Dataset Information

Antibody-associated CNS syndromes without signs of inflammation in the elderly.

ABSTRACT:

Objective

To report the CNS syndromes of patients ?60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.

Methods

This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ?60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.

Results

Among 155 patients ?60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22.6%) fulfilled the indicated criteria. The median age of these 35 patients was 68 years (range 60-88 years). Clinical manifestations included faciobrachial dystonic seizures (FBDS) in 11 of 35 (31.4%) patients, all with LGI1 antibodies; a combination of gait instability, brainstem dysfunction, and sleep disorder associated with IgLON5 antibodies in 10 (28.6%); acute confusion, memory loss, and behavioral changes suggesting autoimmune encephalitis (AE) in 9 (25.7%; 2 patients with AMPAR, 2 with NMDAR, 2 with GABAbR, 2 with LGI1, and 1 with CASPR2 antibodies); and rapidly progressive cognitive deterioration in 5 (14.3%; 3 patients with IgLON5 antibodies, 1 with chorea; 1 with DPPX antibody-associated cerebellar ataxia and arm rigidity; and 1 with CASPR2 antibodies).

Conclusions

In patients ?60 years of age, the correct identification of characteristic CNS syndromes (FBDS, anti-IgLON5 syndrome, AE) should prompt antibody testing even without evidence of inflammation in MRI and CSF studies. Up to 15% of the patients developed rapidly progressive cognitive deterioration, which further complicated the differential diagnosis with a neurodegenerative disorder.

SUBMITTER: Escudero D

PROVIDER: S-EPMC5631166 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature

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Publications

Antibody-associated CNS syndromes without signs of inflammation in the elderly.

Escudero Domingo D Guasp Mar M Ariño Helena H Gaig Carles C Martínez-Hernández Eugenia E Dalmau Josep J Graus Francesc F

Neurology 20170906 14

<h4>Objective</h4>To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.<h4>Methods</h4>This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.<h4>Results</h4>Among 155 patients ...[more]

PMID: 28878050

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Project description:BackgroundNeurocognitive testing is an important concussion evaluation tool, but for neurocognitive tests to be useful, their psychometric properties must be well established. Test-retest reliability of computerized neurocognitive tests can influence their clinical utility. The reliability for a commonly used computerized neurocognitive test, CNS Vital Signs, is not well established. The purpose of this study was to examine test-retest reliability and reliable change indices for CNS Vital Signs in a healthy, physically active college population.HypothesisCNS Vital Signs yields acceptable test-retest reliability, with greater reliability between the second and third test administration compared with between the first and second administration.Study designCohort study.Level of evidenceLevel 3.MethodsForty healthy, active volunteers (16 men, 24 women; mean age, 21.05 ± 2.17 years) reported to a clinical laboratory for 3 sessions, 1 week apart. At each session, participants were administered CNS Vital Signs. Outcomes included standard scores for the following CNS Vital Signs domains: verbal memory, visual memory, psychomotor speed, cognitive flexibility, complex attention, processing speed, reaction time, executive functioning, and reasoning.ResultsParticipants performed significantly better on the second session and/or third session than they did on the first testing session on 6 of 9 neurocognitive domains. Pearson r test-retest correlations between sessions ranged from 0.11 to 0.87. Intraclass correlation coefficients ranged from 0.10 to 0.86.ConclusionClinicians should consider using reliable change indices to account for practice effects, identify meaningful score changes due to pathology, and inform clinical decisions.Clinical relevanceThis study highlights the importance of clinicians understanding the psychometric properties of computerized neurocognitive tests when using them in the management of sport-related concussion. If CNS Vital Signs is administered twice within a small time frame (such as 1 week), athletes should be expected to improve between the first and second administration.

Dataset Information

Antibody-associated CNS syndromes without signs of inflammation in the elderly.

Objective

Methods

Results

Conclusions

Publications

Antibody-associated CNS syndromes without signs of inflammation in the elderly.

Similar Datasets

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