A cerebral arteriovenous malformation mistakenly diagnosed as dry eye and glaucoma: a case report.

BACKGROUND: To report the first case of a cerebral arteriovenous malformation (AVM) with ocular symptoms and review the characteristics of this case and the main point of confusion for the diagnosis of such a case. CASE PRESENTATION: A 58-year-old woman presented to the ophthalmology clinic with 1 and a half years of right eye redness, ocular hypertension and recurrent headache. One and a half years ago she was diagnosed with right eye dry eye and glaucoma and had received treatment according to this diagnosis. However, none of the treatments led to any improvement in redness and headache. Physical examination revealed dry eye and severe corkscrew hyperaemia with dilated vessels in the right eye. The results of fundoscopic examination of both eyes were normal. After we considered that the symptoms may be related to abnormal intracranial vessels, computed tomography angiography and venography (CTA + CTV) were performed, and the results showed an arteriovenous malformation in the right parietal-occipital area in the brain. The AVM was definitively located by further examination with digital subtraction angiography (DSA). After AVM endovascular embolism treatment, the conjunctival congestion of the right eye was significantly relieved, and the intraocular pressure decreased to normal. CONCLUSION: In clinical practice, when corkscrew hyperaemia accompanied by neurological symptoms is found, cerebral vascular diseases might be considered. In this case, the ophthalmologist's diagnosis should combine disease history and imaging examination.

Impact of Pre-operative Embolization With Onyx for Brain Arteriovenous Malformation Surgery.

Objective: To clarify the safety and efficacy of pre-operative embolization using Onyx liquid embolic agent (Onyx; ev3) compared with N-butyl cyanoacrylate (NBCA; Cordis Neurovascular, Inc.) or coils in cerebral arteriovenous malformation (AVM) surgery. Methods: This was a retrospective review of a prospectively collected clinical database of brain AVMs treated at our institute from January 2005 to March 2021. A total of 38 consecutive patients who underwent AVM resection after pre-operative embolization were included. Based on pre-operative embolization materials, the patients were divided into the pre-Onyx group (n = 16), in which NBCA or coils were used for embolization, and the Onyx group (n = 22). Patient characteristics and treatment results were compared between the two groups. Results: Patient characteristics were comparable between the two groups in terms of age, sex, and rupture status. While the Spetzler-Martin grade was also similar between the two groups, the location of the AVM nidus in the eloquent area was slightly higher in patients in the Onyx group (72.7%) than in patients in the pre-Onyx group (43.8%) (P = 0.09). The embolization rate was higher in the pre-Onyx group (mean: 63.0%; range: 12.7-100%) than in the Onyx group (mean: 50.0%; range: 15.8-100%), but the difference was not statistically significant (P = 0.06). The time needed for surgical removal was shorter in the Onyx group (mean: 354.8 min; range: 144-884 min) than in the pre-Onyx group (mean: 457.9 min; range: 240-1,294 min); however, this difference was not statistically significant (P = 0.13). The amount of intraoperative bleeding was significantly lower in the Onyx group (mean: 129.8 ml; range: 20-540 mL) than in the pre-Onyx group (mean: 448.8 mL; range: 120-1,550 ml) (P = 0.0008). The surgical complication rates were comparable between the two groups (pre-Onyx group, 18.8%; Onyx group, 4.5%; P = 0.29). Conclusions: Pre-operative embolization with Onyx can significantly reduce the amount of intraoperative bleeding in AVM resection and may contribute to safe AVM surgery.

A Questionnaire Study on the Use of Complementary and Alternative Veterinary Medicine for Dogs in Sweden.

Complementary and alternative veterinary medicine (CAVM) includes treatment methods that are not part of established veterinary medicine and where there is not as yet sufficient scientific documentation of the proposed effects. The CAVM use in Swedish dogs is largely unknown, and the aim of this study was to explore this use. Electronic questionnaires were distributed to dog owners, small animal veterinary practitioners, and CAVM therapists. A total of 253 dog owners responded. Except for massage, stretching, and laser therapy, more than 75% of the respondents stated that they did not use CAVM for their dogs. Of the 216 responding veterinarians, 39% did not use CAVM. CAVM use was more common among respondents with <10 years of work experience as compared to those with >10 years of work experience (p < 0.05). Almost half of the 124 responding CAVM therapists treated dogs; the most frequently used methods included massage, stretching, and acupressure. A majority of all respondents found the current Swedish regulation of CAVM insufficient. Although CAVM use in Swedish dogs appears to be uncommon, there is a need for scientific assessment of CAVM in dogs as regards safety and effectiveness for different indications.

A Questionnaire Study on the Use of Complementary and Alternative Veterinary Medicine for Horses in Sweden.

Complementary or alternative veterinary medicine (CAVM) includes treatment methods with limited scientific evidence. Swedish veterinarians are legally obliged to base treatments and recommendations on science or well-documented experience, but most CAVM methods are not well documented in animals. The aim of this study was to explore the use of CAVM in Swedish horses. Electronic questionnaires were distributed to horse owners, equine veterinary practitioners and CAVM therapists. Of the 204 responding horse owners, 83% contacted a veterinarian first in case of lameness, while 15% contacted a CAVM therapist. For back pain, 52% stated a CAVM therapist as their first contact and 45% a veterinarian. Only 10-15% of the respondents did not use any CAVM method for prevention or after injury. Of the 100 veterinarians who responded, more than half did not use CAVM themselves but 55% did refer to people who offer this service. Of the 124 responding CAVM therapists, 72% recommended their clients to seek veterinary advice when needed, 50% received referrals from a veterinarian, and 25% did not collaborate with a veterinarian. The two most common methods used by the respondents in all three categories were stretching and massage. Most veterinarians and therapists were not content with the current lack of CAVM regulation.

Cardiac autophagic vacuolation in severe X-linked myopathy with excessive autophagy.

X-linked myopathy with excessive autophagy (XMEA), caused by mutations of the VMA21 gene, is a strictly skeletal muscle disease. Extensive studies in yeast established VMA21 as the master assembly chaperone of V-ATPase, the complex multisubunit proton pump that acidifies organelles and that is vital to all mammalian tissues. As such, skeletal muscle disease exclusivity in XMEA is highly surprising. We now show that the severest VMA21 mutation, c.164-6t>g, does result in XMEA-typical pathology with autophagic vacuolar changes outside skeletal muscle, namely in the heart. However, even patients with this mutation do not exhibit clinical extramuscular disease, including cardiac disease, despite extreme skeletal muscle wasting to the extent of ventilation dependence. Uncovering the unique skeletal muscle vulnerability to defective organellar acidification, and resultant tissue-destructive excessive autophagy, will be informative to the understanding of muscle physiology. Alternatively, understanding extramuscular resistance to VMA21 mutation might disclose heretofore unknown mammalian V-ATPase assembly chaperones other than VMA21.

Leksell Gamma Knife for pediatric and adolescent cerebral arteriovenous malformations: results of 100 cases followed up for at least 36 months.

OBJECT: The goal of this study was to evaluate advantages, risks, and failures of Gamma Knife radiosurgery (GKRS) in a large series of pediatric and adolescent patients with cerebral arteriovenous malformations (cAVMs) who were followed up for at least 36 months. METHODS: Since February 1993, 100 pediatric and adolescent patients (≤ 18 years of age) with cAVMs have undergone GKRS at the authors' institution and were followed up for at least 36 months. Forty-six patients were boys and 54 were girls; the mean age was 12.8 years (range 3-18 years). Hemorrhage, either alone or combined with seizure, was the clinical onset in 70% of cases. The mean pre-GK cAVM volume was 2.8 ml; 92% of cAVMs were Spetzler-Martin (S-M) Grades I-III. Most lesions (94%) were in eloquent or deep-seated brain regions, according to S-M classification. The parameters for mean and range in treatment planning were prescription isodose 53.8% (40%-90%); prescription dose (PD) 20.2 Gy (9.0-26.4 Gy); maximal dose (MD) 37.8 Gy (18-50 Gy); and number of shots 4.7 (1-17). On the day of GKRS, stereotactic CT or stereotactic MRI and digital subtraction angiography were used. RESULTS: Obliteration rate (OR) was angiographically documented in 75 of 84 cases (89.3%) after single-session GKRS, with actuarial ORs at 3 and 5 years of 68.0% and 88.1%, respectively. A repeat treatment was performed in 7 patients (6 with obliteration), and 16 patients with cAVMs underwent staged treatment (9 of them were angiographically cured). Thus, the overall OR was 90%, with actuarial ORs at 3, 5, and 8 years of 59.0%, 76.0%, and 85.0%, respectively. Permanent symptomatic GK-related complications were observed in 11% of cases, with surgical removal of enlarged mass seen on post-RS imaging needed in 5 cases. Hemorrhage during the latency period occurred in 9% of patients, but surgical evacuation of the hematoma was required in only 1 patient. One patient died due to rebleeding of a brainstem cAVM. Radiosurgery outcomes varied according to cAVM sizes and doses: volumes ≤ 10 ml and PDs > 16 Gy were significantly associated with higher ORs and lower rates of permanent complication and bleeding during the latency period. CONCLUSIONS: The data from this study reinforce the conclusion that GKRS is a safe and effective treatment for pediatric and adolescent cAVMs, yielding a high OR with minimal permanent severe morbidity and no mortality. The very low frequency of severe hemorrhages during the latency period further encourages a widespread application of RS in such patients. Univariate analysis found that modified RS-based cAVM score, nidus volume, PD, integral dose, S-M grade, and preplanned treatment (the last 2 parameters were also confirmed on multivariate analysis) significantly influenced OR. Lower S-M grades and single-session planned treatments correlated with shorter treatment obliteration interval on univariate analysis. This statistical analysis suggests that a staged radiosurgical treatment should be planned when nidus volume > 10 ml and/or when the recommended PD is ≤ 16 Gy.

An efficient and non-enzymatic method for isolation and culture of endothelial cells from the nidus of human cerebral arteriovenous malformations.

In this report, we describe an efficient and non-enzymatic method for isolating and culturing endothelial cells (ECs) from the nidus of surgically resected arteriovenous malformation (AVM) specimens. These cultured cells possessed typical phenotypic markers (i.e. von Willebrand factor and CD34), as well as morphological and ultrastructural characteristics of ECs. However, they had activated Notch-1 signaling, which plays a critical role in the development of AVM. The present study suggests that hypoxic endothelial cells from the nidus of human cerebral arteriovenous malformation (CAVMECs) have angiogenic potentials, as our data showed that VEGF gene expression and cell proliferation were more evident with prolonged hypoxia. In our study, we successfully used the vascular tissue explants adherent method to isolate and culture CAVMECs with high purity. This may prove to be a useful tool for studying the molecular mechanisms that mediate abnormal vessel development and maintenance in AVM.