Intracerebral Whipple's disease diagnosed by stereotactic biopsy: a case report and review of the literature.

OBJECTIVE AND IMPORTANCE: This case demonstrates the rare occurrence of intracerebral Whipple's disease in a patient lacking classic systemic manifestations of the disease. Because of the nonspecific presentation and the typically deep-seated location of cerebral lesions in these patients, definitive diagnosis is frequently problematic. We present the first reported use of stereotaxy-guided brain biopsy to confirm the diagnosis of isolated intracranial Whipple's disease. CLINICAL PRESENTATION: The patient was a 36-year-old man who presented with a 4-month history of progressive lethargy, hypersomnia, behavioral changes, and weight gain. The results of the physical examination were remarkable only for findings of hypogonadism. Subsequent laboratory evaluation confirmed the diagnosis of hypogonadotrophic hypogonadism, with low levels of testosterone, luteinizing hormone, cortisol, and prolactin. INTERVENTION: A magnetic resonance image of the brain demonstrated hyperintense lesions on T2-weighted images in the regions of the right fornix, hypothalamus, and putamen that subsequently enhanced with intravenously administered contrast medium. A biopsy was then obtained from the right putaminal lesion under stereotactic guidance. Histopathological analysis of the tissue revealed findings consistent with intracerebral Whipple's disease that were subsequently confirmed using electron microscopy. CONCLUSION: Intracerebral Whipple's disease should be included in the differential diagnosis of patients presenting with progressive dementia and cognitive decline. In these patients, lesions have typically been observed in the hypothalamus, cingulate gyrus, basal ganglia, insular cortex, and cerebellum. As evidenced by our case, stereotaxy affords clinicians the attractive option of a minimally invasive technique by which to obtain tissue from such deep-seated areas. A review of this rare neurosurgical entity is presented.

Complications during anterior surgery of the lumbar spine: an anatomically based study and review.

Procedures involving anterior surgical decompression and fusion are being performed with increasing frequency for the treatment of a variety of pathological processes of the spine including trauma, deformity, infection, degenerative disease, failed-back syndrome, discogenic pain, metastases, and primary spinal neoplasms. Because these operations involve anatomy that is often unfamiliar to many neurological and orthopedic surgeons, a significant proportion of the associated complications are not related to the actual decompressive or fusion procedure but instead to the actual exposure itself. To understand the nature of these injuries, a detailed anatomical study and dissection was undertaken in six cadaveric specimens. Critical structures at risk in the abdomen and retroperitoneum were identified, and their anatomical relationships were categorized and photographed. These structures included the psoas muscle, kidneys, ureters, diaphragm and crura, esophageal hiatus, thoracic duct, greater splanchnic nerves, phrenic nerves, sympathetic chains, medial arcuate ligament, superior and inferior hypogastric plexus, segmental and radicular vertebral vessels, aorta, vena cava, median sacral artery, common iliac vessels, iliolumbar veins, lumbosacral plexus, and presacral hypogastric plexus. Based on these dissections and an extensive review of the literature, the authors provide a detailed anatomically based discussion of the complications associated with anterior lumbar surgery.

Familial occurrence of an arteriovenous fistula with a giant perimedullary pseudoaneurysm of the thoracic spinal cord in 2 young siblings.

Two siblings, a sister at age 3 years and a brother (15 years later) at 4 years of age, both presented with similar clinical pictures consisting of back pain, progressive gait difficulty, lower extremity weakness and hyperreflexia. Imaging studies in both cases showed the presence of a perimedullary mass with expansion of the bony spinal canal at T10-T12. Angiography demonstrated almost identical vascular lesions that in each case was being fed by a single left-sided T8 fistulous vessel that expanded into a giant perimedullary venous pseudoaneurysm. Each malformation was successfully obliterated using endovascular techniques, balloon occlusion of the feeding vessel in the first case and coil embolization of the pseudoaneurysm in the second. Following treatment the neurologic deficits resolved in both patients. These young siblings represent the first known case of familial occurrence of very similar giant perimedullary venous pseudoaneurysms of the thoracic spinal cord.

Optimization of the operative corridor for the resection of craniopharyngiomas in children: the combined frontoorbitozygomatic temporopolar approach. Technical note.

Complete extirpation of tumor remains the primary goal of neurosurgeons in treating intracranial craniopharyngiomas. The intimate relationship of these lesions with the structures of the skull base and the difficulties of obtaining adequate operative visualization often make total removal an elusive goal. The authors describe the use of a combined fronto-orbitozygomatic temporopolar craniotomy to maximize the operative corridor and thereby increase the probability of maximum tumor resection without morbidity and mortality. They applied this approach in four children with craniopharyngiomas that involved the sellar and parasellar, third ventricle, cavernous sinus, and interpeduncular fossa regions. The surgical results are summarized with a presentation of pre- and postoperative imaging from two illustrative cases. A detailed description of the operative procedure is provided with a comparison to other previously described surgical approaches.