Search

Susac's syndrome is a rare autoimmune microangiopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. In many cases, the clinical triad is not fully present at the onset of symptoms. MRI studies often show characteristic punched out lesions of the central fibers of the corpus callosum, and leptomeningeal enhancement and deep gray matter lesions may also be seen. Here we present a case of Susac's syndrome in a middle aged man with the unique clinical finding of cauda equina syndrome and spinal MRI showing diffuse lumbosacral nerve root enhancement. Biopsy specimens of the brain, leptomeninges, and skin showed evidence of a pauci-immune endotheliopathy, consistent with pathology described in previous cases of Susac's syndrome. This case is important not only because it expands the clinical features of Susac's syndrome but also because it clarifies the mechanism of a disorder of the endothelium, an important target for many disorders of the nervous system.

Spontaneous spinal cerebrospinal fluid (CSF) leaks are increasingly recognized in patients presenting with orthostatic headache and ultimately diagnosed with intracranial hypotension. While the precise cause of these spontaneous leaks is unknown, it is thought to result from underlying weakness in the spinal meninges and may be associated with meningeal diverticula or Tarlov cysts. Rarely, calcified intervertebral discs or bony osteophytes can result in CSF leakage, which has been described in the surgery literature but not well recognized in the radiology literature. The authors present three cases of patients presenting with CSF leaks from calcified thoracic disc protrusions that were successfully treated with epidural blood patches.

OBJECTIVE. Perfusion CT is being increasingly used as a diagnostic tool for the evaluation of acute ischemic stroke. It can be performed rapidly and aids in the detection of salvageable tissue (penumbra) from the unsalvageable core infarct. The purpose of this article is to provide an overview of the imaging technique, interpretation pearls, and common pitfalls encountered in perfusion CT of the brain. CONCLUSION. Perfusion CT has proven to be a valuable tool in the diagnosis of acute ischemic stroke. The knowledge provided by these cases will allow the reader not only to confidently identify the presence of acute ischemic stroke, but also to recognize the common pitfalls and limitations of perfusion CT in this setting.

Malignant neoplasia involving contiguous vertebrae and the corresponding intervertebral discs is a rare occurrence that has similar imaging manifestations as pyogenic osteodiscitis. The authors describe the imaging manifestations of two cases of malignant neoplasia mimicking pyogenic osteodiscitis. We present a case of an 83-year-old male with metastatic non-small cell lung carcinoma and an 82-year-old female with a plasmacytoma, both within the thoracic spine. These cases illustrate how the imaging features of pyogenic osteodiscitis may parallel those of malignant neoplasia.

Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Microinfarction is believed to be the basic histologic feature and MR is considered the best imaging modality. This case documents the typical imaging findings of Susac syndrome, as well as the unique MR imaging finding of cranial nerve enhancement.

Oculocerebrorenal syndrome of Lowe (OCRL) is a multisystem disorder characterized by congenital cataracts, hypotonia, and cognitive developmental delay with renal complications developing in the first few months of life. Clinical and laboratory findings of Lowe syndrome are well documented. Though a small number of case reports describe the neuroimaging features and the renal ultrasound manifestations of this disease, a comprehensive review of all the imaging manifestations has not been reported. The authors present a case of OCRL and review the neuroimaging and renal ultrasound manifestations of this multisystem disease.

Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. All three components of the triad usually do not present at the same time, thus delaying time to diagnosis. MRI studies often show characteristic punched out lesions of the central fibers of the corpus callosum. Intracranial leptomeningeal enhancement may be seen, however, cauda equina involvement has not been described to our knowledge. We present a case of Susac syndrome in a middle-aged male with symptoms of cauda equina syndrome, and spinal MRI showing diffuse enhancement of the nerve roots of the cauda equina.

Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis presenting in the 5th through 7th decades of life. Osseous manifestations include symmetrical sclerosis of the long bones and, rarely, the spine. Central nervous system disease commonly affects the white matter tracts as well as the orbits, but epidural disease is rare. To the best of the authors' knowledge, simultaneous epidural and skeletal spine disease has not been reported. The MR imaging characteristics of skeletal spine disease have also not been reported. The authors describe the case of an 87-year-old man with both epidural and skeletal spine disease. The clinical characteristics, imaging manifestations, and the histological features are discussed.

CTP has a growing role in evaluating stroke. It can be performed immediately following NCCT and has advantages of accessibility and speed. Differentiation of salvageable ischemic penumbra from unsalvageable core infarct may help identify patients most likely to benefit from thrombectomy or thrombolysis. Still, CTP interpretation can be complex. We review normal and ischemic perfusion patterns followed by an illustrative series of technical/diagnostic challenges of CTP interpretation in the setting of acute stroke syndromes.

Kikuchi-Fujimoto disease (KFD) is an uncommon disorder that usually presents in young adults of Asian descent with fever and cervical lymphadenopathy. Clinically, this disease is often misdiagnosed as lymphoma or tuberculosis, and biopsy is needed for confirmation. The authors report an unusual case of KFD in a Hispanic male presenting with a subacute subdural hematoma, whose care was complicated by extranodal features not typically associated with KFD. The clinical, histopathologic, and radiographic manifestations of KFD are discussed.

Median palatine cysts are rare, non-odontogenic fissural cysts of the hard palate. These cysts occur in the midline of the hard palate, behind the incisive canal. Only two case reports have documented these cysts on multi-detector computed tomography (MDCT), neither giving detailed descriptions of the cysts. Knowledge of their existence is important and should not be confused with malignant tumors. We present the first case describing the MDCT characteristics of the median palatine cyst.