Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies.

Abstract

Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. Here we have characterized the clinical and laboratory features of two patients presenting with subacute onset, and chronic evolution, of anterograde amnesia and drug-resistant epilepsy associated with thyroid autoimmunity and in absence of tumoral pathology despite long follow-up. Antibodies against onconeural antigens, voltage gated potassium channel and glutamate receptors, which may accompany paraneoplastic as well as non-paraneoplastic LE, were negative. However, biochemical studies showed high titers, and sustained intrathecal synthesis, of antibodies directed against neuronal glutamic acid decarboxylase (GAD). In one patient, plasma exchange determined a dramatic improvement of the neurological deficits along with the decrease of autoantibodies.

• Authors: Sabrina Matà, Gian Carlo Muscas, Ilaria Naldi, Eleonora Rosati, Sergio Paladini, Barbara Cruciatti, Francesca Bisulli, Marco Paganini, Gustavo Mazzi, Sandro Sorbi, Paolo Tinuper
• Journal: Journal of neuroimmunology (J Neuroimmunol) Vol. 199 Issue 1-2 Pg. 155-9 (Aug 13 2008) ISSN: 0165-5728 [Print] Netherlands
• PMID: 18586329 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anticonvulsants
• Autoantibodies
• Autoantigens
• DNA, Viral
• Glutamate Decarboxylase

Topics

• Adult
• Anticonvulsants (therapeutic use)
• Autoantibodies (cerebrospinal fluid, immunology)
• Autoantigens (immunology)
• Brain (pathology)
• DNA, Viral (cerebrospinal fluid)
• Diabetes Mellitus, Type 1
• Female
• Glutamate Decarboxylase (immunology)
• Herpesvirus 6, Human
• Humans
• Limbic Encephalitis (immunology, pathology, therapy)
• Magnetic Resonance Imaging
• Middle Aged
• Plasmapheresis
• Roseolovirus Infections (cerebrospinal fluid, complications)
• Seizures (etiology)