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ISQ – Neuroscience
Published in Bhaskar Punukollu, Michael Phelan, Anish Unadkat, MRCPsych Part 1 In a Box, 2019
Bhaskar Punukollu, Michael Phelan, Anish Unadkat
False – Anterior cerebral artery lesions – (i) upper motor neurone (UMN) palsy of foot and lower limb on contralateral side; (ii) sensory loss on contralateral side – foot and lower limb; (iii) urinary problems. Posterior cerebral artery lesions – (i) alexia without agraphia in left posterior cerebral artery lesions; (ii) Weber’s syndrome; (iii) UMN palsy of whole contralateral side; (iv) ipsilateral oculomotor nerve palsy; (v) superior homonymous quadrantinopia.
Is this capillary malformation? Differential diagnosis and other dermal vascular lesions
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
Two conditions that deserve a special mention are Sturge–Weber syndrome and the PHACES syndrome. In Sturge–Weber syndrome, the CM is typically on a trigeminal cranial nerve V (branches V1-V2) distribution of the face, which can be confused with flat hemangiomas observed in PHACES syndrome. The implications and treatment are significantly different. The hemangioma in PHACES syndrome is typically a large plaque-like hemangioma involving the V1-V2 and even V3 distribution. It is commonly unilateral, although it can be bilateral. Patients with PHACES syndrome may also have other anomalies including a posterior fossa brain malformation (P), hemangioma as described earlier (H), arterial anomalies typically involving the ascending aorta and brachiocephalic arteries (A), cardiac anomalies including atrial and ventricular septal defects and a patent ductus arteriosus (C), eye abnormalities (E), and sternal defects (S). The importance of PHACES syndrome and the large facial hemangioma is to alert the physicians of the potential structural anomalies involving the heart, eyes, central nervous system (CNS), and arterial vascular system. In contrast, patients with Sturge–Weber syndrome need to be screened for brain AVMs as well as CNS abnormalities and, in particular, vascular malformations ipsilateral to the CM. It is worth emphasizing that patients with Sturge–Weber syndrome are at high risk for secondary complications due to episodes of seizures and glaucoma. The absence of seizures and/or glaucoma within the first 4 years of life is used as exclusion criteria for Sturge–Weber syndrome. For more details, see Chapter 90.
Controversies in Pediatric Angle Surgery and Secondary Surgical Treatment
Published in Seminars in Ophthalmology, 2023
Alexander K. Young, Deborah K. Vanderveen
A benefit of a GDD following primary angle surgery is the flexibility to place the drainage device in one of four quadrants between extraocular muscles, rather than be limited to placing a bleb underneath the upper eyelid in the superior quadrant.31 Another benefit of implantation of the drainage devices is the option for a second tube shunt if the refractory glaucoma persists or recurs. This has been shown to raise the five-year success rate.28 The first tube shunt is often left untouched, while the second tube shunt is placed in a different quadrant.32 Of note, Sturge-Weber syndrome has been a particularly challenging syndromic glaucoma that is frequently refractory. Due to the low success rate with primary angle surgery, many surgeons have opted to implant a GDD not only as a secondary surgery but as a primary one. However, the success rate in this subtype has been reported to be as low as 30% at 60 months postoperatively.33
OCT Imaging in Infants
Published in Seminars in Ophthalmology, 2022
Sushma Jayanna, Subhadra Jalali, Tapas R Padhi, Komal Agarwal, Jay Chhablani
Choroidal hemangiomas can be focal or diffuse in nature. Diffuse variety are associated with Sturge-weber syndrome. Diffuse choroidal thickening with retinal vessels tortuosity, hemorrhages, and retinal detachment can be noted clinically. OCT findings include RNFL thinning, focal or diffuse disruption of RPE and choroidal thickening. secondary changes in the overlying retina like intra and subretinal fluid and photoreceptor degeneration. Lesions if involves fovea can cause a vision deterioration. Choroidal thickening is confined to the eye with the tumor and normal choroidal thickness in the other eye. OCT plays an important role in assessing the extent of involvement which helps in precise treatment with either photodynamic therapy or radiation therapy. It also helps in monitoring the lesion after treatment.23,39
Seizure and cognitive outcomes of posterior quadrantic disconnection: a series of 12 pediatric patients
Published in British Journal of Neurosurgery, 2020
Yao Wang, Chao Zhang, Xiu Wang, Lin Sang, Feng Zhou, Jian-Guo Zhang, Wen-Han Hu, Kai Zhang
Except for the age at onset and operation, no differences were observed between the two groups. The seizure-free rate was 85.7% in the acquired group and 100% in the developmental group, similar to the seizure outcomes from Dorfer et al.4 and Mohamed et al.,5 who reported seizure-free rates after PQD for MCD of 80% and 75%, respectively. These results show that MCD is unlikely to be a predictive factor of poor outcomes in PQD patients. Our results differ from those following hemispherectomy, for which seizure outcomes are poor.20,21 MCD patients who undergo hemispherectomy primarily include patients with megalencephaly, microcephaly and focal cortical dysplasia (FCD). The probable reason is that MCD has a diffuse distribution, and the contralateral hemisphere easily becomes involved in patients who underwent hemispherectomy, while it is mostly focal in PQD patients. As in a prior study reported by Jehi et al.,22 surgical pathology other than tumour or high-grade MRI-visible MCDs (types IIA and IIB) increased the risk for seizure recurrence. The authors reported that the recurrence rate of encephalomalacia was 45% after posterior cortex epilepsy surgery, mostly within the first 6 postoperative months. However, encephalomalacia in our study and Sturge–Weber syndrome (SWS) in the Sugano et al.6 study showed seizure outcomes, suggesting that pathology probably cannot be considered a predictor of seizure outcome.