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GABAB Encephalitis: A Fifty-Two-Year-Old Man with Seizures, Dysautonomia, and Acute Heart Failure.

Loftspring MC, Landsness E, Wooliscroft L, Rudock R, Jo S, Patel KR - Case Rep Neurol Med (2015)

Bottom Line: Autoantibodies to the γ-aminobutyric acid receptor, subtype B (GABAB), are a known cause of limbic encephalitis.Here we present a case of GABAB encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure.To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Washington University, St. Louis, MO 63110, USA.

ABSTRACT
Autoantibodies to the γ-aminobutyric acid receptor, subtype B (GABAB), are a known cause of limbic encephalitis. The spectrum of clinical manifestations attributable to this antibody is not well defined at the present time. Here we present a case of GABAB encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure. To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome.

No MeSH data available.


Related in: MedlinePlus

(a) Axial PET demonstrating significant uptake of FDG in the left mesial temporal lobe. (b) Coronal FLAIR MRI with subtle hyperintensity surrounding the left hippocampus (arrowhead).
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fig2: (a) Axial PET demonstrating significant uptake of FDG in the left mesial temporal lobe. (b) Coronal FLAIR MRI with subtle hyperintensity surrounding the left hippocampus (arrowhead).

Mentions: A brain MRI showed FLAIR hyperintensities in left hippocampal body with surrounding mild edema (Figure 2). Continuous video electroencephalogram monitoring captured frequent electrographic and clinical seizures of left posterior temporal onset and moderate to severe generalized slowing. A lumbar puncture was performed with an opening pressure of 20 cmH2O; cerebrospinal fluid studies showed 4 nucleated cells/μL, protein of 35 mg/dL, glucose of 85 mg/dL, and zero oligoclonal bands. Paraneoplastic panel in the CSF was positive for anti-GABAB receptor antibody. This antibody panel is performed at the Mayo Clinic Laboratories (Rochester, MN, USA) and utilizes indirect immunofluorescence on animal brain slices to screen for antibodies reactive to brain antigens. Positive results are further characterized and reflex tests for other autoreactive antibodies are performed based on the staining pattern. Reflex autoantibody tests include those against the NMDA receptor, AMPA receptor, and GAD-65 which were not detected; therefore direct testing for these autoantibodies did not occur. Other relevant antibodies with this presentation are anti-LGI1 anti-GABAA; however these were not screened or tested. Negative antibodies on this panel were ANNA-1, ANNA-2, ANNA-3, anti-glial nuclear antibody, anti-Purkinje cell cytoplasmic antibody, types 1 and 2 and Tr, anti-amphiphysin, and anti-CRMP-5.


GABAB Encephalitis: A Fifty-Two-Year-Old Man with Seizures, Dysautonomia, and Acute Heart Failure.

Loftspring MC, Landsness E, Wooliscroft L, Rudock R, Jo S, Patel KR - Case Rep Neurol Med (2015)

(a) Axial PET demonstrating significant uptake of FDG in the left mesial temporal lobe. (b) Coronal FLAIR MRI with subtle hyperintensity surrounding the left hippocampus (arrowhead).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4644823&req=5

fig2: (a) Axial PET demonstrating significant uptake of FDG in the left mesial temporal lobe. (b) Coronal FLAIR MRI with subtle hyperintensity surrounding the left hippocampus (arrowhead).
Mentions: A brain MRI showed FLAIR hyperintensities in left hippocampal body with surrounding mild edema (Figure 2). Continuous video electroencephalogram monitoring captured frequent electrographic and clinical seizures of left posterior temporal onset and moderate to severe generalized slowing. A lumbar puncture was performed with an opening pressure of 20 cmH2O; cerebrospinal fluid studies showed 4 nucleated cells/μL, protein of 35 mg/dL, glucose of 85 mg/dL, and zero oligoclonal bands. Paraneoplastic panel in the CSF was positive for anti-GABAB receptor antibody. This antibody panel is performed at the Mayo Clinic Laboratories (Rochester, MN, USA) and utilizes indirect immunofluorescence on animal brain slices to screen for antibodies reactive to brain antigens. Positive results are further characterized and reflex tests for other autoreactive antibodies are performed based on the staining pattern. Reflex autoantibody tests include those against the NMDA receptor, AMPA receptor, and GAD-65 which were not detected; therefore direct testing for these autoantibodies did not occur. Other relevant antibodies with this presentation are anti-LGI1 anti-GABAA; however these were not screened or tested. Negative antibodies on this panel were ANNA-1, ANNA-2, ANNA-3, anti-glial nuclear antibody, anti-Purkinje cell cytoplasmic antibody, types 1 and 2 and Tr, anti-amphiphysin, and anti-CRMP-5.

Bottom Line: Autoantibodies to the γ-aminobutyric acid receptor, subtype B (GABAB), are a known cause of limbic encephalitis.Here we present a case of GABAB encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure.To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Washington University, St. Louis, MO 63110, USA.

ABSTRACT
Autoantibodies to the γ-aminobutyric acid receptor, subtype B (GABAB), are a known cause of limbic encephalitis. The spectrum of clinical manifestations attributable to this antibody is not well defined at the present time. Here we present a case of GABAB encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure. To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome.

No MeSH data available.


Related in: MedlinePlus