Moyamoya disease is a rare progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The etiology and pathogenesis are unknown. Clinically, this disease is found with a higher incidence in young female, but there are few cases of moyamoya disease in pregnancy. The disease primarily affects children, but it can also occur in adults. in children. The first symptom of moyamoya disease is often stroke, or recurrent transient ischemic attack, frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures. Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels. The diagnosis of moyamoya disease is made on the basis of angiographic findings and threatment of Moyamoya disease is surgery, or conservative management. We report a case of moyamoya disease that presented as intracerebral hemorrhage and intraventricular hemorrhage during pregnancy complicating severe preeclampsia.
Adult ; Arteries ; Basal Ganglia ; Brain ; Cerebral Hemorrhage ; Cerebrovascular Disorders ; Child ; Diagnosis ; Female ; Hemorrhage ; Humans ; Incidence ; Ischemic Attack, Transient ; Moyamoya Disease* ; Muscle Weakness ; Paralysis ; Pre-Eclampsia* ; Pregnancy ; Seizures ; Stroke

Moyamoya disease is a rare progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The etiology and pathogenesis are unknown. Clinically, this disease is found with a higher incidence in young female, but there are few cases of moyamoya disease in pregnancy. The disease primarily affects children, but it can also occur in adults. in children. The first symptom of moyamoya disease is often stroke, or recurrent transient ischemic attack, frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures. Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels. The diagnosis of moyamoya disease is made on the basis of angiographic findings and threatment of Moyamoya disease is surgery, or conservative management. We report a case of moyamoya disease that presented as intracerebral hemorrhage and intraventricular hemorrhage during pregnancy complicating severe preeclampsia.
Adult ; Arteries ; Basal Ganglia ; Brain ; Cerebral Hemorrhage ; Cerebrovascular Disorders ; Child ; Diagnosis ; Female ; Hemorrhage ; Humans ; Incidence ; Ischemic Attack, Transient ; Moyamoya Disease* ; Muscle Weakness ; Paralysis ; Pre-Eclampsia* ; Pregnancy ; Seizures ; Stroke

PURPOSE: To describe the associated conditions and clinical significance of lenticulostriate uasculopathy (LSV) as demonstrated by cranial sonography. MATERIALS AND METHODS: We retrospectively studied 77 LSV cases who between January 1994 and January 1996 had undergone cranial sonography for neonatal asphyxia, seizure, or bulging of anterior fontanel. Cranial sonography was performed with 7 MHz real-time linear and sector transducers using an Acuson computed sonography unit ; examinations were performed in sagittal and coronal planes. Twenty-six LSV patients underwent color Doppler studies, and in 24 of these, color signal and arterial pulse spectral wave were detected. LSV was grouped as one of three types, according to echogenicity : type I (less echogenic than sylvian fissure), type II (similar to sylvian fissure), and type III (more echogenic than sylvian fissures). We retrospectively evaluated associated sonographic abnormalities of the brain, and reviewed medical records for associated conditions and neurologic sequelae. Follow-up sonographic examinations were performed in 23 patients. RESULTS: There were 56 type I cases (73 %), 17 of type II (22 %) and 4 of type III (5 %). Cranial sonographic results were normal in 36 cases (47 %) and abnormal in 41(53 %). Forty-eight cases of nonspecific causes and 29 cases of perinatal and acquired causes accounted for associated conditions. The echogenicity of LSV had not changed in 21 of 23 follow-up cases. Neurologic examinations were performed in 18 cases and in 14 of these (78 %) there was no neurologic sequela. CONCLUSION: LSV in basal ganglia may be associated with nonspecific neonatal conditions, in addition to well known perinatal causes.
Asphyxia ; Basal Ganglia ; Basal Ganglia Cerebrovascular Disease* ; Brain* ; Cranial Fontanelles ; Follow-Up Studies ; Humans ; Medical Records ; Neurologic Examination ; Retrospective Studies ; Seizures ; Transducers ; Ultrasonography*

Moyamoya disease (MMD) is a cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries. MMD is also accompanied by typical net-like collateral vessels in the basal ganglia. Although the etiology of MMD remains unknown, hereditary, immunogenic, and hemodynamic factors have been implicated as underlying mechanisms of MMD. Here, the authors report on one patient with a rare combination of Graves' disease-associated thyrotoxicosis, myasthenia gravis, and MMD, where cerebrovascular ischemic events occurred during a thyrotoxic state.
Basal Ganglia ; Brain Ischemia ; Carotid Artery, Internal ; Cerebrovascular Disorders ; Constriction, Pathologic ; Graves Disease ; Hemodynamics ; Humans ; Moyamoya Disease ; Myasthenia Gravis ; Thyrotoxicosis

PURPOSE: To investigate clinical characteristics of neonates with lenticulostriate vasculopathy (LSV) and determine the correlation between LSV and clinical characteristics, especially congenital cytomegalovirus (CMV) infection. METHODS: We retrospectively reviewed the medical records of neonates with LSV, born at Cheil General Hospital between January 2005 and December 2015. LSV was graded into three groups based on the number of the LSV lesions and classified into an isolated and combined group showing LSV with coexistent abnormalities noted on brain sonography. We compared clinical data based on the LSV classification. RESULTS: Our study included 102 neonates with LSV, which showed an unilateral pattern in 10 and bilateral pattern in 92 neonates. The numbers of neonates studied based on LSV grading were 33, 53, and 16 in grade 1, 2, and 3, respectively. We observed the isolated LSV in 62 and the combined type in 40 neonates. We observed that 93 (91.2%) of the neonates with LSV did not show specific underlying cause for this condition. Congenital CMV infection was detected in 7 neonates, including 0, 5, and 2 neonates belonging to grade 1, 2, and 3, respectively. Among these, 2 neonates showed the isolated, and 5 showed the combined type of LSV. Statistically, congenital CMV infection was more significantly associated with LSV in grade 2 and 3 than in grade 1 (P < 0.05). Additionally, congenital CMV infection was more commonly observed in the combined than in the isolated LSV type showing a marginal association (P=0.07). CONCLUSION: We observed that LSV was not clinically significant except when associated with CMV infection. We suggest that neonates presenting with a grade 2 or higher of LSV or a combined type of LSV detected via neonatal brain ultrasonography should be evaluated for CMV infection.
Basal Ganglia Cerebrovascular Disease* ; Brain ; Classification ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Hospitals, General ; Humans ; Infant, Newborn ; Medical Records ; Retrospective Studies ; Ultrasonography

Behcet's disease (BD) is characterized by recurrent oro-genital ulcers, skin lesions, and intraocular inflammation, but can also affect various internal organs. Vascular BD usually presents with luminal stenosis, thrombosis, or aneurysm formation in aorta and peripheral arteries. However, intracranial artery involvement has been uncommonly reported in patients with BD and BD cases with lenticulostriate artery aneurysm have been rarely described in the English-language literature. We hereby reported the first case of a Korean BD patient presenting with a ruptured lenticulostriate artery aneurysm, who received medical treatment, and reviewed the literature on reported cases of BD with intracranial aneurysms.
Aneurysm* ; Aorta ; Arteries* ; Basal Ganglia Cerebrovascular Disease ; Constriction, Pathologic ; Humans ; Inflammation ; Intracranial Aneurysm ; Phenobarbital ; Rupture* ; Skin Ulcer ; Thrombosis

The Clinical analysis of 130 spontaneous intracebral & cerebellar hematoma confirmed by brain CT & admitted to this neurosurgical clinic past years & 5 months were made with literature review. 1) The age incidence showed increase over fourth decade and male to female ratio was 56.1:43.9. 2) 50.4% of total patients showed hypertension but 40.3% did not checked their B.P. & 9.3% had normal B.P. before cerebral hemorrhage. Among 50.4% of hypertension cases, 72% received antihypertensive treatment intermittently, 2.9% regularly and the rest 24.3% did not. 3) 69.7% of hematoma located in basal ganglia & thalamus. In nonoperated patients, the morbidity & mortality showed no marked difference in their location except pontine & cerebellar hematomas. In operated patients the morbidity & mortality was prominent in putaminal hemorrhage than in thalamus and cerebellum. Most of putaminal hematoma had semicomatose or comatose mental state and ventricular hemorrhage in 87.5%. 4) The morbidity & mortality increased in proportion to size of hematomas, grade of unconsciousness in admisson and state of intraventricular hemorrhage. 5) About time interval from ictus to operation, the patients within first 24 hours are 29 cases(51.1%) and the next 24 hours are 13 cases(26.5%). The mortality rate of two groups were 55.1% & 53.8% respectively but no death in the patients with operation performed after 48 hours. 6) The morbidity & mortality rate were as follows. In the non operated patients (90 patients), no or mild neurological deficits:22.2% moderate:21.1% severe:20% and moribund or death:36.6%. In the operated patients no or mild neurological deficits:16.3% moderate:18.4% severe:18.4% and moribund or death:46.9%. The results of total patients showed no or mild neurological deficits:20.1%, moderate:20.1%, severe:19.4% and moribund or death:40.2%.
Basal Ganglia ; Brain ; Cerebellum ; Cerebral Hemorrhage ; Coma ; Female ; Hematoma* ; Hemorrhage ; Humans ; Hypertension ; Incidence ; Male ; Mortality ; Putaminal Hemorrhage ; Thalamus ; Unconsciousness

The author analyzed 169 cases of spontaneous intracerebral hemorrhages at the basal ganglia and thalamus, who had been admitted to Jeonju Presbyterian Medical Center from 1975 to 1979. Intracerebral hematoma was confirmed by angiography and the amount of hematoma was divided as small, medium or large according to the angiographic evidence. Among the 169 cases, 145 cases underwent appropriate medical or surgical treatment. 63 cases were treated conservatively and 82 cases were operated ; 22 cases of frontal approach, 51 cases of temporal approach, and 9 cases of extraventricula diainage of clot. Results obtained are as follows : 1. The common pridiection age group was from the fifth to the seventh decades, which was 90.5% of all cases. The ratio of male to female was about 2 to 1. 2. putaminal hemorrhage was 65.1%, and thalamic hemorrhage was 16.6%. 3. Angiographic evidence of arteriosclerosis was seen in 86.4%. 4. The worse prognostic factors were related to age(over 65), site and size of hematoma, and mental state on admission. 5. With conservative management 49.2% were improved, 6.3% not improved, 44.4% moribund or dead. 6. With surgical treatment 58.5% were improved, 3.7% not improved, 37.8% moribund or dead. 7. Microsurgical temporal approach proved to have the following advantages over frontal approach. (1) Better outcome was found in this approach(64.7% vs 54.4%). (2) The distance to the hematoma was closer in temporal approach, and so total removal of hematoma and complete control of bleeding sources with less surrounding structural damages were possible. 8. Early operation seems to be more effective than delayed operation in the cases of large hematoma with deteriorating neurological signs.
Angiography ; Arteriosclerosis ; Basal Ganglia* ; Cerebral Hemorrhage* ; Female ; Hematoma ; Hemorrhage ; Humans ; Jeollabuk-do ; Male ; Protestantism ; Putamen ; Putaminal Hemorrhage ; Thalamus*

The most common etiology of spontaneous, non-traumatic intracerebral hemorrhage is hypertension. One of the most decisive factors for the determination of etiology is location of the hemorrhage. Here, we report on a 53-year-old female patient who was admitted with left putaminal hemorrhage. She did not have prominent vascular risk factors other than fundoscopic signs of hypertensive retinopathy. Magnetic resonance imaging and angiography revealed arteriovenous malformation (AVM) in the basal ganglia, which was fed by the lateral lenticulostriate artery. This case suggests that we should consider an AVM-related hemorrhage in relatively young stroke patients, regardless of hematoma location or presence of accompanying hypertension.
Angiography ; Arteries ; Arteriovenous Malformations ; Basal Ganglia ; Cerebral Hemorrhage ; Female ; Hematoma ; Hemorrhage ; Humans ; Hypertension ; Hypertensive Retinopathy ; Magnetic Resonance Imaging ; Middle Aged ; Putaminal Hemorrhage ; Risk Factors ; Stroke

Hypertensive intracerebral hematoma is a serious and potentially lethal condition. The indication of surgery in this hematoma is still controversial. Authors have treated 335 cases of hypertensive intracerebral hematomas which were confirmed by computerized tomography between July 1, 1982 and June 30, 1986. The hematomas have been classified according to their modes of extension on computerized tomography. The outcome was assessed on their basis of activity daily living. According to our study, moderate and severe types of putaminal hematoma cases have shown better results with surgery than conservative management. Mortality was 23.7 percent in surgery and 35.3 percent in conservative management.
Hematoma* ; Mortality ; Putaminal Hemorrhage