• Journal of Medicine and Life Science
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• v.21 no.1
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• pp.15-19
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• 2024

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels of the body. The two most common ANCAs are myeloperoxidase ANCA and proteinase 3 ANCA. Neurological manifestations are frequent in patients with AAV, including peripheral neuropathy, meningitis, and stroke. AAV-associated ischemic stroke usually affects small vessels supplying the white matter or brainstem. This case report details the presentation and treatment course of a 70-year-old man with rapidly progressive multiple intracranial large artery involvement attributed to myeloperoxidase ANCA-associated vasculitis. Despite treatment with high-dose steroids and a rituximab infusion, the patient developed new speech difficulties and respiratory distress, and brain imaging confirmed new stroke lesions with progressive multiple intracranial large cerebral artery involvement. The patient died from SARS-CoV-2 infection 4 months after the diagnosis. This case emphasized the rare presentation of rapidly progressive large vessel involvement in a patient with myeloperoxidase ANCA-associated vasculitis despite active immunotherapy.

• Journal of Yeungnam Medical Science
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• v.21 no.1
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• pp.96-100
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• 2004

Behcet's disease is a chronic, relapsing multisystem disorder, that may develop into variable neurological manifestations. They include vascular and parenchymal involvement. Vascular involvement is dominated by cerebral venous sinus thrombosis marked by benign intracranial hypertension. Cerebral venous sinus thrombosis can present with all the classical criteria for idiopathic intracranial hypertension, including normal brain CT findings with normal CSF content. But brain MRI is a useful diagnostic method in this situation to confirm the presence of cerebral venous sinus thrombosis. We experienced a case of raised intracranial pressure in a 21-year-old man, caused by cerebral venous sinus thrombosis. We disclosed his symptoms and signs thus fulfilling the diagnostic criteria for Behcet's disease.

• Childhood Kidney Diseases
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• v.28 no.1
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• pp.44-50
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• 2024

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

• The Korean Journal of Nuclear Medicine
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• v.23 no.1
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• pp.85-87
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• 1989

Herpes simplex encephalitis (HSE) is one of the fulminant necrotizing, often fatal sporadic form of the encephalitis caused by herpes simplex type I virus. Characteristically, there is early and almost constant involvement of one or both temporal lobes, although there are common additional areas of involvement. Appropriate early treatment following correct diagnosis by clinical findings, CSF study, EEG and several radiological studies including angiography, redionuclide studies, CT or MRI can reduce its mortality and severity of the sequelae. We report a case of HSE diagnosed by adjuvant study of $^{99m}Tc-HMPAO$ regional cerebral blood flow SPECT, which showed a marked increase in bitemporal cerebral blood flow in a 24-year-old man.

• Korean Journal of Radiology
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• v.24 no.8
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• pp.784-794
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• 2023

Objective: To determine whether dynamic susceptibility contrast-enhanced (DSC) perfusion magnetic resonance imaging (MRI) can be used to evaluate posterior cerebral circulation in pediatric patients with moyamoya disease (MMD) who underwent anterior revascularization. Materials and Methods: This study retrospectively included 73 patients with MMD who underwent DSC perfusion MRI (age, 12.2 ± 6.1 years) between January 2016 and December 2020, owing to recent-onset clinical symptoms during the follow-up period after completion of anterior revascularization. DSC perfusion images were analyzed using a dedicated software package (NordicICE; Nordic NeuroLab) for the middle cerebral artery (MCA), posterior cerebral artery (PCA), and posterior border zone between the two regions (PCA-MCA). Patients were divided into two groups; the PCA stenosis group included 30 patients with newly confirmed PCA involvement, while the no PCA stenosis group included 43 patients without PCA involvement. The relationship between DSC perfusion parameters and PCA stenosis, as well as the performance of the parameters in discriminating between groups, were analyzed. Results: In the PCA stenosis group, the mean follow-up duration was 5.3 years after anterior revascularization, and visual disturbances were a common symptom. Normalized cerebral blood volume was increased, and both the normalized time-topeak (nTTP) and mean transit time values were significantly delayed in the PCA stenosis group compared with those in the no PCA stenosis group in the PCA and PCA-MCA border zones. TTP_PCA (odds ratio [OR] = 6.745; 95% confidence interval [CI] = 2.665-17.074; P < 0.001) and CBV_PCA-MCA (OR = 1.567; 95% CI = 1.021-2.406; P = 0.040) were independently associated with PCA stenosis. TTP_PCA showed the highest receiver operating characteristic curve area in discriminating for PCA stenosis (0.895; 95% CI = 0.803-0.986). Conclusion: nTTP can be used to effectively diagnose PCA stenosis. Therefore, DSC perfusion MRI may be a valuable tool for monitoring PCA stenosis in patients with MMD.

• Biomolecules & Therapeutics
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• v.13 no.2
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• pp.95-100
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• 2005

This study was performed to investigate the mechanism of cerebral blood flow of adenosine $A_{2B}$ receptor agonist in the rats, and to define whether its mechanism is mediated by nitric oxide (NO) and guanylate cyclase. In pentobarbital-anesthetized, pancuronium-paralyzed and artificially ventilated male Sprague-Dawley rats, all drugs were applied topically to the cerebral cortex. Blood flow from cerebral cortex was measured using laser-doppler flowmetry. Topical application of an adenosine $A_{2B}$ receptor agonist, 5'-N-ethylcar-boxamidoadenosine (NECA; $4{\mu}mol/l$) increased cerebral blood flow. This effect of NECA ($4{\mu}mol/l$) was blocked by pretreatment with NO synthase inhibitor, $N^G$-nitro-L-argine methvlester (L-NAME; $40{\mu}mol/l$) and guanylate cyclase inhibitor, LY-83,583 ($10{\mu}mol/l$). These results suggest that adenosine $A_{2B}$ receptor increases cerebral blood flow. It seems that this action of adenosine $A_{2B}$ receptor is mediated via the NO and the activation of guanylate cyclase in the cerebral cortex of the rats.

• Annals of Clinical Neurophysiology
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• v.23 no.1
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• pp.61-64
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• 2021

Adrenoleukodystrophy (ALD) is the most common peroxisomal disorder caused by mutations in the gene, ABCD1, causing abnormal accumulation of very-long-chain fatty acids in the nervous system and adrenal glands. There are various clinical manifestations of ALD. Here we report a 47-year-old male with adrenomyeloneuropathy with cerebral involvement who exhibited progressive gait disturbance and cognitive impairment. A novel frameshift variant (c.95del [p.Val32Alafs^*36]) in exon 1 of ABCD1 was identified. This report provides additional information regarding the various clinical characteristics of ALD.

• Clinical and Experimental Pediatrics
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• v.62 no.9
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• pp.334-339
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• 2019

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

• Journal of Korean Neurosurgical Society
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• v.49 no.2
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• pp.92-96
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• 2011

Objective : We retrospectively evaluated the prognostic factors that can influence long-term survival in patients who suffered acute large cerebral infarction. Methods : Between June 2003 and October 2008, a total of 178 patients were diagnosed with a large cerebral infarction, and, among them, 122 patients were alive one month after the onset of stroke. We investigated the multiple factors that might have influenced the life expectancies of these 122 patients. Results : The mean age of the patients was $70{\pm}13.4$ years and the mean survival was $41.7{\pm}2.8$ months. The mean survival of the poor functional outcome group ($mRS{\geq}4$) was $33.9{\pm}3.3$ months, whereas that of the good functional outcome group ($mRS{\leq}3$) was $58.6{\pm}2.6$ months (p value=0.000). The mean survival of the older patients (270 years) was $29.7{\pm}3.4$ months, whereas that of the younger patients (<70 years) was much better as $58.9{\pm}3.2$ months (p value=0.000). Involvement of ACA or PCA territory in MCA infarction is also a poor prognostic factor (p value=0.021). But, other factors that are also known as significant predictors of poor survival (male gender, hypertension, heart failure, atrial fibrillation, diabetes mellitus, a previous history of stroke, smoking, and dyslipidemia) did not significantly influence the mean survival time in the current study. Conclusion : Age (older versus younger than 70 years old) and functional outcome at one month could be critical prognostic factors for survival after acute large cerebral infarction. Involvement of ACA or PCA territory is also an important poor prognostic factor in patients with MCA territorial infarction.

• The Korean Journal of Pharmacology
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• v.29 no.1
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• pp.1-8
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• 1993

To elucidate involvement of platelet-activating factor (PAF) in cerebral ischemia-reperfusion injury, male Sprague-Dawley rats and albino mice of either sex were subjected to a 10-min bilateral carotid artery occlusion and 6-hr recirculation. The McGraw stroke index in mice was markedly inhibited by PAF antagonists, BN 52021 and CV 6209 (1 mg/kg, i.p., each) When they were administered 10 min before bilateral carotid artery occlusion or 1 hr after reperfusion. The increases in brain water content were significantly attenuated by treatment with BN 52021 or CV 6209 in both animals. BN 52021 exhibited a significant improvement in the postischemic blood pressure change in association with a beneficial effect on the delayed dilatation of pial arterioles after 10 min of ischemia. Thus it is suggested that PAF plays an important role as an endogenous mediator in development of cerebral ischemia-reperfusion injury, and further, specific antagonists to PAF will be able to prevent or reverse the pathological sequelae of cerebral ischemia.