[Von Hippel-Lindau disease].

BACKGROUND: Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies.

OBJECTIVE: To develop guidelines for CCM management.

METHODS: The Angioma Alliance (www.angioma.org), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol.

RESULTS: Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%) were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%).

CONCLUSION: Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines.

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Synopsis of Guidelines for the Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel.

Brainstem Cavernous Malformations: 1390 Surgical Cases from the Literature

BACKGROUND Cavernous malformations (CMs) in deep locations account for 9% to 35% of brain malformations and are surgically challenging. OBJECTIVE To study the clinical features and outcomes following surgery for deep CMs and the complication of hypertrophic olivary degeneration (HOD). METHODS Clinical records, radiological findings, operative details, and complications of 176 patients with deep CMs were reviewed retrospectively. RESULTS Of 176 patients with 179 CMs, 136 CMs were in the brainstem, 27 in the basal ganglia, and 16 in the thalamus. Cranial nerve deficits (51.1%), hemiparesis (40.9%), numbness (34.7%), and cerebellar symptoms (38.6%) presented most commonly. Hemorrhage presented in 172 patients (70 single, 102 multiple). The annual retrospective hemorrhage rate was 5.1% (assuming CMs are congenital with uniform hemorrhage risk throughout life); the rebleed rate was 31.5%/patient per year. Surgical approach depended on the proximity of the CM to the pial or ependymal surface. Postoperatively, 121 patients (68.8%) had no new neurological deficits. Follow-up occurred in 170 patients. Delayed postoperative HOD developed in 9/134 (6.7%) patients with brainstem CMs. HOD occurred predominantly following surgery for pontine CMs (9/10 patients). Three patients with HOD had palatal myoclonus, nystagmus, and oscillopsia, whereas 1 patient each had limb tremor and hemiballismus. At follow-up, 105 patients (61.8%) improved, 44 (25.9%) were unchanged, and 19 (11.2%) worsened neurologically. Good preoperative modified Rankin Score (98.2% vs 54.5%, P = .001) and single hemorrhage (89% vs 77.3%, P < .05) were predictive of good long-term outcome. CONCLUSION Symptomatic deep CMs can be resected with acceptable morbidity and outcomes. Good preoperative modified Rankin Score and single hemorrhage are predictors of good long-term outcome.

Cavernous malformation of brainstem, thalamus, and basal ganglia: a series of 176 patients.

OBJECTIVE\nThe optimal management for patients with cavernous sinus meningiomas remains controversial. We attempt to contribute to the ongoing debate of appropriate surgical indications.\n\n\nMETHODS\nIn this retrospective review, 39 patients, including 27 women and 12 men ranging in age from 24 to 73 years (median, 48 yr), underwent surgical treatment for this condition. Completeness of tumor resection, cranial nerve morbidity, general morbidity, and long-term outcome were studied. The cavernous internal carotid artery was partially encased in 15 patients, totally encased in 11 patients, and narrowed by tumor in 13 patients.\n\n\nRESULTS\nOf eight patients who underwent complete tumor resection, seven had partial encasement of the internal carotid artery. Of 31 patients who underwent subtotal resection, 11 underwent postoperative radiotherapy. There were no deaths in the series. Morbidity was 17.9% for cranial nerves controlling extraocular motor function. Trigeminal nerve function did not improve after surgical treatment. The median follow-up period was 2 years (range, 6 mo-5.3 yr). Symptomatic and radiographic recurrence occurred in two patients who underwent complete tumor resection and in two patients who underwent subtotal resection.\n\n\nCONCLUSION\nBased on our findings and a review of the literature, we conclude the following: 1) the resectability of meningiomas of the cavernous sinus depends on the degree of internal carotid artery involvement; 2) total excision of cavernous sinus meningiomas is possible but rarely achieved in holocavernous meningiomas; 3) cranial nerve morbidity is significant; and 4) subtotal excision with or without postoperative radiotherapy is an effective short-term oncological strategy.

The Surgical Resectability of Meningiomas of the Cavernous Sinus

Background: Cavernomas are rare in the brainstem and account for 18-35% of central nervous cavernomas and can cause recurrent hemorrhages, devastating neurological deficits and mortality. Objectives: To summarize the experience of microsurgical treatment of brainstem cavernomas and to investigate curative effect of microsurgical treatment of brainstem cavernomas. Materials and Methods: A retrospective analysis clinical data of 37 patients with brainstem cavernomas seen between 2003 and 2007. The analysis included age distribution, hemorrhage rates, clinical presentation, location of the lesions, and preoperative and postoperative Karnofsky Performance Scale (KPS) scores. The surgical indications, the timing of surgery and the surgical techniques were also assessed. Results: All the 37 patients received microsurgical resections, there was no surgery-related mortality. Histopathological examination confirmed the diagnosis of cavernoma. Postoperatively, 20 patients had functional improvement, 15 patients had no change in the neurological status, and two patients deteriorated. Early surgery was associated with better outcomes. Mean followed up period was 21.5 months (range 6-36 months). During the follow-up 20 patients had resumed activities of daily living (KPS scores of 90-100), 10 patients were able to self-care with some efforts (KPS scores of 70-80), five patients needed considerable assistance (KPS score of 50-60) and two patients suffered hemiparesis (KPS scores of 40). None of the patient had recurrent hemorrhage. Conclusions: Brainstem cavernomas can safely be resected. Successful resection of brainstem cavernomas can be achived by optimal surgical approaches, feasible entry zone and meticulous microsurgical techniques. The goal of surgical intervention should be the total resection of the lesion without any deteriorative in the neurological deficits.

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Experience of microsurgical treatment of brainstem cavernomas: report of 37 cases.

OBJECT The authors retrospectively analyzed the clinical characteristics, existing problems, and treatment experiences in recently diagnosed cerebral paragonimiasis (CP) cases and sought to raise awareness of CP and to supply reference data for early diagnosis and treatment. METHODS Twenty-seven patients (22 male and 5 female; median age 20.3 years, range 4–47 years) with CP were diagnosed between September 2008 and September 2013. These diagnoses were confirmed by IgG enzyme-linked immunosorbent assays. Follow-up was performed in 24 cases for a period of 6–56 months. RESULTS Cerebral paragonimiasis accounted for 21.6% of paragonimiasis cases (27 of 125). The average duration from onset to praziquantel treatment was 69 days. All patients resided in rural areas. Twenty patients had positive lung results, which included visible lung lesions in 14 cases. The lesions were surgically removed in 8 of these cases. Twenty-four patients had high eosinophil counts (≥ 0.08 × 109/L), and eosinophilic meningitis was n...

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Cerebral paragonimiasis: a retrospective analysis of 27 cases

Characteristic CT and MR imaging findings of cerebral paragonimiasis

Interleukin-18 (IL-18) is a potent proinflammatory cytokine, which can promote hepatitis B virus clearance. The latest studies find that genetic polymorphisms near the IL-28B gene are strongly associated with sustained viral response and spontaneous viral clearance in patients with chronically infected hepatitis C and hepatitis B. We investigated the effect of rs187238 and rs1946518 in IL-18 gene and rs8099917 in IL-28B gene on HBV recurrence in liver transplant patients. A total of 200 liver transplant recipients and relevant donors were enrolled in this study. The patients' mean follow-up was 39 month (range 10-65 month). All liver transplant recipients were in a stable stage. The total recipients (n = 200) were divided into end-stage liver disease secondary to hepatitis B (n = 140) and end-stage liver disease secondary to other diseases (n = 60) before transplantation. Recipients (n = 140) with hepatitis B before transplantation were defined to nonHBV recurrence group (n = 119) or HBV recurrence group (n = 21), which was positive for HBsAg or elevatory in HBV DNA (>2.0 × 10(2) IU mL(-1)) after transplantation. For the recipients (n = 140) had hepatitis B before transplantation, we studied the single-nucleotide polymorphisms (SNPs) of IL-18 gene (rs187238 and rs1946518) and IL-28B gene (rs8099917) by high-resolution melting (HRM) curve analysis. The serum levels of IL-18 and IFN-γ were tested by ELISA. The serums levels of IFN-γ were lower in HBV recurrence group than that in nonHBV recurrence group (P < 0.01). The genotype of IL-28B gene rs8099917 was associated with alanine aminotransferase (ALT) levels and aspartate aminotransferase (AST) levels in HBV-related liver transplant recipients (n = 140). The recipients with allele G (GG+GT) had higher ALT and AST levels (P < 0.05). No association was found between IL-18 gene and IL-28B gene polymorphisms with HBV recurrence in the liver transplant recipients or the donors. We identified that the IFN-γ was a protective factor of HBV recurrence after liver transplantation. The allele G of rs8099917 was associated with hepatitis B-related hepatocytes injury. The rs8099917 G allele subgroup should reinforce antiviral therapy.

Association of polymorphisms in interleukin-18 and interleukin-28B with hepatitis B recurrence after liver transplantation in Chinese Han population.

Background and Purpose—We retrospectively analyzed the clinical and imaging characteristics, diagnosis, and treatment outcomes of 10 patients with hemorrhagic cerebral paragonimiasis (CP), and we evaluated the influence of Paragonimus infection on cerebrovascular damage. Methods—Ten patients (7 male and 3 female; median age 15.7 years, range 4–46 years) with hemorrhagic CP were diagnosed between April 2009 and January 2013. All patients underwent the head computed tomography scans and 9 patients underwent MRI examinations. Four patients underwent computed tomographic angiography, magnetic resonance angiography, and digital subtraction angiography. Liquid-based cytological examination of cerebrospinal fluid was performed in 7 patients. Follow-up examinations were performed for 9 cases for a period of 12 to 62 months. Results—Hemorrhagic CP accounted for 37% of CP cases (10/27). No patients were initially diagnosed with CP. The major symptoms of hemorrhagic CP included acute headache, vomiting, hemiparalysi...

Jing Chen

Papers

Experience of microsurgical treatment of brainstem cavernomas: report of 37 cases.

Cerebral paragonimiasis: a retrospective analysis of 27 cases

Characteristic CT and MR imaging findings of cerebral paragonimiasis

Association of polymorphisms in interleukin-18 and interleukin-28B with hepatitis B recurrence after liver transplantation in Chinese Han population.

Hemorrhagic Stroke and Cerebral Paragonimiasis