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Teaching NeuroImages Neurology

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Presentation on theme: "Teaching NeuroImages Neurology"— Presentation transcript:

1 Teaching NeuroImages Neurology
A woman in her 80’s with cognitive dysfunction and abnormal left hand posture Teaching NeuroImages Neurology López Chiriboga et al. © 2017 American Academy of Neurology

2 Vignette An 81- year-old woman was diagnosed with corticobasal degeneration(cognitive decline and left hand posturing). She developed Hyponatremia and bilateral faciobrachial dystonic seizures (FBDS). Neurological examination revealed persistent involuntary continuous left 3rd and 4th finger flexor activation, and delayed relaxation. EMG revealed high frequency spontaneous discharges, including neuromyotonia and fast mykomia of the left flexor digitorum superficialis and pronator teres. LGI-1 IgG was detected in serum via CBA. López Chiriboga et al. © 2017 American Academy of Neurology

3 Imaging A B C D E López Chiriboga et al.
A) Persistent left III and IV digit flexion. B) Electromyography shows abnormal high intraburst frequency discharges. Serum was tested for IgG reactive with LGI1 or CASPR2 proteins by a cell-based immunofluorescence assay using as substrate fixed HEK 293 cells that were nontransfected or transfected with plasmid-encoding human LGI1 or CASPR2 proteins. We detected bound IgG by use of fluorescein isothiocyanate conjugated goat IgG specific for human IgG. Serum is reactive with LGI-1 transfected cells (C), but not CASPR-2 transfected cells (D) or control “mock-transfected cells” (E). LGI1 transfected CASPR2 transfected Control mock-transfected López Chiriboga et al. © 2017 American Academy of Neurology

4 Acquired Neuromyotonia in LGI-1 Autoimmunity
Symptoms improved significantly after administration of IVIG. Encephalopathy coexisting with peripheral nerve hyperexcitability, can mimic neurodegeneration. Testing for CASPR2/LGI1 should be performed. REFERENCES López Chiriboga et al. © 2017 American Academy of Neurology

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