Eosinophilia-myalgia syndrome (L-tryptophan-associated neuromyopathy).

Histopathologic study of skeletal muscle biopsy in a patient with eosinophilia-myalgia syndrome following L-tryptophan use showed prominent lymphocytic perineuritis, neuritis, and perimysial fasciitis. The presence of perineuritis and neuritis provides a histopathologic basis for clinical features of neuropathy in eosinophilia-myalgia syndrome and occurred in conjunction with a fasciitis or interstitial myositis that was predominantly perimysial and focally endomysial.

Factors determining the degree of endometrial exfoliation and their diagnostic implications in endometrial adenocarcinoma.

Since the degree of endometrial exfoliation largely determines the ability of cytology to diagnose endometrial adenocarcinomas by the study of cervicovaginal smears, five features of their subsequent histologically diagnosed endometrial adenocarcinomas were studied in 28 patients whose smears contained endometrial cells. The surface area occupied by abnormal endometrium was found to be an important determinant in the degree of endometrial exfoliation. The tumor grade, endocervical involvement, pattern of growth and squamous-cell component were all factors that influenced cellular shedding. The extent of myometrial involvement did not affect the likelihood that an endometrial adenocarcinoma would be diagnosed cytologically prior to histologic examination.

Cytopathologic features of adenoid cystic carcinoma. Case report and literature review.

A case of primary bronchial adenoid cystic carcinoma in a 40-year-old white female is reported. Cytologic findings in a bronchial wash specimen obtained at the time of bronchoscopic examination are described, with an emphasis on correlation with the histologic features of the tumor. The literature is briefly reviewed with respect to the occurrence of primary adenoid cystic carcinoma in the bronchial tree.

Thiamine triphosphate levels and histopathology. Correlation in Leigh disease.

Thiamine and thiamine triphosphate (TTP) values were assayed in various brain regions in 11 controls and 13 patients with subacute necrotizing encephalomyelopathy (SNE, Leigh disease). The TTP values of normal brain were 5% of the total thiamine value. The relative TTP (or % TTP) level was consistently low in the pons, midbrain, and cerebellum of all the SNE brains. Twenty-five percent of the SNE brains had normal TTP levels in the frontal region. The TTP values correlated with the degrees of pathologic involvement in all sampled regions of the brain except the cerebellum. The concentration of thiamine in the mammillary bodies exceeded its concentration elsewhere in both control and SNE brains. The finding of low TTP levels in morphologically abnormal regions supports the hypothesis that TTP deficiency is etiologically related to SNE.

Infantile diffuse cerebral degeneration with hepatic cirrhosis.

Four children had progressive degeneration of the cerebral cortex, with hepatic cirrhosis. They and four previously described ones, are representative of a distinct form of hepatocerebral degeneration. Onset of the neurological disorder is between ages 1 and 3 years, at times with mild developmental delay. Explosive onset of intractable convulsions, leaving the child in a stuporous and demented state, is characteristic. Generalized hypotonia or hemiparesis were observed in several affected children. Clinical evidences of hepatic disease, including ascites and jaundice, occurred late, if at all. The illness ended fatally within ten months of onset of convulsions. Pathological findings in the brain are neuronal loss and gliosis, in a pattern that is indistinguishable from that in degeneration of the cerebral gray matter in infancy (Alpers disease). The hepatic lesions consist of cirrhosis or of subacute hepatitis, with superimposed fatty infiltration of hepatocytes. The disorder is genetically determined, with recessive inheritance.

Stercoraceous perforation of the cecum: report of two cases.

Two cases of stercoraceous perforation of the cecum with adjacent mass formation simulating a neoplasm are described. The predisposing factors for the formation of fecalomas and the mechanisms of perforation are discussed and a review of the literature on this subject is presented. There were no demonstrable predisposing colonic lesions to account for the fecaloma formation in these casesmboth patients recovered completely after surgical treatment of the lesions.

Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver.

The clinical, biochemical, and pathological features of an unusual expression of infantile spongy degeneration of the central white matter are presented with emphasis on neuropathological observations. The topographical distribution of the spongy change along with the observed defect in myelination were such as to suggest an arrest in development of the white matter in late foetal life. Of additional interest, in the present case, is the observed deposition of glycogen in the brain, heart, and liver along with a markedly fatty liver. Our findings are compared with those in other cases of so-called spongy degeneration, as well as with certain of the aminoacidurias and, as a consequence, we wish to suggest that the basic pathogenetic factor probably lies in a disturbance of the biochemical energy supply system rather than in a disorder of myelin lipid metabolism per se.