Schilder's (1912) disease. Total cerebral blindness due to acute demyelination.

A 51-year-old woman became blind from involvement of both occipital lobes with a confluent lesion demonstrated by computed tomographic scan. Biopsy showed demyelination consistent with Schilder's 1912 variant of diffuse sclerosis. No cytoplasmic inclusions were found on electron microscopy.

Periventricular white matter lucencies in senile dementia of the Alzheimer type and in normal aging.

The significance of periventricular lucencies in the white matter on CT in demented patients is not understood. We studied the relationship of these changes to mental status of subjects with senile dementia of the Alzheimer type. A semiquantitative method showed more numerous and extensive lucencies in demented than in healthy elderly. Neuropathologic examination of five subjects with these changes and confirmed Alzheimer's disease revealed diffuse white matter pallor without infarction. There were no hypertensive vascular changes, although limited hyaline thickening was present.

Localized neurofibromatosis of the female genitourinary tract.

The internal female genital tract is rarely involved in neurofibromatosis. This report details a case of diffuse neurofibroma with prominent Wagner-Meissner body differentiation involving uterine cervix and endocervix, vagina, and vulva. The urinary bladder, urethra, and one ureter were also extensively affected. The anatomic localization of the lesions in this case is consistent with segmental neurofibromatosis restricted to the distribution of the second, third, and fourth sacral nerves. Failure to consider the possibility of genitourinary involvement in neurofibromatosis, the potential for localized manifestations in the absence of other easily recognized stigmata, and the subtle histologic features in small tissue biopsies may cause difficulty in diagnosis.

Juvenile pilocytic astrocytoma of the cerebrum in adults. A distinctive neoplasm with favorable prognosis.

Seven adults (15 years of age or older) with juvenile pilocytic astrocytoma of the cerebrum were treated at Barnes Hospital in St. Louis, Missouri, from 1950 to 1981. This group comprised 7% of the total adult population with well differentiated astrocytoma and astrocytoma with anaplastic foci treated over a 30-year period. All seven patients were less than 30 years old at the time of presentation. The tumors were characterized by distinctive histology, a predilection for the temporal lobe, the presence of a major cyst, and relative gross circumscription making total surgical resection feasible. In contrast to patients with diffusely infiltrating cerebral astrocytoma, those with juvenile pilocytic astrocytoma have had an extremely favorable course, regardless of treatment. Four were treated with surgery alone after total gross tumor excision, and three received postoperative irradiation after subtotal excision. Six were alive 27, 24, 17, 9, 5, and 2 years after initial surgery. One patient died 7 years after surgery and radiation as a result of cerebral radiation necrosis. Juvenile pilocytic astrocytoma of the cerebrum is a recognizable, distinctive entity with natural history and pathological characteristics similar to the relatively benign cerebellar astrocytoma of childhood.

The clinical significance of "adenoid" formations of neoplastic astrocytes, imitating metastatic carcinoma, in gliosarcomas. A review of five cases.

Five cases of supratentorial gliosarcomas occurring in middle-aged and elderly patients are reported. In the gliomatous portions of the tumors there were areas with clusters and ribbons of cuboidal cells forming a cribriform pattern and structures resembling ducts and glands of a metastatic adenocarcinoma. Where the adenoid structures were embedded in a mucoid matrix, an imitation of mucus-producing ("colloid") carcinoma was created. In one patient who had a large pelvic mass (later identified as a benign leiomyoma) the histology of the brain tumor suggested a metastatic müllerian carcinosarcoma. The pseudoglandular elements in each case could be identified as being composed of primitive astrocytes, partly by finding transitions to more mature and typical astrocytes and partly by positive staining with the immunoperoxidase method for glial fibrillary acidic protein (GFAP). The recognition of this morphologic variant of malignant glial tumors is important, particularly in patients of the age group in which primary carcinomas elsewhere in the body occur with increasing frequency. In such patients the correct diagnosis of their brain tumor can avoid the added post-craniotomy discomfort and expense of a "workup for a primary tumor."

Astrocytic tumors of the retina. Differentiation of sporadic tumors from phakomatosis-associated tumors.

A single, 1.3-cm, intraocular tumor, composed of interlacing, spindle-shaped astrocytes, involved the retina and optic disc of a 13-month-old girl. Neither the patient nor her family had stigmas of a phakomatosis. We found 42 previous cases of histologically documented astrocytic tumors of the retina. Twenty-four patients (57%) had tuberous sclerosis, six patients (14%) had neurofibromatosis, and 12 patients (29%) were otherwise normal. Patients with tuberous sclerosis usually had multiple, peripheral, retinal tumors containing giant "astrocytes." Patients with neurofibromatosis and otherwise normal patients more frequently had disc-based tumors. However, three patients (50%) with neurofibromatosis had multiple tumors, whereas multiplicity occurred in only one of the patients in the sporadic group. We conclude that if the tumor is single, is disc based, and lacks giant cells, the patient is not at great risk of tuberous sclerosis.

Cerebral astrocytic neoplasms in the adult: contribution of histologic examination to the assessment of prognosis.

Cerebral astrocytomas are the most common primary central nervous system (CNS) tumors in adults. As a group these tumors are characterized by histologic variability and inconstant prognosis. Nevertheless, microscopic examination of these tumors with identification of certain histologic features provides useful prognostic information. Evidence of anaplasia including necrosis, vascular endothelial proliferation, mitotic activity, cytologic pleomorphism, and foci of increased cellularity, when present in diffusely infiltrating astrocytic gliomas, is associated with aggressive behavior. In particular, the occurrence of tumor necrosis in anaplastic astrocytomas is reliably predictive of a highly unfavorable outcome. Specific histologic subtypes of cerebral astrocytoma with relatively benign course may also be identified. These include juvenile pilocytic astrocytoma, subependymal giant-cell astrocytoma, and, in some cases, pleomorphic xanthoastrocytoma. The critical influence of tumor location and the limitations imposed by potentially nonrepresentative biopsy material must be appreciated when assessing prognosis in cerebral astrocytomas. Prognostic data provided by histologic examination are useful in selecting treatment regimens and for evaluation of newly proposed therapies.

Neoplastic angioendotheliomatosis. Histologic, immunohistochemical, and ultrastructural findings in two cases.

Two cases of neoplastic angioendotheliomatosis are described. Both patients presented with subacute development of dementia punctuated by focal neurologic signs. Postmortem examination in both cases disclosed a vasculocentric distribution of neoplastic cells in many organs. Two characteristic histologic patterns were seen: (1) distension of vascular spaces by free-floating, intraluminal cells and (2) asymmetric, subendothelial tumor growth. Intravascular tumor and secondary vascular changes were associated with microinfarcts in involved organs. Immunohistochemical localization of factor VIII-related antigen in the neoplastic cells of one case provides evidence for their endothelial origin. The neoplastic cells in both cases exhibited similar but nonspecific ultrastructural features. In addition, dramatic fine structural changes including the acquisition of numerous pinocytotic vesicles and bundles of fine filaments with periodic densities were found in the non-neoplastic endothelial cells.

Anaplastic astrocytoma of the adult cerebrum. Prognostic value of histologic features.

Initial biopsy and resection specimens from 78 cerebral astrocytomas with anaplastic foci occurring in adults were examined for the presence of five specific histologic features traditionally considered indicative of anaplasia. Anaplastic astrocytomas with foci of tumor necrosis had been classified as glioblastoma multiforme and excluded from this analysis. Median actuarial survival was shorter for patients whose tumors contained vascular endothelial proliferation (3.5 years versus 5.5 years) or greater than 1 mitosis per 10 high power microscopic fields (1 year versus 5.5 years). No correlation with survival was found for estimation of nuclear pleomorphism and cell density or the presence of secondary structures. Vascular endothelial proliferation and increased mitotic activity may serve as prognostically significant histologic markers in cerebral astrocytomas of intermediate malignancy.

The Chiari II malformation: Part IV. The hindbrain deformity.

Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary "kinking"; anterior displacement and sequential sagittal compression of each protrusion by the protrusions posterior to it; compression of all protrusions by the posterior lip of foramen magnum and the posterior arch of C1; and associated cervical hydromyelia, cervical diastematomyelia, and cervical arachnoid cysts.

The value of radiation therapy in addition to surgery for astrocytomas of the adult cerebrum.

A retrospective review of 86 adults (age 15 years or older) treated from 1950 to 1979 for well-differentiated astrocytoma or anaplastic astrocytoma of the cerebrum at Washington University Medical Center-Barnes Hospital was undertaken to determine the influence of postoperative radiation therapy on survival and neurologic function. Analysis was facilitated by a temporal change in treatment approach, with reliance on surgery alone before 1960 and routine use of postoperative irradiation after 1970. Six patients had astrocytomas with a juvenile pilocytic histologic pattern. Outcome was uniformly favorable in these cases regardless of therapy (100% survival; median follow-up, 14 years). Actuarial survival for the 80 patients with diffusely infiltrating astrocytoma was significantly better with the addition of postoperative irradiation than with surgery alone (median survival, 5 versus 2.2 years, respectively; 5-year survival, 50% versus 21%, respectively). Posttreatment neurologic function was also superior in the group managed with surgery and postoperative irradiation. The relationship to survival of age, degree of histologic anaplasia, extent of surgical resection, tumor size, and radiation dose was also investigated.

Malignant mixed müllerian tumor of the uterus with neuroectodermal differentiation.

A case of malignant mixed müllerian tumor of the uterus with neuroectodermal differentiation is reported. The most notable feature of the tumor was its large component of central nervous system tissue containing both glial and neuronal elements. The islands of neural tissue were admixed with more conventional carcinomatous and sarcomatous elements typically found in tumors of this type. The neuroectodermal component was conspicuous in the residual uterine tumor removed after radiotherapy, but not in the initial biopsy specimens. The patient died after developing local recurrence and distant metastases, which were treated with chemotherapy.

Chronic varicella-zoster virus myelitis without cutaneous eruption in a patient with AIDS: report of a fatal case.

We describe a fatal case of varicella-zoster virus myelitis that was preceded by neurological symptoms for 10 months in a patient with human immunodeficiency virus infection and an extremely low CD4 cell count (20/microL). The patient was also receiving chronic acylovir therapy for suppression of herpes complex. Despite chronic unilateral periauricular and facial pain, which was later accompanied by upper- and lower-extremity weakness, a cutaneous eruption never developed. It is hypothesized that a blunted inflammatory response in the spinal cord--possibly related to a very low CD4 cell count--and long-term acylovir administration might have contributed to the atypical manifestation might have contributed to the atypical manifestation of varicella-zoster virus-related neurological disease in this immunocompromised patient.

Cysts of the subependymal germinal matrix: sonographic demonstration with pathologic correlation.

Five neonates who were born at term and who had well-defined cystic lesions in the subependymal germinal matrix demonstrated by real-time cranial ultrasonography are reported. None had evidence of subependymal or intraventricular hemorrhage. Two of the neonates had congenital viral infections, one each with rubella virus and cytomegalovirus. Two of the other three neonates died, and pathologic examination of their brains confirmed the presence of the cysts and showed no evidence of prior subependymal hemorrhage. Cranial CT examinations of three patients failed to detect the subependymal cysts. Although central liquefaction in an area of prior hemorrhage is probably the most common cause of subependymal cyst formation in neonates, other conditions such as neurotropic infection should also be considered.

Sickle cell anemia and central nervous system infarction: a neuropathological study.

CNS infarcts were demonstrated in 12 of 24 autopsied patients with sickle cell anemia. The infarcts occurred most extensively in the territory supplied by the distal branches of the internal carotid artery, especially the anterior-middle cerebral artery boundary zone. They were regularly associated with organizing and recanalizing thrombi involving the distal cervical and proximal intracranial divisions of the internal carotid system. On the basis of these findings, we infer that the pathogenesis of the infarction involves perfusion failure or intraarterial embolization in addition to intravascular sickling.

Hypoxic-ischemic encephalopathy in term infants: diagnosis and prognosis evaluated by ultrasound.

The results of cranial ultrasonography in 32 term infants who had hypoxic-ischemic encephalopathy (HIE) and in 26 control infants were reviewed. Small or nonvisualized ventricles were present in 94% of the patients with HIE and in 62% of the control subjects. In 20 of the 32 patients (63%) with HIE, ultrasound demonstrated parenchymal abnormalities; the most common finding was periventricular hyperechogenicity (12/32 patients). There was a single false positive result in the control group. Fifty per cent of patients with HIE who had abnormalities demonstrated on ultrasonograms died (within 15 days of birth), whereas none of the infants who had normal ultrasound findings died. Moreover, 80% of surviving patients who had abnormal ultrasound findings had residual neurological deficits versus only 11% of those with normal ultrasound findings. Our results indicate that ultrasound is effective in detecting parenchymal changes in patients with HIE, parenchymal echoes are a more reliable sign of HIE than ventricular size, and the finding of parenchymal lesions on ultrasound scans appears to be predictive of abnormal motor development.

Adnexal metaplasia in carcinoma in situ of the skin.

Two cases of epidermoid carcinoma in situ of the skin, one with mucinous metaplasia and the other with sebaceous metaplasia, are reported. These unique cases suggest a spectrum of differentiation possible by neoplastic epidermal keratinocytes, which may help in the understanding of the histogenesis of certain skin neoplasms.

Suprasellar germinoma: radiation therapy.

From 1974 to 1984, nine patients with suprasellar germinoma were treated with megavoltage radiation therapy. The entire craniospinal axis was irradiated in all patients, with median doses of 45.6 Gy, 31.6 Gy, and 25.2 Gy delivered to the tumor volume, whole brain, and spinal cord, respectively. Two patients have died (14, 11.5 months) without evidence of tumor: one of uncontrolled endocrine dysfunction due to patient noncompliance, the other of hyperosmolar coma. There have been no tumor recurrences with a median 68-month follow-up among seven survivors. The dose usually recommended for treatment of intracranial germinoma is 50-55 Gy. Our data suggest that 45 Gy may be sufficient, if treatment volume is adequate. Posttherapy endocrine testing is also essential.