Integrative Rehabilitation for Hypertrophic Olivary Degeneration after Operation of Brain Stem Cavernous Malformation: A Case Report / 中国康复理论与实践

Objective To summarize the integrative rehabilitation treatment of traditional Chinese medicine (TCM) and Western medicine in hypertrophic olivary degeneration (HOD) secondary to the operation of brain stem cavernous malformation. Methods The clinical data of medication, rehabilitation and follow-up of a patient with HOD secondary to operation on brain stem cavernous malformation was retrospectively analyzed. Results Three months after operation, limb static and motor tremor, dysarthria, palate spasm, eye movement disorder and walking difficulty appeared. The patient was diagnosed as HOD according to clinical features and brain magnetic resonance imaging (MRI). He was treated with pertinence rehabilitation training combined with TCM including acupuncture and herbs. After integrative rehabilitation, he could stand and walk independently, the tremor was alleviated, the balance function improved, the activities of daily living improved, and the dosage of oral western medicine also decreased. Conclusion After intracranial surgery, secondary neurodegeneration and movement disorder may appear, and it could be improved by integrative rehabilitation treatment of TCM and Western medicine

Integrative Rehabilitation for Hypertrophic Olivary Degeneration after Operation of Brain Stem Cavernous Malformation: A Case Report / 中国康复理论与实践

Objective To summarize the integrative rehabilitation treatment of traditional Chinese medicine (TCM) and Western medicine in hypertrophic olivary degeneration (HOD) secondary to the operation of brain stem cavernous malformation. Methods The clinical data of medication, rehabilitation and follow-up of a patient with HOD secondary to operation on brain stem cavernous malformation was retrospectively analyzed. Results Three months after operation, limb static and motor tremor, dysarthria, palate spasm, eye movement disorder and walking difficulty appeared. The patient was diagnosed as HOD according to clinical features and brain magnetic resonance imaging (MRI). He was treated with pertinence rehabilitation training combined with TCM including acupuncture and herbs. After integrative rehabilitation, he could stand and walk independently, the tremor was alleviated, the balance function improved, the activities of daily living improved, and the dosage of oral western medicine also decreased. Conclusion After intracranial surgery, secondary neurodegeneration and movement disorder may appear, and it could be improved by integrative rehabilitation treatment of TCM and Western medicine

Novel technologies and models for studying pharmacological activities of traditional Chinese medicine for promoting blood circulation and removing blood stasis / 中国药理学与毒理学杂志

OBJECTIVE Our previous studies demonstrated that various ingredients from the traditional Chinese medicine (TCM) for promoting blood circulation and removing blood stasis, as exemplified by cryptotanshinone and salvi?anolic acid B, exerted striking effects on modulating angiogenesis and vascular permeability, which suggests that they may be effective in treating vascular leak-driven diseases (e.g. tumor, cerebral cavernous malformation and diabetic reti?nopathy). However, the lack of reliable and advanced technologies and models sets up difficult hurdles for better under?standing the role of TCM for promoting blood circulation and removing blood stasis. To this end, this study is to outline numerous cutting-edge platforms that can be utilized for exploring the function of TCM for promoting blood circulation and removing blood stasis in vascular leak-driven diseases. METHODS Two-photon laser scanning fluorescence micros?copy was used to observe the interactions between neutrophils and blood vessels in a real-time manner. Dynamic flow system was employed to mimic the in vivo behaviors of neutrophils. RIP1-Tag5 spontaneous pancreatic cancer model was used to study the function of tumor blood vessels. CCM2ECKO (deletion of CCM2 in endothelial cells) mice were employed to establish the cerebral cavernous malformation (CCM) animal model. Micro-computed tomography (micro-CT) was utilized to assess the CCM lesion. Müller cell-knockout mouse model was used to study the progression of dia?betic retinopathy. Vascular permeability in this model was assessed by fluorescein angiography. RESULTS The interac?tions between neutrophils and endothelial cells involve a series of complicated processes, including rolling, adhesion, intraluminal crawling and transmigration, which were all monitored in vivo by two-photon laser scanning fluorescence microscopy in a real-time manner. Dynamic flow system was capable of recapitulating the biological behaviors of neutro?phils in vitro. Tumor vascular function in particular vascular perfusion could be assessed in the RIP1-Tag5 spontaneous pancreatic cancer model. In terms of CCM studies, specific deletion of CCM2 in endothelial cells resulted in the initiation of CCM lesion. The size and number of CCM lesions could be visualized and quantified by micro-CT. Furthermore, the Müller cell-knockout mouse model was able to precisely reflect the clinical symptoms of diabetic retinopathy. Vascular leak could be monitored at different time points using fluorescein angiography. CONCLUSION An array of high technol?ogies and animal models can be used in investigating the occurrence and progression of multiple vascular leak-driven diseases. The pre-clinical and clinical studies of TCM for promoting blood circulation and removing blood stasis provide fundamental support for the application of the above-mentioned platforms, with the purpose of uncovering the scientific basis of TCM for promoting blood circulation and removing blood stasis.

Malformaciones cavernosas cerebrales, presentación de 14 casos y revisión de literatura / Brain cavernous malformations, presentation of 14 cases and literature review

Objetivo: Presentar los resultados de tratamiento quirúrgico obtenidos en una serie de 14 casos de malformación cavernosa, situadas en diferentes localizaciones encefálicas, además de realizar una revisión bibliográfica sobre el tema. Material y métodos: En el periodo de los años 2014-2019, se diagnosticaron y protocolizaron 14 pacientes por medio de la consulta externa de neurocirugía del Hospital Juárez de México. Todos menos 2, fueron intervenidos quirúrgicamente. Resultados: En 12 de los 14 casos que recibieron tratamiento quirúrgico, se documentó mejoría neurológica posterior a la resección total en 10 pacientes, 1 paciente de cavernoma gigante temporal se hizo resección subtotal, en 1 paciente con lesión de localización protuberancial se le realizó únicamente drenaje de hematoma. El déficit preoperatorio tendió a mejorar progresivamente en las lesiones de mayor tamaño y en ningún caso se documentaron complicaciones, las crisis convulsivas se controlaron disminuyendo progresivamente la dosis de fármacos anticonvulsivantes en el periodo postquirúrgico de este grupo de pacientes. Y dos pacientes, uno con lesión mesencefálica y el otro con cavernomatosis solo se sometieron a observación. Conclusiones: La cirugía es el método de elección hoy en día para el tratamiento de las malformaciones cavernosas, siendo los mejores resultados a menor tamaño de la lesión y con localizaciones más superficiales. Los resultados quirúrgicos de nuestros pacientes son similares a lo reportado en la literatura mundial.

Objectives: To present the surgical outcomes obtained in a series of 14 cases of cavernous malformation, located in different brain locations, in addition to conducting a literature review on the subject. Method: Between the years, 2014 and 2019, 14 cases were diagnosed and protocolized in neurosurgery department of Hospital Juárez of México. All patients except two, were surgically treated. Results: In 12 of the 14 cases received surgical treatment, neurological improvement was documented after the total resection in ten patients, one patient with giant temporal cavernoma performed a subtotal resection, other case with a lesion in the pontine location a hematoma drainage was performed. All surgical patients the preoperative clinical deficit tended to improve progressively in larger lesions and no complications were documented. Seizures were controlled by gradually decreasing the dose of anticonvulsant drugs in the post-surgical period of this group of patients. And two patients, one with mesencephalic lesion and another with cavernomatosis, were only observe. Conclusion: Surgery is the method of choice today for the treatment of cavernous malformations, with the best outcome being the smallest size of the lesion and with more superficial locations. The surgical outcomes in our patients are similar to those reported in the world literature

Resección microquirúrgica de cavernoma del receso lateral derecho a través de abordaje telovelar / Microsurgical resection of a cavernoma of the right lateral recess through the telovelar approach

A partir de la introducción de las técnicas modernas en diagnóstico por Resonancia Magnética por Imágenes (RMI), revolucionó la comprensión de las malformaciones cavernosas, permitiendo su diagnóstico preciso. En paralelo, el avance de las técnicas microquirúrgicas y el conocimiento de las zonas seguras de acceso al tronco cerebral han definido los accesos para su resección segura. Se presenta un caso de paciente varón de 25 años de edad, sin antecedentes patológicos, consulta por cefalea de un año de evolución. En RM donde se observa lesión nodular en relación con el receso lateral derecho. Se realiza abordaje suboccipital medial, con acceso telovelar hacia el receso lateral con la exéresis completa de la lesión. Un adecuado conocimiento sobre la anatomía del IV ventrículo, seleccionando el abordaje microquirúrgico más apropiado, con técnica quirúrgica meticulosa es prioritario en la resección de cavernomas en esta localización. El devastador resultado neurológico que puede ocurrir en un paciente con una malformación cavernosa del tronco encefálico cuando estos sangran, plantean a la resección microquirúrgica como la mejor opción en aquellas lesiones accesibles a través de zonas seguras.

From the introduction of modern imaging techniques with Magnetic Resonance Imaging, it revolutionized the understanding of cavernous malformations, allowing for accurate diagnosis. In parallel, the advancement of microsurgical techniques and the knowledge of safe areas of access to the brain stem have defined access for safe resection. We present a case of male patient of 25 years of age, without pathological history, consultation for headache of one year of evolution. In MRI where nodular lesion is observed in relation to the right lateral recess. A medial suboccipital approach was performed, with telovelar access to the lateral recess with complete excision of the lesion. An adequate knowledge about the anatomy of the IV ventricle, selecting the most appropriate microsurgical approach, with meticulous surgical technique is a priority in the resection of cavernous in this location. The devastating neurological outcome that can occur in a patient with a cavernous malformation of the brainstem when they bleed, posed to microsurgical resection as the best option in those lesions accessible through safe areas.

De novo cavernous malformation after radiosurgery for cerebellar arteriovenous malformation: A case report

Stereotactic radiosurgery, including gamma knife radiosurgery (GKS), can in rare cases result in de novo cavernous malformations (CMs). Here, we present a case of de novo CM induced by GKS following treatment of a cerebellar arteriovenous malformation (AVM). A 48-year-old woman was diagnosed with left unilateral Moyamoya disease. Conventional cerebral angiography also revealed an AVM in the left cerebellum. The patient underwent GKS using a 50% isodose of 15 Gy at the margin of the left cerebellar AVM. Magnetic resonance imaging (MRI) taken 3 years after GKS revealed small chronic hemorrhages with perilesional edema in the left cerebellum. Five years later, the lesions became aggravated, but were asymptomatic. Eight years following GKS, the patient was admitted complaining of headache and dizziness. Brain MRI revealed a 1.3cm hemosiderin deposit with an inner hyperintense nodular portion that was enhanced in the left cerebellum. An open craniotomy was performed and the mass was removed, from which pathological findings were compatible with those for CM. The patient recovered to the prehemorrhagic state. This case shows that De novo CMs can rarely develop after radiosurgery. Most CMs have been reported to develop following radiosurgery for brain tumors. As shown in this patient, CMs can also develop after radiosurgery for cerebellar AVM in adults.

CCM3 down-regulating the lead acetate-induced migration of human umbilical vein endothelial cells / 中国职业医学

OBJECTIVE: To investigate the effects of knockout cerebral cavernous malformation(CCM) virulence gene CCM3 on the migration induced by lead acetate in immortalized human umbilical vein endothelial cells(HUVECs) and to explore the possible mechanism of endoplasmic reticulum stress(ERS). METHODS: CCM3 wildtype(CCM3-WT) and CCM3 knockout(CCM3-KO) HUVECs were used as experimental cells. a) CCM3-WT and CCM3-KO HUVECs were treated with lead acetate at 0,10,50 and 200 μmol/L for 24 hours. The migration of these cells was observed by woundhealing assay. b) CCM3-WT and CCM3-KO HUVECs were treated with lead acetate at 0,10,50 and 200 μmol/L for 24 hours,and at 50 μmol/L for 0,6,12,24 and 48 hours,and the mRNA expression of genes of unfolded protein response pathway were detected by quantitative real-time polymerase chain reaction; the protein expression of glucose-regulated protein 78(GRP78) was detected by Western blotting. c) CCM3-WT and CCM3-KO HUVECs were divided into lead exposure group and tauroursodeoxycholic acid(TUDCA) group. The former was treated with 50 μmol/L lead acetate for 24 hours,and the latter was pre-treated with ERS inhibitor TUDCA,followed by 50 μmol/L lead acetate. The migration of these cells was observed by wound-healing assay. RESULTS: a) The migration of CCM3-WT and CCM3-KO cells decreased and showed a dose-effect relationship with the increase of lead acetate concentration(P < 0. 05). b) The mRNA relative expression of the GRP78,protein kinase-like endoplasmic reticulum kinase(PERK),transcription activator 4(ATF4) and CCAAT enhancer binding homologous protein(CHOP) in CCM3-KO cells treated with 10,50 and 200 μmol/L lead acetate were higher than that in CCM3-WT cells at the same doses,except for the GRP78 in CCM3-KO cells treated with10 μmol/L lead acetate(P < 0. 05). The mRNA expression of PERK and CHOP in CCM3-KO cells increased in a timeeffect relationship with the increase of lead-exposure time(P < 0. 05). The mRNA relative expression of the four genes in CCM3-KO cells were higher than those in CCM3-WT cells at 48 hours(P < 0. 05). When cells were treated with 50μmol/L lead acetate,the protein expression of GRP78 in CCM3-KO cells was higher than that in CCM3-WT cells(P <0. 05),and the protein expression of GRP78 in CCM3-KO cells increased in a time-effect relationship with the increase of lead-exposure time(P < 0. 05). c) The cell migration of TUDCA group was lower than that of lead-exposure group(P <0. 05). CONCLUSION: Lead acetate may activate ERS by activating the PERK-ATF4-CHOP signaling pathway,thereby reducing the migration of HUVECs. CCM3 gene has a protective effect on cell migration.

Surgical Approaches for Symptomatic Cerebral Cavernous Malformations of the Thalamus and Brainstem

OBJECTIVE: Surgical resection of thalamic and brainstem cerebral cavernous malformations (CCMs) is associated with significant operative morbidity, but it may be outweighed, in some cases, by the neurological damage from recurrent hemorrhage in these eloquent areas. The goals of this retrospective cohort study are to describe the technical nuances of surgical approaches and determine the postoperative outcomes for CCMs of the thalamus and brainstem. MATERIALS AND METHODS: We reviewed an institutional database of patients harboring thalamic or brainstem CCMs, who underwent surgical resection from 2010 to 2014. The baseline and follow-up neuroimaging and clinical findings of each patient and the operative details of each case were evaluated. RESULTS: A total of eight patients, including two with thalamic and six with brainstem CCMs, were included in the study cohort. All patients had progressive neurological deterioration from recurrent CCM hemorrhage, and the median modified Rankin Scale (mRS) at presentation was 3. The median CCM maximum diameter and volume were 1.7 cm and 1.8 cm³, respectively. The thalamic CCMs were resected using the anterior transcallosal transchoroidal and supracerebellar infratentorial approaches each in one case (13%). The brainstem CCMs were resected using the retrosigmoid and suboccipital trans-cerebellomedullary fissure approaches each in three cases (38%). After a median follow-up of 11.5 months, all patients were neurologically stable or improved, with a median mRS of 2. The rate of functional independence (mRS 0-2) was 63%. CONCLUSION: Microneurosurgical techniques and approaches can be safely and effectively employed for the management of thalamic and brainstem CCMs in appropriately selected patients.

A case involving anesthesia for cesarean section followed by resection of ruptured cavernous malformation of pons :A case report

Cavernous malformation is an important cause of cerebral hemorrhage in pregnant women. We experienced a case of cesarean section followed by neurosurgery for resection of ruptured cavernous malformation of pons in a 24-year-old pregnant woman. Establishing integrated treatment strategy for pregnant women with cavernous malformation is difficult because of the small number of cases. Therefore, the cooperation between the brain surgeon, anesthesiologist and obstetrician is essential when surgical intervention is needed. Despite aspiration pneumonia, the patient successfully underwent surgery. We reported this case along with a brief literature review.

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management / Neurocisticose, cavernoma cerebral familiar e calcificações intracranianas: diagnóstico diferencial e acompanhamento adequado

ABSTRACT Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.

RESUMO A neurocisticercose (NCC) é um importante problema endêmico de saúde pública em algumas áreas do mundo, sendo epilepsia sua manifestação clínica mais comum. Múltiplas lesões intracranianas, geralmente com calcificações visualizadas em tomografia computorizada de crânio, são interpretadas como um dos critérios diagnósticos na fase crônica da doença. A ressonância magnética é o melhor teste de imagem para identificar a doença em diferentes estágios de sua forma cística mas apresenta limitações para demonstrar lesões calcificadas. Malformações cavernosas cerebrais, ou cavernomas, são malformações vasculares comuns ao sistema nervoso e epilepsia é também a sua forma mais frequente de apresentação. Na sua forma familiar cavernomas apresentam-se tipicamente com múltiplas lesões encefálicas e, frequentemente, com focos de calcificações na TC. Em alguns países, e determinadas regiões geográficas, onde neurocisticercose é endêmica, a neuroimagem mais usada para diagnóstico é a TC de crânio. Nesse contexto torna-se importante estabelecer bases para o diagnóstico diferencial entre as duas doenças, devido às diferentes formas de acompanhamento e tratamento adequado.

Two cases of familial cerebral cavernous malformation caused by mutations in the CCM1 gene / 소아과

Cerebral cavernous malformation (CCM) is a vascular malformation characterized by abnormally enlarged capillary cavities without any intervening neural tissue. We report 2 cases of familial CCMs diagnosed with the CCM1 mutation by using a genetic assay. A 5-year-old boy presented with headache, vomiting, and seizure-like movements. Brain magnetic resonance imaging (MRI) revealed multiple CCM lesions in the cerebral hemispheres. Subsequent mutation analysis of his father and other family members revealed c.940_943 del (p.Val314 Asn315delinsThrfsX3) mutations of the CCM1 gene. A 10-month-old boy who presented with seizure-like movements was reported to have had no perinatal event. His aunt was diagnosed with cerebral angioma. Brain and spine MRI revealed multiple angiomas in the cerebral hemisphere and thoracic spinal cord. Mutation analysis of his father was normal, although that of the patient and his mother revealed c.535C>T (p.Arg179X) mutations of the CCM1 gene. Based on these studies, we suggest that when a child with a familial history of CCMs exhibits neurological symptoms, the physician should suspect familial CCMs and consider brain imaging or a genetic assay.

Systemic Expression of Vascular Endothelial Growth Factor in Patients with Cerebral Cavernous Malformation Treated by Stereotactic Radiosurgery

OBJECTIVE: Increased expression of angiogenic factors, such as vascular endothelial growth factor (VEGF), is associated with the pathogenesis of cerebral cavernous malformations (CCMs). The purpose of this study was to investigate plasma levels of VEGF in normal subjects and in patients with CCM and to evaluate change in these levels following stereotactic radiosurgery (SRS). METHODS: Peripheral venous blood was collected from 6 patients with CCM before SRS using Gamma Knife and at the 1 week, 1 month, 3month, and 6 month follow-up visits. Plasma VEGF levels were measured using commercially available enzyme-linked immunosorbent assay kits. Peripheral blood samples were obtained from 10 healthy volunteers as controls. RESULTS: Mean plasma VEGF level of 41.9 pg/mL (range, 11.7–114.9 pg/mL) in patients with CCM at baseline was higher than that of the healthy controls (29.3 pg/mL, range, 9.2–64.3 pg/mL), without significant differences between CCM patients and controls (p=0.828). Plasma VEGF level following SRS dropped to 24.6 pg/mL after 1 week, and decreased to 18.5 pg/mL after 1 month, then increased to 24.3 pg/mL after 3 months, and 32.6 pg/mL after 6 months. Two patients suffering from rebleeding after SRS showed a higher level of VEGF at 6 months after SRS than their pretreatment level. CONCLUSION: Plasma VEGF levels in patients with CCM were elevated over controls at baseline, and decreased from baseline to 1 month after SRS and increased further for up to 6 months. Theses results indicated that anti-angiogenic effect of SRS might play a role in the treatment of CCMs.

Elevated Cellular Retinoic Acid Binding Protein-I in Cerebrospinal Fluid of Patients with Hemorrhagic Cerebrovascular Diseases : Preliminary Study

OBJECTIVE: Elevated cellular retinoic acid binding protein-I (CRABP-I) is thought to be related to the abnormal proliferation and migration of smooth muscle cells (SMCs). Accordingly, a higher CRABP-I level could cause disorganized vessel walls by causing immature SMC phenotypes and altering extracellular matrix proteins which could result in vulnerable arterial walls with inadequate responses to hemodynamic stress. We hypothesized that elevated CRABP-I level in the cerebrospinal fluid (CSF) could be related to subarachnoid hemorrhage (SAH). Moreover, we also extended this hypothesis in patients with vascular malformation according to the presence of hemorrhage. METHODS: We investigated the CSF of 26 patients : SAH, n=7; unruptured intracranial aneurysm (UIA), n=7; arteriovenous malformation (AVM), n=4; cavernous malformation (CM), n=3; control group, n=5. The optical density of CRABP-I was confirmed by Western blotting and presented as mean+/-standard error of the measurement. RESULTS: CRABP-I in SAH (0.33+/-0.09) was significantly higher than that in the UIA (0.12+/-0.01, p=0.033) or control group (0.10+/-0.01, p=0.012). Hemorrhage presenting AVM (mean 0.45, ranged 0.30-0.59) had a higher CRABP-I level than that in AVM without hemorrhage presentation (mean 0.16, ranged 0.14-0.17). The CRABP-I intensity in CM with hemorrhage was 0.21 and 0.31, and for CM without hemorrhage 0.14. Overall, the hemorrhage presenting group (n=11, 0.34+/-0.06) showed a significantly higher CRABP-I intensity than that of the non-hemorrhage presenting group (n=10, 0.13+/-0.01, p=0.001). CONCLUSION: The results suggest that elevated CRABP-I in the CSF could be related with aneurysm rupture. Additionally, a higher CRABP-I level seems to be associated with hemorrhage development in vascular malformation.

Delayed Diagnosis of Probable Radiation Induced Spinal Cord Vascular Disorders

Occasionally, unexpected neurological deficits occur after lumbar spinal surgery. We report a case of monoparesis after lumbar decompressive surgery. A 63-year-old man, who had undergone decompression of L4-5 for spinal stenosis 4 days previously in the other hospital, visted the emergency department with progressive weakness in the left leg and hypoesthesia below sensory level T7 on the right side. He had been cured of lung cancer with chemotherapy and radiation therapy 10 years previously, but detailed information of radiotherapy was not available. Whole spine magnetic resonance (MR) imaging showed fatty marrow change from T1 to T8, most likely due to previous irradiation. The T2-weighted MR image showed a high-signal T4-5 spinal cord lesion surrounded by a low signal rim, and the T1-weighted MR image showed focal high signal intensity with focal enhancement. The radiological diagnosis was vascular disorders with suspicious bleeding. Surgical removal was refused by the patient. With rehabilitation, the patient could walk independently without assistance 2 months later. Considering radiation induced change at thoracic vertebrae, vascular disorders may be induced by irradiation. If the spinal cord was previously irradiated, radiation induced vascular disorders needs to be considered.

Two Cases of Cavernous Malformation Presenting with Unusual and Large Intracerebral Hemorrhages

Cavernous malformations (CMs), characterized by the presence of a hemosiderin rim and intralesional hemorrhage, are relatively common intracranial vascular malformations. Extralesional hemorrhages arising from CMs are seen in a minority of cases, but most of them show typical CM findings on magnetic resonance imaging. Here, the authors report two cases of pathologically confirmed CM presenting with unusual and large intracerebral hemorrhages, which were not surrounded by the typical hemosiderin rim. CMs presenting with large intracerebral hemorrhage should be considered in the differential diagnosis of massive intracerebral hemorrhages.

Unusual Presentation of Cerebral Cavernous Malformation

Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage.

Chronic Encapsulated Intracerebral Hematoma Associated with Cavernous Malformation

Chronic encapsulated intracerebral hematoma (CEIH) is a rare cerebrovascular disease that behaves as a slowly expanding lesion with a gradual onset. It is well established that CEIH is associated with arteriovenous malformations; however, CEIH associated with cavernous malformation (CM) is extremely rare. We herein report a case of CEIH associated with CM, and discuss its pathogenesis. A 12-year-old female was admitted to our hospital because of a one week history of progressive headache and nausea. Brain computed tomography scan and magnetic resonance imaging showed an intracerebral hematoma surrounded by edema in the right frontal lobe. One week later, her headache and nausea worsened, and a brain computed tomography scan revealed the enlargement of hematoma. A right frontal craniotomy was performed. The capsule, mass, and hematoma were totally removed. Histological examination confirmed the diagnosis of CEIH associated with CM. Immunohistochemical analysis revealed increased expression of vascular endothelial growth factor (VEGF) and the VEGF receptor-1 in the endothelium and fibroblasts. Our findings suggest that the activated VEGF pathway might have positively contributed to development of CEIH in the present patient.

Microsurgical Treatment and Outcome of Pediatric Supratentorial Cerebral Cavernous Malformation

OBJECTIVE: The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. METHODS: Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. RESULTS: The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. CONCLUSION: With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.

Brainstem cavernous malformation / Cavernomas de tronco cerebral

Cavernous malformation (CM) of the central nervous system (CNS) are acquired or developmental vascular malformations that represent the 5% to 15% of all vascular malformations of the CNS. Eighty to ninety percent of CM are supratentorial, 15% infratentorial, and 5% occur in the spinal cord. The subset of brainstem malformation presents as a very difficult paradigm for treating clinicians. The widespread use of magnetic resonance imaging (MRI) has increased the recognition of this disease. Clinical presentation, pathophysiology and treatment are discussed in this article...

Os cavernomas do sistema nervoso central (SNC) são malformações vasculares do desenvolvimento ou adquiridas que representam 5% a 15% de todas as malformações vasculares do SNC. Dos cavernomas, 80% a 90% são supratentoriais, 15% são infratentoriais e 5% ocorrem na medula espinhal. As malformações do tronco encefálico se apresentam como um paradigma de decisão de tratamento muito difícil para os cirurgiões. O amplo uso das imagens por ressonância magnética aumentou o reconhecimento dessa patologia. A apresentação clínica, a fisiopatologia e o tratamento serão discutidos neste artigo...

Giant Cystic Cerebral Cavernous Malformation with Multiple Calcification: Case Report

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2x4.6x6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review.