Rapidly progressive global cerebral atrophy in the setting of anti-LGI1 encephalitis

Yelam, Anudeep; Nagarajan, Elanagan; Bollu, Pradeep C.

doi:10.1136/bcr-2018-228428

Cited by 5 publications

(4 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some studies suggest ( 9 – 11 , 23 ) that effective and long-term immunotherapy should be given to prevent long-term complications, including hippocampal atrophy ( 23 ) and sustained memory impairment ( 13 ). Second-line drugs can be added to the therapy of patients ( 23 , 24 ) who did not respond well to the first-line drugs or had a recurrence, including rituximab, MMF, or cyclophosphamide. Once cognitive impairment is confirmed, patients should receive immunotherapy ( 9 – 11 ) and long-term maintenance therapy to relieve their symptoms ( 23 , 24 ), improve prognosis, and avoid intractable epilepsy and hippocampal atrophy.…”

Section: Discussionmentioning

confidence: 99%

“…Second-line drugs can be added to the therapy of patients ( 23 , 24 ) who did not respond well to the first-line drugs or had a recurrence, including rituximab, MMF, or cyclophosphamide. Once cognitive impairment is confirmed, patients should receive immunotherapy ( 9 – 11 ) and long-term maintenance therapy to relieve their symptoms ( 23 , 24 ), improve prognosis, and avoid intractable epilepsy and hippocampal atrophy. Anti-LGI1 Ab encephalitis may recur or become chronic, as well as legacy cognitive sequelae.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

et al. 2020

View full text Add to dashboard Cite

Introduction: Anti-leucine-rich glioma-inactivated 1 antibody (anti-LGI1) encephalitis is one of the most common autoimmune encephalitis. Anti-LGI1 encephalitis presented with subacute or acute onset of cognitive impairment, psychiatric disturbances, faciobrachial dystonic seizures (FBDSs), convulsions, and hyponatremia. The common sequela of anti-LGI1 encephalitis is cognitive disorder, but there are few studies on the recovery of cognitive function after immunotherapy. This study aimed to explore clinical characteristics of cognitive impairment and 1-year outcome in patients with anti-LGI1 encephalitis. Methods: The clinical data and characteristics of cognitive impairment of 21 patients with anti-LGI1 encephalitis from 2016 to 2019 in Nanjing Brain Hospital were analyzed retrospectively. At the time of onset of hospitalization and 1 year after discharge, the cognitive functions in these patients were assessed using two cognitive screening scales—Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment-Basic (MoCA-B). Results: Among the 21 patients, 13 were male and 8 were female, aged 51.10 ± 14.69 (age range 20–72) years. Nineteen patients, comprising 90.48%, had recent memory deterioration. Routine electroencephalography (EEG) results of 13 cases were abnormal. EEG results were epileptic or slow-wave activity involving the temporal lobes. Eleven cases of brain MRI were abnormal, and the focus involved the hippocampus and mediotemporal lobe. The decrease of short-term memory [recall scores: 0.57 ± 0.81 (MMSE), 0.76 ± 1.34 (MoCA-B)] is the most obvious at the time of admission. After intravenous (IV) injection of methylprednisolone and/or immunoglobulin, the clinical symptoms of the patients improved obviously. Total MMSE and MoCA-B scores of patients were significant increased after 1 year (21.19 ± 3.54 vs. 26.10 ± 3.02, P < 0.001; and 19.00 ± 4.38 vs. 25.19 ± 4.25, P < 0.001, respectively). Recall scores and orientation scores of MoCA-B were significantly improved after 1 year (0.76 ± 1.34 vs. 3.24 ± 1.48, P < 0.001; and 3.10 ± 1.26 vs. 5.00 ± 1.22, P < 0.001, respectively). However, 3/21 (14.29%) patients still have obvious short-term memory impairment (recall scores ≤ 1). Conclusion: Cognitive impairment is one of the most common manifestations of anti-LGI1 encephalitis, with the main prominent being acute or subacute short-term memory loss. Although most patients with anti-LGI1 encephalitis respond well to immunotherapy, a small number of patients still have cognitive disorders, mainly recent memory impairment, after 1 year.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Compared with reports of literature, 59% of pediatric patients showed normal brain MRI and for those with abnormal results, the majority showed lesions of temporal lobe and hippocampus, which were also in accordance with adult anti-LGI1 encephalitis. Progressive cerebral atrophy was reported only in one adult case (Yelam et al, 2019). However, Szots et al (2017) found the existence of progressive global brain atrophy in patients with LGI1 limbic encephalitis, especially in temporal limbic structures, frontal lobe and the cerebellum, using a volumetric analysis method of the T1-weighted MRI data, despite early immunotherapy.…”

Section: Discussionmentioning

confidence: 99%

Anti-LGI1 encephalitis with initiating symptom of seizures in children

et al. 2023

View full text Add to dashboard Cite

BackgroundAnti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is infrequently reported but more and more recognizable in children. Here we give detailed description of the clinical features and long-term outcome of three cases of childhood onset anti-LGI1 encephalitis.MethodsThree anti-LGI1 encephalitis patients were hospitalized in the Department of Pediatrics at Qilu Hospital of Shandong University. Data about the clinical manifestations, treatments and long-term follow-up outcomes were described in detail.ResultsCase 1 showed an adolescent girl with initiating symptom of acute-onset frequent focal seizures. Her serum LGI1-antibody test was positive, and she had a good response to antiseizure medication (ASM) and IVIG. Case 2 showed a preschool-age boy with long-period refractory focal seizures and recent behavioral change. Both serum and cerebrospinal fluid (CSF) tests of LGI1-antibody were positive, and the MRI showed progressive atrophy in the left hemisphere. The symptoms got improved after receiving second-line immunotherapy initially but there are still the sequelae of drug-resistant epilepsy and mild to moderate intellectual disability. Case 3 showed an adolescent boy with initiating symptom of acute-onset frequent focal seizures. Both serum and CSF tests of LGI1-antibody were positive, and he had a good response to immunotherapy. By analyzing all literature-reported 19 pediatric cases, we found pediatric anti-LGI1 encephalitis is more common in female and adolescent. Seizures and behavioral changes were the most common symptoms. CSF pleocytosis and LGI1-antibodies results were mostly negative. Most patients showed good response to immunotherapy.ConclusionChildhood onset anti-LGI1 encephalitis is a heterogeneous clinical syndrome, ranging from typical limbic encephalitis to isolating focal seizures. It is important to test autoimmune antibodies when encountering similar cases and repeat antibody testing if necessary. Timely recognition leads to earlier diagnosis and more rapid initiation of effective immunotherapy and potentially better outcomes.

show abstract

“…Since antibody titers are yet not established to accurately monitor the clinical course, some authors suggest a comprehensive approach including seizure control, neuropsychological evaluation, and even MRI metrics, such as brain atrophy or white matter integrity (Yelam et al, 2019;Szots et al, 2017). Nonetheless, the latter seems difficult to implement, mainly due to the current barriers of individual brain atrophy measurements (e.g.…”

Section: Discussionmentioning

confidence: 99%

LGI1-antibody associated epilepsy successfully treated in the outpatient setting

Uribe-San-Martín

Ciampi

Santibáñez

et al. 2020

Journal of Neuroimmunology

View full text Add to dashboard Cite

We report six patients with anti-LGI1 associated epilepsy. Two patients presented with new-onset generalized tonic-clonic seizures, four developed faciobrachial dystonic seizures and two piloerection. All patients had significant cognitive complaints at the time of diagnosis. All patients described seizure reduction during the first week of carbamazepine, and seizure freedom was obtained at a median of 13 days (range 7-22), sustained after the initiation of immunosuppression. Median time from symptom onset to carbamazepine initiation was 164 days (range 38-206 days). We discuss the particular seizure response to sodium channel blocking antiepileptic drugs, alone or associated with immunosuppression in this antibody mediated seizures.

show abstract

Rapidly progressive global cerebral atrophy in the setting of anti-LGI1 encephalitis

Cited by 5 publications

References 22 publications

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

Anti-LGI1 encephalitis with initiating symptom of seizures in children

LGI1-antibody associated epilepsy successfully treated in the outpatient setting

Contact Info

Product

Resources

About