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Limbic encephalopathy

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
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
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...
August 18, 2016: Journal of Neurovirology
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
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
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 23, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
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...
July 17, 2016: Lupus
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
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
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
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
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
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
M Carmen Temboury Molina, M Luisa Ruiz-Falco Rojas, Isabel Palmi Cortés, Raquel Villamor Martín
No abstract text is available yet for this article.
June 2016: Endocrinología y Nutrición: órgano de la Sociedad Española de Endocrinología y Nutrición
Shu-Ping Peng, Yi-Ning Li, Jun Liu, Zhi-Yuan Wang, Zi-Shu Zhang, Shun-Ke Zhou, Fang-Xu Tao, Zhi-Xue Zhang
Cerebral blood flow is strongly associated with brain function, and is the main symptom and diagnostic basis for a variety of encephalopathies. However, changes in cerebral blood flow after mild traumatic brain injury remain poorly understood. This study sought to observe changes in cerebral blood flow in different regions after mild traumatic brain injury using pulsed arterial spin labeling. Our results demonstrate maximal cerebral blood flow in gray matter and minimal in the white matter of patients with mild traumatic brain injury...
February 2016: Neural Regeneration Research
Keiko Tanaka
Paraneoplastic neurological syndrome with limbic encephalopathy tends to progress rapidly, presenting with physical symptoms such as ataxia or sensory disturbance. However, some affected patients demonstrate amnesia, inactivity, or abnormal behavior, which lead to the diagnosis of dementia. It is important to perform an extensive differential diagnosis with autoantibody-examination and tumor survey, so as not to overlook potentially treatable dementia.
April 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Li Zhang, Qiang Lu, Hong-Zhi Guan, Jun-Hua Mei, Hai-Tao Ren, Ming-Sheng Liu, Bin Peng, Li-Ying Cui
BACKGROUND: Morvan syndrome is a rare disorder characterized by the combination of peripheral nerve hyperexcitability, encephalopathy and dysautonomia with marked insomnia. It was reported to have association to antibodies to voltage-gated potassium channels including contactin associated protein-like 2 antibodies (CASPR2-Ab) and leucine-rich glioma inactivated protein 1 antibodies (LGI1-Ab). LGI1-Ab was reported to associate with seizures, amnesia, confusion, hyponatraemia and a good prognosis, while CASPR2-Ab with peripheral presentations, probable risk for tumor and a poor prognosis...
2016: BMC Neurology
Matteo Gastaldi, Anaïs Thouin, Angela Vincent
Over the last 15 years it has become clear that rare but highly recognizable diseases of the central nervous system (CNS), including newly identified forms of limbic encephalitis and other encephalopathies, are 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 1 protein and contactin-associated protein like 2, that are associated with voltage-gated potassium channels...
January 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Matthew C Loftspring, Eric Landsness, Lindsey Wooliscroft, Robert Rudock, Sally Jo, Kevin R Patel
Autoantibodies to the γ-aminobutyric acid receptor, subtype B (GABAB), are a known cause of limbic encephalitis. The spectrum of clinical manifestations attributable to this antibody is not well defined at the present time. Here we present a case of GABAB encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure. To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome.
2015: Case Reports in Neurological Medicine
Vikram Puvenna, Madeline Engeler, Manoj Banjara, Chanda Brennan, Peter Schreiber, Aaron Dadas, Ashkon Bahrami, Jesal Solanki, Anasua Bandyopadhyay, Jacqueline K Morris, Charles Bernick, Chaitali Ghosh, Edward Rapp, Jeffrey J Bazarian, Damir Janigro
Repetitive traumatic brain injury (rTBI) is one of the major risk factors for the abnormal deposition of phosphorylated tau (PT) in the brain and chronic traumatic encephalopathy (CTE). CTE and temporal lobe epilepsy (TLE) affect the limbic system, but no comparative studies on PT distribution in TLE and CTE are available. It is also unclear whether PT pathology results from repeated head hits (rTBI). These gaps prevent a thorough understanding of the pathogenesis and clinical significance of PT, limiting our ability to develop preventative and therapeutic interventions...
January 1, 2016: Brain Research
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