Hemangioblastoma of the spinal cord. Review and report of five cases.

Eighty-five cases of hemangioblastoma of the spinal cord are reviewed, including five new ones. While current views of the incidence, histology, and cytogenesis are presented, the main purpose of this article is to call attention to the identifying clinical and radiological characteristics of these spinal tumors. Median age at onset of symptoms was 30 years. The ratio of men to women was 1.1:1. Presenting symptoms were usually radicular pain or posterior column sensory loss or both. The lesions were most often single (79%), intramedullary (60%), and located in the cervical or thoracic spinal cord. There was associated syringomyelia in 67% of intramedullary cases and meningeal varicosities in 48% of all cases. Lindau disease and hemangioblastomas in other central nervous system locations were present in 33% of cases. The characteristic roentgenographic picture is a densely vascular tumor in association with a larger avascular syrinx and meningeal varicosities. Surgery is the only definitive treatment.

Stroke in a 15-year-old girl secondary to terminal carotid dissection.

A 15-year-old girl died 14 days after hemiplegia suddenly developed. On arteriography, intimal separation of the middle cerebral arteries showed as a long attenuated column of dye--the "string" sign. Pathologic examination showed intimal separation starting at the distal bifurcation of the right internal carotid artery and extending into the middle and anterior cerebral arteries. The arteriographic string sign as evidence of dissection may aid diagnosis of this cause of childhood hemiplegia.

Progressive thrombosis after treatment of diffuse large cell non-Hodgkin's lymphoma and concomitant lupus anticoagulant.

We report a case of diffuse large cell non-Hodgkin's lymphoma with concomitant lupus anticoagulant at initial diagnosis. Progressive thrombosis occurred despite radiologically proven response of the lymphoma after chemotherapy treatment. Extraordinary bone scintigraphy with multiple "cold" lesions probably due to bone ischemia is described.

Cerebral vasospasm with ruptured saccular aneurysm--the clinical manifestations.

Fifty cases of verified intracranial ruptured saccular aneurysm were analyzed to investigate the relationship of the development of a delayed cerebral ischemic deficit to the presence of cerebral vasospasm visualized on angiography. Twenty-five patients developed a delayed ischemic deficit (DID), and all showed Grade 3+ or 4+ vasospasm. Nineteen patients had Grade 0, 1+, or 2+ vasospasm, and none developed a DID. It was concluded that in this study vasospasm accounted for all DID's and that in the absence of vasospasm DID did not occur. The DID occurred most often on Day 8 (7 of 25 cases).

A safe technique for the precise localization of carotid-cavernous fistula during balloon obliteration. Technical note.

The authors report a technique to precisely localize a fistulous opening in the carotid artery. The patient is heparinized and a Prolo catheter is introduced into the internal carotid artery and inflated distal to the approximate site of the fistula. Heparinization allows the balloon to be inflated long enough to obtain and analyze high-quality angiography film without fear of thromboembolism generated by the temporary balloon occlusion. Contrast material injected through the Prolo catheter proximal to the balloon reveals a small segment of cavernous carotid artery between the inflated balloon distally and the fistula proximally. The venous structures are now only faintly opacified and cannot obscure the morbid anatomy of the exact fistulous tear in the carotid artery. If the balloon is placed exactly opposite to the site of the fistula, a standing, stagnant column of dye forms a cast of the cavernous, petrous, and cervical carotid artery. Once the fistula is localized with this method, it may be obliterated by any therapeutic means preferred. If the Prolo catheter is used for intraluminal occlusion, then a transfemoral contralateral carotid angiogram is done before the heparin is reversed to confirm that the balloon has not been placed proxial to the fistula.

Secondary syringomyelia due to intramedullary spinal cord metastasis. Case report and review of literature.

Intramedullary spinal cord metastasis is relatively rare. We describe a patient having intramedullary spinal cord metastasis associated with syringomyelia, confirmed by magnetic resonance imaging, in a patient who had poorly differentiated carcinoma of the lung. The patient responded to treatment with steroids and radiotherapy, with complete resolution of neurologic symptoms and syringomyelia.

Spontaneous dissection of cervico-cerebral arteries.

Sixteen cases of spontaneous dissection of the cervical internal carotid artery (6 verified) are described. The mean age was 45 years. The clinical picture varied from simply headache and a bruit to hemiplegia and aphasia. Eleven patients had transient ischemic attacks. Headache, facial pain, a subjective bruit, oculo-sympathetic palsy and transient monocular blindness were present in various combinations in two-thirds of cases and their presence suggested the correct diagnosis. Examples of suspected dissection of the intracranial internal carotid, middle cerebral, posterior cerebral and extracranial vertebral arteries are also presented. Spontaneous dissection is more common than the literature indicates.

The diagnosis of diastematomyelia by computed tomography.

CT scanning has proven efficacy for the demonstration of normal and altered anatomy of the soft tissues of the head, providing information not available with any other modality. The introduction of total body CT has enabled us to study the entire spine and its contents. This new modality is extremely useful in diagnosing a variety of abnormalities of the spine, including diastematomyelia. A case is presented and the application of CT scanning to the spine is discussed.

Computed tomography in extra-dural epidermoid and xanthoma.

Two cases of extradural epidermoid cyst and one case of xanthoma are reported in order to describe the computed tomographic and other radiographic findings. Contrast enhancement of the margin was seen in all of the cases. Absorption values almost as low as those found with cerebrospinal fluid were present in the central region of the lesions.

Complementary role of computed tomography and other neuroradiologic procedures.

Although a probable initial diagnosis may be suggested by computed cranial tomography, the diagnostic accuracy is often increased by the complementary use of other neuroradiologic studies. The use of CT scanning is conjunction with other more invasive neuroradiological studies results in improved diagnosis and patient management in almost the entire spectrum of neurosurgical diseases-- subarachnoid hemorrhage due to aneurysm, stroke, trauma, normal pressure hydrocephalus, and especially neoplasms.

CSF enhancement for computerized tomography.

Metrizamide cisternography, combined with hypocycloidal tomography, has been performed in 12 patients with possible posterior fossa or parasellar mass lesions with finely detailed images of the basal cisterns resulting. Computed tomography was performed in nine of these patients and produced exceptional images of the cisternal anatomy. The technique of cerebrospinal fluid enhancement may be efficacious in identifying small basal masses not shown with conventional computed tomography, and thus may form an important complement to enhancement by intravenous injection of contrast medium.

Surgical treatment of extracranial carotid occlusive disease.

This report has reviewed the preoperative evaluation, operative technique, postoperative care, results, and representative pathological findings in 225 carotid endarterectomies. Surgery is generally indicated in patients with: 1. TIA's, and carotid stenosis (lumen diameter less than 2 mm.) or an ulcerated plaque. 2. A stable, mild to moderate neurological deficit with or without TIA's, and carotid stenosis (lumen diameter less than 2 mm.) or an ulcerated plaque. 3. An acute progressive or fluctuating neurological deficit, and carotid stenosis (lumen diameter less than 2 mm.) or carotid occlusion. Surgery should be considered in some patients with: 1. TIA's, and ipsilateral carotid occlusion. 2. An acute partial persistent neurological deficit, and carotid stenosis (lumen diameter less than 2 mm.) or occlusion. 3. No symptoms, and carotid stenosis (lumen diameter less than 2 mm.). Surgery is generally not indicated in patients with an acute severe persistent neurological deficit, and carotid stenosis or occlusion.

Clinico-radiologic profile of spinal cord multiple sclerosis in adults.

MRI is extremely useful for the assessment of initial disease burden and to identify the dissemination of the multiple sclerosis (MS) in time and space. Though MRI of the spinal cord is not used to establish the diagnosis of MS, spinal cord is frequently involved in this disease and there has been increasing emphasis of the spinal imaging in making clinical decision in the management of MS. We undertook a retrospective study of patients with diagnosed MS: 1) to identify radiologic pattern of spinal cord involvement in MS and 2) to correlate radiologic findings with clinical presentation. We reviewed radiologic records from 2004 to 2009 of patients with abnormal T2 signal intensity of the spinal cord with radiologic concern of demyelinating disease. Patients in this cohort who met the Revised McDonald MS Diagnostic Criteria were included in this study. 166 patients were included in the study. There was preference for cervical spinal cord particularly posterior aspect of the spinal cord. Enhancement of the lesions was rare (4.1%). Mean lesion length was 18.2 mm. The average number of lesions per patient was 2.04. Sensory symptoms were predominating and most of the patients had relapsing-remitting course. Patients with sensory symptoms, bladder and bowel involvement and motor symptoms had almost equally distributed lesions among anterior, posterior and central spinal cord. However, all of the patients presented with posterior column signs and gait abnormality had involvement of the posterior spinal cord. Radiologic manifestation of spinal cord MS is extremely variable and can involve the entire length of the spinal cord. Clinical symptoms may or may not be associated with radiologic presentation of the lesions.

JC virus infection of the brain.

Since its initial description, there have been significant changes in the epidemiology, pathogenesis, and clinical and imaging manifestations of JCV infection of brain. The most common clinical manifestation is PML. Other recently described CNS manifestations are JCE, JCVGCN, and JCM. Although AIDS is the most common predisposing factor for JCV reactivation, there is increasing incidence of brain manifestations of JCV reactivation in non-HIV settings, including different rheumatologic, hematologic, and oncologic conditions; monoclonal antibody therapy; transplant recipients; primary immunodeficiency syndromes; and even in patients without any recognizable immune deficiency. IRIS may develop secondary to restoration of immunity in HIV-positive patients with PML receiving antiretroviral therapy. This is of profound clinical significance and needs to be diagnosed promptly. Imaging plays a crucial role in the diagnosis of the disease, monitoring of treatment response, identifying disease progression, and predicting prognosis. In this article, current understanding of the epidemiology, pathogenesis, clinical presentations, and all aspects of imaging of JCV infection of the brain have been comprehensively reviewed.