Cytomegalovirus in the nervous system of patients with the acquired immune deficiency syndrome.

Clinicopathological features of infection of the nervous system by cytomegalovirus (CMV) in 31 patients with the acquired immune deficiency syndrome (AIDS) are reviewed. Neuropathology was variable, ranging from rare isolated CMV inclusions in brain without associated inflammation or necrosis, to severe necrotizing ependymitis and meningoencephalitis. In 1 patient, CMV had produced a necrotizing meningoradiculitis which presented clinically as ascending paralysis. In the brains and spinal cords of 6 patients, evidence of human immunodeficiency virus (HIV) infection of neural parenchyma was seen in close proximity to CMV infection. Both viruses individually or together were associated with low grade (microglial nodule) encephalitis. In retrospect, the diagnosis of CMV had been a difficult one to make clinically in neurologically impaired patients with AIDS. The results suggest that CMV may also localize in the nervous system without significant clinical sequelae. Imaging studies and analysis of cerebrospinal fluid revealed abnormalities in many patients, but none of them (short of culture of CMV itself in two cases) appeared to be specific to this neurological complication of the immunodeficiency.

Cytochemical and competitive protein binding assays for estrogen receptor in breast disease. A comparative study of 62 cases.

Estrogen receptor content of breast lesions was estimated using a fluorescent cytochemical technique and a competitive protein binding assay. Of 48 cancers examined, an equal proportion contained significant quantities of receptor by either method (62.5%). The concordance between methods for individual patients was also 62.5%. A greater proportion (26%) of patients younger than 45 years of age had receptor-positive cancers using the cytochemical method than were found by the biochemical method (10%). Benign breast disease was also studied using the fluorescent cytochemical method. A greater proportion of lesions containing estrogen receptors was found compared with that cited in the literature for the competitive protein-binding assay. Because of the methodologic simplicity of the fluorescent cytochemical method, further study for routine use is indicated.

Constriction of the umbilical cord as a cause of fetal demise following midtrimester amniocentesis.

Two cases of constriction of the umbilical cord resulting in fetal demise following midtrimester amniocentesis are presented. In both cases, real-time ultrasonography prior to amniocentesis revealed a viable fetus. Fetal demise was identified immediately following the procedure in the first case and one month later in the other. A localized constriction at the fetal end of the umbilical cord in both, with torsion of the constricted segment in the second case, was observed. Wharton's jelly was noted to be deficient in this segment of the cord in the first case. The mechanism of fetal demise is discussed. It is suggested that this abnormality should be considered when fetal demise follows midtrimester amniocentesis.

Cytopathologic presentation of cerebral histiocytosis.

Histiocytoses involving the central nervous system are rare, and those in which the brain and meninges are the the only systems affected are rarer still. This paper presents such a case, in which cytologic study of cerebrospinal fluid during life showed large numbers of abnormal histiocytes and necropsy showed infiltration of brain and meninges by masses of similar cells, without other systems being involved. The cytologic features and differential diagnosis are discussed.

Cell clusters in urinary cytology.

Clusters of cells in cytologic preparations from the urinary tract have been commented on repeatedly. To determine their significance we studied 88 patients with documented papillary carcinomas, Grades I-IV, who had cytologic examination of their urine. We defined two distinct cluster types, "rounded", and "papillary", which were easily distinguishable from artifactual excoriated cell aggregates. By cytologic techniques 64 per cent of the specimens were positive and 18 per cent suspicious for malignancy, with the lowest sensitivity for Grade I tumors, and the highest for Grade IV tumors. Clusters were observed in 39 per cent of the cases, and also were more common in the most undifferentiated tumors. However, many cases which were cytologically positive had no clusters, and in only one case were clusters present without other cytologic features of malignancy. It appears that the presence of cell clusters is not a more sensitive indicator of papillary lesions than usual cytologic criteria applied to single cells. Clusters may indicate a higher grade tumor.

Disseminated choroid plexus papilloma. An ultrastructural study.

Choroid plexus papilloma is a rare intracerebral neoplasm that occasionally disseminates along the neuraxis. The fine structure of such a metastatic papilloma appears to correspond very closely to that of normal choroid plexus. Lack of capillary endothelial pores is the only meaningful difference. There does not appear to be an ultrastructural feature that is unique to disseminating choroid plexus papilloma.

Systemic giant-cell histiocytosis: report of a case and a review of the adult form of Letterer-Siwe disease.

The multiple classifications of histiocytic disorders, and the lack of specificity in their application, have degraded the utility of such diagnostic terms as "Letterer-Siwe Disease" and "Malignant Histiocytosis". A case is presented of a systemic histiocytic proliferation with erythrophagocytosis and giant-cell formation which does not fit into any presently accepted diagnostic niche. No similar case was found in the literature. Moreover, it appears that the entity "Adult form of Letterer-Siwe disease" has been uncritically accepted on the basis of a few poorly documented cases, and should be abandoned.

Cerebral lymphomatoid granulomatosis. A report of two cases, with disseminated necrotizing leukoencephalopathy in one.

Neuropathologic findings in 2 cases of cerebral lymphomatoid granulomatosis with sequelae are presented. A 30-year old male with macular rash and pulmonary lymphomatoid granulomatosis responded to Prednisone terapy but developed acute intracranial hypertension with coma. A necrotizing hemorrhagic lesion was evident in the left putamen surrounded by diffuse and perivascular atypical lymphoplasmacytic infiltration. An 18-year old girl developed pulmonary lymphomatoid granulomatosis, diplopia, slurred speech and right hemiparesis. Brain scan, angiography and EEG suggested a left fronto-parietal mass assumed to represent lymphomatoid granulomatosis. She responded well to cerebral irradiation, intrathecal methotrexate and cytoxan but relapsed with seizures and increasing respiratory insufficiency. At autopsy, stigmata of cerebral lymphomatoid granulomatosis were absent but a parenchymatous degeneration consistent with disseminated necrotizing leukoencephalopathy following antileukemic therapy in children, was found.