Imaging feature of Leukoencephalopathy with calcifications and cysts (Labrune syndrome).

Labrune syndrome is an uncommon CNS disorder characterized by leukoencephalopathy, cerebral calcifications, and cysts on brain imaging. The basic pathology is microangiopathy resulting from a mutation in the SNORD118 gene. Radiological imaging is the hallmark of the disease.

Functional and Neuropsychological Outcome After Surgical Treatment of Moyamoya Disease.

BACKGROUND: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive stenosis of the supraclinoid internal carotid artery. As a result of chronically decreased brain perfusion, eloquent areas of the brain become hypoperfused, leading to cognitive changes in patients. Repeated infarcts and bleeds produce clinically apparent neurologic deficits. OBJECTIVES: 1) To study the functional and neuropsychological outcome in MMD after revascularization surgery. 2) To find postrevascularization correlation between functional and neuropsychological improvement and radiologic improvement. METHODS: A single-center prospective and analytic study was carried out including 21 patients with MMD during the study period from March 2021 to December 2022. Patients were evaluated and compared before and after revascularization for functional, neuropsychological, and radiologic status. RESULTS: Postoperative functional outcome in terms of modified Rankin Scale score showed improvement in 33.33% of cases (P = 0.0769). An overall improving trend was observed in different neuropsychological domains in both adult and pediatric age groups. However, the trend of neuropsychological improvement was better in adults compared with pediatric patients. Radiologic outcome in the form of the Angiographic Outcome Score (AOS) significantly improved after revascularization (P = 0.0001). There was a trend toward improvement in magnetic resonance imaging (MRI) perfusion in the middle cerebral artery and anterior cerebral artery territories, 4.7% (P = 0.075) and 9.33% (P = 0.058) respectively, compared with preoperative MRI perfusion. CONCLUSIONS: After revascularization, significant improvement occurred in functional and neuropsychological status. This result was also shown radiologically as evidenced by improvement in MRI perfusion and cerebral angiography.

Cortical malformation adjacent to a large pial arteriovenous fistula.

The coexistence of an arteriovenous fistula (AVF) and neuronal migration abnormalities is a rare phenomenon. The underlying pathophysiology responsible for these anomalies remains elusive. Neuronal architectural irregularities arise from complex neuronal formation, migration and organisation dysfunctions. Isolated cases of these associations are rarely described in the literature. Here, we present an unusual case involving the coexistence of a pial AVF and a pachygyria-polymicrogyria complex in an early childhood boy. We have provided a detailed description of the neuroimaging characteristics and the therapeutic embolisation in this case, along with follow-up. Additionally, we conduct a comprehensive review of potential hypotheses about the association, referencing prior case reports. The presence of an aberrant blood supply or deviant venous drainage from the developing cortex may contribute to a variety of neuronal migration anomalies.

Reconstructive endovascular treatment of petrous ICA pseudoaneurysm in skull base osteomyelitis: a hidden catastrophe.

Skull base osteomyelitis can be more life-threatening in immunocompromised patients and patients with diabetes. Here, we present a case of a petrous internal carotid artery pseudoaneurysm resulting from skull base osteomyelitis in a diabetic male in his 50s. This case report highlights the need to be conscious of the various complications associated with skull base osteomyelitis, be proficient in detecting them and treat them as early as possible for better outcomes. After adequate control of the disease process with medical treatment, immediate management of the aneurysm with balloon angioplasty and stenting was done. Acknowledging the trivial nasal and ear bleed, radiological evaluation is necessary to rule out rare complications like pseudoaneurysms in a diagnosed case of skull base osteomyelitis. In the discussion, we have cited the various treatment methods and similar cases of pseudoaneurysm caused by osteomyelitis. Currently, the patient continues to live a disease and disability-free life.

Nonbifurcating carotid artery-embryological scrutiny, intriguing associations, and review of literature.

Nonbifurcating carotid is quite a rare anatomical variant of carotid anatomy. The embryology of carotid development is more intricate and convoluted than is presently known. This case report finds an unusual case of a nonbifurcating carotid artery whose embryological basis cannot be explained by popular prevalent theories. It also stresses about an abundance of case reports from East Asia on this topic, to the best of the authors' knowledge. The formation of atherosclerotic plaque requires more than abnormal fluid hemodynamic, which has been briefly pointed out.