Convergence substitution for paralysed horizontal gaze.

Three patients with paralysed horizontal gaze are presented. Involuntary use of convergence to assist horizontal gaze was noted as a late feature. All patients showed (1) unilateral or bilateral horizontal gaze palsy (two patients had one and a half syndrome, the other had bilateral nuclear sixth nerve palsies), (2) adduction of both eyes on attempted gaze into the paralysed field, (3) miosis which coincided with adduction. Convergence substitution should be considered in the differential diagnosis of gaze induced strabismus.     

Sudden total deafness in sickle cell disease

Sickle cell disease is a world-wide problem which has been noted to cause high tone sensorineural hearing loss. We report a case with sudden onset bilateral hearing loss which progressed to total sensorineural deafness. To our knowledge there has been no report of such a case occurring previously.     

Congenital absence of extraocular muscles: MR demonstration.

We describe a case of an infant with complete congenital absence of the extraocular muscles, noted at surgery and confirmed by multiplane MR, which found no evidence of extraocular muscle tissue. This almost certainly represents an extreme form of bilateral congenital fibrosis syndrome.     

The radiologic manifestations of alveolar soft-part sarcoma

Alveolar soft-part sarcoma is a rare soft-tissue tumor of unknown cellular origin that is characterized histologically by its organized "pseudoalveolar" pattern. The radiologic findings in 11 patients with this neoplasm were reviewed. The six men and five women were 16-48 years old (mean, 27 years). Nine patients had untreated primary tumors (thigh, four; forearm, two; and buttock, rectus abdominis muscle, and infratemporal fossa, one each) and two had locally recurrent masses (one each in the retroperitoneum and retrocrural space). All patients were evaluated by conventional radiography, two by sonography, eight by CT, five by angiography, and three by MR. Conventional radiographs showed the soft-tissue mass in only four patients; four lesions caused destruction of adjacent bone and two had soft-tissue calcification. Unenhanced CT showed low-attenuation lesions in four of five patients. The lesions were hypervascular on contrast-enhanced CT or angiography in each of nine patients studied. Prominent draining veins were shown by CT or angiography in five patients. Three lesions had a prolonged capillary stain on angiography. Alveolar soft-part sarcoma should be considered in the differential diagnosis of a hypervascular soft-tissue mass, particularly in the thigh of a young adult.     

Dendritic cell populations in Leishmania major-infected skin and draining lymph nodes

Using a metacyclic promastigote ear infection model of cutaneous leishmaniasis, we examined the phenotype, parasite load, and cytokine production of dendritic cells in the skin and draining lymph nodes of resistant C57BL/6J and susceptible BALB/c mice. Five dendritic cell populations were isolated from the skin and lymph nodes, and the main difference between the groups of mice was an increased number of plasmacytoid dendritic cells in the lymph nodes of the susceptible mice. Although similar cell types were present in the skin emigrants of both strains, there was a 10-fold larger number of cells in BALB/c mouse skin early in infection than in C57BL/6J mouse skin. None of the dendritic cells in the lymph nodes harbored parasites until 3 weeks after infection, with the Langerhans cells having the largest load and the plasmacytoid dendritic cells having the smallest load but the longest lasting infection. Although parasites could be detected in the lymph nodes a few hours after infection, none of the skin emigrants harbored parasites, indicating that they are not the vehicle that ferries the parasites from the skin to the lymph nodes. The presence of larger numbers of plasmacytoid cells in infected BALB/c mice, the more protracted infection of these cells, and their production of alpha interferon point to a complex and important role for the plasmacytoid cells in leishmaniasis.     

Neuroticism,symptom reports,and Type A behavior: Interpretive cautions for the Framingham scale

The Framingham Type A Scale (FTAS) is one of three primary measures of Type A behavior. Unlike the structured interview (SI) and Jenkins Activity Survey (JAS), the FTAS is correlated with neuroticism. Further, neuroticism and FTAS scores predict angina-like chest pain complaints but not more definitive coronary heart disease (CHD) end points. Thus, the FTAS may be unique among Type A measures in its susceptibility to the neuroticism-symptom reporting confound. The present study examined associations of the SI, JAS, and FTAS with neuroticism and symptom reporting in two independent samples of undergraduate males. Unlike the SI and JAS, the FTAS was correlated with neuroticism and symptom reporting. Further, the correlations of FTAS scores and symptoms were due to shared variance with neuroticism. The findings are discussed in terms of a possible alternative interpretation of the Framingham Study and the need to consider neuroticism in studies of personality and health.     

Direct prenatal chromosome diagnosis of a malignancy

A fetal tumor was suspected at 31 weeks of gestation. The occurrence of polyhydramnios led to an ultrasound examination, which revealed deformation of the fetal head, face, eye, and neck. This was confirmed by computerized tomography. Amniocentesis yielded cells with an inverted duplication of chromosome #1. This abnormality of chromosome #1 marked the malignant teratoma cells in the amniotic fluid. Cytogenetic analysis of tumor tissue and of normal tissue obtained postnatally confirmed that the abnormality of chromosome #1 observed in amniotic fluid cells was confined to the tumor. The constitutional karyotype was normal. To our knowledge, this is the first report of the direct chromosomal detection of malignancy before birth.