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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]

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