Background Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory central nervous system (CNS) disorder that usually presents as steroid responsive encephalitis, meningitis, myelitis, or meningoencephalomyelitis. Hypertrophic pachymeningitis (HP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Depending on the etiology, HP can be idiopathic or secondary to a wide variety of other diseases. There are no reports of autoimmune GFAP astrocytopathy presenting as HP. Methods In this case report, we describe a rare case of pediatric HP possibly associated with anti-GFAP antibody. Results A 13-year-old previously healthy girl presented with headache for nearly 8 months with left-sided peripheral facial palsy and left-sided abductor nerve palsy in the second month of course. Magnetic resonance imaging (MRI) of the brain revealed contrast enhancement of hypertrophic dura mater. Anti-GFAPα antibodies were positive in serum and cerebrospinal fluid. The patient improved clinically after steroid treatment with partial resolution of abnormal intracranial MRI lesions. Conclusion The present study suggests that HP may be one of the clinical phenotypes for autoimmune GFAP astrocytopathy or GFAP antibody is a biomarker for HP.

The clinical data of patients with Kikuchi–Fujimoto disease (KFD) combined with the involvement of the central system is limited, particularly for children. This study aimed to investigate the clinical features and prognosis of KFD associated with the central nervous system in children. We collected the clinical data of patients diagnosed with KFD combined with the involvement of the central system, hospitalized at Children's Hospital of Chongqing Medical University (CHCMU). Furthermore, we summarized the clinical features and outcomes in children by reviewing the published literature. Together with the two patients from CHMCU enrolled in this study, we identified 19 children diagnosed with KFD combined with the involvement of the central nervous system, including eight males and 11 females. The onset age ranges from 6 to 18 years (the median age was 14 and the interquartile range was 9–16). Sixteen patients were diagnosed with aseptic meningitis. Headache (78.9%, 15/19) was the most common symptom. Cerebrospinal fluid pressure was increased in 3 patients (60.0%, 3/5), cell count was elevated in 14 patients (73.7%, 14/19), glucose was decreased in 4 patients (21.0%, 4/19), and protein was elevated in 12 patients (63.1%, 12/19). A total of 9 patients had cranial imaging changes. Electroencephalogram (EEG) was performed on seven patients and 4 patients presented abnormal EEG (57.1%, 4/7). A total of 12 patients received steroid therapy; two were combined with antiepileptic treatment. In children, KFD complicated with the involvement of the central nervous system was rare and aseptic meningitis was the primary manifestation. Most patients had a good prognosis and a few had residual sequelae of neurological damage.

To investigate and summarize the magnetic resonance imaging (MRI) manifestations of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children. Methods: We retrospectively analyzed data from 17 pediatric patients with autoimmune GFAP astrocytopathy confirmed by the detection of GFAP autoantibodies in cerebrospinal fluid in our single-center. Furthermore, we reviewed current literature and summarized previous findings on the MRI characteristics of this disease in children. Results:In these 17 patients, the clinical manifestations and results of CSF analysis were suggestive of autoimmune disorder, with a good improvement. The lesions on MRI were most commonly located in the bilateral basal ganglia (70.6%), thalamus (64.7%), cerebral white matter (29.4%). 93.3% of the cerebral lesions were relatively scattered and small, 80% of the spinal lesions presented as longitudinally extensive ones. Both periventricular radial linear (PVRL) (53.8%) and punctate or linear enhancement in basal ganglia and thalamus (53.8%) were commonly observed, followed by the leptomeningeal enhancement (46.2% in the brain and 62.5% in the spinal cord). We then included 55 pediatric patients with MRI data from current literature in our analysis (n = 72, 44 males). Our results revealed similar MRI findings but the enhancement pattern between our series and previously published cases, that is, leptomeningeal enhancement in the brain 46.2% vs 31.4%, in spinal cord 62.5% vs 18.4%, and PVRL enhancement 53.8% vs 11.2%. There were no detailed reports on punctate or linear enhancement. Conclusion:The MRI characteristics of autoimmune GFAP astrocytopathy in children could be suggestive. Scattered and small lesions (especially punctate or linear) in the bilateral thalamus, basal ganglia, and white matter, as well as longitudinally extensive spinal cord lesions (if present), with punctate, PVRL and leptomeningeal enhancement might be a distinct indication for the early diagnosis of this disorder.

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