Objective: To localize the neuroanatomical substrate of rapid eye movement sleep behavior disorder (RBD) and to investigate the neuroanatomical locational relationship between RBD and α-synucleinopathy neurodegenerative diseases. Materials and Methods: Using a systematic PubMed search, we identified 19 patients with lesions in different brain regions that caused RBD. First, lesion network mapping was applied to confirm whether the lesion locations causing RBD corresponded to a common brain network. Second, the literature-based RBD lesion network map was validated using neuroimaging findings and locations of brain pathologies at post-mortem in patients with idiopathic RBD (iRBD) who were identified by independent systematic literature search using PubMed. Finally, we assessed the locational relationship between the sites of pathological alterations at the preclinical stage in α-synucleinopathy neurodegenerative diseases and the brain network for RBD. Results: The lesion network mapping showed lesions causing RBD to be localized to a common brain network defined by connectivity to the pons (including the locus coeruleus, dorsal raphe nucleus, central superior nucleus, and ventrolateral periaqueductal gray), regardless of the lesion location. The positive regions in the pons were replicated by the neuroimaging findings in an independent group of patients with iRBD and it coincided with the reported pathological alterations at post-mortem in patients with iRBD. Furthermore, all brain pathological sites at preclinical stages (Braak stages 1-2) in Parkinson's disease (PD) and at brainstem Lewy body disease in dementia with Lewy bodies (DLB) were involved in the brain network identified for RBD. Conclusion: The brain network defined by connectivity to positive pons regions might be the regulatory network loop inducing RBD in humans. In addition, our results suggested that the underlying cause of high phenoconversion rate from iRBD to neurodegenerative α-synucleinopathy might be pathological changes in the preclinical stage of α-synucleinopathy located at the regulatory network loop of RBD.
Brainstem infarction due to vertebrobasilar insufficiency is a rare initial presentation of systemic lupus erythematosus (SLE) patients and small-vessel dissection as the direct cause of infarction has not been reported. We report the case of a 20-year-old female with acute infarction on the right side of the pons due to a small artery (pontine perforator) dissection, identified on digital subtraction angiography and high-resolution vessel wall MRI (vwMRI). She was diagnosed with SLE based on the presence of neurologic disorders and relevant laboratory findings. The pontine perforator-dissecting aneurysm had occluded and the right distal vertebral artery had resolved on subsequent vwMRI. She had a modified Rankin Scale (mRS) score of 1 at discharge with mild symptom improvement, and exhibited no further aggravation of symptoms at 3 or 12 months, maintaining an mRS score of 1.
Cancer progression is driven by genetic mutations, environmental factors, and intricate interactions within the tumor microenvironment (TME). The TME comprises of diverse cell types, such as cancer cells, immune cells, stromal cells, and neuronal cells. These cells mutually influence each other through various factors, including cytokines, vascular perfusion, and matrix stiffness. In the initial or developmental stage of cancer, neurotrophic factors such as nerve growth factor, brain-derived neurotrophic factor, and glial cell line-derived neurotrophic factor are associated with poor prognosis of various cancers by communicating with cancer cells, immune cells, and peripheral nerves within the TME. Over the past decade, research has been conducted to prevent cancer growth by controlling the activation of neurotrophic factors within tumors, exhibiting a novel attemt in cancer treatment with promising results. More recently, research focusing on controlling cancer growth through regulation of the autonomic nervous system, including the sympathetic and parasympathetic nervous systems, has gained significant attention. Sympathetic signaling predominantly promotes tumor progression, while the role of parasympathetic signaling varies among different cancer types. Neurotransmitters released from these signalings can directly or indirectly affect tumor cells or immune cells within the TME. Additionally, sensory nerve significantly promotes cancer progression. In the advanced stage of cancer, cancer-associated cachexia occurs, characterized by tissue wasting and reduced quality of life. This process involves the pathways via brainstem growth and differentiation factor 15-glial cell line-derived neurotrophic factor receptor alpha-like signaling and hypothalamic proopiomelanocortin neurons. Our review highlights the critical role of neurotrophic factors as well as central nervous system on the progression of cancer, offering promising avenues for targeted therapeutic strategies.
Purpose: An association between very low birth weight infants(VLBWI) and hearing loss has long been recognized. Early identification and intervention for hearing loss benefits language and speech/cognitive development. We investigated the risk factors and clinical outcomes of hearing loss among VLBWI. Methods: We analyzed the results of auditory brainstem response (ABR) testing of VLBWI. These infants were admitted to the neonatal intensive care unit (NICU) of Pusan National University Yangsan Hospital between December 2008 and February 2011. A follow-up was conducted subsequently. Results: ABR evaluations were performed on 65 infants, and 31 showed abnormal results (47.7%). Among the 31 infants, 10 were classified with moderate/severe/profound hearing loss (15.4%). The infants with abnormal ABR had a higher incidence of low birth weight, prolonged ventilator care, cumulative dose of furosemide, and the lowest $PaO_2$ (P<0.05). Those with moderate/severe/profound hearing loss had a higher incidence of low Apgar scores at 5 minutes (odds ratio[OR],0.34; 95% confidence interval[CI],0.13-0.89), prolonged ventilator care (OR,1.06; 95% CI,1.01-1.12), and mild hearing loss compared to those without profound hearing loss. Follow-up evaluations on eight infants with ABR reveled improvements 5.6${\pm}$3.9 months later. One infant, who had profound hearing loss in both ears, used a hearing aid. Conclusion: Factors influencing hearing loss at the first VLBWI hearing screening test included lower Apgar scores at 5 min and prolonged use of a ventilator. Most VLBWI with hearing losses were expected to recover after several months of follow-up.
Seo, Jeong Il;Yoo, Si Uk;Gong, Sung Hyeon;Hwang, Gwang Su;Lee, Hyeon Jung;Kim, Joong Pyo;Choi, Hyeon;Lee, Bo Young;Mok, Ji Sun
Clinical and Experimental Pediatrics
/
v.48
no.7
/
pp.706-710
/
2005
Purpose : Early diagnosis of congenital hearing loss through the neonatal hearing screening test minimizes language defect. This research intends to identify frequency of congenital hearing loss in infants through neonatal hearing screening test with the aim of communicating the importance of hearing test for infants. Methods : From May 20, 2003 to May 19, 2004, infants were subjected to Automated Auditory Brainstem Response test during one month of birth to conduct the test with 35 dB sound. Infants who passed the 1st round of hearing test, were classified into 'pass' group whereas those who did not were classified into 'refer' group. Infants who did not 'pass' in the hearing test conducted within one month of birth were subjected to re-test one month later, and if classified as 'refer' during the re-test, they were subjected to the diagnosis for validation of hearing loss by requesting test to the hearing loss clinic. Results : There was no difference among the 'pass' and 'refer' group in terms of form of childbirth, weight at birth and gestational age. In the 1st test, total of 45 infants were classified into 'refer' group. Six among 35 who were subjected to re-test(17%) did not pass the re-test, and all were diagnosed with congenital hearing loss. This corresponds to 0.35%(3.5 per 1,000) among total number of 1,718 subjects. Conclusion : In our study the congenital hearing loss tends to be considerably more frequently than congenital metabolic disorder. Accordingly, newly born infants are strongly recommended to undergo neonatal hearing screening test.
Kong, Seung Hyun;Kang, Jang Hee;Hwang, Kwang Su;Kim, Joong Pyo;Lee, Hyeon Jung;Choi, Hyeon;Mok, Ji Sun;Kim, Jung Young
Clinical and Experimental Pediatrics
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v.49
no.8
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pp.845-850
/
2006
Purpose : To assess the incidence of neonatal hearing loss in a neonatal intensive care unit and the relative importance of risk factors for hearing imparement in a neonatal intensive care unit which the Joint Committee on Infant Hearing(JCIH) had recommended. Methods : One thousand, two hundred and one newborns admitted to the Good Moonhwa Intensive Care Unit from May 2003 to December 2005 were assesed using the automated auditory brainstem response(AABR). The screening was performed on those aged more than 36 weeks and weighing more than 2,200 g. We divided the infants into two groups, 'pass' and 'refer'. The 'refer' group were retested one month later, and if classified as 'refer' during the retest, were referred to a hearing impairment clinic. Results : From the 1,201 neonates, 1,187(98.8 percent) passed the test and 14(1.2 percent) failed. 293(24.4 percent) of the 1,201 neonates had a risk factor for hearing impairment; 282(96.2 percent) passed the test and 11(3.8 percent) failed. The group with risk factors were shown to have a higher incidence of hearing loss(P<0.001). The neonates in the refer group were shown to have a higher incidence of ototoxic drugs(P<0.001), low birth weight(<1,500 g)(P<0.001) and craniofacial anomalies(P=0.007). On the other hand, there were no statistical differences between the pass and refer groups in congenital infection, hyperbilirubinemia, bacterial meningitis, low Apgar scores, prolonged mechanical ventilation and syndromes known to include hearing loss. Conclusion : In order to identify hearing-impaired infants within an appropriate period, neonatal hearing screening tests and identification of the risk factors for neonatal hearing loss are important.
Purpose : To describe MR imaging features of hypoxic brain damage in relation to time elapse and prognosis of patients. Materials and methods : We reviewed 19 MR studies of 18 patients with hypoxic brain damage. MR imaging studies were performed between 1 to 20 days after the hypoxic insults (mean 8.6 days). MR images were analyzed with regard to the locations of abnormal signal intensities, the presence of brain edema. And imaging findings were correlated with the time elapse after the insults and the prognosis of patients. Results : On 19 cases of MR studies, abnormal high intensities on T2-weighted images were found in the basal ganglia (15, 78.9%), cerebral cortex (13, 68.4%), white matter (9, 47.4%), thalamus (6, 31.6%), cerebellum (4, 21.1%) and brainstem (1, 5.3%), respectively. Cerebral cortical involvement was typically bilateral and diffuse, but sometimes limited to the parieto-occipital area. The brainstem and cerebellar involvement was rare and in all cases, cerebral cortical lesions accompanied. Most of the white matter lesions were accompanied with cortical and deep gray matter lesions and found in subacute period(>6 days). The cortical high signal intensity lesions on T1-weighted image were found mostly in subacute stage, but in some cases involvement was also found in acute stage ($\leq$ 6 days). The cortical edema is found on 11 cases in acute and subacute stages. In cases of recovered consciousness, cortical involvement and edema on MR were rare. Conclusion : MR findings of hypoxic brain damage were various, but diffuse bilateral involvement of cortex and/or deep gray matter was found in most of the cases. White matter involvement was rarely found in acute stage and usually found in subacute stage. In cases of good pronosis, cortical involvement and edema were rare.
Kim, Tae-Wan;Jung, Tae-Young;Jung, Shin;Kim, In-Young;Moon, Kyung-Sub;Jeong, Eun-Hui
Journal of Korean Neurosurgical Society
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v.51
no.5
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pp.272-275
/
2012
Objective : Choroid plexus papillomas (CPPs) are generally regarded as benign tumors with typical radiologic and pathologic findings. However, they sometimes have unusual findings. We have analyzed radiologic findings and pathologic growth patterns on CPPs. Methods : The study group included 5 male and 5 female patients (age range, 3 months to 58 years : median, 29 years). The study group included 3 pediatric and 7 adult patients. All patients underwent surgery; 9 patients had a gross total resection and 1 patient had a subtotal resection. We analyzed the radiologic findings (location, size, mottle-like appearance, enhancement, calcifications, and hydrocephalus) and pathologic growth patterns (typical papillary, papillary and solid, and papillary and tubular). Results : The median follow-up duration was 21.3 months (range, 4-47.8 months). There were no recurrences after initial treatment. All patients had benign CPPs. Pediatric CPPs were 3.2 cm masses (range, 2.7-4 cm) with homogeneous enhancement and a mottle-like appearance, which pathologically showed the papillary growth pattern. Hydrocephalus was present in all pediatric patients. Postoperatively, subdural hygroma had occurred in two patients. In adults, CPPs were located in the fourth ventricle in 6 patients and suprasellar area in 1 patient. The size varied from 0.5-4.2 cm. Hydrocephalus and calcifications occurred in 3 and 4 patients, respectively. Three patients showed the heterogeneous enhancement without a mottle-like appearance and pathologically showed combined papillary and solid growth in 2 patients and papillary and tubular growth in one. Postoperatively, two patients with large masses had injuries of the brainstem and underwent shunt procedures for aggravation of hydrocephalus. Conclusion : CPPs may show unusual radiologic findings, which preoperatively give the difficulty to be differentiated from other tumors. CPPs with unusual radiologic findings showed the combined pathologic growth patterns.
Objective : Aneurysms of vertebral artery and its branches make up approximately 3% of all intracranial aneurysms. As the aneurysm have an intimate relationship with lower cranial nerves and medulla, surgical management of the aneurysms are one of the challenging neurosurgical problems. The authors analyzed the management outcomes for aneurysms arising from vertebral artery and its branches. Methods : At the authors' institution between May 1989 and Jan. 2000, 42 patients were treated with transcranial and endovascular surgery for aneurysms of vertebral artery and its branches. The medical records and neuroimaging studies of the patients were reviewed retrospectively. Results : Forty two patients were comprised of 28 female and 14 male patients aged from 26-80 year old(mean : 51.8). Of the 42 patients, 37 patients(88%) had subarachnoid hemorrhage. Of the 37 patients with subarachnoid hemorrahge, 35 patients(95%) were in good neurological status(Hunt Hess grade I-III), 2 patients(5%) in poor grade(H-H grade IV-V) before operation. Location of the aneurysm were 16 in vertebral artery, 12 in vertebro-PICA junction, and 14 in the peripheral PICA. Twenty nine patients were treated with transcranial surgery and 13 patients with endovascular surgery. The management outcome of the transcranial surgery was : Glasgow outcome scale(GOS) I and II ; 24, GOS III ; 2, GOS IV ; 1 and GOS V(death) ; 2. The causes of mortality related to transcranial surgery were rebleeding after failure in clipping in one and suspected brainstem infarct in one. Morbidity was attributed to vasospasm(3), lower CN palsy(7, including temporary dysfunction) and pseudomeningocele(1). The management outcome of the endovascular surgery was : Glasgow outcome scale(GOS) I-II ; 9, GOS III ; 1, GOS IV ; 1, and GOS V(death) ; 2. The causes of mortality related to endovascular surgery were sepsis from pneumonia(1) and vasospasm(1). There were one cerebellar infarct and one lateral medullary syndrome. Conclusion : Excellent and good surgical results can be expected in 80% of the patients with aneurysms of vertebral arery and its branches. The outcomes of endovascular surgery in treating vertebral artery aneurysm were satisfactory and endovascular surgery may offer a therapeutic alternative especially in vertebral dissecting aneurysm.
Lee, Sung Yeal;Son, Eun Ik;Kim, Ok Bae;Choi, Tae Jin;Kim, Dong Won;Yim, Man Bin;Kim, In Hong
Journal of Korean Neurosurgical Society
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v.29
no.8
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pp.1030-1036
/
2000
Objective : The aim of this study was to retrospectively analyze the safety and effect of Linac based Photon Knife Radiosugery System(PKRS) for treatment of cerebral arteriovenous malformation. Patients and Methods : The authors analyzed the clinical method and results of ten patients who were followed up more than two years, among the 18 patients who had radiosurgery on arteriovenous malformation from June, 1992, to Dec. 1997, with Linac based Photon knife radiosurgery system(PKRS) which was developed in our hospital. Results : The average age of the patients was 30.4(with the range of 13-49), and the sex was seven males and three females. For the initial clinical symptoms, there were five patients with headache, three with seizure, one with hemiparesis, and one with vomiting. Before the radiosurgery, computed tomography, MRI, and cerebral angiogram were done. For the location of arteriovenous malformation, it was found on six patients of cerebral hemisphere, two of thalamus, one of brainstem, and one of corpus callosum. Regarding the size of nidus, there were seven patients of smaller than 3cm, and three patients of larger than 3cm. Computed tomography, MRI, and cerebral angiogram were done periodically for sixth months, first year, and second year after the radiosurgery of PKRS for the completeness of obliteration. Six cases showed complete obliteration, and four partial obliterations were observed among ten cases, and interestingly, six cases of complete obliteration were observed among seven cases of small AVM of smaller than 3cm(the rate of complete obliteration : 85.7%). All patients tolerated the treatment and no significant complication were seen. Conclusion : In this study, linac based radiosurgery using PKRS onto arteriovenous malformation showed excellent effects, therefore authors believe that it is an ideal method for small sized or deep seated AVM.
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