UNASSIGNED: In pediatrics, a leukemoid reaction in severe DKA cases with cerebral edema has never been reported. The fluid management was challenging as it required balancing rates to ensure improvement of the condition while preventing neurological sequelae.
UNASSIGNED: The combination of diabetic ketoacidosis (DKA), cerebral edema, and leukemoid reaction in pediatrics has never been reported before in the literature. It may lead to significant morbidity and high mortality. Here, we report a case of DKA-induced cerebral edema associated with severe leukocytosis (WBC 98 × 109/L), which had many challenges in fluid therapy.

BACKGROUND: The dialysis disequilibrium syndrome (DDS) is a complication in those undergoing dialysis for chronic kidney disease (CKD) or acute kidney injury (AKI), characterized by nonspecific symptoms that may progress to coma and death secondary to cerebral edema. This syndrome is associated with rapid change in electrolytes during dialysis with changes in intracranial pressure (ICP) and may have a higher incidence in the elderly neurosurgical patient population.
METHODS: Literature review and illustrative case example.
RESULTS: A 62-year-old female presented with acute mental status change during hemodialysis (HD), with a history of a nonsurgical acute subdural hematoma (SDH) 10 days prior. Imaging showed a conversion of the acute SDH to chronic SDH of 12.2 mm in size with a 14.1 midline shift, for which she underwent a hemicraniectomy with SDH evacuation, with a gradual return to baseline. The literature review identified 5 publications meeting the inclusion criteria. Major theories of DDS include a reverse urea effect, intracerebral acidosis, idiogenic osmoles, and local inflammation. This complication may occur more frequently in the elderly neurosurgical patient population, likely due to age-related comorbidities, preexisting neurological insult, and increased permeability of the blood-brain barrier (BBB), leading to cerebral edema.
CONCLUSIONS: DDS is a rare and potentially fatal complication of HD that may have a higher incidence in the elderly neurosurgical patient population, yet remains to be fully understood. Further study is recommended to characterize the pathophysiological mechanism and incidence of DDS in neurosurgical patients.

High-altitude cerebral edema (HACE) is serious, sometimes fatal clinical condition visualized in unacclimatized individuals climbing high altitudes. The current case report highlights a 39 year old male with a recent history of high-altitude mountain climbing and presented with memory impairment. The radiological findings revealed edema and microhemorrhages at genu and splenium of corpus callosum. Two months later the subject displayed complete resolution of edema, with persistent microhemorrhages. Herein, we report the radiological features of this rare clinical event. The lack of advanced imaging centers at higher altitudes elicit this clinical condition as less described entity.

Background: Systemic lupus erythematosus (SLE) is a common autoimmune disease with various symptoms involving multiple organs. Neuropsychological manifestations are various and generally serious. Leukoencephalopathy is particularly rare but life-threatening in patients with SLE. Results: Here, we describe the case of a young woman who developed a subacute onset intracranial hypertension, papillar edema on fundus examination, diffuse cerebral edema on brain CT scan, and diffuse leukoencephalopathy on brain magnetic resonance imaging (MRI). The immunological workup was positive for antinuclear antibodies, anti-DNA and anti-extractable nuclear antigens (ENA) antibodies. She was ultimately diagnosed with SLE and experienced significant improvement after treatment with high dose of corticosteroids, acetazolamide, and immunosuppressant. We additionally review the previously reported cases of SLE with diffuse cerebral edema and leukoencephalopathy with a focus on the possible pathophysiological mechanisms of such association. Conclusions: We highlight, through this case report and the literature review, the importance of considering SLE in patients with cerebral edema and diffuse leukoencephalopathy and treating it aggressively.

Most influenza B infections are self-limited, but in some instances, they can cause substantial morbidity and mortality due to complications. Acute fulminant cerebral edema (AFCE) is one of the rare complications. AFCE, a consequence of acute encephalitis, presents as acute onset of alteration in mental status, seizure, and/or headache followed by rapidly progressive encephalopathy, often leading to death. The exact pathophysiology of AFCE is unknown, but many pathomechanisms have been proposed. We present a case of an 18-year-old female in excellent physical condition who presented with respiratory insufficiency after being recently diagnosed with influenza B infection. Three days later, she developed acute encephalopathy, leading to brain death. To our knowledge, this rare case of AFCE developing following influenza B infection is the first reported case outside the pediatric population.

BACKGROUND: Infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can lead not only to respiratory symptoms but also to neurologic symptoms with various levels of severity. After the worldwide prevalence of Omicron variant, severe neurological manifestations of coronavirus disease 2019 (COVID-19) such as febrile seizure, demyelinating disease, and cerebrovascular disease, have been reported. However, reports of acute encephalopathy in patients with COVID-19 are quite limited. Especially in terms of cytokine storm-inducing hemorrhagic shock and encephalopathy syndrome (HSES), there is no case reported related to COVID-19.
METHODS: We describe the case of an 8-year-old girl who presented with fatal HSES associated with pediatric SARS-CoV-2 infection. Status epilepticus occurs after the onset of fever and diarrhea and lasted for at least an hour. Unconsciousness was followed by circulatory failure and ultimately leading to death within 2 days after the fever onset. Analysis of forty-eight cytokines and chemokines measured in three consecutive serum samples revealed that interferon (IFN)-γ, interleukin (IL)-6, IL-10, IL-17A, tumor necrosis factor (TNF)-a, IL-8, Interferon gamma inducible protein (IP)-10, and Monocyte chemoattractant protein (MCP)-1, were increased within an hour after the onset of impaired consciousness.
CONCLUSIONS: Here, we describe a case of fatal fulminant encephalopathy with rapid progression because of HSES associated with COVID-19. High levels of cytokines and chemokines observed in this case may be because of the SARS-CoV-2-associated cytokine storm. This study is the first COVID-19-associated case of HSES.

High altitude cerebral edema (HACE) is a clinical spectrum of high-altitude illness. The working diagnosis of HACE should be based on the history of rapid ascent with signs of encephalopathy. Magnetic resonance imaging (MRI) can be crucial in the timely diagnosis of the condition. A 38-year-old female was airlifted from Everest base camp due to sudden onset of vertigo and dizziness. She had no significant medical or surgical history, and routine laboratory tests showed normal results. MRI was performed, which showed no abnormalities except for the detection of subcortical white matter and corpus callosum hemorrhages on susceptibility-weighted imaging (SWI). The patient was hospitalized for 2 days and treated with dexamethasone and oxygen, and had a smooth recovery during follow-up. HACE is a serious and potentially life-threatening condition that can occur in individuals who rapidly ascend to high altitudes. MRI is a valuable diagnostic tool in the evaluation of early HACE, and can detect various abnormalities in the brain that may indicate the presence of HACE, including micro-hemorrhages. Micro-hemorrhages are tiny areas of bleeding in the brain that may not be visible on other MRI sequences but can be detected on SWI. Clinicians especially radiologists, should be aware of the importance of SWI in the diagnosis of HACE, and ensure that it is included in the standard MRI protocol for evaluating individuals with high altitude-related illnesses for early diagnosis and appropriate treatment to prevent further neurological damage and improve patient outcomes.

The neurologic manifestations of coronavirus disease 2019 (COVID-19) may range from mild symptoms such as headache or confusion to profound encephalopathy with variable outcomes and sequelae. Here, we reported a case of fatal COVID-19-associated encephalitis with acute fulminant cerebral edema, presenting first with visual hallucination and then a rapid progression into comatose status in a few hours. Serial brain computed tomography depicted cerebral edematous changes from bilateral ventral temporal lobe to the whole brain leading to brain herniation. Multiple cytokines in serum and cerebrospinal fluid (CSF) were increased, with a more prominent rise in the CSF. Therefore, we postulated a hypothesis regarding the mechanism of this fulminant encephalitis that the SARS-CoV-2 virus attacked ventral temporal lobes initially, triggered a severe cytokine storm, and then led to subsequent disruption of the blood-brain barrier, diffuse brain edema, and brain herniation. The trend of cytokine profiles over time may aid in diagnosing and evaluating the severity and prognosis of COVID-19-associated encephalitis.

UNASSIGNED: Diabetic ketoacidosis (DKA) is associated with a high mortality rate, especially if cerebral edema develops during the disease course. It is rarer and more severe in adults than in children. We present cases of two patients with cerebral edema-related DKA.
UNASSIGNED: The first patient was a 38-year-old man with diabetes mellitus who presented with DKA-related disturbed consciousness. Although glycemic correction was performed slowly, he showed pupil dilation 11 h later. He underwent emergency ventricular drainage, but died of cerebral herniation. The second patient was a 25-year-old woman who presented with impaired consciousness secondary to DKA. Head computed tomography showed subarachnoid hemorrhage and cerebral edema. No related intraoperative findings were observed; it was concluded that the first computed tomography scan revealed pseudo-subarachnoid hemorrhage.
UNASSIGNED: Diabetic ketoacidosis-related cerebral edema develops despite treatment according to guidelines and is difficult to predict. Therefore, adult patients should be treated cautiously during DKA management.

BACKGROUND: Early-onset progressive encephalopathy with brain edema and/or leukoencephalopathy-1 (PEBEL1) is a rare autosomal recessive severe neurometabolic disease. The aim of this study was to investigate the clinical characteristics and genetic pathogenicity of PEBEL1 caused by rare NAXE (or APOA1BP)-related defects.
METHODS: The patient was a girl aged 2 years and 10 mo. She was hospitalized due to walking disorder for > 40 d. The clinical manifestations were ataxia, motor function regression, hypotonia, and eyelid ptosis. Within 1 mo of hospitalization, she developed sigh breathing, respiratory failure, cerebellar edema and brain hernia, and finally she died. Changes were found in cranial imaging, including cerebellar edema accompanied by symmetrical myelopathy. Through whole exome sequencing, we detected NAXE compound heterozygous variation (NM 144772.3) c.733A>C (p. Lys245Gln, dbSNP: rs770023429) and novel variation c.370G>T (p.Gly124Cys) in the germline gene. The clinical features and core phenotypes of this case were consistent with 18 previously reported cases of PEBEL1.
CONCLUSIONS: This is the first case of NAXE-related PEBEL1 with severe clinical phenotype in Mainland China. The p.Gly124Cys mutation discovered in this case has enriched the pathogenic variation spectrum of NAXE.