scholarly journals Limbic autoimmune encephalitis with antibodies to glutamate decarboxylase (GAD)

2020 ◽  
Vol 19 (2) ◽  
pp. 67-71
Author(s):  
L. U. Ulukhanova ◽  
M. M. Yaraliev ◽  
S. M. Attaeva
Keyword(s):  
Membrane Proteins ◽  
Glutamate Decarboxylase ◽  
Clinical Case ◽  
Limbic Encephalitis ◽  
Autoimmune Encephalitis ◽  
Neuronal Membrane ◽  
Neurological Syndrome ◽  
Antibodies To Glutamate ◽  
Current Events

Limbic encephalitis (LE) is a rare neurological syndrome, in most cases has a paraneoplastic nature, but recently there has been a large number of observations of idiopathic autoimmune LE associated with antibodies to neuronal membrane proteins. A clinical case of a disease in a 13-year-old patient with the detection of antibodies to glutamate decarboxylase (GAD) is described. The debut of the disease is marked by a decrease in memory for current events after the first convulsive attack during 2 months.

Enoxaparin-induced Wunderlich syndrome in a young patient with anti-GAD 65-associated opsoclonus and limbic encephalitis: a rare complication in a rare disease

2021 ◽  
Vol 14 (10) ◽  
pp. e244916
Author(s):  
Saranya B Gomathy ◽  
Animesh Das ◽  
Awadh Kishor Pandit ◽  
Achal Kumar Srivastava
Keyword(s):  
Rare Disease ◽  
Young Patient ◽  
Clinical Condition ◽  
Limbic Encephalitis ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Rare Condition ◽  
Gad 65 ◽  
Good Improvement

Wunderlich syndrome is a rare condition characterised by acute spontaneous non-traumatic renal haemorrhage into the subcapsular and perirenal spaces. Our case of anti-GAD65-associated autoimmune encephalitis (AE), aged 30 years, developed this complication following use of enoxaparin and was managed by selective glue embolisation of subsegmental branches of right renal cortical arteries. Our case had opsoclonus as one of the clinical manifestations, which has till now been described in only two patients of this AE. This patient received all forms of induction therapies (steroids, plasmapheresis, intravenous immunoglobulin and rituximab) following which she had good improvement in her clinical condition. The good response to immunotherapy is also a point of discussion as this has been rarely associated with anti-GAD65 AE.

scholarly journals Fulminant thymomatous AMPAR-antibody limbic encephalitis with hypertonic coma, bruxism, an isoelectric electroencephalogram and temporal cortical atrophy, with recovery

2019 ◽  
Vol 12 (2) ◽  
pp. e227893
Author(s):  
Nicolás Urriola ◽  
Kavie Soosapilla ◽  
James Drummond ◽  
Mark Thieben
Keyword(s):  
Botulinum Toxin ◽  
Glutamate Receptor ◽  
Limbic Encephalitis ◽  
Botulinum Toxin Injection ◽  
Autoimmune Encephalitis ◽  
Cortical Atrophy ◽  
Impaired Consciousness ◽  
Clinical Recovery ◽  
Clinical Presentations ◽  
Electroencephalogram Eeg

Autoimmune encephalitides are a potentially devastating group of treatable disorders with a wide variety of clinical presentations. The most studied autoimmune encephalitis is caused by antibodies to the N-methyl-D-aspartate glutamate receptor. A rarer cause is due to antibodies against the evolutionarily related α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR). The full assortment of electroencephalogram (EEG) and clinical descriptions of the latter are yet to be fully described. A 44-year-old woman with impaired consciousness and subsequent coma characterised by an isoelectric EEG was diagnosed with AMPAR-antibody limbic encephalitis. MRI revealed temporal T2 hyperintensities that improved with immunosuppression, although leaving marked cortical atrophy. Gradual clinical improvement saw the development of aggressive bruxism requiring botulinum toxin injection with eventual meaningful clinical recovery. This case expands the clinical spectrum of AMPAR limbic encephalitis to include aggressive bruxism, and highlights that despite poor clinical and EEG findings at the outset, recovery is still possible.

scholarly journals Anti-voltage-gated potassium channel complex antibody–mediated limbic encephalitis: a case report of a 53-year-old man admitted to intensive care psychiatric unit with psychotic mania

2020 ◽  
Vol 33 (4) ◽  
pp. e100196
Author(s):  
Homayun Shahpesandy ◽  
Rosemary Mohammed-Ali ◽  
Ahmed Oladosu ◽  
Tarik Al-Kubaisy ◽  
Moses Anene ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Medial Temporal Lobe ◽  
Short Term Memory ◽  
Limbic Encephalitis ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Clonic Seizure ◽  
Neurological Symptoms ◽  
Psychiatric Symptomatology ◽  
Voltage Gated

Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psychosis, short-term memory impairment as well as faciobrachial and grand mal seizures. We aim to present a case of a 53-year-old man with positive anti–voltage-gated potassium channel (VGKC) complex antibodies who initially presented with symptoms of psychotic mania. Six weeks post-psychiatric symptomatology, he presented with neurological symptoms such as faciobrachial jerking and tonic-clonic seizure. The patient had no previous psychiatric history and was initially treated with psychotropic medications. Our experience emphasises the fact that limbic encephalitis is not easy to identify as most patients initially present with psychiatric symptomatology than neurological symptoms. Furthermore, immunological and laboratory testing takes a rather long time to determine the diagnosis. What is more, few psychiatrists consider autoimmune nature of the neuropsychiatric presentation. Therefore, it is important to consider autoimmune encephalitis in patients with new-onset psychosis or mania who also present with neurological symptoms at some stage of their illness. Characteristic indicators of autoimmune encephalitis include neurological symptoms such as facial twitching, seizures, confusion and cognitive decline.

scholarly journals The behavioral variant of frontotemporal dementia: a clinical case

2021 ◽  
Vol 13 (1) ◽  
pp. 67-73
Author(s):  
Yu. V. Kotsyubinskaya ◽  
V. A. Mikhailov ◽  
I. K. Stulov ◽  
N. I. Ananyeva ◽  
L. I. Sitnik
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Case ◽  
Alcohol Intoxication ◽  
Cerebral Atrophy ◽  
Behavioral Changes ◽  
Self Awareness ◽  
Obsessive Compulsive ◽  
Temporal Lobes ◽  
Personality Changes ◽  
Current Events

The paper is devoted to frontotemporal dementia (FTD), one of the most common forms of frontotemporal degeneration. The main symptoms of the disease include disinhibition, lack of empathy, obsessive-compulsive symptoms, apathy, cognitive impairment, appetite changes, and progressive changes in social behavior. In parallel, there are personality changes that are characterized by lower levels of self-awareness and by progressive psychological and social maladaptation of patients in society.The paper describes a clinical case of FTD in a female patient with marked behavioral changes and personality disorder. A 52-year-old woman was admitted to the alcoholism treatment department for alcohol intoxication and symptoms of mental confusion. According to her relatives, drinking too much alcohol every day, she was found to become rude, indifferent to others and her own duties, sharply limited the range of her activities and communication, and showed a decline in memory for current events. Psychopathological examination determined a distinct motivational-volitional decrease, the patient’s inability to mobilize mental activity, non-critical thinking, and indifference. X-ray diagnosis revealed the changes characteristic of frontotemporal neurodegeneration (atrophy of the frontal and temporal lobes prevails). The described case confirms that alcohol abuse can mask organic disorders that develop in systemic cerebral atrophy.

Autoimmune Encephalitis

2017 ◽  
Author(s):  
Andrew McKeon
Keyword(s):  
Multiple Sclerosis ◽  
Plasma Membrane ◽  
Limbic Encephalitis ◽  
Autoimmune Encephalitis ◽  
Cytotoxic T Cell ◽  
Diagnostic Laboratory ◽  
Cns Disorders ◽  
Membrane Antigens ◽  
Specific Igg

Autoimmune encephalitis clinically encapsulates a spectrum of disorders including limbic encephalitis, and other autoimmune CNS disorders, which often have a paraneoplastic cause. Unlike multiple sclerosis, autoimmune encephalitides are unified by well-characterized neural-specific IgG biomarkers detectable in serum or CSF. Diagnostic laboratory, in vitro and neuropathological studies have demonstrated two broad groups. The first, characterizable by the detection of neuronal nuclear, cytoplasmic, or nucleolar antibodies (such as ANNA-1, aka anti-Hu), likely have a cytotoxic T cell mediated pathogenesis. The second, characterizable by the detection of antibodies targeting plasma membrane antigens, such as NMDA receptor, are likely mediated (at least in part) by pathogenic antibody.

scholarly journals Autoimmune Limbic Encephalitis in a Patient with Acute Encephalopathy and Hyponatremia

2019 ◽  
Vol 2019 ◽  
pp. 1-5 ◽  
Author(s):  
K. A. Agarwal ◽  
M. Albertolle ◽  
M. Tiru
Keyword(s):  
Hospital Admissions ◽  
Limbic Encephalitis ◽  
Autoimmune Encephalitis ◽  
Developed Countries ◽  
Acute Encephalopathy ◽  
Neurological Outcomes ◽  
Common Clinical Presentation ◽  
Autoimmune Limbic Encephalitis ◽  
The Developed Countries ◽  
Mri Changes

Acute encephalopathy is a common clinical presentation for hospital admissions. Autoimmune encephalitis is a rare cause of encephalopathy which has increasingly been recognized over the last decade. The detection of various neuronal antibodies has helped diagnose these syndromes, but they have limited availability, mostly in the developed countries. We present a case of a middle-aged female presenting with memory impairment, gait disturbances, and hyponatremia. A clinical diagnosis of autoimmune limbic encephalitis was made based on faciobrachial dystonic seizures, SIADH, and MRI changes 10 days prior to autoantibody titer returned. Prompt treatment with steroids and intravenous immunoglobulin was started with improvement in her neurological symptoms. This case highlights the importance of considering autoimmune encephalitis syndromes in the differential diagnosis of patients with classical neurological presentations and prompt diagnosis and immunotherapy to improve neurological outcomes.

Herpes-associated autoimmune encephalitis (clinical case)

2019 ◽  
Vol 17 (7) ◽  
pp. 167-169
Author(s):  
Z. A. Goncharova ◽  
◽  
V. A. Megeryan ◽  
N. M. Yarosh ◽  
S. M. Sehwail ◽  
...  
Keyword(s):  
Clinical Case ◽  
Autoimmune Encephalitis

Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment

2020 ◽  
Vol 19 (8) ◽  
pp. 584-598
Author(s):  
Mette Scheller Nissen ◽  
Matias Ryding ◽  
Morten Meyer ◽  
Morten Blaabjerg
Keyword(s):  
Limbic Encephalitis ◽  
Current Knowledge ◽  
Autoimmune Encephalitis ◽  
Epileptic Seizures ◽  
Surface Proteins ◽  
Future Research ◽  
Cell Surface Antigens ◽  
Treatment Regimens ◽  
Nmda Receptor Encephalitis ◽  
Disease Mechanisms

Autoimmune Encephalitides (AE) comprises a group of diseases with antibodies against neuronal synaptic and cell surface antigens. Since the discovery of the most common subtype, NMethyl- D-Aspartate (NMDA) receptor encephalitis, an astonishing number of novel disease-causing antibodies have been described. This includes other glutamatergic and GABAergic receptor antibodies and antibodies against various other surface proteins. Many of these novel conditions present as limbic encephalitis with memory impairment, psychiatric features and epileptic seizures, often alongside subtype specific clinical features. Others present with a clinical disease course specific to the antibody. In contrast to the well-known paraneoplastic syndromes with antibodies directed against intracellular antigens (e.g. limbic encephalitis with Hu antibodies), autoimmune encephalitides are often highly responsive to immunotherapy, with a good outcome if diagnosed and treated early. Prognosis depends on aggressive immunotherapy, often with a combination of corticosteroids, intravenous immunoglobulin, plasma exchange or in some cases anti-CD20 therapy and cyclophosphamide. Other treatment regimens exist, and prognosis varies between disease subtypes and occurrence of underlying cancer. We review current knowledge on subtype-specific clinical presentation, disease mechanisms, diagnosis including pitfalls, treatment paradigms and outcome in autoimmune encephalitides, and provide suggestions for future research.

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