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Isolated seizures are a common early feature of paraneoplastic anti-GABA B receptor encephalitis.
- Source :
-
Journal of neurology [J Neurol] 2019 Jan; Vol. 266 (1), pp. 195-206. Date of Electronic Publication: 2018 Nov 20. - Publication Year :
- 2019
-
Abstract
- Objective: To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABA <subscript>B</subscript> receptor antibodies (GABA <subscript>B</subscript> R-Abs).<br />Methods: Retrospective clinical study of CSF-confirmed cases of GABA <subscript>B</subscript> R-Abs encephalitis.<br />Results: We identified 22 patients (4 female) with GABA <subscript>B</subscript> R-Abs, with a median age of 64 years (range 55-85). All were paraneoplastic: 20 small-cell lung cancer, one malignant thymoma, and one uncharacterized lung mass. The most frequent first symptom was the isolated recurrent seizures without cognitive inter-ictal impairment in 17 patients (77%). In the other, three presented the first behavioral disorders and two presented de novo status epilepticus (SE). After a median delay of 10 days (range 1-30), the recurrent seizures' phase was followed by an encephalitic phase characterized by confusion in 100% of cases and SE in 81% (n = 17), with 53% (n = 9) non-convulsive SE. Dysautonomic episodes were frequent (36%, n = 8, bradycardia and central apnea) and killed three patients. CSF study was abnormal in 95% of the cases (n = 21). At the encephalitic phase, MRI showed a temporal FLAIR hypersignal in 73% (n = 16) of the cases. First-line immunotherapy was initiated after a median delay of 26 days (range 6-65) from disease onset, and a partial response was observed in 10 out of 20 patients (50%). There was no complete response. Two years after onset, a massive anterograde amnesia affected all still alive patients. Nine patients died from cancer progression (median survival: 1.2 years).<br />Conclusion: Paraneoplastic GABA <subscript>B</subscript> R-Abs encephalitis is characterized by a stereotype presentation with an epilepsy phase before an encephalitic phase with dysautonomia. The functional prognosis is poor.
- Subjects :
- Aged
Aged, 80 and over
Amnesia, Anterograde etiology
Amnesia, Anterograde physiopathology
Autoantibodies cerebrospinal fluid
Encephalitis diagnosis
Encephalitis therapy
Female
Humans
Immunotherapy
Lung Neoplasms complications
Lung Neoplasms physiopathology
Male
Middle Aged
Paraneoplastic Syndromes, Nervous System diagnosis
Paraneoplastic Syndromes, Nervous System therapy
Retrospective Studies
Seizures diagnosis
Seizures therapy
Small Cell Lung Carcinoma complications
Small Cell Lung Carcinoma physiopathology
Encephalitis physiopathology
Paraneoplastic Syndromes, Nervous System physiopathology
Receptors, GABA-B immunology
Seizures physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 266
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 30460450
- Full Text :
- https://doi.org/10.1007/s00415-018-9132-0