Abstract
Introduction: Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is an autoimmune disorder associated with antibodies to voltage-gated potassium channels (VGKC). It is a non-paraneoplastic and partially reversible encephalitis that can be diagnosed via serological testing. Untreated LGI1-LE can be associated with neurocognitive as well as neuropsychiatric sequelae. Here we report the neuropsychological and clinical profile of a patient with LGI1-LE following three different treatment approaches: plasmapheresis (PA), intravenous immunoglobulin (IVIG), and corticosteroids (CO). Method: We investigated our patient with 10 neuropsychological evaluations obtained over a 9-year follow-up period. Multiple MRI scans, EEG recordings, neurological examinations, and serum tests were also obtained. Results: The neurocognitive profile of our patient was characterized by long-term memory impairment (verbal and visual-spatial), and deficits in aspects of executive functioning and language. Neuropsychiatric symptoms of depression and anxiety were noted intermittently. Conclusions: Non-specific treatment prior to diagnosis had marginal effects on neurocognitive profile, neuropsychiatric symptoms, or control of epileptic seizure. In contrast, specific treatments for LGI1-LE following diagnosis resulted in neurocognitive improvement and epileptic control. Among the three treatments, IVIG and CO had the most beneficial impact on neurocognitive status, likely due to the continuity of administration.
| Original language | English |
|---|---|
| Journal | Journal of Clinical and Experimental Neuropsychology |
| DOIs | |
| Publication status | Published - Jan 1 2019 |
| Externally published | Yes |
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Keywords
- Anti-leucine-rich glioma-inactivated 1
- Antibodies against anti-voltage-gated-potassium-channel (VGKC)
- Autoimmune epilepsy
- Limbic encephalitis
- neuropsychological assessment
ASJC Scopus subject areas
- Clinical Psychology
- Neurology
- Clinical Neurology
Cite this
A 9-year neuropsychological report of a patient with LGI1-associated limbic encephalitis. / Zangrandi, Andrea; Gasparini, Federico; Marti, Alessandro; Bhalla, Rishi; Napoli, Manuela; Angelini, Damiano; Ghidoni, Enrico; Rizzi, Romana.
In: Journal of Clinical and Experimental Neuropsychology, 01.01.2019.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - A 9-year neuropsychological report of a patient with LGI1-associated limbic encephalitis
AU - Zangrandi, Andrea
AU - Gasparini, Federico
AU - Marti, Alessandro
AU - Bhalla, Rishi
AU - Napoli, Manuela
AU - Angelini, Damiano
AU - Ghidoni, Enrico
AU - Rizzi, Romana
PY - 2019/1/1
Y1 - 2019/1/1
N2 - Introduction: Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is an autoimmune disorder associated with antibodies to voltage-gated potassium channels (VGKC). It is a non-paraneoplastic and partially reversible encephalitis that can be diagnosed via serological testing. Untreated LGI1-LE can be associated with neurocognitive as well as neuropsychiatric sequelae. Here we report the neuropsychological and clinical profile of a patient with LGI1-LE following three different treatment approaches: plasmapheresis (PA), intravenous immunoglobulin (IVIG), and corticosteroids (CO). Method: We investigated our patient with 10 neuropsychological evaluations obtained over a 9-year follow-up period. Multiple MRI scans, EEG recordings, neurological examinations, and serum tests were also obtained. Results: The neurocognitive profile of our patient was characterized by long-term memory impairment (verbal and visual-spatial), and deficits in aspects of executive functioning and language. Neuropsychiatric symptoms of depression and anxiety were noted intermittently. Conclusions: Non-specific treatment prior to diagnosis had marginal effects on neurocognitive profile, neuropsychiatric symptoms, or control of epileptic seizure. In contrast, specific treatments for LGI1-LE following diagnosis resulted in neurocognitive improvement and epileptic control. Among the three treatments, IVIG and CO had the most beneficial impact on neurocognitive status, likely due to the continuity of administration.
AB - Introduction: Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1-LE) is an autoimmune disorder associated with antibodies to voltage-gated potassium channels (VGKC). It is a non-paraneoplastic and partially reversible encephalitis that can be diagnosed via serological testing. Untreated LGI1-LE can be associated with neurocognitive as well as neuropsychiatric sequelae. Here we report the neuropsychological and clinical profile of a patient with LGI1-LE following three different treatment approaches: plasmapheresis (PA), intravenous immunoglobulin (IVIG), and corticosteroids (CO). Method: We investigated our patient with 10 neuropsychological evaluations obtained over a 9-year follow-up period. Multiple MRI scans, EEG recordings, neurological examinations, and serum tests were also obtained. Results: The neurocognitive profile of our patient was characterized by long-term memory impairment (verbal and visual-spatial), and deficits in aspects of executive functioning and language. Neuropsychiatric symptoms of depression and anxiety were noted intermittently. Conclusions: Non-specific treatment prior to diagnosis had marginal effects on neurocognitive profile, neuropsychiatric symptoms, or control of epileptic seizure. In contrast, specific treatments for LGI1-LE following diagnosis resulted in neurocognitive improvement and epileptic control. Among the three treatments, IVIG and CO had the most beneficial impact on neurocognitive status, likely due to the continuity of administration.
KW - Anti-leucine-rich glioma-inactivated 1
KW - Antibodies against anti-voltage-gated-potassium-channel (VGKC)
KW - Autoimmune epilepsy
KW - Limbic encephalitis
KW - neuropsychological assessment
UR - http://www.scopus.com/inward/record.url?scp=85067566894&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85067566894&partnerID=8YFLogxK
U2 - 10.1080/13803395.2019.1617836
DO - 10.1080/13803395.2019.1617836
M3 - Article
JO - Journal of Clinical and Experimental Neuropsychology
JF - Journal of Clinical and Experimental Neuropsychology
SN - 1380-3395
ER -