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Background: Acute high-altitude illness (AHAI) refers to a series of syndromes including acute mountain sickness (AMS), high-altitude pulmonary edema (HAPE) and high-altitude cerebral edema (HACE). Among these, HACE is a severe and potentially life-threatening condition that can occur when individuals ascend to high altitudes. It is often characterized by ataxia, confusion, and altered mental status. Without appropriate treatment, HACE can rapidly progress to coma, but seizures are infrequent in occurrence. Case presentation: Here, we report a severe HACE patient with coma and status epilepticus. The patient is a 23-year-old male who was visiting Lhasa for the first time. He initially experienced headaches and dizziness on the first day, and then he was found in coma with limb convulsions on the next day. Immediate medical attention was sought, and brain CT and MRI scans showed reversible white matter lesions, especially in the corpus callosum and subcortical white matter. Although the lesions disappeared on T1 and T2 sequences, microbleeds were observed on the SWI sequence. After treatment with tracheal intubation, glucocorticoids and hyperbaric oxygen, the cerebral edema has resolved and the clinical symptoms improved, the patient has no seizures anymore. Conclusions: HACE typically follows AMS and poses a significant risk to life. Clinical manifestations mainly include ataxia, alterations of behavior, and impaired consciousness, with severe cases progressing to coma. Seizures, though rarely observed, may occur. Imaging shows reversible white matter lesions, with microbleeds being a significant and persistent imaging marker over time. Administration of glucocorticoids plays a crucial role in treatment. Despite experiencing seizures, this patient did not experienced any further episodes once his condition improved. [ABSTRACT FROM AUTHOR]

Objective: To report a series of atypical presentations of Aicardi–Goutières syndrome. Methods: Clinical, neuroimaging, and genetic data. Results: We report a series of six unrelated patients (five males) with a subacute loss of developmental milestones, pyramidal signs, and regression of communication abilities, with onset at ages ranging from 7 to 20 months, reaching a nadir after 4 to 24 weeks. A remarkable improvement of lost abilities occurred in the follow-up, and they remained with residual spasticity and dysarthria but preserved cognitive function. Immunization or febrile illness occurred before disease onset in all patients. CSF was normal in two patients, and in four, borderline or mild lymphocytosis was present. A brain CT scan disclosed a subtle basal ganglia calcification in one of six patients. Brain MRI showed asymmetric signal abnormalities of white matter with centrum semi-ovale involvement in five patients and a diffuse white matter abnormality with contrast enhancement in one. Four patients were diagnosed and treated for acute demyelinating encephalomyelitis (ADEM). Brain imaging was markedly improved with one year or more of follow-up (average of 7 years), but patients remained with residual spasticity and dysarthria without cognitive impairment. Demyelination relapse occurred in a single patient four years after the first event. Whole-exome sequencing (WES) was performed in all patients: four of them disclosed biallelic pathogenic variants in RNASEH2B (three homozygous p.Ala177Thr and one compound heterozygous p.Ala177Thr/p.Gln58*) and in two of them the same homozygous deleterious variants in RNASEH2A (p.Ala249Val). Conclusions: This report expands the phenotype of AGS to include subacute developmental regression with partial clinical and neuroimaging improvement. Those clinical features might be misdiagnosed as ADEM. [ABSTRACT FROM AUTHOR]

Background and Purpose: Acute blood pressure elevations lead to wide spectrum of neurologic manifestations, ranging from no overt neurologic symptoms to catastrophic events like ICH. Little is known regarding the determinants of this clinical variability. We determined clinical and imaging features of hypertensive crisis patients with normal neurological examination, ICH and posterior reversible encephalopathy syndrome (PRES). Methods: Cranial MRI was performed in patients with hypertensive urgency or emergency but normal neurological examination. Their clinical characteristics, and imaging features regarding cerebral small vessel disease were compared to ICH and PRES patients. Results: Hypertensive ICH patients (n = 58) were older, less likely to have hyperlipidemia, less commonly used calcium channel blockers, and had higher burden of chronic cSVD features in comparison to hypertensive crisis patients with normal neurological findings (n = 51). Multivariate analyses revealed cSVD burden score (p = 0.003) to be related with ICH, while higher admission blood pressure levels (p < 0.001), hyperlipidemia (p = 0.006) and calcium channel blocker usage (p = 0.005) were more common in patients with normal neurological examination. The PRES (n = 9) group was comprised of younger patients with recent history of hypertension and low burden of cSVD. Conclusions: Hypertensive surge is associated with ICH when cSVD burden is high, probably caused by microvascular dysfunction secondary to long-standing hypertension, while the episode causes no structural damage if this burden is less. Although our observations are exploratory, short term but severe hypertension manifests with PRES possibly due to the absence of adaptive changes. [ABSTRACT FROM AUTHOR]

Introduction: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinically bewildering encephalopathy syndrome which can be encountered in emergency room or in an already hospitalised patient. Its occurrence and mechanisms have been widely published mainly in the population of patients with transplant necessitated immunosuppression and not many studies have been carried out in the setting of many other risk factors. Aim: To study the frequency of primary clinical conditions causing or associated with PRES in general radiology practice. To study the frequency and distribution of abnormal findings in specific regions of brain. Materials and Methods: Radiology database of a freestanding imaging center and a multi-specialty hospital was scrutinised for reports citing PRES, hypertensive encephalopathy, eclampsia, renal disorders or any state of neurotoxicity on brain imaging, between July 2011 and June 2015. Reports showing complete or partial PRES pattern on imaging during the clinical neurotoxic syndrome were included in the study. Results: Total 84 patients were identified with PRES, out of them 80 were adults (mean age- 40.48±13.03 years). Among them 56 were men, 24 were women. There were four children (mean age- 12±2.19 years; with renovascular hypertension due to Takayasu’s arteritis). Twenty patients were normotensive and four patients were positive for Human Immunodeficiency Virus (HIV). Conclusion: There are many causes and associations of PRES but all of them are ‘systemic’ in nature. Spectrum of these systemic diseases may vary in population/practice. Hypertension is not an absolute requirement for diagnosis of PRES though it is the strongest association. Non posterior (non parieto-occipital) locations being frequent it is advisable to drop ‘posterior’ component from its name.

Background: Reversible leukoencephalopathy syndrome (RLS) is an acute neurological syndrome associated with altered mental status and visual disturbances described in patients with sudden elevations in systemic blood pressure and other medical conditions. In this process, neuroimaging studies usually demonstrate diffuse edema involving the subcortical structures of the posterior regions of the brain. Triple H (HHH) therapy is an established treatment for symptomatic vasospasm following subarachnoid hemorrhage (SAH). RLS has not been reported in the scientific literature as a complication of HHH therapy with perfusion computed tomography (CTP) imaging documentation. Case: A 73-year-old woman developed iatrogenic RLS during HHH therapy for SAH-related vasospasm. The computed tomography (CT) revealed bilateral parieto-occipital hypodensities. The CTP study showed increased cerebral blood volume and blood flow as well as decreased mean transit time in both parietal-occipital regions, which is compatible with vasogenic edema. Conclusion: The induction of hypertension as part of HHH therapy for SAH-related cerebral vasospasm may result in RLS. Therefore, it should be considered as a potentially reversible cause in the differential diagnosis of neurological deterioration in SAH patients while on HHH therapy. CTP study can offer an alternative for the assessment of this cerebrovascular syndrome. [ABSTRACT FROM AUTHOR]

Reports on an episode of reversible leukoencephalopathy syndrome (RLS), a rare brain disorder, in a 22-year-old male patient on chronic hemodialysis with well-controlled moderate hypertension who presented with de novo headache and generalized seizures. Patient's receipt of a kidney transplant 15 months after RSL onset and cyclosporine since the second post-transplant day; Need for the investigation for the presence of an underlying RLS by magnetic resonance imaging.

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic entity not yet understood, that presents with transient neurologic symptoms and particular radiological findings. Few papers show the differences between pregnant and non-pregnant patients. We review the cases of 38 women diagnosed with PRES, in order to find significant differences between pregnant (18) and non-pregnant (20) patients. We found differences among the age of patients (25.83 years old in pregnant and 29.31 years old in non pregnant; P=0.001); in the mean of highest systolic blood pressure, that was higher in nonpregnant group (185:162 mmHg; P=0.121); and in creatinine levels that was higher in non-pregnant group (3.47:1.04 mg/dL; P=0.001). To our knowledge, just a few papers analyzed whether PRES syndrome presented in the same way in pregnant and non-pregnant patients. The differences and the possible pathophisiology of this syndrome still remain enigmatic. [ABSTRACT FROM AUTHOR]

Objective: We report a case of contrast-induced encephalopathy (CIE) after repeated percutaneous transluminal angioplasty (PTA) for acute middle cerebral artery (MCA) occlusion., Case Presentation: An 88-year-old woman with left hemiparesis was transferred to our hospital by ambulance. MRI revealed acute MCA M1 occlusion. We performed intravenous tissue plasminogen activator therapy and PTA for right MCA occlusion, leading to complete recanalization and improvement in hemiparalysis. After approximately one week, restenosis of right MCA developed and PTA was performed again on day 11. However, her left hemiparesis exacerbated shortly thereafter. CT demonstrated leakage of contrast medium, and an extensive high-intensity area (HIA) on the white matter in the right cerebral hemisphere was noted on MRI FLAIR. The HIA on MRI and neurological deficits gradually improved after conservative treatment, but diffuse atrophy of the right cerebral hemisphere occurred and higher brain dysfunction remained., Conclusion: Repeated ischemia and reperfusion, and the frequent use of contrast media were considered the causes of CIE., Competing Interests: The authors declare no conflicts of interest., (©2022 The Japanese Society for Neuroendovascular Therapy.)

Reversible Posterior leukoencephalopathy syndrome is a proposed cliniconeuroradiological entity characterized by headache, altered mental status, cortical blindness, seizures, and other focal neurological signs, and a diagnostic magnetic resonance imaging picture. A variety of different etiologies have been reported like hypertension, pre-eclampsia/eclampsia, cyclosporin A or tacrolimus neurotoxicity, uraemia and porphyria. With early diagnosis and prompt treatment, the syndrome is usually fully reversible. Reversible Posterior leuko encephalopathy Syndrome (RPLS)) is a very rarecause of sudden onset vision loss in pregnancy. There are only few case reports in literature. We report a case of of RPLS with sudden onset vision loss in pregnancy. Clinicians as well as radiologists must be familiar with this clinically frightening, under diagnosed condition to assure timely diagnosis and treatment to prevent persistent deficits. [ABSTRACT FROM AUTHOR]

Abstract: Sweet disease, also known as acute febrile neutrophilic dermatosis, is an idiopathic multisystem inflammatory disorder characterised by erythematous skin lesions and fever. “Neuro-Sweet disease” is a rare central nervous system involvement that coexists with the characteristic cutaneous lesions of Sweet disease. Here, we report a case of Sweet disease complicated with acute encephalopathy. This case showed extensive lesions in the cerebral white matter on magnetic resonance imaging (MRI) and high protein concentration in the cerebrospinal fluid (CSF) without pleocytosis. After steroid therapy, the patient''s clinical syndrome recovered completely with no significant neurological deficits or abnormal findings on brain MRI, and normalization of the high CSF protein concentration. Both clinical and MRI findings suggested that the marked leukoencephalopathy in this case was mainly due to reversible oedema rather than destructive structural alterations in the cerebral parenchyma. [Copyright &y& Elsevier]

Abstract: We describe a case of a severe, immediate postpartum HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) with reversible posterior leukoencephalopathy (RPLS) characterized by generalized seizure, altered mental status, and visual loss in a 37-year-old primigravid woman. Magnetic resonance brain imaging showed diffuse subcortical edema, which resolved completely after 2 weeks along with complete symptom regression. The pathogenesis of RPLS is discussed and the importance of a prompt diagnosis is emphasized, as is the crucial role of rapid blood pressure reduction. [Copyright &y& Elsevier]

Posterior Reversible Encephalopathy Syndrome (PRES) is a neurological complication associated with several medical conditions and it has been described in clinical findings of seizures, headache, vomiting, altered mental status, and visual changes and focal neurologic deficit, in conjunction with radiological findings of primarily posterior cerebral white matter edema of both cerebral hemispheres. PRES can develop in a wide array situations including pregnancy and postpartum in patients with or without symptoms and signs of eclampsia. A prompt diagnosis of PRES by magnetic resonance imaging and an immediate antihypertensive and anticonvulsant therapy can help to prevent serious complications. The clinical case presented deals with a 35 year-old pregnant woman whose history of eclampsia was observed after a cesarean section.