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1.
Article in English | WPRIM | ID: wpr-913833

ABSTRACT

Purpose@#Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. @*Materials and Methods@#Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. @*Results@#Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). @*Conclusion@#This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

2.
Article in English | WPRIM | ID: wpr-913603

ABSTRACT

Background@#In addition to vascular endothelial cells, vascular smooth muscle cells (VSMCs) are subject to continuous shear stress because of blood circulation. The angiogenic properties of VSMCs in extracranial arteriovenous malformations (AVMs) may exceed those of normal blood vessels if the body responds more sensitively to mechanical stimuli. This study was performed to investigate the hypothesis that rapid angiogenesis may be achieved by mechanical shear stress. @*Methods@#VSMCs were obtained from six patients who had AVMs and six normal controls. The target genes were set to angiopoietin-2 (AGP2), aquaporin-1 (AQP1), and transforming growth factor-beta receptor 1 (TGFBR1). Reverse-transcriptase polymerase chain reaction (RT-PCR) and real-time PCR were implemented to identify the expression levels for target genes. Immunofluorescence was also conducted. @*Results@#Under the shear stress condition, mean relative quantity values of AGP2, AQP1, and TGFBR1 in AVM tissues were 1.927±0.528, 1.291±0.031, and 2.284±1.461 when compared with neutral conditions. The expression levels of all three genes in AVMs were higher than those in normal tissue except for AQP1 under shear stress conditions. Immunofluorescence also revealed increased staining of shear stress-induced genes in the normal tissue and in AVM tissue. @*Conclusions@#Shear stress made the VSMCs of AVMs more sensitive. Although the pathogenesis of AVMs remains unclear, our study showed that biomechanical stimulation imposed by shear stress may aggravate angiogenesis in AVMs.

3.
Article in English | WPRIM | ID: wpr-914661

ABSTRACT

Background and Objectives@#The clinical implications of the BRAF V600E mutation in papillary thyroid microcarcinoma (PTMC), defined as ≤1.0 cm of tumor size, remain controversial. We investigated the association between the BRAFV600E mutation and PTMC recurrence in a retrospective cohort of patients with thyroid cancer. @*Materials and Methods@#This study included 2319 patients with PTMC (median age, 50 years [interquartile range (IQR), 41-57 years]) who underwent thyroid surgery from 2010 to 2019 at a single tertiary medical center. The median follow-up time was 75 months (IQR, 30-98 months). Tumor recurrence was confirmed by histological, cytological, radiographic, and biochemical criteria, combined with persistent and recurrent disease. @*Results@#A total of 60.2% (1395/2319) patients with PTMC had the BRAF V600E mutation. The tumor recurrence rate was 2.1% (19/924) in BRAF mutation-negative patients and 2.9% (41/1395) in BRAF mutation-positive patients, with a hazard ratio (HR) of 1.05 (95% confidence interval [CI], 0.61-1.84) after adjusting for clinicopathological risk factors. Similar results were found in patients with high-risk PTMC (adjusted HR, 1.09; 95% CI, 0.56-2.11) who had lymph node metastasis (LNM), extrathyroidal extension (ETE), or distant metastasis (DM) at diagnosis and in patients with low-risk PTMC (adjusted HR, 1.00; 95% CI, 0.35-2.83) who had no LNM, ETE, or DM. @*Conclusion@#The finding that the BRAF V600E mutation was not associated with tumor recurrence in our cohort of Korean patients with PTMC, especially in patients with low-risk PTMC, suggests that its value in the prediction of disease progression is limited.

4.
Article in English | WPRIM | ID: wpr-913590

ABSTRACT

Background@#Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision. @*Methods@#Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement. @*Results@#Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group. @*Conclusions@#Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.

5.
Article in English | WPRIM | ID: wpr-901389

ABSTRACT

Amyloidosis has been reported to be associated with non-Hodgkin lymphoma. Amyloidosis and lymphoma can be related in two ways: lymphoma-associated systemic amyloidosis and peritumoral amyloidosis with lymphoma. We report a rare case of peritumoral amyloidosis in a patient with head and neck mucosa-associated lymphoid tissue lymphoma. On CT, the oropharyngeal mass showed an irregularly shaped soft-tissue density with multifocal amorphous calcifications and heterogeneous enhancement. On MRI, the mass showed heterogeneous low signal intensity on both T1- and T2-weighted images. On contrast-enhanced MR images, the mass showed good enhancement with several inner non-enhancing foci. Concurrent pathologies, such as peritumoral amyloidosis, should be considered when calcifications are noted in patients with pre-treatment lymphoma.

6.
Article in English | WPRIM | ID: wpr-899876

ABSTRACT

Increasing rates of coronavirus disease 2019 (COVID-19) vaccination coverage will result in more vaccine-related side effects, including acute myocarditis. In Korea, we present a 24-year-old male with acute myocarditis following COVID-19 vaccination (BNT162b2).His chest pain developed the day after vaccination and cardiac biomarkers were elevated.Echocardiography showed minimal pericardial effusion but normal myocardial contractility.Electrocardiography revealed diffuse ST elevation in lead II, and V2-5. Cardiac magnetic resonance images showed the high signal intensity of T2- short tau inversion recovery image, the high value of T2 mapping sequence, and late gadolinium enhancement in basal inferior and inferolateral wall. It was presumed that COVID-19 mRNA vaccination was probably responsible for acute myocarditis. Clinical course of the patient was favorable and he was discharged without any adverse event.

7.
Article in English | WPRIM | ID: wpr-897074

ABSTRACT

Background@#Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. @*Methods@#Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. @*Results@#Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. @*Conclusion@#Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

8.
Article in English | WPRIM | ID: wpr-894293

ABSTRACT

Background@#Oral propranolol has recently been introduced as a successful treatment for infantile hemangioma (IH).Though, there are limited reports on this treatment including large number of Korean patients with IH covering a long-term powder and solution formulation period. @*Objective@#We investigated the effectiveness and side effects of two different formulations of oral propranolol treatment in patients with IH at a Korean tertiary university hospital. @*Methods@#From June 2011 to October 2019, 375 patients were treated with powder- or solution-type oral propranolol starting at 1 mg/kg/day and increasing up to 3 mg/kg/day. Drug effectiveness was evaluated on four scales through sequential photographs by two dermatologists. Side effects were recorded on a medical chart. @*Results@#Overall, the mean improvement scale was 2.61±0.73 at 3 months after treatment initiation. The scale was higher for solution-type than for powder-type oral propranolol at the 3-month follow-up (2.71±0.79 vs. 2.54±0.67, p<0.05). The patients’ mean duration of treatment was 8.56±5.85 months, which was shorter for solution-type than for powder-type oral propranolol (6.0 vs. 10.69 months, p<0.05). Among the total number of patients, 22 reported mild side effects, including loose stools and noticeable sleep disturbance, and few serious side effects such as grunting, while two patients required medical intervention. @*Conclusion@#The patients in our study were effectively treated for IH with oral propranolol without significant side effects and had a shorter treatment duration with solution-type oral propranolol than with powder-type oral propranolol.

9.
Article in English | WPRIM | ID: wpr-893685

ABSTRACT

Amyloidosis has been reported to be associated with non-Hodgkin lymphoma. Amyloidosis and lymphoma can be related in two ways: lymphoma-associated systemic amyloidosis and peritumoral amyloidosis with lymphoma. We report a rare case of peritumoral amyloidosis in a patient with head and neck mucosa-associated lymphoid tissue lymphoma. On CT, the oropharyngeal mass showed an irregularly shaped soft-tissue density with multifocal amorphous calcifications and heterogeneous enhancement. On MRI, the mass showed heterogeneous low signal intensity on both T1- and T2-weighted images. On contrast-enhanced MR images, the mass showed good enhancement with several inner non-enhancing foci. Concurrent pathologies, such as peritumoral amyloidosis, should be considered when calcifications are noted in patients with pre-treatment lymphoma.

10.
Article in English | WPRIM | ID: wpr-892172

ABSTRACT

Increasing rates of coronavirus disease 2019 (COVID-19) vaccination coverage will result in more vaccine-related side effects, including acute myocarditis. In Korea, we present a 24-year-old male with acute myocarditis following COVID-19 vaccination (BNT162b2).His chest pain developed the day after vaccination and cardiac biomarkers were elevated.Echocardiography showed minimal pericardial effusion but normal myocardial contractility.Electrocardiography revealed diffuse ST elevation in lead II, and V2-5. Cardiac magnetic resonance images showed the high signal intensity of T2- short tau inversion recovery image, the high value of T2 mapping sequence, and late gadolinium enhancement in basal inferior and inferolateral wall. It was presumed that COVID-19 mRNA vaccination was probably responsible for acute myocarditis. Clinical course of the patient was favorable and he was discharged without any adverse event.

11.
Article in English | WPRIM | ID: wpr-889370

ABSTRACT

Background@#Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. @*Methods@#Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. @*Results@#Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. @*Conclusion@#Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

12.
Article in English | WPRIM | ID: wpr-901997

ABSTRACT

Background@#Oral propranolol has recently been introduced as a successful treatment for infantile hemangioma (IH).Though, there are limited reports on this treatment including large number of Korean patients with IH covering a long-term powder and solution formulation period. @*Objective@#We investigated the effectiveness and side effects of two different formulations of oral propranolol treatment in patients with IH at a Korean tertiary university hospital. @*Methods@#From June 2011 to October 2019, 375 patients were treated with powder- or solution-type oral propranolol starting at 1 mg/kg/day and increasing up to 3 mg/kg/day. Drug effectiveness was evaluated on four scales through sequential photographs by two dermatologists. Side effects were recorded on a medical chart. @*Results@#Overall, the mean improvement scale was 2.61±0.73 at 3 months after treatment initiation. The scale was higher for solution-type than for powder-type oral propranolol at the 3-month follow-up (2.71±0.79 vs. 2.54±0.67, p<0.05). The patients’ mean duration of treatment was 8.56±5.85 months, which was shorter for solution-type than for powder-type oral propranolol (6.0 vs. 10.69 months, p<0.05). Among the total number of patients, 22 reported mild side effects, including loose stools and noticeable sleep disturbance, and few serious side effects such as grunting, while two patients required medical intervention. @*Conclusion@#The patients in our study were effectively treated for IH with oral propranolol without significant side effects and had a shorter treatment duration with solution-type oral propranolol than with powder-type oral propranolol.

13.
Article in English | WPRIM | ID: wpr-918028

ABSTRACT

Objective@#Anterior cervical corpectomy using a titanium mesh cage may result in delayed nonunion and thus a change in cervical alignment, and patients may require revision surgery.We investigated the radiologic and clinical outcomes of cervical corpectomy and the risk factors for subsidence. @*Methods@#We studied 74 patients who underwent single-level anterior cervical corpectomy for cervical spondylotic myelopathy with or without ossification of the posterior longitudinal ligament between 2007 and 2014. Graft subsidence was considered present when there was a reduction in the anterior and posterior heights by an average of 4 mm or more 2 years after the operation. We measured cervical parameters before surgery, immediately after surgery, and 6, 12, and 24 months after surgery. The clinical outcomes were the neck and arm visual analog scale scores and reoperation rate. @*Results@#In the subsidence group, these values gradually decreased over the 24 months. The radiologic parameters did not differ between the 2 groups for 24 months after the onset of subsidence. There were no differences in clinical outcome or reoperation rate. In the analysis of the risk factors, subsidence occurred with a large T1 slope and a large change in the C27 Cobb angle (p=0.020 andp=0.026, respectively). @*Conclusion@#Subsidence gradually occurred after single-level anterior cervical corpectomy for up to 24 months. However, the presence of subsidence did not affect the radiologic and clinical outcomes. When the T1 slope was large and the C27 Cobb angle change was severe, more subsidence occurred.

14.
Article in English | WPRIM | ID: wpr-811390

ABSTRACT

BACKGROUND/OBJECTIVES: North Korean refugees (NKRs) in South Korea are a unique population as they must adapt in a new country with similar cultural traits but different social, political, and economic systems, but little research has been conducted on diet and nutrition in this population. This study examined food security, dietary behaviors, and nutrient intakes among adult NKRs living in South Korea and compared them to those of South Koreans.SUBJECTS/METHODS: The subjects were 139 adult NKRs (25 men, 114 women) living in the Seoul metropolitan area, and 417 age- and sex-matched South Korean controls (SKCs; 75 men, 342 women) selected from the Korea National Health and Nutrition Examination Survey (KNHANES). Food security and dietary behaviors (meal skipping, eating-out, meals with family, nutrition education and counseling, and nutrition label knowledge and utilization) were obtained using self-administered questionnaires. Nutrient intakes were assessed by 24-hr recall. The statistical analysis was performed using IBM SPSS ver. 23.0.RESULTS: In South Korea, food security had improved over the previous 12 months, but remained significantly poorer for NKR women than SKC women. Meal skipping was three times more frequent than for SKCs and eating-out was rare. Average energy intake was 1,509 kcal for NKR men and 1,344 kcal for NKR women, which was lower than those of SKCs (2,412 kcal and 1,789 kcal, respectively). Significantly more NKRs (men 24.0%, women 21.9%) showed simultaneously deficient intake in energy, calcium, iron, vitamin A, and riboflavin than SKCs (men 2.7% (P = 0.003), women 7.0% (P < 0.001)). NKR women had a significantly higher index of nutrient quality (INQ) for some nutrients than SK women.CONCLUSIONS: This study reports significant differences in food security, dietary behaviors, and nutrient intakes between NKRs and SKCs. Generally, NKRs reported lower intakes despite improved food security, but relatively good INQs across nutrients. Further research is needed to understand processes of food choice and consumption among NKRs to provide appropriate support aimed at improving diets.

15.
Article in English | WPRIM | ID: wpr-833476

ABSTRACT

Objective@#: Spine surgery is associated with higher morbidity and mortality rates in elderly patients. The modified Frailty Index (mFI) is an evaluation tool to determine the frailty of an individual and how preoperative status may impact postoperative survival and outcomes. This study aimed to determine the usefulness of mFI in predicting postoperative complications in patients aged ≥75 years undergoing surgery with instrumentation. @*Methods@#: We retrospectively reviewed the perioperative course of 137 patients who underwent thoracolumbar-instrumentation spine surgery between 2011 and 2016. The preoperative risk factors were the 11 variables of the mFI, as well as body mass index (kg/cm2), preoperative hemoglobin, platelet, albumin, creatinine, anesthesia time, operation time, estimated blood loss, and transfusion amount. The 60-day occurrences of complication rates were used for outcome assessment. @*Results@#: Major complications after spinal instrumentation surgery occurred in 34 of 138 patients (24.6%). The mean mFI score was 0.18±0.12. When we divided patients into a pre-frail group (mFI, 0.09–0.18; n=94) and a frail group (mFI ≥0.27; n=44), only the rate of sepsis was statistically higher in the frail group than in the pre-frail group. There were significantly more major complications in patients with low albumin levels or in patients with infection or who had experienced trauma. The mFI was a more useful predictor of postoperative complications than the American Society of Anesthesiologists physical status score. @*Conclusion@#: The mFI can successfully predict postoperative morbidity and mortality in patients aged ≥75 years undergoing spine surgery. The mFI improves perioperative risk stratification that provides important information to assist in the preoperative counselling of patients and their families.

16.
Article | WPRIM | ID: wpr-831814

ABSTRACT

Oral sulfate solution (OSS) is an emerging cleansing agent for bowel preparation. However, data comparing OSS to other conventional bowel preparations in Asian patients are limited. Therefore, the objective of this study was to compare the efficacy and tolerability of OSS to ascorbic acid plus polyethylene glycol (AA + PEG) in Asian patients. Methods: This was a prospective, randomized, parallel, investigator-blind study performed in two university hospitals in Korea. Bowel preparation efficacy was evaluated using both the Ottawa Bowel Preparation Scale (OBPS) and Boston Bowel Preparation Scale (BBPS). Results: Among 173 patients, 86 received OSS while 87 received AA + PEG for bowel preparation. Total OBPS score was 2.80 ± 2.48 in the OSS group and 4.49 ± 3.08 in the AA + PEG group, indicating significantly (p < 0.001) better efficacy with OSS. Total BBPS was higher in the OSS group (7.43 ± 1.49 vs. 6.51 ± 1.76, p < 0.001), indicating superior bowel preparation quality with OSS. Preparation-related adverse events were generally acceptable. Patients receiving OSS had more nausea (1.92 ± 0.94 vs. 1.54 ± 0.76, p = 0.004) and abdominal cramping (1.45 ± 0.78 vs. 1.17 ± 0.51, p = 0.006) than those receiving AA + PEG. However, overall satisfaction and taste were similar between the two groups. Conclusions: OSS had a non-inferior bowel cleansing efficacy than AA + PEG regardless of colon segment.

17.
Article | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

18.
Article | WPRIM | ID: wpr-830604

ABSTRACT

Sinus pericranii is a rare vascular anomaly characterized by abnormal venous communication betweenthe inner and outer regions of the cranial cavity. Here, we report a case of sinus pericraniiand venous malformations in the right periorbital region of a 2-year-old girl. Radiologic findingsshowed venous malformations in the right parietal region communicating with the superior sagittalsinus in the intracranial region. There were notable improvements following surgical resectionfor the abnormal venous lesions and several sclerotherapies. Presence of a bluish and pulsatingmass on the scalp, which showed bruit on auscultation, may indicate sinus pericranii, whichshould be included in the differential diagnosis.

19.
Article in English | WPRIM | ID: wpr-759989

ABSTRACT

Vertebral artery injuries associated with C1 lateral mass screw insertion rarely occur during C1-2 fusion. The posterior inferior cerebellar artery (PICA) is uncommonly located at the C1 lateral mass insertion position. A 71-year-old woman with atlanto-axial subluxation and cord compression underwent C1-2 fusion. Sixth nerve palsy and diplopia were detected postoperatively, and decreased consciousness occurred on postoperative day 4. Brain magnetic resonance image (MRI) and computed tomography (CT) revealed PICA infarction. In the preoperative CT angiography, the PICA originated between the C1 and C2 level. In the postoperative CT scan, the PICA was not visible. The patient was treated conservatively for two weeks and recovered. PICA originating between the C1 and C2 level comprises 1.1–1.3% of cases. Therefore, vertebral artery anomalies should be evaluated prior to C1-2 fusion to prevent vessel injuries.


Subject(s)
Abducens Nerve Diseases , Aged , Angiography , Arteries , Brain , Brain Infarction , Consciousness , Diplopia , Female , Humans , Infarction , Pica , Tomography, X-Ray Computed , Vertebral Artery
20.
Article in English | WPRIM | ID: wpr-759988

ABSTRACT

Involuntary movement of the cervical spine can cause damage to the cervical spinal cord. Cervical myelopathy may occur at an early age in involuntary movement disorders, such as tics. We report the case of a 21-year-old man with Tourette syndrome, who developed progressive quadriparesis, which was more severe in the upper extremities. The patient had abnormal motor tics with hyperflexion and hyperextension of the cervical spine for more than 10 years. High-signal intensity intramedullary lesions were observed at C3-4-5-6 level on T2 weighted magnetic resonance imaging. Examinations were performed for high-signal intensity intramedullary lesions that may occur at a young age, but no other diseases were detected. Botulinum toxin injection to the neck musculature and medication for tic disorders were administered. However, the myelopathy was further aggravated, as the involuntary cervical movement still remained. Therefore, laminoplasty was performed at C3-4-5-6, with posterior fixation at C2-3-4-5-6-7 to alleviate the symptoms. The neurological signs and symptoms improved dramatically. The management of tic disorders should be the first priority during treatment. However, surgical treatment may be necessary, if symptoms worsen after appropriate treatment.


Subject(s)
Botulinum Toxins , Cervical Cord , Dyskinesias , Humans , Laminoplasty , Magnetic Resonance Imaging , Neck , Quadriplegia , Spinal Cord Diseases , Spine , Spondylosis , Tic Disorders , Tics , Tourette Syndrome , Upper Extremity , Young Adult
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