PMC5771906_01
MRI of the brain. (A) T2 FLAIR sequence shows a hyperintense 17 mm x 14 mm nodular lesion within the right mesial frontal lobe. (B) Diffusion-weighted imaging sequence shows increased signal without an ADC reduction, which suggests edema. (C) ADC map of the corresponding diffusion-weighted image. (D) T1-weighted postcontrast image shows no contrast enhancement. (E and F). Magnetic resonance spectroscopy of the lesion shows overall nonneoplastic spectrum.
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The lesion stayed stable in the subsequent 4 months (Fig. 1).
EEG. (A) Displayed on average reference montage, the long-term video EEG showed recurrent, small-amplitude, negative, blunted sharp waves (black stars), with the maximum over C4 lead. These sharp waves did not always precede the left foot twitching. These frontal sharp waves and the patient's jerking of the left limbs intensified after anti-epileptic medications were discontinued. (B) Displayed on the average reference montage, the EEG was obtained on the following day of A, which showed recurrent sharp waves maximum over C4, P4, and Cz with higher amplitude compared with those of A. Of note, the sensitivity of B was decreased to 10 uV/mm. Eleven minutes after B, the patient developed a generalized tonic-clonic seizure that was triggered by putting her left foot on the floor (reflex seizure).
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The video EEG evolved into one generalized tonic-clonic seizure, which was triggered by standing on the floor (reflex seizure) (Fig. 2).
Serial change of serum TPOAb and TgAb.
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The serum TPOAb and TgAb remained in the normal range during the first 7 months of EPC onset (Fig. 3). TgAb was mildly elevated at 32 IU/mL (Ref 1-4 IU/mL), but TPOAb remained within the normal range 0.5 IU/mL (Ref 0-9 IU/mL) (Fig. 3). The repeat Low-dose steroids - prednisone (10 mg-20 mg daily) were added but they failed to halt the EPC progression (Fig. 3). TPOAb and TgAb were elevated further to 70 IU/mL and 172 IU/mL (Fig. 3), respectively. Therefore, the patient was administered high-dose IV methylprednisolone 1000 mg daily for 3 days. With steroid treatment, clinical improvement was accompanied by a parallel decrease in the serum TPOAb and TgAb levels (Fig. 3).
Histopathology. (A) Hematoxylin and eosin stain sections show the cortex with reactive astrocytes. (B) Perivascular lymphocytic infiltrate. (C) Luxol fast blue staining for myelin shows no significant myelin loss. (D) Immunohistochemistry with antiglial fibrillary acidic protein antibody highlights numerous reactive astrocytes. MIB 1 staining showed a proliferation index of less than 1%; p53 was negative (not shown). IDH1/2 mutation was not detected.
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The lesion was found to be gliotic with marked perivascular and parenchymal inflammation (Fig. 4).
MRI of the brain after the lesionectomy. (A) Seven months after onset of EPC, T2 FLAIR sequence shows an area of encephalomalacia over the right frontal cortex in the region of surgery. (B) Twelve months after onset of EPC, T2 FLAIR sequence shows stable postsurgical changes over the right frontal cortex in the region of surgery despite the worsening of EPC. (C) Twelve months after onset of EPC, T1 postcontrast sequence shows no contrast enhancement around the area of encephalomalacia. (D) Two years after onset of EPC, T2 FLAIR sequence shows stable postsurgical changes. In addition, no asymmetric brain volume loss was noted.
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Although the EPC worsened after the lesionectomy, a follow-up MRI of the brain 7 months after the onset did not show any new changes (Fig. 5).