Poster Abstracts Tuesday, November 8, 2005 $271

Conclusion: Therapeutic trial was successful on this boy who had progressive encephalopathy without definitive causes identified. Autoinmmne process which induced the leukoencephalopathy was sp eculated in tiffs case because o f the antecedent infection. Therapeutic trial with steroid might be considered when facing a case of progressive encephalopathy with related white matter lesion on MRI.

0674 Increased antigen Slmdfic CSF/semm indices of anti~B-crys~aUin antibodies in Guillain-Barr6 synthfOiile

Wanschitz, j1, Elding, R 1, Loescher, W 1, Reindl, M 1, Berger, T 1. ~ Clinical Department of Neurology, Innsbruck Medical University, Austria

The small heat-shock protein ~B-crystallin was identified as a highly innnunogerfic antigen to human T cells in multiple sclerosis. The aim of tiffs study was to investigate humoral responses to ~B-crystallin in Guillain-Barr6 syndrome (GBS).

Using human full-length recombinant ~B-crystallin we measured anti-~B-crystallin antibodies by an enzyme-linked immunosorbent assay in cerebrospinal fluid (CSF) and serum of 30 patients with GBS (mean age at onset 54.95 years). Severity of disease varied among patients with a Hughes score ranging from 1-5. By electrophysiology GBS patients were initially classified as normal, primary demyelinat- ing, primary mxonal, inexitable, and equivocal. Patients with multiple sclerosis, meningitis and other non-inflammatory neurological diseases were used as controls.

Anti-~B-crystallin antibody indices (anti-~B-crystallin antibodies in CSF × total IgG in CSF / ~B-crystallin antibodies in serum x total IgG in sermn) were significantly elevated in patients with GBS as compared to controls (p < 0.0001, non-parametric ANOVA). The subgroup of GBS patients with mxonal involvement demonstrated significantly lower anti-~B-crystallin antibodies in CSF than GBS patients without axonal pathology (p < 0.01, Mann-Wtfitney U test). By inmmnolffstochenffcal exanffnation of sural nerve biopsies from two patients with GBS we for the first time demonstrate raised expression of czB-crystallin on disintegrating nerve fibers, which was not found in unaffected nerves.

Further studies are planned to clarify whether anti<zB-crystallin antibodies are primarily involved in the pathogenesis o f GBS or mirror the tissue stress response. We hypothesize that attti-~B-crystallin antibodies may be used as additional tools in sub-typing of GBS or as predictors of clinical outcome.

0675 CNS Lupus in tile absence of systenfie disease activity

Everett, CM 1, Graves, TD 1 , Lad, S. 1 , Jfiger, HR 2, Thom, M 3 , lsenberg, DA 4, Hanna, MG 1. ~Departrnent of Clinkal Neurology, National Hospital for Neurology & Neurosurgery, Queen Square, London, UR) 2Lysholm Department of Neuroradiology, National Hospital for Neurology & Neurosurgery, Queen square, London, UK; 3Department of Neuropathology, Institute of Neurology, Queen Square House, London, UK; 4Department of Rheumatology, Middlesex Ho@ital, University College London Foundation Trust, Arthur Stanley House, Tottenham Street, London, UK

Baekgrounfl: Neurological features including psychosis, stroke and seizures complicate systemic lupus erythematosus (SLE). CNS SLE vascalopathy is usually a non-inflannnatory process affecting small arterioles and capillaries leading to micro-infarcts and haemorrhages. Rarely CNS vasculitis occurs, usually accompanied by clinical and serological evidence of systemic, activity. We present a case of CNS vasculifis in an SLE patient without systemic, disease activity. Method: A 35 year old woman with a 10 year history of SLE developed seizures, followed by a rapid neurological decline leading to coma within 2 days. She had no systemic evidence of active SLE. She was initially treated for herpes simplex virus (HSV) encephalitis based on

MRI brain appearances but with rapid deterioration a brain biopsy was performed. Results: MRI brain showed bilateral hippocampal high signal extending into the right temporal lobe wlffte matter, progressing to involve the right corona radiata and insula, the left temporal lobe white matter and brainstem, bmnunological markers for SLE including complement, ANA, double stranded DNA and ENA antibodies were normal. ESR and CRP were both elevated. CSF was sterile with an elevated protein (10.71 g/L), normal glucose and 2 lymphocytes/ml present. HSV PCR was negative and oligoclonal bands absent. A brain biopsy revealed an active small vessel lymphocytic vasculitis Conclusion: This case highlights the importance of considering active CNS SLE in the absence of systemic disease activity. Initially, the absence of serological markers for SLE activity and the imaging appearances obscured the diagnosis. A high index of suspicion led to the correct diagnosis.

0676 Autoantibodies against the amino terminal of alpha-enolase are a useful diagnogtic marker for Hashimoto's Encephalopathy

Fujii, A 1, Yoneda, M 1, Kuriyama, M 1. ~University ofFukui, Fukui, Japan

Background: A neurological disorder associated with Hashimoto's thyroiditis (HT) has recently come to be regarded as a new clinical entity, Hashimoto's encephalopathy (HE), distinct from myxoedema encephalopathy associated with hypothyroidism. HE has a wide variety of clinical features and lacks any specific diagnostic marker, thus can be underdiagnosed or misdiagaosed. We investigated autoantibodies in HE and identified their epitope(s). Methods: 1) Two-dimensional electrophoresis (2-DE) was carried out using lysates of human brain, and immunoblotted with serum from an HE patient. 2) Brain protein spots recognized by serum from the HE patient were removed from the gel, denatured, trypsinized, and subjected to a matrix-assisted laser absorption iorfization time-of- flight (MALDDTOF)/mass spectroscopic (MS) analysis. 3) The amino (NHa)-terminal, carboxyl (COOH)-ten-ninal and mid-region of the identified molecule were expressed in cultured human cells and compared inmmnoreactivity among 6 HE patients, 17 HT patients and controls (125 healthy controls and 25 controls with other neurological disorders). Results: 1) A 48 kDa protein was selectively reacted with serum from an HE patient on 2-DE. 2) The protein was identified as alpha-enolase by MALDI-TOF/MS. 3) The recombinant NH2-terminal region of alpha-enolase was lffghly reactive by HE patients (83%), much less reactive in HT (12%), and unreactive in all controls Qv value < 0.005 between HE and HT). In contrast, the COOH-temrinal region reacted with all sera, and the mid-region did not react any of samples. Conclusion: HE antibodies specifically recognize the NH2-terminal of alpha-enolase and are a useful marker for the diagnosis of HE.

0678 Variable cognitive Ihncfion in voltage-gated potassimn "channel antibody positive Limbic Encephalitis

Harrower TP l, Kartsounis, LD ~, Vincent, A ~, De Silva, RN ~ . 1Regional Centre for Neurosurgery and Neurology, Waterloo Road, Romford, Essex, UK; 2Neurosciences Group, Institute of Molecular Medicine, Yohn Radcliffe Ho@ital, O:~ford, UK

Background: Voltage gated potassium channel antibody positive limbic encephalitis is usually associated with seizures, Morvan's syndrome, cognitive impairment and behavioral disturbances in the absence of a malignancy. Milder cases have as yet not been described. Methods: Description of the presentation, investigation and treatment of a case of lmibic encephalitis associated with anti-Voltage gated