Morphological analyses of paraspinal muscles: Comparison of progressive lumbar kyphosis (camptocormia) and narrowing of lumbar canal by disc protrusions

Progressive lumbar kyphosis (camptocormia), a rare, usually familial disease in elderly patients, is characterized by inability to immobilize the lumbar spine in relation to the pelvis. CT scan reveals selective involvement of the spinal muscles with a heterogeneous appearance and is in favour of a primary disorder of these muscles. Our aim was to define the muscular lesions and clarify their nature in this particular disorder. Biopsies of the paravertebral muscles of 14 patients with lumbar kyphosis and of 20 operated on for disc herniation or narrowed lumbar canal, were studied by light microscopy, histochemistry and electron microscopy. In both groups, type 1 fibre predominance and atrophy of type 2 fibres were observed. Ragged-red fibres with abnormal mitochondria also occurred. The differential feature was increased frequency of extensive diffuse or lobulated fibrosis in camptocormia. Other features related to pathogenesis could not be determined.     

Imaging of the aging brain. Part II. Pathologic conditions

The most common pathologic disorders in the elderly result in dementia, movement disorders, and/or focal neurologic deficit. Neuroimaging, particularly magnetic resonance (MR), provides a unique window into the diagnosis and understanding of such diseases. The most common cause of dementing illness is Alzheimer disease (enlarged cerebrospinal fluid spaces, focal medial temporal lobe abnormal signal intensity, cortical iron). Since the initiation of MR imaging, important questions are being asked about the role of white matter disease (signal hyperintensities) in primary degenerative dementia and the nosology of vascular dementia. Parkinsonian disorders are characterized by generalized brain atrophy and dopaminergic site (e.g., putamen, pars compacta of the substantia nigra) iron or pigmentary accumulation best visualized in disease that responds poorly to dopamine replacement therapy. A key role of imaging in the elderly is the noninvasive exclusion of mass lesion (e.g., hematoma, neoplasm). MR imaging is exquisitely sensitive for the detection of the two common abnormalities that cause focal neurologic deficit in the elderly--infarction and intracerebral hematoma.     

Human β-mannosidase deficiency associated with peripheral neuropathy

Human β-mannosidosis is an inherited lysosomal storage disorder described in only seven families. We present a further case in a black African 14-year-old boy with severely deficient β-mannosidase activity, bilateral thenar and hypothenar amyotrophy, electrophysiologically demonstrable demyelinating peripheral neuropathy, and cytoplasmic vacuolation of skin fibroblasts and lymphoid cells. The clinical and biochemical features of our patient are compared to those of previously reported patients.     

Lack of self-renewal capacity in Fancc-/- stem cells after ex vivo expansion

Treatments of the hematological manifestation in Fanconi anemia (FA) are first supported by attempts to stimulate hematopoiesis with androgens or hematopoietic growth factors. However, the long-term curative treatment of the hematological manifestation in FA patients is bone marrow (BM) or cord blood stem cell transplantation. The success rate for BM transplantation is fairly high with HLA-matched sibling donors but is, unfortunately, low with HLA-matched unrelated donors. An alternative curative treatment for those patients with no sibling donors might be gene transfer into hematopoietic stem cells. Because FA patients have reduced numbers of stem/progenitor cells, ex vivo expansion of hematopoietic stem cells would be a crucial step in gene transfer protocols. Using the FA mouse model, Fancc-/-, we tested the ability of CD34- hematopoietic stem cells to support ex vivo expansion. We determined that Fancc-/- CD34- stem cells have reduced reconstitution ability and markedly reduced self-renewal ability after culture, as shown by secondary transplants. These results indicate that FA stem cells may not be well suited for ex vivo expansion before gene transfer or transplantation protocols.     

Central neurocytoma. Immunohistochemical study: MIB1, p53 and bcl-2. Report of 5 cases

To further characterize central neurocytoma, a rare intraventricular tumour described in 1982, we analyzed six tumours by immunohistochemistry for MIB1, p53 and bcl-2. bcl-2, an inhibitor of p53-mediated apoptosis is frequently expressed in gliomas, especially in tumors with wild-type p53. Its expression in peripheral neuroblastomas suggests a down-regulation during final terminal differentiation. Six tumors from five patients (one female/four males, age ranged from 18 to 63 years) were examined. All patients were alive from 2 to 88 months after initial surgical resection. On histological sections, tumours demonstrated a typical pattern. Synaptophysin staining was seen in all cases. Proliferation index was low (< 4.5%). bcl-2 was never expressed. p53 expression varied but within low values (< 10% of cells). These latter antibodies were rarely analyzed until now in this usually benign neoplasm which represents a well differentiated variant of neuron derived tumors.     

Image digitizing in conventional radiology

Since early 1991 a new procedure has been tested at the H?pital du St-Sacrement that uses a phosphorous plate instead of a standard x-ray cassette. The phosphorous plate is exposed to x-rays using standard equipment and is then read by a computer that transfers the resulting image onto either a plate or a cathode screen. This new technology lends importance to the radiology technician's role in image manipulation and determination of the ideal sensitometric curve for each x-ray examination. The technician can generate an image with adjustments in latitude and contrast, depending on the radiologist's needs and notwithstanding the technical parameter settings used during the x-rays.     

Acute ischemia of a limb as a complication of multiple hereditary exostoses. Case report and literature review

The authors present a case of acute ischemia of a limb as a complication of multiple hereditary exostoses. They discuss surgery, complications, and review the literature.     

Laparoscopic esophagomyotomy with posterior partial fundoplication for primary esophageal motility disorders

Background: The outcomes of a laparoscopic esophagomyotomy with posterior partial fundoplication were compared between groups of patients with primary motility disorders. Methods: In this study, 47 patients (26 women and 21 men, ages 24 to 77 years; mean, 47 years) with significant dysphagia or chest pain who failed conservative treatment underwent a laparoscopic esophagomyotomy and posterior partial fundoplication. Preoperative evaluation revealed four groups of primary motility disorders: achalasia (n= 12), nutcracker esophagus (n= 12), hypertensive lower esophageal sphincter (LES) (n= 16), and diffuse esophageal spasm (n= 7). Statistical analysis was performed by Cramer's V test. Results: Average follow-up period was 30.3 months. There was no mortality or early morbidity. Late morbidity included dysphagia or chest pain over 6 weeks in 10 patients (21%), recurrent gastroesophageal reflux disease (GERD) in 3 patients (6%), and recurrent motility disorder in 2 patients (4%). Overall, 94% of the patients ultimately had complete resolution of dysphagia or chest pain. There was no significant difference in outcomes between groups. Conclusion: Early results suggest that laparoscopic esophagomyotomy with posterior partial fundoplication provides safe and effective relief from dysphagia and chest pain in patients with each of the primary motility disorders. Received: 18 February 1999/Accepted: 16 December 1999/Online publication: 13 June 2000     

Caregiver behaviors and resources influence child height-for-age in rural Chad

The purpose of this study was to identify caregiver characteristics that influence child nutritional status in rural Chad, when controlling for socioeconomic factors. Variables were classified according to the categories of a UNICEF model of care: caregiving behaviors, household food security, food and economic resources and resources for care and health resources. Sixty-four households with 98 children from ages 12 to 71 mo were part of this study. Caregivers were interviewed to collect information on number of pregnancies, child feeding and health practices, influence on decisions regarding child health and feeding, overall satisfaction with life, social support, workload, income, use of income, and household food expenditures and consumption. Household heads were questioned about household food production and other economic resources. Caregiver and household variables were classified as two sets of variables, and separate regression models were run for each of the two sets. Significant predictors of height-for-age were then combined in the same regression model. Caregiver influence on child-feeding decisions, level of satisfaction with life, willingness to seek advice during child illnesses, and the number of individuals available to assist with domestic tasks were the caregiver factors associated with children's height-for-age. Socioeconomic factors associated with children's height-for-age were the amount of harvested cereals, the sources of household income and the household being monogamous. When the caregiver and household socioeconomic factors were combined in the same model, they explained 54% of the variance in children's height-for-age, and their regression coefficients did not change or only slightly increased, except for caregiver's propensity to seek advice during child illnesses, which was no longer significant. These results indicate that caregiver characteristics influence children's nutritional status, even while controlling for the socioeconomic status of the household.