Mixed connective tissue disease. A clinical, histologic, and immunofluorescence study of eight cases

OBJECTIVES: The diagnosis of solvent-induced chronic toxic encephalopathy is commonly based on case histories of exposure to solvents, symptoms, and deficits on psychometric tests. It has previously been demonstrated that long-term solvent-exposed workers have disturbances of the equilibrium system. The correlation between these disturbances and the diagnosis of chronic toxic encephalopathy has been analyzed in the present study. MATERIAL AND METHODS: Sixty men, consecutively admitted due to the suspicion of this syndrome, were investigated and classified into 3 groups--solvent-induced chronic toxic encephalopathy, incipient chronic toxic encephalopathy and nonchronic toxic encephalopathy. They were all examined using an otoneurological test battery, including analysis of saccades, smooth pursuit, visual suppression of the vestibular ocular reflex, and dynamic posturography. RESULTS: Compared with healthy referents several of the subjects, even in the nonchronic toxic encephalopathy group, showed a reduced visual suppression ability, a prolonged latency of saccades, and pathological posturographic results. Some otoneurological tests correlated with the duration of exposure and the results of psychometric tests representing memory and perceptual skills. Nevertheless, there was no significant group correlation between the otoneurological findings and the diagnosis of chronic toxic encephalopathy. CONCLUSION: Disturbances revealed by an otoneurological investigation have so far not been considered in the diagnosis of chronic toxic encephalopathy. Our results indicate that an otoneurological test battery adds worthwhile information about lesions within the brainstem-cerebellar complex not revealed by a psychometric investigation.     

Postpartum thyroiditis and Basedow disease

BACKGROUND: The postpartum period is characterized by a rebound in autoimmunity secondary to immune tolerance induced by pregnancy, creating favorable conditions for flare up of Graves' disease or autoimmune thyroiditis. Postpartum thyroiditis is a recognized clinical entity. CASE REPORT: Six years after onset of Graves' disease treated with antithyroid drugs, a 25-year-old woman had a high serum level of antithyroperoxidase antibodies a few months before she became pregnant. Six weeks after delivery, she developed signs of hyperthyroidism and goiter. The diagnosis of postpartum thyroiditis was retained. Her condition regressed spontaneously to euthyroidism then hypothyroidism. DISCUSSION: The therapeutic options involved underline the importance of distinguishing between Graves' disease and postpartum thyroiditis. The diagnosis of postpartum thyroiditis is based on history taking, clinical findings, and laboratory tests, especially isotope uptake.     

Secretory meningioma: clinical, histologic, and immunohistochemical findings in 31 cases

BACKGROUND: Secretory meningioma is a rare histologic variant characterized by a unique epithelial differentiation of meningothelial cells resulting in the production of hyaline inclusions. Most previous reports have presented single case observations. The authors selected 31 cases for a clinicopathologic study to characterize this type of tumor further. METHODS: Clinical data were compiled and the extent of peritumoral edema was assessed from preoperative computed tomography or magnetic resonance imaging scans. Preparations of surgical specimens of all tumors were studied after both conventional histologic and immunohistochemical preparations were made. Immunostaining was performed by either the avidin-biotin complex method or the alkaline phosphatase-antialkaline phosphatase method using 22 primary antibodies. RESULTS: In the tumor collection used in this study, secretory meningiomas represented 3% of meningiomas. The female-to-male ratio was 9:1. Most tumors were located at the sphenoid ridge or at the frontal convexity, and recurrences were not observed. Eighty-four percent of tumors presented with slight to marked peritumoral edema. The MIB-1 staining index showed a mean of 3.8%. Inclusions and surrounding cells consistently expressed epithelial membrane antigen, cytokeratins, carcinoembryonic antigen, and carbohydrate antigen 19-9. In decreasing frequency, they also contained alpha1-antitrypsin, immunoglobulin (Ig)A, alpha1-antichymotrypsin, IgM, and IgG. Cells positive for vimentin and S-100 did not contain inclusions. All tumors were positive for progesterone receptors. Macrophages were stained with antibodies to factor XIIIa, human leukocyte antigen-DR, and alpha1-antitrypsin. In 64% of cases, tumor vessels lacked expression of glucose transporter protein 1. CONCLUSIONS: The classification of secretory meningioma as a distinct variant has been justified on clinical, histologic, and immunohistochemical grounds. The unique epithelial features call attention to the broad spectrum of differentiation properties found in meningiomas.     

Effect of non-erupted third molars on roots of approximal teeth. A radiographic, clinical and histologic study

This study was undertaken to evaluate clinically and histologically root resorption in extracted human second molars in close proximity to non-erupted third molars. The control group consisted of extracted second molars that were proximal to fully erupted third molars. Eight out of the 11 teeth in the study group presented different degrees of radiographic root resorption, nine presented clinical resorption, and all 11 had histologic evidence of root resorption. In the control group, no signs of root resorption were seen radiographically or clinically. Histologically, limited sites of resorption were identified in all teeth, which were partially repaired by cellular cementum. Histologic observation of study specimens revealed root surface resorption in 10 out of the 11 teeth, one showing replacement resorption as well. Inflammatory resorption was observed in the three most advanced cases in the study group. Reparative cementum partially lining resorbed areas was evident in all teeth with surface resorption. Within the limits of this study, radiographic identification of distal root resorption of second molars in close proximity to non-erupted third molars appears reliable. The findings may support the hypothesis that the presence of a non-erupted third molar in close proximity to the distal root of the second results in root resorption.     

Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases

The role of 5-hydroxytryptamine (5-HT) in neural reflexes regulating secretion was examined in muscle-stripped segments of guinea-pig colon set up in modified flux chambers. A 15-microL pulse of 5-HT (100 microM) to the mucosal bath (1.5 mL), which was continuously perfused, evoked an increase in short-circuit current (Isc). The 5-HT-induced increase in Isc was inhibited by tetrodotoxin, N-acetyl-5-hydroxytryptophyl-5-hydroxytryptophan amide (5-HTP-DP), GR82334 and atropine, but not by tropisetron. 5-HTP-DP reduced the response to a 5-HT pulse over the concentration range of 1 nM to 1 microM. The Isc response to a 5-HT pulse was unaffected by the cyclooxygenase inhibitor, piroxicam. This contrasted with a reduction in the Isc response to mucosal stroking with a brush by piroxicam. The results suggest that a 5-HT pulse, like mucosal stroking, activates a secretory reflex that includes tachykinin and cholinergic neurons but, unlike mucosal stroking, does not release prostaglandins.     

Kaposi's sarcoma following long-term immunosuppressive therapy: clinical, histologic, and ultrastructural study

Multifocal Kaposi's sarcoma in a patient with chronic myeloid leukemia treated with busulfan, a cytostatic and suppressive drug, is reviewed. After five years of treatment, during which temporary remissions occurred, the patient experienced a relapse of leukemia and a considerable immune deficiency. This was expressed by a decrease in the ratio of CD4/CD8 lymphocytes in the peripheral blood. The relation of Kaposi's sarcoma with leukemia, as well as with the state of immunity in this case, does not evoke any doubts. Verification of oncologic treatment brought about a remission of leukemia, an improvement in the patient's immune state, as well as an inhibition of new foci of the Kaposi's sarcoma in the skin in the course of a few months of follow-up evaluation.     

A spindle cell varient of thymic carcinoid tumor. A clinical, histologic, and fine structural study with emphasis on its distinction from spindle cell thymoma

We describe the clinical, histologic, and fine structural features of two thymic carcinoid tumors that had a major spindle cell component, and present the reason for classifying our two cases as variants of thymic carcinoid tumors. These tumors pursued an aggressive clinical course, demonstrated histologic features such as vascular invasion and mitotic activity, ultrastructurally contained numerous dense-core granules, and lacked prominent tonofilaments and well-formed desmosomes. Radiotherapy, even for apparently encapsulated cases, may be indicated for this aggressive mediastinal neoplasm, which can be clearly separated from the thymoma by clinical, histologic, and fine structural criteria.     

Conservative management of epidural haematomas. Prospective study of 15 cases

Conservative management of epidural haematoma (EDH) depends on a balance between expansion and resorption rate of the clot. 15 patients with EDH whose CT scans demonstrated a small EDH and were asymptomatic or with minor symptoms or with a delayed diagnosis were treated conservatively. The thickness of haematoma ranged between 4.9-40.8 mm. In two patients, the haematoma extended from the posterior fossa to the supratentorial region. In 7 patients, additional intracranial pathology was detected. None of the patients had neurological deterioration on follow up. The second CT was performed on second day at the earliest, in fourth week at the latest. We conclude that the patients with EDH who are neurologically stable during the first 24 hours after trauma, with small EDH and with minor or no symptoms or signs, might be candidates for conservative management. An absolute precondition for conservative management is close supervision of the patient.     

The Papillon-Lefèvre syndrome. A clinical and ultrastructural study of 2 cases

Two cases of Papillon-Lefèvre syndrome, a rare hereditary affection of unknown etiology, whose clinical signs are hyperkeratosis palmoplantaris and parodontitis, are described. The results obtained with the ultrastructural examination of the lesions are shown. A therapeutic protocol which can reduce the evolution of the pathology, is also suggested.     

Striato-nigral degeneration. Apropos of a clinical, therapeutic, and anatomic study of 2 cases

The authors report two anatomo-clinical cases of striato-nigral degeneration with trial of L-Dopa; one of them included an olivoponto-cerebellar degeneration. The features of this degeneration are compared with the literature and related to heredo-degenerative diseases of the nervous system.     

Intrathyroidal lymphoepithelial cyst. A report of two cases not associated with Hashimoto's thyroiditis

Two cases of intrathyroidal lymphoepithelial cyst are described. Both of them were solitary, one being found incidentally in a patient operated on for a multinodular goiter, the other being clinically obvious as a cold nodule. They exhibited features of cysts of branchial cleft origin, i.e. squamous cell lining epithelium and abundant lymphoid tissue with reactive germinal centers. The thyroid gland parenchyma showed a discrete lymphoid infiltration consistent with the diagnosis of focal lymphocytic thyroiditis. In the first case a single epidermoid solid cell nest was found. The histogenesis of intrathyroidal lymphoepithelial cysts remains unclear, but their origin from cystically degenerated ultimobranchial body remnants (solid cell nests) seems to be most probable. This assumption is supported by a similar immunohistochemical profile of solid cell nests and epithelial cells lining the cysts and also by the presence of one solid cell nest in the proximity to the cyst in one of our cases.     

A comparative study of chemonucleolysis with recombinant human cathepsin L and chymopapain. A radiologic, histologic, and immunohistochemical assessment

STUDY DESIGN: Investigation of the effects of recombinant human cathepsin L on intervertebral discs and comparison with the effects of chymopapain. OBJECTIVE: To evaluate the effects of cathepsin L on intervertebral discs as an agent for chemonucleolysis. SUMMARY BACKGROUND DATA: Cathepsin L is a typical cysteine proteinase that belongs to the papain superfamily. It plays a major role in intracellular proteolysis and is not believed to induce anaphylactic reactions. METHODS: In vivo: Rabbit intervertebral discs were injected with recombinant human cathepsin L, its buffer solution, and chymopapain. After 1, 4, and 16 weeks the animals were killed, and radiologic and histologic examinations were performed. In vitro: The enzymatic actions of recombinant human cathepsin L and chymopapain on human intervertebral disc proteoglycans were examined immunohistochemically using antiproteoglycan antibodies. RESULTS: In rabbit models, roentgenography showed that disc spaces treated with cathepsin L and chymopapain had become narrower 1 week after injection. Histologically, loss of safranin-O staining was observed in the anulus fibrosus of discs treated with cathepsin L. After 16 weeks, nucleus pulposus had regenerated with chondrocyte-like cells, and the safranin-O staining characteristics of the matrix also had recovered. In an immunohistochemical study, all components of the proteoglycan stained weakly after chymopapain digestion. After cathepsin L digestion, unsulfated chondroitin and core protein staining was weaker, but the chondroitin 6-sulfate staining was unaffected. CONCLUSIONS: Cathepsin L seems to be an effective agent for chemonucleolysis. Its enzymatic action on proteoglycan appears to be different from that of chymopapain.     

15 years CA-125 antigen: usefulness and limits. Apropos of 3 clinical cases

Fifteen years ago, the first clinical assay of the serum tumour marker CA 125 was introduced as a commercial kit. This test is currently the most widely used tumour marker in gynaecologic oncology. Studies have shown that the CA 125 is a valuable tool in the follow-up management of ovarian cancer. As a teaching exercise we present the analysis of three clinical cases, in which CA 125 was determined. Two of these patients did not have an ovarian cancer. It appears that this tumour marker very often is not only used in the follow-up of ovarian cancer, but also as a screening test and as a diagnostic tool. This inappropriate use (due to the lack of specificity and therefore many false positive results) can be avoided by considering it's limits.     

Autosomal recessive cornea plana. A clinical and genetic study of 78 cases in Finland

Occupational exposure to nickel by inhalation may result in impaired olfactory sense. Recent studies have shown that nickel is transported from the olfactory epithelium along the axons of the primary olfactory neurons to the brain. In the present study 63Ni2+ was applied in the olfactory chambers of pikes (Esox lucius) and the rate at which the metal was transported in the primary olfactory neurons was determined by beta-spectrometry. The results showed a wave of 63Ni2+ in the olfactory nerves, which slowly moved toward the olfactory bulbs. The maximal 63Ni2+ transport rate corresponding to the movement of the base of the wave front was found to be about 0.13 mm/h at the experimental temperature (10 degrees C). This rate of 63Ni2+ transport falls into the class of slow axonal transport. Radioluminography of tape sections of a pike given 63Ni2+ in the right olfactory chamber showed a selective labeling of the right olfactory nerve. The subcellular distribution of 63Ni2+ in the olfactory nerves and the olfactory epithelium of the pikes was studied in tissues subjected to homogenizations and centrifugations, and these methods were also used to examine the subcellular distribution of 63Ni2+ in tissues of the olfactory system of rats given the metal intranasally. It was found that the 63Ni2+, in both the pike and the rat, was present in the cytosol and also in association with various particulate cell constituents. Gel filtrations of the cytosols showed that the 63Ni2+ mainly was eluted at a Ve/Vo ratio corresponding to a MW of about 250. The same coefficient was obtained in gel filtrations performed with 63Ni2+ mixed with histidine in vitro. It is likely that the cytosolic nickel may be bound to histidine or possibly to other amino acids which are similar in size to histidine. Additionally, in the olfactory tissues of the rat the 63Ni2+ was partly present in the cytosol in association with a component with a MW of about 25,000. It is concluded that (i) 63Ni2+ is transported in the primary olfactory neurons by means of slow axonal transport, (ii) in this process the metal is bound to both particulate and soluble cytosolic constituents, and (iii) the metal shows this subcellular distribution also in other parts of the olfactory system.     

Gonadoblastoma: histologic, ultrastructural, and histochemical observations in five cases

In this series of gonadoblastomas it appeared that the germ cells were the motivating force underlying either tumor proliferation or regression. The ultrastructural morphology confirmed the presence of undifferentiated gonadal cells with active steroid synthesis by the interstitial cells. The Call-Exner-like bodies which showed extensive calcification were composed of basement membrane material containing a sulfated mucosubstance. A histochemical comparison with the noncalcifying Call-Exner bodies of a granulosa-cell tumor differed only in their glycoprotein content. The calcium deposits were identified as oriented hydroxyapatite crystals by electron diffraction, and it is proposed that the basement membrane material serves as a nucleation site for calcification. High serum testosterone levels were correlated with the presence solely of interstitial cells in one case. The finding of a gonadoblastoma without the presence of a Y chromosome contradicts earlier proposals concerning the requirement of a Y chromosome for germ cell proliferation.     

Lymphocytic infiltration in endometrial adenocarcinoma. Correlation with histologic differentiation, clinical staging and prognosis

Lymphocytic infiltration in endometrial adenocarcinoma was evaluated in curettings from 50 patients and correlated with histologic differentiation, clinical staging and prognosis. In spite of the absence of any significant correlation between lymphocytic infiltration and the various parameters investigated, the presence of a marked infiltrate in 46% of the cases suggests that in endometrial adenocarcinoma, as in other neoplasms, a cell-mediated immune response does indeed exist, but warrants further investigation by more refined methods.