Charles E. Rawlings

Ependymomas: A clinicopathologic study

Since 1924, when ependymomas were first classified as a distinctive glial neoplasm by Bailey, much has been published concerning these tumors, but there are important points of interest that are still not clear. In order to study more fully the clinical and pathologic characteristics of the ependymoma, we identified 62 patients with histologically proven neoplasms. Twenty-two were supratentorial, 21 were infratentorial, and 19 were intramedullary spinal cord tumors. These groups had mean ages of 17, 7, and 41 years, respectively, at the time of first symptoms. The presenting and accompanying symptoms were related to location and included headaches, nausea, visual changes, hemiparesis, and neck, back, and radicular pain. Neurological signs included papilledema, nystagmus, gait disturbance, cranial nerve palsies, altered mental status, paraparesis, and sensory dysfunction. Radiologic modalities of particular importance included computed tomography and myelography. Surgery and radiation therapy were the primary treatment modalities with median survival times from first symptoms being 92, 36, and 117 months for the above groups, respectively. Based on computer-generated survival curves, several characteristics significantly affected survival. These included tumor site, age, and neuraxis metastases. In patients with supratentorial tumors, cranial nerve palsies, microcystic changes, and mitotic figures were important, while in patients with infratentorial tumors, widened sutures, increased head circumference, age, epithelial features, and subependymal features significantly affected survival. Patients who had complete gross resection of a spinal cord tumor had no recurrences or mortality.

Clinicopathologic correlations in the oligodendroglioma

To determine the prognostic significance of histologic variables in oligodendroglial neoplasms, the presence and degree of 15 such variables were correlated with postoperative survival rates in 71 patients. By univariate analysis, prognostically significant factors, in order of decreasing importance, were mitoses (log), necrosis, nuclear cytologic atypia, vascular hypertrophy, and vascular proliferation. When studied by stepwise regression, necrosis and the number of mitoses contained all of the prognostically useful information. When each of the five variables significant by univariate analysis was tested in the Cox model by adding a variable to the model containing the other four, necrosis was found to be the only independently significant variable. There were significant positive pairwise correlations between each of the five significant histologic variables except for cytologic atypia with necrosis. The only histologic variable with a significant association with older age was the number of mitoses. These results suggest that necrosis and, to a lesser extent, the mitotic count are features that, in the appropriate setting, can be used to identify the “anaplastic” oligodendroglioma.

Ganglioglioma: a clinical study with long-term follow-up

Gangliogliomas are uncommon tumors of mixed neoplastic glial and neuronal elements. Because of their low incidence, few large series exist that fully describe the clinical characteristics of patients afflicted with this tumor. We have reviewed the medical records of 20 patients at Duke University Medical Center with histologically proven gangliogliomas. These patients typically presented within the first three decades of life and their most common presenting symptom was seizures. Therapies included surgical resection, either partial or total, radiation therapy, and/or chemotherapy. Long-term follow-up was achieved by chart review and by telephone interview. Patients who underwent gross total resection alone seemed to fare the best when comparing all treatment groups, and we therefore recommend this as the main form of treatment.

Oligodendroglioma. An analysis of the value of radiation therapy.

The role of radiation therapy in the treatment of supratentorial oligodendrogliomas is controversial. To evaluate the role of radiation therapy, the Duke University Medical Center series was retrospectively analyzed. Clinical history, radiation dosages, and pathologic materials were reviewed. Seventy‐one patients were identified as having histologically proven oligodendroglioma. Analysis of the patient population demonstrated it to be similar in all major parameters to other populations previously reported in the literature. Multivariate statistical analysis of the demographic, clinical and radiographic variables of these patients showed that a poorer prognosis was associated with persons of increased age (P = 0.052) and black persons (P = 0.014), and in those with papilledema (P = 0.07), hemiparesis (P = 0.001), intellectual deficits (P = 0.0002), and necrosis (P = 0.041). All patients had a surgical procedure as first treatment while 18 and three patients, respectively, underwent a second and third surgical procedure. Thirty‐seven patients had a subsequent course of radiotherapy. Univariate and multivariate statistical analysis comparing the patients treated with surgery alone those treated with surgery plus radiotherapy revealed no significant population or prognostic differences between the groups. The median times until clinical deterioration were 39 versus 27 months, the median times until documented tumor recurrence were 27 versus 28 months and the median survival times were 4.5 versus 5.2 years, for nonirradiated versus irradiated patients. These data, from a large and rigidly evaluated population, demonstrated no statistically significant difference in the symptom‐free interval, time until tumor recurrence, or survival between the groups nor did radiation appear beneficial to any subgroup evaluated. The results suggest the need for a prospective clinical trial to evaluate the true role of radiation therapy in the treatment of this tumor.

A case of Ollier's disease associated with two intracranial gliomas.

Olliers disease or multiple enchondromatosis is a deforming dysplastic disease of cartilage involving primarily the metaphyses and diaphyses of long bones. It is only rarely associated with sarcomatous degeneration of the enchondromas or other generalized neoplasms. A related disease, Maffuccis syndrome, is, however, associated with generalized tumors. We present the case of a 29-year-old, albino, black man with Olliers disease who, as a child, underwent a number of orthopedic procedures for multiple limb deformities and fractures. At age 25, he developed hydrocephalus, progressive cranial nerve palsies, and a large enchondroma of the skull base. He subsequently underwent multiple shunt procedures and two suboccipital craniectomies. Eighteen months later, a brain computed tomographic (CT) scan revealed an intracerebellar mass, which was found to be an anaplastic astrocytoma. Two years later, he developed a right hemiparesis and sensory dysfunction with a diffuse supratentorial mass on CT scan. A stereotactic biopsy showed this to be a similar anaplastic astrocytoma. The literature concerning Olliers disease is reviewed, and the intracerebral lesions associated with both Olliers disease and Maffuccis syndrome are examined.

The DREZ Procedure: An update on technique

The DREZ operation was first done in 1975 on a patient with arm pain following a brachial plexus avulsion. Since then approximately 500 patients have undergone the DREZ procedure under our care for treatment of various pain syndromes including deafferentation pain, post-herpetic neuralgia, and post-paraplegia pain. We report several modifications in instrumentation and technique. Currently, we use two types of electrodes for lesion production. The first is the standard 0.25 mm diameter, thermocouple, temperature monitoring electrode which has a 2 mm long tip for introduction into the spinal cord. A second type, recently modified from the original, is used only for lesioning the nucleus caudalis in patients with trigeminal post-herpetic neuralgia. Its tip is 3 mm long with insulation along the first 1 mm. This allows lesioning of the caudalis nucleus while sparing the more superficial spinocerebellar tracts. We no longer lesion only the dorsal root entry zones at each root level but include all the contiguous substantia gelatinosa between roots. With lesions only 1 mm apart this greatly increases the number of lesions and decreases the incidence of incomplete postoperative pain relief. In patients undergoing caudalis lesioning, we make two rows of lesions, one above the other, from C2 to slightly above the obex. This prevents sparing of the facial midline with resultant residual pain. Finally, lesions are made by heating the electrode tip to 75 degrees C for exactly 15 sec, thus allowing for a more uniform lesion. With these modifications, we have a decreased incidence of incomplete pain relief as well as a decreased incidence of complications, especially in patients undergoing caudalis lesioning.

Franz Josef Gall and his contribution to neuroanatomy with emphasis on the brain stem

Franz Josef Gall, born in 1758, studied medicine in Strasbourg and Vienna and focused his research in two areas. His most notorious research was in craniofacial morphology that gradually evolved into the pseudoscience of phrenology. His second research area was neuroanatomy. Although Gall is usually best remembered for his inglorious introduction of phrenology, his neuroanatomic contributions have more lasting significance. In this vignette we explore Galls neuroanatomic contributions especially as they relate to brain stem anatomy. By means of blunt dissection he was the first to describe the origins of several cranial nerves, including the trigeminal nucleus. It will be apparent that Gall--his dubious introduction of phrenology notwithstanding--was a pioneer in the description of brain stem anatomy.

THE HISTORY OF NEUROSURGICAL PROCEDURES FOR THE RELIEF OF PAIN

Pain has been a major medical problem from the beginning of recorded history. Since the earliest medical writings, there have been innumerable procedures designed to relieve pain and its suffering. In this study, we have reviewed both the early medical writings of various civilizations and the first modern publications, to compile a history of neurosurgical procedures for the relief of pain.

Traumatic Extradural Spinal Cysts: A case report and review of the literature

Extradural spinal cysts, although unusual, are well-documented entities. The majority are of congenital origin while only a few with a clear cut traumatic pathogenesis are reported. We present a case of a traumatic extradural spinal cyst which presented as a progressive radiculopathy following a penetrating injury. We also review the literature concerning traumatic extradural spinal cysts and show that they can occur following a wide variety of spinal trauma. The trauma must involve sufficient force to cause a dural rent and iatrogenic causes are included. The differences and similarities between traumatic and congenital cysts are shown, and treatment is discussed. The prognosis following surgical resection is shown to be excellent.

Syringomyelia in a man with sarcoidosis

Sarcoidosis, generally a systemic ailment, uncommonly affects the central nervous system. It only rarely affects the spinal cord alone or in combination with other areas. Syringomyelia has never been associated with sarcoidosis. We report the case of a 32-year-old man with sarcoidosis who presented with surgically verified syringomyelia. The pertinent literature is reviewed. The mechanisms by which sarcoidosis could cause a syrinx are discussed.