJECTIVE: Analyse the clinical profile, associated tumour types, and response to treatment of paraneoplastic neurological syndromes associated with antibodies against Ma proteins. METHODS: A retrospective study of patients with antibodies against Ma proteins identified in a neuroimmunology laboratory of reference. RESULTS: Of the 32 patients identified, 20 showed reactivity against Ma2 only (anti-Ma2 antibodies), 11 against Ma1 and Ma2 (anti-Ma antibodies), and 1 with reactivity against Ma1 only (anti-Ma1 antibodies)...
July 25, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/27432809/encephalopathy-with-upper-body-hypertonia-and-myoclonus-in-patient-with-systemic-lupus-erythematosus-and-anti-caspr2
#14
A Castro, J C Romeu, R Geraldes, J A Pereira da Silva
Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET).Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies...
January 2017: Lupus
https://www.readbyqxmd.com/read/27431532/non-paraneoplastic-limbic-encephalitis-and-central-nervous-hhv-6b-reactivation-causality-or-coincidence
#15
Pitt Niehusmann, Guido Widman, Anna M Eis-Hübinger, Susanne Greschus, Barbara K Robens, Alexander Grote, Albert J Becker
Autoantibody-related encephalopathies represent an important differential diagnosis in adult onset epilepsy. Here, we report the case of a 25-year-old patient with new-onset epilepsy and psychotic syndrome, who underwent biopsy resection for etiological classification. MRI analysis and neuropathological examination showed a T-lymphocytic dominated encephalitis with involvement of the limbic system. An indirect immunohistochemistry approach identified autoantibodies against glutamic acid decarboxylase (GAD) in cerebral spinal fluid and serum, which were confirmed by affinity purification / mass spectrometry analysis...
August 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27371488/the-clinical-spectrum-of-caspr2-antibody-associated-disease
#16
Agnes van Sonderen, Helena Ariño, Mar Petit-Pedrol, Frank Leypoldt, Peter Körtvélyessy, Klaus-Peter Wandinger, Eric Lancaster, Paul W Wirtz, Marco W J Schreurs, Peter A E Sillevis Smitt, Francesc Graus, Josep Dalmau, Maarten J Titulaer
OBJECTIVE: To report a large cohort of patients with antibodies against contactin-associated protein-like 2 (Caspr2) and provide the clinical spectrum of this disorder. METHODS: Serum and CSF samples were assessed at 2 neuroimmunology centers in Barcelona and Rotterdam. Patients were included if Caspr2 antibodies were confirmed with 2 independent techniques, including brain immunohistochemistry and cell-based assay. Clinical information was obtained by the authors or provided by treating physicians after patients' informed consent...
August 2, 2016: Neurology
https://www.readbyqxmd.com/read/27356651/anti-glutamic-acid-decarboxylase-antibody-positive-neurological-syndromes
#17
REVIEW
Hassaan Tohid
A rare kind of antibody, known as anti-glutamic acid decarboxylase (GAD) autoantibody, is found in some patients. The antibody works against the GAD enzyme, which is essential in the formation of gamma aminobutyric acid (GABA), an inhibitory neurotransmitter found in the brain. Patients found with this antibody present with motor and cognitive problems due to low levels or lack of GABA, because in the absence or low levels of GABA patients exhibit motor and cognitive symptoms. The anti-GAD antibody is found in some neurological syndromes, including stiff-person syndrome, paraneoplastic stiff-person syndrome, Miller Fisher syndrome (MFS), limbic encephalopathy, cerebellar ataxia, eye movement disorders, and epilepsy...
July 2016: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/27112681/autoimmune-dementia-and-encephalopathy
#18
Eoin P Flanagan, Daniel A Drubach, Bradley F Boeve
Autoimmune dementia and encephalopathies (ADE) are complex disorders that can cause immune-mediated cognitive deficits and have confusing nomenclature. Presentation varies from acute limbic encephalitis to subacute or chronic disorders of cognition mimicking neurodegenerative dementia. It may occur as a paraneoplastic phenomenon or an idiopathic autoimmune phenomenon. The presence of a personal/family history of autoimmunity, inflammatory spinal fluid, serologic evidence of autoimmunity (neural or nonorgan-specific), or mesial temporal magnetic resonance imaging abnormalities are clues to diagnosis...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27112679/anti-nmdar-encephalitis-and-other-glutamate-and-gaba-receptor-antibody-encephalopathies
#19
Marienke A A M De Bruijn, Maarten J Titulaer
Over the last few year, antibodies to various central nervous system receptors, particularly the glutamate and γ-aminobutyric acid (GABA) receptors, have been found to be associated with autoimmune neurologic disorders. The receptors include the N-methyl-d-aspartate receptor (NMDAR), the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), the metabotropic glutamate receptors (mGluRs), and GABA type A and B receptors (respectively GABAAR and GABABR). Compared to the previously described paraneoplastic antibodies directed at intracellular targets, the patients with receptor antibodies are often younger, they less frequently have malignancies, and they respond better to immunotherapy...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27112678/voltage-gated-potassium-channel-complex-autoimmunity-and-associated-clinical-syndromes
#20
REVIEW
Sarosh R Irani, Angela Vincent
Voltage-gated potassium channel (VGKC)-complex antibodies are defined by the radioimmunoprecipitation of Kv1 potassium channel subunits from brain tissue extracts and were initially discovered in patients with peripheral nerve hyperexcitability (PNH). Subsequently, they were found in patients with PNH plus psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) with prominent amnesia and frequent seizures. Most recently, they have been described in patients with pure epilepsies, especially in patients with the novel and distinctive semiology termed faciobrachial dystonic seizures (FBDS)...
2016: Handbook of Clinical Neurology
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