Successful immune treatment for non-paraneoplastic limbic encephalitis

Mori, Masahiro; Kuwabara, Satoshi; Yoshiyama, Mitsuharu; Kanesaka, Toshihide; Ogata, Tsuyoshi; Hattori, Takamichi

doi:10.1016/s0022-510x(02)00188-0

Cited by 37 publications

(16 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In our case, disease progression despite steroid treatment [6,7] required a more aggressive immunotherapy with immunoglobulin [8] and azathioprine, which apparently are good alternative treatment options in NPLE without sufficient response to steroid treatment. Under this medication, there was a partial neuropsychological improvement and a decline of the VGKC-Ab levels.…”

Section: Sirsmentioning

confidence: 78%

Successive affection of bilateral temporomesial structures in a case of non-paraneoplastic limbic encephalitis demonstrated by serial MRI and FDG-PET

et al. 2009

View full text Add to dashboard Cite

Sirs,Non-paraneoplastic limbic encephalitis (NPLE) often results in an unfavorable clinical course with progressive neuropsychological deficits and epileptic seizures despite treatment [1][2][3]. Magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG-PET) and antibodies against voltage-gated potassium channels (VGKC-Ab) are helpful in monitoring the course of the disease [3][4][5]. We present a follow-up of a NPLE patient over a 3-year period with successive affection of bilateral temporomesial structures.A 67-year-old man with no relevant medical history presented with recurrent episodes of visual hallucinations, memory impairment, disorientation and myoclonic jerks. Neurological examination was unremarkable; however neuropsychological testing revealed severe verbal (shortterm) memory, speech fluency and visual recognition deficits. EEG showed an intermittent theta-focus as well as intermittent right temporal ictal rhythmic activity. On MRI hyperintensity and diffuse swelling of the right hippocampus on T2-and diffusion-weighted (DWI) images was found. FDG-PET revealed right temporomesial hypermetabolism and bilateral hypometabolism in the temporal lobes (Fig. 2a). There were no signs of tumor in the wholebody PET examination. CSF (including virological analysis) was normal except for elevated VGKC-Ab levels ([9085 pM, controls \100 pM). Thyroid function and autoantibodies tests were negative. The diagnosis of NPLE was made and methylprednisolone (MP; 4000 mg i.v. over 5 days) as well as gabapentin treatment was initiated. Figure 1 shows the course of the disease, examination results and therapeutic management.Three months later, the patient presented with a deterioration of symptoms. MRI showed an atrophy of the right hippocampus, with mild residual T2-hyperintensity. FDG-PET showed left temporal hypermetabolism and a right temporomesial ''cold'' lesion (Fig. 2b). VGKC-Ab were still elevated ([8067 pM). Treatment with MP (5000 mg i.v. over 5 days) was started again. Six months later he was re-admitted with progressive memory impairment, confirmed by neuropsychological examination. Now on MRI, left hippocampal edema was found and FDG-PET demonstrated severe hypometabolism in both temporomesial regions. After 2 months of stable condition, there was once again neuropsychological deterioration. There were no significant changes on MRI compared to the previous examinations and the FDG-PET showed left temporomesial hypometabolism. IV immunoglobulin therapy (150 g over 3 days) was applied. Two and 4 months later MRI and FDG-PET findings remained unchanged. VGCK-Ab levels were almost normal. Memory impairment had temporarily deteriorated before improving again, this time under immunosuppressive treatment with azathioprine (100 mg/ d). At the last two visits (after 2 and 3.5 years) the patient was seizure-free under gabapentin and showed an improvement of his cognitive functions under azathioprine treatment. MRI showed left hippocampal atrophy and FDG-PET hypometabolism in both temporomes...

show abstract

Section: Sirsmentioning

confidence: 78%

Successive affection of bilateral temporomesial structures in a case of non-paraneoplastic limbic encephalitis demonstrated by serial MRI and FDG-PET

et al. 2009

View full text Add to dashboard Cite

show abstract

“…LE can be classified, according to its pathogenesis, into three categories; paraneoplastic, infectious, and autoimmune-related [17][18][19]. Recent studies indicate that some central nervous system antibodies such as VGKC-Abs are related to autoimmune-mediated LE [20].…”

Section: Discussionmentioning

confidence: 99%

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Sekiguchi¹,

Takahashi²,

Mori³

et al. 2008

Journal of the Neurological Sciences

Self Cite

View full text Add to dashboard Cite

“…The 4 antibody-positive patients had no findings of viral infection or cancer. Several reports have been published concerning the immunotherapy response form of nonparaneoplastic limbic encephalitis [21,22,23,24,25,26]. Recently, a VGKC antibody has been detected in patients with reversible autoimmune limbic encephalitis [27,28,29,30,31,32].…”

Section: Discussionmentioning

confidence: 99%

Autoantibodies against Glutamate Receptor ε<sub>2</sub>-Subunit Detected in a Subgroup of Patients with Reversible Autoimmune Limbic Encephalitis

et al. 2007

View full text Add to dashboard Cite

We investigated the presence of autoantibodies againstglutamate receptor (GluR) Ε₂ in serum and cerebrospinal fluid (CSF) samples from 12 consecutive patients with acute encephalitis/encephalopathy by immunoblotting using recombinant GluRΕ₂ as antigen. In 4 patients, IgM autoantibodies against GluRΕ₂ were detected in CSF in the early phase of the disease but were not detectable after several months. Seizures and psychiatric symptoms were noted during the acute phase of the disease in these 4 patients, who showed various degrees of residual amnesia. Immunotherapy was performed on 3 patients (patients 1, 3 and 4), and they showed marked improvements. Immunohistochemistry using these patients’ sera showed that immunoreactivity is specifically detected in the cytoplasm of rat hippocampal and cortical neurons. The clinical features and neuroimaging findings of patients with IgM autoantibodies against GluRΕ₂ in CSF resemble those of patients with reversible autoimmune limbic encephalitis.

show abstract

Successful immune treatment for non-paraneoplastic limbic encephalitis

Cited by 37 publications

References 8 publications

Successive affection of bilateral temporomesial structures in a case of non-paraneoplastic limbic encephalitis demonstrated by serial MRI and FDG-PET

Successive affection of bilateral temporomesial structures in a case of non-paraneoplastic limbic encephalitis demonstrated by serial MRI and FDG-PET

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Autoantibodies against Glutamate Receptor ε<sub>2</sub>-Subunit Detected in a Subgroup of Patients with Reversible Autoimmune Limbic Encephalitis

Contact Info

Product

Resources

About