Chorea in patients with AIDS.

OBJECTIVE: To describe differing etiologies and possible anatomoclinical correlates of choreic movements in a series of AIDS patients. METHODS: We analyzed the clinical records and neuroimaging data of 5 consecutive AIDS patients who developed choreic movements at our center from January, 1994 to December, 1996. RESULTS: There were 2 cases of focal choreic dyskinesias, 1 of right hemichorea, and 2 of generalized chorea. Onset was acute and febrile in 1 case, and subacute in the other 4. In 1 patient the chorea was the AIDS onset symptom; in another choreic movements were the first neurological symptom following AIDS diagnosis; in 2 patients AIDS had a neurological onset other than chorea; and in the fifth patient buccofacial dyskinesias appeared following the development of bacterial encephalitis. CONCLUSION: Chorea was associated with cerebral toxoplasmosis in 2 patients, progressive multifocal leukoencephalopathy in 1, subacute HIV encephalopathy in another, and was probably iatrogenic in the last. Chorea is not unusual in AIDS, however the causes are variable and careful neuroradiological and clinical evaluation is required to identify them. AIDS-related disease should be considered in young patients presenting with chorea without a family history of movement disorders.

Hepato-splenic gammadelta T-cell lymphoma: a rare entity mimicking the hemophagocytic syndrome.

Hepatosplenic gammadelta T-cell lymphoma is a rare histologic type of the peripheral T-cell lymphomas, clinically characterized by predominant involvement of liver and spleen, no or little adenopathy, and an often aggressive course; it affects mainly adolescents and young adults, with a male predominance. Postthymic T-cell malignancies are heterogeneous in their clinical and laboratory features. Among the gammadelta postthymic T-cell lymphomas, two distinct entities (cutaneous and hepatosplenic, respectively) are reported in the literature. The former shows predominant multiple involvement of the skin and subcutaneous tissue; it occurs mostly in older patients and the phenotype is CD3-, CD4-, CD8-. Because of the small number of reports, the course of the disease was unknown. The latter shows a clinical picture characterized by hepatosplenomegaly, no or little adenopathy, and sometimes systemic symptoms (fever, cytopenias likely due to hypersplenism); it presents a peculiar sinusoidal involvement of liver and spleen. The bone marrow histologic feature often reveals a little infiltration, especially sinusoidal and easily underestimated phenotype: CD2+, CD3+, CD7+, CD5-, CD4-, CD8-, CD44+. Few cases of this lymphoma associated by hemophagocytic syndrome are described (Sun, 1990; Kadin, 1981; Jaffe, 1983). We report a case of a young man with a rapid and fatal course in which the more important clinical feature was hemophagocytosis. The diagnosis of lymphoma was very difficult because of paucity of histologic involvement, and only the rearrangement of TCR gamma chain gene by polymerase chain reaction on paraffin sections confirmed a clonal T-cell proliferation.

Evaluation of atherosclerotic lesions using NMR microimaging.

Magnetic resonance imaging (MRI) has been proposed as a potential tool in the evaluation of atherosclerotic lesions. However, two basic difficulties have to date prevented the full exploitation of the potentials of this technique: the poor spatial resolution of the conventional tomographs and the wide variety of the lesions as well as their intrinsic dishomogeneity. In this study the in vitro morphology of normal and atherosclerotic vascular tissue specimens has been evaluated using a high resolution spectometer equipped with a microimaging device. Morphological features of the vessel walls as small as 10(-1) mm have been detected and the distribution of lipids and of calcified or necrotic regions has been evidenced in atherosclerotic plaques. Different techniques, such as local spectroscopy performed on volumes of 1 mm3 and localized magnetization recovery measurements, have been employed to characterize specific regions of the vessel walls from the chemical and the physical point of view. The good agreement of NMR findings with histological data allows us to conclude that NMR microimaging represents a suitable technique for the in vitro detection and characterization of atheromatous lesions.

[Neuropathology of the acquired immunodeficiency syndrome]. / Neuropatologia della sindrome da immunodeficienza acquisita.

Neuropathology of acquired immunodeficiency syndrome. The Central Nervous System (CNS) has been examined at autopsy in 60 patients who died of AIDS in a 6-year period in our hospital. Most of the patients were intravenous drug abusers, the mean age was of 34 years, with a high prevalence of males. Neurologic symptoms were present in 62% of patients, while histologic lesions have been observed in 51 cases (85%). Opportunistic infections were found in 27 patients, the commonest being T. gondii (12) and Cytomegalovirus (7); Progressive Multifocal Leukoencephalopathy was observed in 2 cases. HIV-associated lesions included 21 cases of Multifocal Giant Cell Encephalitis (MGCE), 15 of Progressive Diffuse Leukoencephalopathy (PDL) and 7 cases of Vacuolar Myelopathy. Primary CNS lymphoma was noted in 8 patients and secondary deposits were observed in 3 cases. Simultaneous CNS lesions by more than one pathogen were frequently encountered. The main pathogenetic mechanisms for characterization of all the lesions and their relationship with clinical features of the disease are discussed. It is supposed that MGCE and PDL represent two different patterns of HIV-encephalopathy.

Antiapolipoprotein B cryoglobulinemia associated with normal plasma lipids and severe atherosclerosis.

A middle-aged female presented with diffuse planar xanthomas, associated with normal plasma lipids and a severe crescendo angina. Hematological testing disclosed the presence of a cryoglobulin binding to apolipoprotein B. The patient died after a short and dramatic clinical course. At autopsy, a markedly thickened aorta with diffuse atheromatous lesions of the major peripheral arteries were observed. Intracytoplasmic filaments (50-100 A), comparable to those described in amyloid deposits, were also detected. Endothelial cells were filled with lipid-containing vacuoles without extracellular lipid deposits. The reported case indicates that benign gammopathies against 'atherogenic' lipoprotein components, without clinical myeloma, may result in the development of severe arterial lesions.