PMC9393788_01
Patient's clinical course. CSF, cerebrospinal fluid; IVIg, intravenous immunoglobulins; IVMP, intravenous methylprednisolone; LEV, levetiracetam; MDZ, midazolam; MRI, magnetic resonance imaging; PB, phenobarbital; PCR, polymerase chain reaction; PHT, phenytoin; S.E., status epilepticus; TPM, topiramate; TPS, thiopental; VPA, valproate.
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F1
Figure 1 summarizes the patient's clinical course. Both serum and CSF showed intense neuropilar staining selectively involving the striatum (Figure 2A, serum titer: 1:1,200, CSF titer: 1:5), suggesting the presence of antibodies directed against an uncharacterized neuronal target.
Panel (A) Diffuse staining is typical of certain anti-neuronal antibodies, such as anti-LGI 1 antibodies (A); in their presence, staining involves the hippocampus, cerebellum, thalamus, striatum (shown in detail), and cortical areas. The patient's serum shows LGI1-like staining, but with selective involvement of the striatum (B, in detail). As shown in (C), no staining is observed with a control serum. Panel (B) MRI at day 40 (first row) vs. follow-up MRI 2 weeks later (second row). In the first row, axial and coronal T2-weighted images (A,B) show bilateral slight enlargement and hyperintensity of the claustrum (black arrows) and mild T2 hyperintensity of the left thalamic pulvinar (white arrow). Also note the diffuse widening of the pericerebral CSF spaces. After 2 weeks, axial and coronal T2-weighted images (C,D) show complete resolution of signal alterations and a quite normal appearance of the pericerebral CSF spaces.
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F2
Both serum and CSF showed intense neuropilar staining selectively involving the striatum (Figure 2A, serum titer: 1:1,200, CSF titer: 1:5), suggesting the presence of antibodies directed against an uncharacterized neuronal target. At this point, MRI showed slight bilateral enlargement and hyperintensity of the claustrum on T2-weighted sequences and mild T2 hyperintensity of the left thalamic pulvinar, without diffusion restriction or contrast enhancement after IV gadolinium administration (Figure 2B).