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Biomedical subjects

K Berry

Publications and source records attributed to K Berry.

At least 37 records · Page 2Linked to original sources

Examination of brains of AIDS cases for human immunodeficiency virus and human cytomegalovirus nucleic acids.

The role of direct virus infection as a determining factor in acquired immunodeficiency syndrome (AIDS) dementia was investigated using in situ hybridisation for human immunodeficiency virus (HIV) and human cytomegalovirus (HCMV). Four of the five AIDS dementia patients in this series demonstrated HIV infected cells distributed in widely different parts of the brain, but only one case showed HCMV infected cells. The greater abundance of HIV was in subcortical white matter in nodular areas consisting of monocyte/macrophage infiltrates. The cells were occasionally arranged as a multinucleated syncitium. In two cases, a few large cells with the appearance of neurons were positive for HIV hybridisation. By appropriate treatment with ribonuclease, it was shown that hybridisation was primarily to HIV RNA. HCMV infected cells were observed in small numbers in only one of the positive cases, suggesting that HCMV is not a determining factor in AIDS dementia. HCMV positive cells were located in the grey matter, with an appearance suggestive of neurons. Cells expressing the MHC-class II antigen HLA-DR, a marker of reactive microglia and macrophages, were observed to be extensive in affected brain sections in the one case examined. These cells were present in greater number than HIV infected cells. In this case, extensive numbers of HIV infected cells were noticed along the peripheral margin of the substantia innominata. This could indicate infection in this case of a critical brain region from the cerebrospinal fluid.

Acquired Immunodeficiency Syndrome

Duodenal tuberculosis.

The clinical and radiological features in 30 cases of duodenal tuberculosis are presented. The patients are divisible into two groups on the basis of their presenting features. Twenty-two had symptoms and signs of gastric or duodenal obstruction, and eight patients had dyspepsia suggesting peptic ulceration. In the first group, laporotomy showed that the duodenal obstruction was due to extrinsic compression by matted tuberculous lymph nodes in the majority; there was only five intrinsic strictures. In patients with dyspepsia there were bulbar and post-bulbar ulcers accompanied by more widespread mucosal changes, induration, and periduodenal lymphadenopathy. Bypass procedures were performed to relieve obstruction; no resection was possible because of the adherence of the lymph node masses.

Adolescent

Lymphocytic adenohypophysitis.

Lymphocytic adenohypophysitis (LAH) is an uncommon disorder in the spectrum of pituitary disease. Twenty-three cases proven by biopsy or at autopsy have been reported since 1962. We report 2 further cases and review the etiology, immunology and pathology of the disease. The diagnosis should be considered in a female patient who presents during the post-partum period with the clinical picture of a non-functional or prolactin cell pituitary adenoma and evidence of hypopituitarism.

Adult

Brain lesions in a Pacific white-sided dolphin (Lagenorhynchus obliquidens).

A young, male, free-ranging Pacific white-sided dolphin (Lagenorhynchus obliquidens) was found disoriented and died after being held in captivity for several months. Malacic lesions in several areas of the brain were associated with helminth eggs. The appearance and location of these eggs suggested they were of the genus Nasitrema.

Animals

Cytochrome c oxidase deficiency in Leigh syndrome.

We studied 6 mitochondrial enzymes in crude extracts and isolated mitochondria from 5 children with pathologically proven subacute necrotizing encephalomyelopathy (Leigh syndrome). Samples were taken from brain (5 patients), skeletal muscle (4 patients), liver (4 patients), kidney (4 patients), heart (1 patient), and cultured fibroblasts (3 patients). An isolated defect of cytochrome c oxidase (COX) activity was found in brain (decrease of activity to 15 to 39% of the normal mean), muscle (9 to 20%), kidney (1 to 67%), and in the 1 available heart (4%) from a patient with cardiopathy. COX activity was also decreased in liver of 3 patients (2 to 13% of normal) and in cultured fibroblasts of 2 patients (18 and 27%), but it was normal in both liver and fibroblasts from 1 patient. Immunotitration using polyclonal antibodies against human heart COX showed essentially normal amounts of cross-reacting enzyme protein in various tissues from different patients. Electrophoresis of COX immunoprecipitated from brain mitochondrial extracts showed normal patterns of COX subunits in 2 patients. This study confirms the theory that COX deficiency is an important cause of Leigh syndrome.

Brain

Acetylcholine receptor antibodies in myasthenia gravis: use of a qualitative assay for diagnostic purposes.

We have modified the techniques of Lindstrom and of Tindall to measure serum acetylcholine receptor antibody using human antigen bound to 125I-alpha Bungarotoxin. By using 10 microliters of serum and precipitating antigen-antibody complexes with an excess of staph A, we found that only one out of 43 patients with clinically diagnosed active generalized Myasthenia Gravis had no antibodies. In pooling these results with the results of tests done for diagnostic purposes we found positive results in 54/55 generalized active MG, 8/21 MG in remission, 16/37 ocular MG and 0/55 healthy controls. Two out of 38 non MG were also positive and their clinical diagnosis of botulism and penicillamine treated rheumatoid arthritis have been confirmed by a one year follow-up. Most of these sera were also tested for reactivity with fetal calf AchR. Six out of 49 samples positive with the human receptor were negative with calf receptor. We conclude that our technique is extremely useful for the diagnosis of Myasthenia Gravis and that fetal calf antigen cannot replace human antigen in the assay.

Animals

Pathological and clinical study of Müller's muscle in Graves' ophthalmopathy.

Specimens of Müller's muscle from patients with Graves' disease were examined for evidence of morphologic, histochemical, immunohistochemical and electron microscopic changes. No scarring, significant inflammation or shared antigenicity between Müller's muscle and thyroglobulin-related antigens was found. There were moderate numbers of mast cells in 48 of 66 specimens. Computed tomography in 16 patients with Graves' ophthalmopathy showed enlargement of the superior rectus-levator complex in 17 of 28 orbits with lid retraction. However, no correlation was found between the amount of lid retraction and the degree of enlargement of the muscle complex.

Ciliary Body

Cytoplasmic body myopathy. Report on a family and review of the literature.

A 15-year-old girl who was seen for scoliosis presented with cardiorespiratory failure associated with a respiratory infection. She was found to have weakness predominant in the face, sternomastoid, proximal limb, respiratory, spinal and cardiac muscles. The serum creatine kinase level was slightly elevated and the electrocardiogram was abnormal. The electromyograph was consistent with a myopathy. The course was malignant. Her 14-year-old brother had similar findings and succumbed at the age of 14 and one-half years from cardiorespiratory failure. The mother had minimal weakness of proximal limb muscles since early life. The tendon reflexes were normal as was the serum creatine kinase level. The course was benign. On light microscopy the muscle biopsy in the girl showed fibre diameter variation, centrally placed nuclei, necrosis, fibrosis and cytoplasmic bodies. The muscle biopsy in the brother and mother had similar findings except that the inclusion bodies were not seen in the mother. On electron microscopy, the girl showed typical cytoplasmic bodies, involving predominantly type 1 fibres. The mother also had these structures. The literature is reviewed and the origin, pathogenesis and aetiology of the cytoplasmic body are discussed.

Adolescent

Inclusion body myositis (IBM): myopathy or neuropathy?

Inclusion body myositis (IBM) is described in six elderly patients (three women) and in a young familial patient. They all showed the morphologically characteristic vacuoles containing osmiophilic membranous whorls and intracytoplasmic or intranuclear inclusions. There is a well-delineated bimodal age spectrum of IBM, with onset in the second and sixth decades, but otherwise the disorder seems to be a specific entity. Clinical, electrophysiologic, and morphologic features suggest a neurogenic origin in some cases.

Aged

Acute hemorrhagic leukoencephalopathy. A clinical, pathological, and radiological correlation.

Two patients with acute hemorrhagic leukoencephalopathy, one of which was pathologically proven, were serially studied with CT scanning. Both patients showed marked distinctive low density white matter changes throughout both hemispheres, which correlated with clinically involved areas. One patient recovered from the disease, perhaps due to steroid treatment, and showed slow but complete resolution of CT scan changes. We feel that CT scan findings significantly help in the diagnosis of this disease, which may be amenable to early treatment with steroids.

Acute Disease

Malignant meningioma: clinical and pathological features.

The records of 15 patients with a diagnosis of malignant meningioma were reviewed. In one of these patients, in whom invasion of the brain and pituitary gland was the only unusual feature, the tumor was reclassified as benign. Seven tumors, four hemangiopericytomas and two transitional and one syncytial meningioma, were considered to be only borderline-malignant despite necrosis and invasion of the brain, because of few mitoses and regular architecture. Of this group of patients, four men and three women, two are alive and well, three died after incomplete resections, and two succumbed to recurrent tumor that had become inoperable. The other seven patients, six men and one woman, had lesions classified as histologically frankly malignant, on the basis of marked anaplasia and numerous mitoses. These comprised three hemangiopericytomas and three syncytial and one fibrous meningioma. One of these patients is alive and well and the others are dead, three a a result of metastases. The initial clinical course of malignant meningiomas tends to be short but is otherwise indistinguishable from that of benign meningiomas. The chances of recurrence and eventual death are high, and extracranial metastases are not rare. The tumors are most often hemangiopericytomas, but not exclusively so, and men are particularly at risk.

Adult

Surgical closure of the larynx for intractable aspiration.

Surgical closure of the glottis is reserved for patients who have suffered progressive loss of sphincteric and phonatory laryngeal function. In the prevention of life-threatening aspiration, a triple-layer closure is performed with use of a superiorly based sternohyoid muscle flap. Surgical closure is theoretically reversible should sufficient neuromuscular function return in any given patient.

Adult

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Pathological study of two cases.

Superficial temporal artery to middle cerebral artery (STA-MCA) anastomoses were examined at autopsy in two patients who survived 2 years and 5 1/2 years, respectively, after the cerebral revascularization procedure. Identification of the actual anastomotic sites at autopsy was rendered impossible because of marked fibrosis in the region of the surgery. However, in both patients intimal fibrosis and medial damage were noted in the STA immediately adjacent to the anastomosis. In the patient who survived 2 years, the lumen of the STA was only slightly compromised by these changes, but in the patient who survived 5 1/2 years, the artery was almost totally occluded. The MCA's adjacent to the anastomosis were widely patent and showed focal areas of intimal hyperplasia with no abnormalities of the media.

Cerebral Arteries