Terson syndrome is defined as intraocular hemorrhage associated with intracranial bleeding. This syndrome can occur in the event of intracranial hemorrhage or elevated intracranial pressure. To our knowledge, it has never been associated with chronic myeloid leukemia. A 45-year-old woman suffering from chronic myeloid leukemia was referred to our clinic with Terson syndrome after intracranial bleeding. We followed this patient for a year, performing visual acuity assessment, fundus examination, color retinography, and A-scan and B-scan ultrasonography. At presentation, her best-corrected visual acuity on the right was 20/63 and on the left was 20/320. In the right eye, retinoscopy showed blurring of the optic margins surrounded by retinal and preretinal hemorrhages, preretinal fibrosis of the optic disc along the vascular arcades, and perivascular retinal infiltrates. In the left eye, the optic disc was surrounded by retinal and preretinal hemorrhages, and massive fibrosis with hard exudates and severe preretinal hemorrhage were observed at the posterior pole. Roth spots and many circular hemorrhages were noted at the periphery of the retina. A-scan and B-scan ultrasonography did not show intraocular leukemic infiltration. The clinical picture remained stable over the following 12 months. In this patient, we observed the ophthalmoscopic features of chronic myeloid leukemia, but also coexistence of features typical of Terson syndrome. To our knowledge, no similar cases have been reported previously.
retinal disease; chronic myeloid leukemia; eye hemorrhage; intracranial hemorrhage
Depressive symptoms are common in neurological diseases, at times posing dilemma in organic or functional origin. Cerebrovascular disease may predispose, precipitate, or perpetuate some geriatric depressive syndromes that resemble primary depressions both clinically and therapeutically in about half of the patients following acute stroke. Terson's syndrome is the direct occurrence of vitreous hemorrhage following subarachnoid/subdural hemorrhage, often overlooked in the acute setting. Autosomal dominant (adult) polycystic kidney disease may be associated with berry aneurysms and hypertension, and may lead to intracranial bleeds. We report an unusual case of organic depression and Terson's syndrome in a 50-year-old female with polycystic kidney disease and hypertension, following anterior communicating artery aneurysmal subarachnoid bleed with bilateral subdural extension. Management included anti-hypertensives, antiepileptics, neodymium: YAG laser photocoagulation, and aneurysmal clipping.
Depression; intracranial aneurysm; polycystic kidney disease; subarachnoid hemorrhage; syndrome; vitreous hemorrhage
The present study reports the case of a 33-year-old male who presented with Terson syndrome with no cerebral hemorrhage secondary to traumatic brain injury (TBI). A computed tomography scan of the patient, who had sustained an impact injury to the right occipital region, showed no cerebral lesion. Ophthalmoscopy clearly demonstrated vitreous hemorrhage in both eye globes. Vitreous hemorrhage, which results from an abrupt increase in intracranial pressure (ICP), is associated with TBI. In this case, the visual disturbance was attributed to Terson syndrome secondary to TBI. Therefore, close ophthalmological and radiological evaluation is required in patients with TBI, in order to enable the diagnosis of Terson syndrome and an early vitrectomy.
cerebral hemorrhage; intracranial pressure; traumatic brain injury; Terson syndrome; vitreous hemorrhage
Intraocular hemorrhage in patients suffering from aneurysmal subarachnoid hemorrhage is known as Terson's syndrome and is an underestimated but common pathology. We therefore designed a prospective single-blinded study to evaluate the validity of ocular ultrasound compared to the gold standard indirect funduscopy in the diagnosis of Terson's syndrome.
Material and Methods
Fifty-two patients (104 eyes in total) suffering from aneurysmal subarachnoid hemorrhage were enrolled in this study. Two investigators independently performed a single-blinded ocular ultrasound using a standard intensive care ultrasound system to detect an intraocular hemorrhage. Indirect funduscopy following iatrogenic mydriasis served as the gold standard for confirmation or exclusion of an intraocular hemorrhage. Statistical analyses were performed to evaluate the sensitivity and specificity, positive and negative predictive values of the method as well as the learning curve of ocular ultrasound.
Indirect funduscopy detected Terson's syndrome in 11 of 52 (21.2%) respectively in 21 of 104 (20.2%) eyes in patients suffering from subarachnoid hemorrhage. Sensitivity and specificity increased with the number of ocular ultrasound examinations for both investigators, reaching 81.8% and 100% respectively. Positive and negative predictive values were different for both investigators (63.6% vs. 100% positive and 100% vs. 95.7% negative) but were both correlated to the amount of intraocular hemorrhage. A low Glasgow Coma scale (p = 0.015) and high Hunt & Hess grade (p = 0.003) was associated with a higher rate of Terson's syndrome.
Ocular ultrasound using standard ultrasound equipment has been confirmed as a reliable, easy-to-handle bedside screening tool for detecting Terson's syndrome. Nevertheless funduscopy remains the gold standard to detect Terson's syndrome.
This report describes a middle-aged man presenting to the ophthalmologist with history of seeing floaters before both eyes since 2-weeks duration. A history of intermittent headache and dizziness of recent onset was elicited on questioning. Ocular examination showed bilateral early papilloedema and mild vitreous hemorrhage. Brain computed tomography (CT) disclosed features suggestive of colloid cyst of the third ventricle in the region of foramen of Monro with moderate hydrocephalus. Emergency craniotomy and excision of the cyst was done, and the patient is doing well for the last 18 months after the surgical intervention. The mechanism of this presentation, importance of early investigations, and timely intervention are highlighted in order to avoid serious neurological sequelae. The literature was extensively reviewed for atypical presentations of intraventricular colloid cyst.
Colloid Cyst; Obstructive Hydrocephalus; Third Ventricle; Vitreous Haemorrhage
Intraventricular hemorrhage during pregnancy is usually followed by a poor recovery. When caused by moyamoya disease, ischemic or hemorrhagic episodes may complicate the management of high intracranial pressure. A 26-year-old Caucasian woman presented with generalized seizures and a Glasgow Coma Score (GCS) of 3 during the 36th week of pregnancy. The fetus was delivered by caesarean section. The brain CT in the mother revealed bilateral intraventricular hemorrhage, a callosal hematoma, hydrocephalus and right frontal ischemia. Refractory high intracranial pressure developed and required bilateral ventricular drainage and intensive care treatment with barbiturates and hypothermia. Magnetic resonance imaging and cerebral angiography revealed a moyamoya syndrome with rupture of the abnormal collateral vascular network as the cause of the hemorrhage. Intracranial pressure could only be controlled after the surgical removal of the clots after a large opening of the right ventricle. Despite an initially low GCS, this patient made a good functional recovery at one year follow-up. Management of refractory high intracranial pressure following moyamoya related intraventricular bleeding should require optimal removal of ventricular clots and appropriate control of cerebral hemodynamics to avoid ischemic or hemorrhagic complications.
Pregnancy; Moyamoya syndrome; Intraventricular hemorrhage; Ventriculostomy; Intensive care management
Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India.
To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India.
Material and Methods:
Relevant investigations were done to rule out other causes of moyamoya syndrome.
We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs.
Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.
Cerebral infarction; moyamoya; transient ischemic attack
Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.
Cerebral infarction; intraventricular hemorrhage; Moyamoya disease; pediatric
Moyamoya disease is a rare cerebrovascular disease characterized by stenosis or occlusion of the arteries around the circle of Willis with arterial collateral circulation. Patients with Moyamoya have significant risk for brain hemorrhage. Recognition of this disease in the patient with recurrent strokes requires a high degree of clinical suspicion.
A 57-year-old man with a past history of diabetes, hyperlipidemia and hypertension presented to the emergency department with right-sided weakness and aphasia. A head CT on admission without contrast showed a small left frontal gyrus stroke with no acute hemorrhage and he was managed medically. Laboratory testing on admission was remarkable for elevated hemoglobin A1c and LDL cholesterol. The patient's neurologic symptoms improved initially, however on Day 3 of hospitalization, the patient developed worsening of his symptoms. Physical examination was notable for a right-sided facial droop, hemiparesis in his right upper extremity, decreased strength in lower right extremity and mixed aphasia. Repeat head CT without contrast revealed a large left middle cerebral artery infarction and CTA of the neck showed multiple intracranial stenoses, specifically occlusion of the left M1 artery and moderate stenosis of the right M1 artery. Multiple collateral vessels reconstituting M2 segments were also noted, which were suspicious for Moyamoya disease. His proximal right upper extremity weakness improved mildly, but on Day 4 the patient again developed worsening right-sided weakness and a repeat head CT showed an evolving left middle cerebral artery infarction. Other stroke workup including transthoracic echocardiogram and carotid duplex ultrasound were unremarkable. He was medically managed with aspirin, atorvastatin, lisinopril, and insulin. He continued to receive speech, occupational, and physical therapy throughout his hospital stay and was transferred to an inpatient rehabilitation facility for further care after stabilization of his neurologic symptoms.
This case illustrates the clinical presentation of recurrent strokes, which is common in the context of Moyamoya disease. The diagnosis of Moyamoya disease is made with MRA or CTA which shows significant stenosis or occlusion of the cerebral arteries with a prominent collateral circulation. It is important to consider this disease in a patient with recurrent strokes because of the progressive nature of the disease and increased risk for cerebral hemorrhage.
Moyamoya syndrome, a vasculopathy characterized by chronic progressive stenosis at the apices of the intracranial internal carotid arteries, is an increasingly recognized entity which is associated with cerebral ischemia. Diagnosis is made on the basis of clinical and radiographic findings, including a characteristic stenosis of the internal carotid arteries in conjunction with abundant collateral vessel development. Adult moyamoya patients often present with hemorrhage, leading to rapid diagnosis. In contrast, children usually present with transient ischemic attacks or strokes, which may prove more difficult to diagnose because of patient's inadequate verbal and other skills, leading to delayed recognition of the underlying moyamoya. The progression of disease can be slow, with rare, intermittent events, or it can be fulminant, with rapid neurologic decline. However, regardless of the course, it is apparent that moyamoya syndrome, both in terms of arteriopathy and clinical symptoms, inevitably progresses in untreated patients.
Surgery is generally recommended for the treatment of patients with recurrent or progressive cerebral ischemic events and associated reduced cerebral perfusion reserve. Many different operative techniques have been described, all with the main goal of preventing further ischemic injury by increasing collateral blood flow to hypoperfused areas of the cortex, using the external carotid circulation as a donor supply. This article discusses the various treatment approaches, with an emphasis on the use of pial synangiosis, a method of indirect revascularization. The use of pial synangiosis is a safe, effective, and durable method of cerebral revascularization in moyamoya syndrome and should be considered as a primary treatment for moyamoya, especially in the pediatric population.
Moyamoya; pial synangiosis; ischemia; stroke
Prevention of rebleeding plays an important role in the treatment of hemorrhagic moyamoya disease, because rebleeding results in high mortality and morbidity. We discuss possible treatment for patients with moyamoya disease accompanied with distal choroidal artery aneurysms and review the literature to summarize clinical treatment and mechanisms. The cases of three male patients who suffered from intraventricular hemorrhage are presented. Computed tomography (CT) and digital subtractive angiography (DSA) revealed that bleeding was believed to be caused by ruptured aneurysms originating from distal choroidal artery aneurysms. Two patients successfully underwent superficial temporal artery (STA)-middle cerebral artery (MCA) bypass combined with encephalo-duro-myo-synangiosis (EDMS) and the obliteration of the aneurysm. The follow-up DSA or CT scan demonstrated that the aneurysms completely disappeared with the patency of the reconstructed artery. Neither of the patients experienced rebleeding during the follow-up period (up to 34 months). Given conservative treatment, the third patient experienced recurrent hemorrhages 4 months after the first ictus. This study describes treatment for moyamoya disease accompanied with distal choroidal artery aneurysms. Our experience suggests that cerebral revascularization combined with obliteration of the complicated distal aneurysm in the same session is a possible treatment.
Aneurysm; Intracranial hemorrhage; Revascularization; Moyamoya disease
Methods: Papers relating to vitreous haemorrhage in patients with subarachnoid haemorrhage were retrieved. The only studies considered were those with at least 10 consecutive cases of subarachnoid haemorrhage with or without vitreous haemorrhage. The frequency of vitreous haemorrhage in such cases was calculated in prospective and retrospective studies. Mortality was compared in patients with and without Terson's syndrome.
Results: 154 papers were reviewed. Three prospective studies and six retrospective studies satisfied the inclusion criteria. Of 181 patients with subarachnoid haemorrhage assessed prospectively (mean age, 51.7 years), 24 (13%) had vitreous haemorrhage; among 1086 retrospective records, 37 (3%) had documented vitreous haemorrhage (p<0.001). Patients with Terson's syndrome had higher Hunt and Hess grades than those without (mean grade, 3.6 v 2.6). Patients with Terson's syndrome were also more likely to die (13 of 30 (43%) v 31 of 342 (9%); odds ratio 4.8; p<0.001).
Conclusions: Prospective studies show a higher frequency of Terson's syndrome than retrospective studies, suggesting that vitreous haemorrhage is not well documented. Vitreous haemorrhage is an adverse prognostic finding in patients with subarachnoid haemorrhage.
Background and Purpose: Moyamoya disease has been classically described by the Asian experience, yet clinical aspects of moyamoya phenomena in the United States remain vastly undefined. The multifocal occlusive arterial disorder may be linked with numerous conditions; however, later stages of this syndrome share common vascular pathophysiology. This study is aimed at characterizing inpatient moyamoya cases in the United States over a broad time span. Methods: A comprehensive analysis of the Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project (Releases 1–13, 1988–2004) based on ICD-9-CM code 437.5 was performed. Annual percentages and trends analyses were conducted for demographic variables, admission characteristics, co-morbidities, and procedures. Result: 2247 admissions of moyamoya cases were analyzed from a wide geographic distribution throughout the United States between 1988 and 2004. Age at admission varied considerably (mean 29.6 ± 18.5 years), affecting women more frequently than men (61.9%). Various racial groups were identified (35.4% White, 19.7% African American, 5.6% Hispanic, 8.3% Asian or Pacific Islander, 1.4% Native American). Admissions were typically emergent (38.8%) or urgent (18.7%), although elective admissions occurred (24.4%). Aside from moyamoya, sickle cell disease was diagnosed in 13.6%, ischemic stroke in 20.7%, intracerebral hemorrhage in 7.4%, transient ischemic attack in 3.4%, and subarachnoid hemorrhage in 3.1%. Cerebral angiography was performed in 24.9% while extracranial–intracranial bypass was done in 8.4% of patients. Conclusion: Moyamoya in the United States is a heterogeneous condition affecting individuals of all ages across a diverse racial spectrum and wide geographic distribution. Further recognition of moyamoya syndrome may facilitate ongoing research and future therapeutic approaches.
moyamoya; collateral; stroke; cerebral ischemia
We report a case of Terson syndrome, providing the first intrasurgical imaging of subinternal limiting membrane blood diffusion in Terson syndrome. We highlight some remarkable in vivo anatomical findings that may give a contribution to the debate about its pathogenesis. Here we hypothesize that the subretinal space might be unlikely to be a primary source of intraocular hemorrhage, and we support the two generally accepted theories about blood diffusion from the retinal vasculature or from the perivascular spaces.
Moyamoya disease is characterized by a progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is still unknown, recent genome-wide and locus-specific association studies identified RNF213 as an important susceptibility gene of moyamoya disease among East Asian population. A polymorphism in c.14576G>A in RNF213 was identified in 95% of familial patients with moyamoya disease and 79% of sporadic cases, and patients having this polymorphism were found to have significantly earlier disease onset and a more severe form of moyamoya disease, such as the presentation of cerebral infarction and posterior cerebral artery stenosis. The exact mechanism by which the RNF213 abnormality relates to moyamoya disease remains unknown, while recent reports using genetically engineered mice lacking RNF213 by homologous recombination provide new insight for the pathogenesis of this rare entity. Regarding biomarkers of moyamoya disease, moyamoya disease is characterized by an increased expression of angiogenic factors and pro-inflammatory molecules such as vascular endothelial growth factors and matrix metalloproteinase-9, which may partly explain its clinical manifestations of the pathologic angiogenesis, spontaneous hemorrhage, and higher incidence of cerebral hyperperfusion after revascularization surgery. More recently, blockade of these pro-inflammatory molecules during perioperative period is attempted to reduce the potential risk of surgical complication including cerebral hyperperfusion syndrome. In this review article, we focus on the genetics and biomarkers of moyamoya disease, and sought to discuss their clinical implication.
Moyamoya disease; Genetics; Biomarkers; RNF213; Susceptibility gene
PURPOSE: To examine the ophthalmologic experience with the shaken baby syndrome (SBS) at one medical center, including clinical findings, autopsy findings, and the visual outcome of survivors. METHODS: One hundred sixteen patients admitted from 1987 to 1998 for subdural hematomas of the brain secondary to abuse were included. RESULTS: Retinal hemorrhages were detected in 84% of the children, but this important finding had been missed often by nonophthalmologists. Poor visual response, poor pupillary response, and retinal hemorrhage correlated strongly with demise of the child. One child who died had pigmented retinal scars from previous abuse, a condition not previously observed histopathologically. The clinical and autopsy findings varied somewhat, probably because of the differing conditions for examination. No correlation could be made between computerized tomography scans done during life and the subdural hemorrhage of the optic nerve found on autopsy. Half of the surviving patients were known to have good vision. One fourth of the patients had poor vision, largely due to cerebral visual impairment from bilateral injury posterior to the optic chiasm. Severe neurologic impairment correlated highly with loss of vision. CONCLUSION: This series provides information on the frequency of eye findings in SBS patients. No fundus finding is pathognomonic for SBS. When retinal hemorrhages are found in young children, the likelihood that abuse occurred is very high. The difficulty that nonophthalmologists have in detecting retinal hemorrhage may be an important limiting factor in finding these children so they may be protected from further abuse.
To investigate the reasons for postvitrectomy diabetic vitreous hemorrhage (PDVH), and to analyze the time of PDVH onset, the treatment of PDVH, the visual outcome of the treatment, and factors that affect visual acuity after treatment.
Materials and Methods:
Overall, 292 eyes from 236 patients with proliferative diabetic retinopathy (PDR) underwent vitrectomy from 2006 to 2010. Fifty eyes out of 43 patients had severe postoperative vitreous hemorrhage. The average follow-up duration was 6.8 ± 3.8 months (range, 2–12 months).
Recurrent vitreous hemorrhage (VH) after primary vitrectomy occurred in 40 eyes (80%) with an average time of VH onset of 62.5 ± 32.8 days (range, 3–170 days). VH occurred after silicone oil removal occurred in 10 eyes (20%), with an average time of VH onset of 27.4 ± 20.3 days (range, 1–60 days). The reasons for PDVH included chronic errhysis from retinal neovessels (47.1% of the eyes), residual fibrous vascular membrane (12.8% of the eyes), fibrovascular ingrowth at sclerotomy sites (4.3% of the eyes), iris neovessels and neovascular glaucoma (4.3% of the eyes), retinal vein occlusion (2.8% of the eyes), retinal tears (8.1% of the eyes), retinotomy (1.4% of the eyes), epichoroidal bleeding (1.4% of the eyes), polycythemia rubra vera (1.4% of the eyes), hypoperfusional retinopathy (4.3% of the eyes), and unknown reasons (12.8% of the eyes). Visual acuity increased in 43 eyes (86%) after surgical or nonsurgical treatment. The improvement in visual acuity after treatment was not affected by age, sex, duration of diabetes, time of PDVH onset, frequency of surgery, or treatment methods.
Postvitrectomy diabetic vitreous hemorrhage commonly occurs two months after vitrectomy. Residual epiretinal neovascularization is the most common cause of PDVH. Active surgical or nonsurgical treatment for severe vitreous hemorrhage can obviously improve the patients’ visual prognosis.
Hemorrhage; proliferative diabetic retinopathy; vitrectomy
TNF-α is an inflammatory cytokine that plays a central role in promoting the cascade of events leading to an inflammatory response. Recent studies have suggested that TNF-α may play a key role in the formation and rupture of cerebral aneurysms, and that the underlying cerebral inflammatory response is a major determinate of outcome following subrarachnoid hemorrhage (SAH).
We studied 14 comatose SAH patients who underwent multimodality neuromonitoring with intracranial pressure (ICP) and cerebral microdialysis as part of their clinical care. Continuous physiological variables were time-locked every 8 hours and recorded at the same point that brain interstitial fluid TNF-α was measured in brain microdialysis samples. Significant associations were determined using generalized estimation equations.
Each patient had a mean of 9 brain tissue TNF-α measurements obtained over an average of 72 hours of monitoring. TNF-α levels rose progressively over time. Predictors of elevated brain interstitial TNF-α included higher brain interstitial fluid glucose levels (β=0.066, P<0.02), intraventricular hemorrhage (β=0.085, P<0.021), and aneurysm size >6 mm (β=0.14, p<0.001). There was no relationship between TNF-α levels and the burden of cisternal SAH; concurrent measurements of serum glucose, or lactate-pyruvate ratio.
Brain interstitial TNF-α levels are elevated after SAH, and are associated with large aneurysm size, the burden of intraventricular blood, and elevation brain interstitial glucose levels.
tumor necrosis factor-α; intraventricular hemorrhage; cerebral inflammatory response; cerebral microdialysis; brain interstitial fluid
The presentation of moyamoya disease (MMD) as an aneurysmal subarachnoid hemorrhage (SAH) is relatively frequent and in the absence of aneurysms is extremely rare.
A 53-year-old male patient suddenly developed severe headache associated with dysarthria and an altered state of consciousness. At the time of admission, he was found drowsy with global aphasia, stiff neck, right hemiparesis and right Babinski's sign. A non-contrast brain computed tomography was performed and a small bleeding in the subarachnoid space over the left frontal and parietal cortex was observed. Four-vessel cerebral angiography showed bilateral stenosis of the internal carotid arteries, with multiple tortuous vessels branching from the anterior and middle cerebral arteries. These abnormal vessels were anastomosing with branches from the posterior cerebral and middle meningeal arteries. With this information, a diagnosis of MMD was made. A three-dimensional reconstruction from digital angiography ruled out aneurysms or vascular malformations. After 4 weeks, another angiography was performed and remained the same as previous one.
Clinical and radiological characteristics of this case are consistent with previous reports, supporting the theory that non-aneurysmal SAH in MMD is caused by rupture of fragile moyamoya vessels.
Moyamoya; subarachnoid hemorrhage; vascular anastomosis
The brain MR images of 23 patients with angiographically proved moyamoya disease were reviewed to evaluate the capability of MR to demonstrate vascular and parenchymal abnormalities. All the MR images were obtained on a 2.0 T superconducting system and included T1-weighted sagittal and T2-weighted axial images without implementation of flow compensation (FC). The vascular abnormalities demonstrated on MR images were narrowing of the cavernous internal carotid artery (ICA) (73%), narrowing or occlusion of the supraclinoid ICA (87%) and proximal middle cerebral artery (MCA) (91%), and multiple collateral vessels in the basal ganglia and/or thalamus (96%). The parenchymal abnormalities included ischemic infarctions (74%), predominantly located in watershed areas, hemorrhagic infarctions (26%), intracerebral hematomas (13%), and intraventricular hemorrhage (13%). In conclusion, MR imaging was a useful diagnostic modality for detecting both vascular and parenchymal abnormalities associated with moyamoya disease. This may obviate the need for invasive angiography as far as the diagnosis is wanted at the non-quantitative level.
Whether unilateral moyamoya disease (MMD), confirmed by steno-occlusive lesion at the terminal portion of internal carotid artery with formation of moyamoya vessels unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) MMD remains controversial. It is well-known that adult patients with MMD tend to suffer from cerebral hemorrhage, occasionally due to the rupture of aneurysm arising from moyamoya vessel.
A 61-year-old woman was diagnosed as unilateral MMD incidentally and followed by magnetic resonance imaging annually. Seven years after the diagnosis, cerebral aneurysm appeared on the moyamoya vessel. Before further examination, the aneurysm ruptured and resulted in massive cerebral hemorrhage.
Even in the unilateral MMD, cerebral hemorrhage may occur due to the rupture of cerebral aneurysm. Careful follow-up is recommended and early treatment is required once cerebral aneurysm is detected.
Cerebral aneurysm; Intracerebral hemorrhage; moyamoya disease; Unilateral
Terson’s syndrome has had multiple definitions. The original definition is unlikely to have included Terson’s original case. An updated definition based on mechanism is proposed and related to the original case described by Terson.
Terson’s syndrome; Vitreous hemorrhage; Subarachnoid hemorrhage
We report a rare case of Williams syndrome accompanying moyamoya disease in whom postoperative global cerebral infarction occurred unpredictably. Williams syndrome is an uncommon hereditary disorder associated with the connective tissue abnormalities and cardiovascular disease. To our knowledge, our case report is the second case of Williams syndrome accompanying moyamoya disease. A 9-year-old boy was presented with right hemiparesis after second operation for coarctation of aorta. He was diagnosed as having Williams syndrome at the age of 1 year. Brain MRI showed left cerebral cortical infarction, and angiography showed severe stenosis of bilateral internal carotid arteries and moyamoya vessels. To reduce the risk of furthermore cerebral infarction, we performed indirect anastomosis successfully. Postoperatively, the patient recovered well, but at postoperative third day, without any unusual predictive abnormal findings the patient's pupils were suddenly dilated. Brain CT showed the global cerebral infarction. Despite of vigorous treatment, the patient was not recovered and fell in brain death one week later. We suggest that in this kind of labile patient with Williams syndrome accompanying moyamoya disease, postoperative sedation should be done with more thorough strict patient monitoring than usual moyamoya patients. Also, we should decide the revascularization surgery more cautiously than usual moyamoya disease. The possibility of unpredictable postoperative ischemic complication should be kept in mind.
Moyamoya disease; Williams syndrome; Postoperative infarction; Ischemic complication
Moyamoya syndrome associated with protein S deficiency is rarely encountered and is usually reported in paediatric cases with cerebral ischaemia.
A 32-year-old woman had symptoms of sudden-onset severe headache, projectile vomiting, impaired consciousness, and slight neck stiffness. The computed tomography scan of her brain showed primary intraventricular haemorrhage, and the subsequent four vessel cerebral angiographies revealed stage 3 to 4 Moyamoya disease according to Suzuki and Takaku’s angiographic classification. The coagulation profile showed the presence of protein S deficiency. The patient was treated with external ventricular drainage and conservative management until blood clot resolution. The patient was discharged with normal neurological examination findings after her initial impaired consciousness and orientation defect gradually recovered.
This case report would alert physicians to the possible coexistence of Moyamoya syndrome and protein S deficiency, even in adult cases presenting with primary intraventricular haemorrhage.
Intraventricular haemorrhage; Moyamoya syndrome; protein S deficiency
Patients with angioid streaks are prone to developing subretinal hemorrhage after ocular or head injury due to the brittleness of Bruch’s membrane. However, there have been no reports of any angioid streak patients in whom choroidal neovascularization occurred after vitrectomy surgery. We report herein a patient with angioid streaks who developed choroidal neovascularization after vitrectomy surgery for epiretinal membrane.
A 76-year-old man presented with distorted vision in his left eye, with a best corrected visual acuity of 1.2 and 0.6 in his right and left eyes, respectively. Fundus examination showed angioid streaks in both eyes and epiretinal membrane only in the left eye. The patient underwent 23-gauge three-port pars plana vitrectomy with removal of the epiretinal membrane combined with cataract surgery. Internal limiting membrane in addition to the epiretinal membrane were successfully peeled and removed, with indocyanine green dye used to visualize the internal limiting membrane. His left best corrected visual acuity improved to 0.8. An elevated lesion with retinal hemorrhage due to probable choroidal neovascularization was found between the fovea and the optic disc in the left eye at 7 weeks after surgery. Since best corrected visual acuity decreased to 0.15 and the hemorrhage expanded, posterior sub-Tenon injection of triamcinolone acetonide was performed. However, no improvement was observed. Even though intravitreal bevacizumab injection was performed a total of five times, his best corrected visual acuity remained at 0.1. Subsequently, we performed a combination treatment of a standard-fluence photodynamic therapy and intravitreal ranibizumab injection, with additional intravitreal ranibizumab injections performed 3 times after this combination treatment. Best corrected visual acuity improved to 0.5 and the size of the choroidal neovascularization markedly regressed at 4 months after the combined treatment.
Development of choroidal neovascularization could possibly occur in elderly patients with angioid streaks after vitrectomy surgery. In such cases, a combination of photodynamic therapy and intravitreal ranibizumab injection may be considered for initial treatment of the choroidal neovascularization.
Angioid streaks; Choroidal neovascularization; Vitrectomy surgery; Anti-vascular endothelial growth factor treatment; Photodynamic therapy; Pseudoxanthoma elasticum