Cerebral blood flow abnormalities with central sparing on arterial spin labeling in mild encephalopathy associated with excitotoxicity: a case report.

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) and mild encephalopathy associated with excitotoxicity (MEEX) are the most frequent acute encephalopathies in pediatric patients in Japan. AESD typically presents with biphasic seizures and delayed reduced diffusion in the subcortical area, called bright tree appearance (BTA), on radiological examination. In patients with AESD, arterial spin labeling (ASL) shows decreased cerebral blood flow (CBF) in the hyperacute stage and increased CBF in the acute stage, suggesting the usefulness of ASL for the early diagnosis of AESD. Additionally, proton magnetic resonance spectroscopy (MRS) shows elevated glutamate (Glu) and glutamine (Gln) in AESD. MEEX is a group of mild encephalopathies with transient elevation of Gln on MRS similar to that in AESD; however, MEEX does not include any clinical biphasic course or abnormalities, including BTA on diffusion-weighted imaging. Although the usefulness of ASL for AESD has been reported, there are no reports for patients with MEEX. In this study, we report our experience with a 4-year-old girl diagnosed with MEEX who showed unique findings on ASL. CASE PRESENTATION: The patient was a 4-year-old girl admitted to the emergency room with febrile status epilepticus. Considering the possibility of AESD, vitamin therapy was initiated. ASL-MR imaging (MRI) of the brain performed on the second day showed increased blood flow in the frontal, temporal, and occipital regions with spared central sulcus, which indicated AESD with central sparing. The patient was diagnosed with AESD, and the treatment included pulse steroid therapy and immunoglobulin therapy from day 3. The patient remained mildly unconscious but gradually became conscious by day 7 with no seizures. Brain MRI performed on day 8 did not show any characteristic AESD findings, such as BTA. Furthermore, MRS showed elevated Gln, which, along with the clinical course, led to the diagnosis of MEEX. The patient was discharged on day 16 without obvious sequelae. CONCLUSIONS: ASL may be useful in the early diagnosis of MEEX as well as AESD, facilitating early intervention.

Chronic Neutropenia in Children With Abscess Forming Cervical Lymphadenitis Caused by Staphylococcus aureus.

BACKGROUND: Suppurative cervical lymphadenitis is rare in children with limited information about the frequency of neutropenia in any series. METHODS: We conducted a retrospective review of pediatric patients in whom suppurative cervical lymphadenitis was diagnosed between April 2011 and March 2016 at a tertiary children's hospital in Tokyo, Japan. Microbiologically confirmed cases of cervical lymphadenitis with abscess formation were included in the analysis. RESULTS: Twenty-five patients (median age, 1 yr old; 64% female) with abscess formation caused by Staphylococcus aureus were identified. Among these 25 patients, 5 (20%) met the criteria for chronic neutropenia and 4 (80%) received the final diagnosis of autoimmune neutropenia based on the identification of the serum anti-CD16 antibodies. CONCLUSIONS: Chronic neutropenia and autoimmune neutropenia were relatively common among infants with suppurative cervical lymphadenitis caused by S. aureus.