Spontaneous regression and recurrence of a tumefactive perivascular space

Perivascular spaces can occasionally appear mass-like (tumefactive or giant perivascular space), and can be associated with clinical symptoms. Spontaneous regression of a tumefactive perivascular space is a very rare phenomenon with only two reported cases in the English medical literature. Spontaneous regression of a tumefactive perivascular space along with resolution of clinical symptoms, followed by spontaneous recurrence associated with symptom recurrence is an extremely rare occurrence, which to the best of our knowledge, has not been reported in the medical literature. We describe a case of spontaneous regression of a tumefactive perivascular space, three years after its initial detection, followed by spontaneous recurrence after two years

MRI patterns of global hypoxic-ischemic injury in adults

Background and purpose: To assess MRI patterns and associated clinical outcome in adults with global hypoxic-ischemic injury. Materials and methods: In order to identify the patients with evidence of global hypoxic-ischemic injury, we retrospectively searched our radiology information system for reports of brain MRI studies from 01/01/2004 to 12/31/2010, containing the keywords - "hypoxia", "hypoxic", "anoxia" and "anoxic". A board certified neuroradiologist visually inspected the corresponding MR images for the presence, location and extent (focal versus diffuse) of ischemic findings. Clinical data for these patients was collected from the electronic medical records, including mechanism of the hypoxic-ischemic injury, and clinical outcome was measured using modified Rankin Scale (mRS). Results: Review of radiology reports identified 151 cases, of which 64 patients remained after exclusion of normal studies ("no hypoxia" in the report), pediatric patients and patients with remote perinatal hypoxia. Five patients had relatively favorable clinical outcome (mRS of 1 to 3) and 59 had poor outcome (mRS of 4 to 6). Patterns associated with relatively favorable clinical outcome were: a) watershed pattern and b) basal ganglia without cortical involvement. Patterns associated with poor clinical outcome were: a) diffuse cortical and deep grey matter pattern, with and without perirolandic sparing; (b) medial occipital with perirolandic involvement; c) precentral gyrus involvement; d) diffuse white matter involvement; e) brainstem involvement; f) cerebellar involvement and g) hippocampal involvement. Conclusion: The vast majority of patients with MRI patterns of hypoxic-anoxic injury have a poor clinical outcome, independently of the observed pattern, with the only relative exception being the watershed pattern and the basal ganglia pattern without cortical involvement. (C) 2012 Elsevier Masson SAS. All rights reserved

Congenital X-linked Stapes Gusher Syndrome in an Infant A Case Report

Congenital X-linked deafness with stapes gusher is a rare, but well-known clinical entity with characteristic radiological features. Recognition of these findings is important as it changes the treatment course and precludes stapedectomy, which if done can lead to stapes gusher in these patients. We present the case of a nine-month-old male infant with hearing loss. CT scan showed typical radiological features of X-linked stapes gusher syndrome. In addition, the superior semicircular canals appeared dehiscent which could be due to immature ossification or less likely due to defective development as part of the disease. To the best of our knowledge this is the youngest patient prospectively identified by imaging in the English medical literature

Isolated contralateral sudden sensorineural hearing loss: an unusual manifestation of pontine infarct

Unilateral, acute onset sensorineural hearing loss ("sudden sensorineural hearing loss" [SSNHL]) as an isolated event without other associated neurological deficits usually results from a lesion of the cochlea. Lesions in the ascending central auditory pathways cranial to the cochlear nucleus seldom result in unilateral hearing loss due to decussation of the central auditory pathways at multiple levels. We describe a patient with a tiny acute infarct in the right pons resulting in isolated acute onset left-sided SSHNL, without any other associated acute neurological deficits

Imaging of Tumor Syndromes

Tumor predisposition syndromes represent a heterogeneous group of multiorgan disorders, with many having substantial central nervous system involvement. This article highlights the common and uncommon manifestations of these syndromic disorders, the underlying genetic pathways, and the imaging findings. Radiologists must be aware of the diagnostic criteria, optimal imaging techniques (both for diagnosis and surveillance), as well as the innumerable imaging manifestations of these syndromes. Multidisciplinary approach and teamwork are essential in managing these patients, with imaging having a central role as more of these patients get diagnosed earlier and survive longer

Susac syndrome in a young child

Susac syndrome is a microangiopathy of unknown origin affecting the brain, retina and inner ear. This rare entity is often misdiagnosed as a demyelinating condition such as multiple sclerosis or acute disseminated encephalomyelitis. A high index of suspicion must be present as the majority of patients do not have the complete clinical triad at the time of onset of symptoms. The radiologist plays an important role when the disease is suspected and helps orient the investigations. The syndrome has characteristic imaging features on MRI that include multifocal white matter and occasional grey matter lesions, the corpus callosum being always involved. The predominant central callosal lesions, especially with rapid cystic transformation (central callosal holes) can be considered pathognomonic of this condition in the appropriate clinical setting. This disease is extremely rare in children. We report a case of Susac syndrome in a 9-year-old girl to increase the awareness among paediatric radiologists of this entity, which is usually not considered as a differential diagnosis of multifocal white matter involvement in this age group

Enhancement of multiple cranial and spinal nerves in vanishing white matter: expanding the differential diagnosis

Abnormal cranial or spinal nerve contrast enhancement on MRI in cases of suspected pediatric leukodystrophy is recognized as an important clue to the diagnosis of either metachromatic leukodystrophy or globoid cell leukodystrophy (Krabbe disease). We report a case of genetically confirmed childhood vanishing white matter with enhancement of multiple cranial and spinal nerves in addition to the more typical intracranial findings. This case expands the limited differential diagnosis of cranial nerve or spinal nerve enhancement in cases of suspected leukodystrophy and may aid in more efficient work-up and earlier diagnosis of vanishing white matter

Bilateral Massive Angiomyolipomatosis Associated with Tuberous Sclerosis

Angiomyolipoma is an uncommon tumor consisting of vascular, smooth muscle, and fatty elements. Angiomyolipoma can present in two forms; as an isolated unilateral lesion which occurs sporadically or as bilateral lesions associated with tuberous sclerosis and Von Hippel-Lindau syndrome. Bilateral massive angiomyolipomatosis is a very rare entity. Only three cases have been reported in medical literature. We present a case of bilateral massive angiomyolipomatosis associated with tuberous sclerosis

FDG Positron Emission Tomography and Computed Tomography Demonstration of Carcinoma Arising in an Epiphrenic Diverticulum

Esophageal carcinoma arising within an epiphrenic diverticulum is rare. We describe a case of a carcinoma in a long-standing epiphrenic diverticulum in a 62-year-old patient. Fluorine-18-2-Fluoro-2-Deoxy-D-Glucose Positron Emission Tomography and Computed Tomography, and endoscopic ultrasound revealed a hypermetabolic mass within the diverticulum. A preoperative diagnosis was made via endoscopic biopsy. The patient underwent Ivor-Lewis esophagectomy. He remains well and free of recurrence 18 months after surgery