Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases
Authors:
- 1 January 2016
- journal article
- case report
- Published by Japanese Society of Internal Medicine in Internal Medicine
- Vol. 55 (16) , 2285-2289
Abstract
Sjögren's syndrome (SS) may be complicated by neurological manifestations. We herein report three women (age range 26-60 years old) who all presented with limbic encephalitis (LE) as the predominant clinical feature 3 months to 15 years after the diagnosis of SS. The 26-year-old patient also developed acute motor axonal neuropathy one week after autoimmune encephalitis. All three patients showed contrast-enhanced MRI lesions and inflammatory cerebrospinal fluid findings, while not displaying any anti-neuronal antibodies and showing a remarkable response to immunotherapy. SS is often overlooked when the symptoms are mild. Therefore, in LE cases with no identifiable cause, serological screening for rheumatologic disorders is recommended.Keywords
This publication has 21 references indexed in Scilit:
- Acute motor axonal neuropathy in association with Sjögren syndromeMuscle & Nerve, 2010
- Neurological manifestations of primary Sjogren's syndromeCurrent Opinion in Neurology, 2010
- Peripheral Nervous System Manifestations of Sjögren SyndromeThe Neurologist, 2010
- Involvement of Nervous System Pathways in Primary Sjögren's SyndromeRheumatic Disease Clinics of North America, 2008
- Autoimmune limbic encephalitis in 39 patients: immunophenotypes and outcomesJournal of Neurology, Neurosurgery & Psychiatry, 2006
- Primary Sjögren's syndrome: new clinical and therapeutic conceptsAnnals of the Rheumatic Diseases, 2005
- Neurologic Manifestations in Primary Sjögren SyndromeMedicine, 2004
- Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus GroupAnnals of the Rheumatic Diseases, 2002
- Motor Dominant Neuropathy in Sjoegren's Syndrome: Report of Two Cases.Internal Medicine, 2002
- Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndromeAnnals of Neurology, 1990