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PubMed2000

Linomide in relapsing and secondary progressive MS: part II: MRI results. MRI Analysis Center of the University of Texas-Houston, Health Science Center, and the North American Linomide Investigators.

OBJECTIVE: To determine the safety and efficacy of roquinimex (linomide) in the management of relapsing-remitting and secondary progressive MS as monitored by MRI. BACKGROUND: Preclinical studies and several short term randomized trials of linomide suggested clinical and MRI-measured benefits with acceptable risk for closely followed MS patients. METHODS: The North American Linomide Trial formally screened 853 individuals for relapsing or secondary progressive, clinically definite MS; recent disease activity or progression; and an Expanded Disability Status Scale score at entry of 3.0 to 6.5 inclusive. MRI was obtained on 811 subjects at pre-enrollment, 718 cases at enrollment, and then at three monthly intervals until the trial was prematurely terminated for unacceptable toxicity. RESULTS: Enhancement was found on 40.2% of 718 entry scans. Statistically robust correlations were found between clinical demographic data and several entry MRI measures including CSF volume, a reflection of brain atrophy. Assessment of the effect of treatment on MRI-measured disease was limited by early trial termination. However, active treatment for 3 months reduced the proportion of patients with one or more enhancements. An exploratory analysis suggested that 2.5 mg was the most active of three doses tested in limiting the total volume of enhanced tissue, the proportion of MRI-defined lesions designated as "black holes," and by a novel MRI composite disease measure. CONCLUSIONS: The short term signature of the effect of linomide on MRI-measured aspects of the disease suggests that safer drugs of this class might be useful in the management of MS. The use of a composite index of the heterogeneous nature of the pathology of MS as captured by MRI may have merit as an outcome measure in clinical trials.

Adjuvants, Immunologic↗
PubMed1992

Sensory evoked potentials: PERG, VEP, and SEP.

The review summarizes current developments in pattern electroretinography practice and theory: generator sources and clinical applications. This is particularly relevant to neuronal disease of the central nervous system (CNS). Visual evoked potentials (VEPs) are discussed in reference to the evolution and follow up of multiple sclerosis (MS) and in conjunction with magnetic resonance imaging (MRI) demonstrated abnormalities. These reveal that the techniques are complementary. Sensory evoked potentials (SEPs) in conjunction with MRI studies are highlighted as quantitatively comparable in spinal cord disorders.

Central Nervous System Diseases↗
PubMed1996

Multiple extrahepatic portosystemic shunts in dogs: 30 cases (1981-1993).

OBJECTIVE: To describe the long-term outcome in dogs with naturally developing multiple extrahepatic portosystemic shunts (PSS). DESIGN: Retrospective case series. ANIMALS: 30 dogs with multiple PSS. PROCEDURE: Medical records of dogs with multiple PSS were reviewed. Follow-up data were obtained by 1 or more of the following methods: recheck at the veterinary teaching hospital (n = 6) or telephone contact with the referring veterinarian (n = 18) or owner (n = 10). The chi 2 or Mann-Whitney rank sum test was used to determine the association of clinical factors with long-term outcome. Survival curves were generated by the Kaplan-Meier product limit method. RESULTS: Median age at diagnosis was 1 year. Findings on exploratory surgery in 25 dogs included ascites; numerous tortuous vessels connecting the portal vein with systemic veins; a small, misshapen liver; and an enlarged portal vein. The most common lesions on histologic evaluation of hepatic tissue specimens were hepatocellular atrophy, portal vascular duplication, cirrhosis, inflammation, and bile duct proliferation. Twelve dogs were treated surgically with vena caval banding, whereas 13 dogs were treated conservatively with dietary restriction of protein and administration of antibiotics, diuretics, and other drugs. Long-term survival and quality of life were similar in dogs from both treatment groups. Median follow-up interval in dogs that survived hospitalization was 24 months (range, 1 to 54 months). CLINICAL IMPLICATIONS: On the basis of these findings, vena caval banding in dogs with multiple PSS is not superior to medical and nutritional treatment.

Animals↗
PubMed2001

Critical illness polyneuropathy.

Critical illness polyneuropathy is a self-limited acute axonal neuropathy that develops during treatment of severely ill patients and remits spontaneously once the critical condition is under control. Clinical manifestations include muscle weakness and atrophy, delayed weaning from the respirator, and prolongation of the mobilization phase. The pathogenesis is not understood in detail but most authors assume that the inflammatory cascade that mediates the systemic inflammatory response and multiple organ failure play a pivotal role. This review summarizes current knowledge of this common neuropathic complication during intensive care treatment.

Diagnosis, Differential↗
PubMed1990

[Central nervous symptoms and findings in patients with systemic lupus erythematosus].

Systemic lupus erythematosus is a disease characterized by multiple autoimmune phenomena, and a broad clinical spectrum. Involvement of the central nervous system is common, and in the majority of patients occurs mainly as an organic brain syndrome or as migraineous headache. Cerebral atrophy as judged by CT scan is common. Cerebral infarction occurs in a minority of patients, mainly those with high disease activity and a high titer of anti-phospholipid antibodies. Systemic lupus erythematosus should be considered as a possible diagnosis in patients with certain central nervous system aberrations, especially young females.

Adult↗
PubMed1999

Spectral tuning of a circadian photopigment in a subterranean 'blind' mammal (Spalax ehrenbergi).

The atrophied subcutaneous eyes of Spalax ehrenbergi (the blind mole rat) express a long wavelength sensitive (LWS) cone opsin. Our data provide strong evidence that this photopigment is spectrally tuned to enhance photon capture in the red light environment of the eye. Furthermore, novel mechanisms appear partially responsible for this sensory fine-tuning. These data support the hypothesis that the LWS opsin of Spalax acts as a functional photopigment and that it is not a 'residue' of the pre-subterranean visual system. As the eye of Spalax has only one known function, the entrainment of circadian rhythms to environmental light, the LWS photopigment is implicated in this task. These results, together with our recent findings that rod and cone photopigments are not required for murine photoentrainment, suggest that multiple photopigments (classical and novel) mediate the effects of light on the mammalian circadian system.

Amino Acid Sequence↗
PubMed2002

Immunoserological changes in the cerebro-spinal fluid and serum in systemic lupus erythematosus patients with demyelinating syndrome and multiple sclerosis.

The aim of the present study was to investigate the role of antinuclear and antiphospholipid antibodies in the pathogenesis of systemic lupus erythematosus (SLE) with demyelinating syndrome and several forms of multiple sclerosis (MS). Paired samples of serum and cerebrospinal fluid (CSF) were investigated using laser nephalometric and enzyme linked immunosorbant assay (ELISA) methods, and the parameters of intrathecal synthesis were calculated. Elevation of the concentrations of antiribosomal P protein antibodies in the CSF and serum, and intrathecal synthesis anticardiolipin (aCL) antibodies were characteristic in all patient groups. The immunoserological changes were more pronounced in the SLE patients. A similar pathogenetic role of antiphospholipid antibodies in central nervous system (CNS) damage in SLE patients with demyelinating syndrome and of MS patients can be assumed.

Adult↗
PubMed1973

Proteins from human cerebrospinal fluid: binding with nucleic acids.

Cerebrospinal fluid (CSF) contains two groups of proteins that bind tightly to DNA and to polyriboguanylic acid, respectively. In certain diseases the amounts of a given nucleic acid bound by a constant volume of CSF may increase, while in others the amount of such proteins may be reduced. Binding of polyriboguanylic acid increased in CSF samples from patients with brain tumors, stroke, multiple sclerosis, and communicating hydrocephalus, but it significantly decreased in CSF samples from patients with obstructive hydrocephalus. These increases may or may not be proportional to the rise in total CSF proteins characteristic for these diseases. Elevated binding of DNA was observed in samples from patients with hydrocephalus, epilepsy, and cortical atrophy. The technique described may be applicable to the diagnosis of a variety of diseases of the central nervous system.

Alcoholism↗
PubMed1981

Intralesional corticosteroids.

Intralesional corticosteroids are commonly used in clinical dermatology. They are an adjunct to topicals and a possible substitution for systemic corticosteroids. Intralesional corticosteroids can be injected via a syringe and needle or with a Dermo-Jet. Although multiple preparations are available, the triamcinolones have more acceptable physical characteristics. Systemic absorption and consequent adverse effects are rarely a problem because of low dosage administered. Local adverse effects (the most prominent of which is atrophy) depend on the strength of the preparation, the quantity used, the area of the body, and the skin condition being treated.

Absorption↗
PubMed1986

Familial juvenile parkinsonism with multiple systems degenerations. A clinicopathological study.

An unusual case of familial multisystemic degeneration is reported. Two siblings had juvenile parkinsonism, areflexia, and retinal degeneration of slow progression. The main neuropathological findings in case 1 were pallidoluysian, nigral, dentate, and dorsal columns degeneration. The authors draw a comparison between this case and juvenile parkinsonism, dentato-rubro-pallido-luysian atrophy, and spino-cerebello-nigral degeneration.

Adult↗
PubMed1994

An electron microscopic study of muscle capillary wall thickening in systemic lupus erythematosus.

Capillary vessel wall thickening may contribute to the muscle fibre atrophy and impaired pulmonary gas transfer notable in SLE/MCTD. Using electron microscopy we measured the thickness of unilaminar capillary basement membranes (CBM) and quantified basement membrane laminae and pericyte layers in the skeletal muscle biopsies of 31 patients with SLE, including 14 with MCTD, and 11 controls. Capillary basement membrane thickness was significantly increased in SLE compared with controls (P < 0.01) and was significantly associated with the level of C3dg (P < 0.01). Patients with MCTD had significantly greater layering of pericytes and laminae compared with patients with SLE alone (P < 0.0001 for both variables). Multiple stepwise regression analysis indicated that ANA titre was a good predictor of pericyte layering (R = 0.63, P = 0.003) and of basement membrane lamination (P < 0.0001), with steroid therapy having a significant negative association with the number of laminae (P < 0.02). A negative association between KCO and the number of layers of basement membrane (R = 0.51, P = 0.01) was documented. However, multiple regression analysis revealed no association between parameters of thickened vessel walls and the presence of muscle fibre atrophy.

Adult↗
PubMedDate not supplied

[Magnetic resonance in the diagnosis and treatment of multiple sclerosis].

Although the diagnosis of multiple sclerosis is still based on clinical criteria, confirmation by magnetic resonance imaging (MRI) is considered to be essential, thank to its high sensitivity in demonstrating the spatial dissemination of the demyelinating plaques in the brain and spinal cord. Additionally, MRI can establish an approximation of the pathological substrate of the multiple sclerosis plaques and it has proven useful for studying the natural history of the disease and monitoring the effects of new treatments. This capacity of MRI is based on its ability to estimate the degree of demyelination, gliosis, edema, inflammation and axonal damage and to detect diffuse involvement of the normal appearing white matter. The selective identification of the lesions that contribute most to the patient's disability and clinical progression, such as severe demyelination and axonal damage, improves the MRI correlation with the neurological impairment scales. However, a correct application of MRI in the study of multiple sclerosis requires standardization of the techniques and sequences used in the different clinical forms of the disease and of the systems for measuring the lesion load. In this way multiple sclerosis can serve as a true biological marker of the severity of the disease.

Atrophy↗
PubMed2006

Mutations in OSTM1 (grey lethal) define a particularly severe form of autosomal recessive osteopetrosis with neural involvement.

UNLABELLED: We report three novel osteopetrosis patients with OSTM1 mutations and review two that have been previously described. Our analysis suggests that OSTM1 defines a new subset of patients with severe central nervous system involvement. This defect is also present in the gl mouse, which could represent a good model to study the role of the gene in the pathogenesis of this disease. INTRODUCTION: Autosomal recessive osteopetrosis (ARO) is a severe hereditary bone disease whose cellular basis is in the osteoclast, but with heterogeneous molecular defects. In addition to the TCIRG1 and the ClCN7 genes, whose mutations account for approximately 55% and 10% of cases, respectively, the OSTM1 gene has been described thus far in only two ARO patients. materials and methods: We report here three novel ARO patients presenting with severe primary central nervous system involvement in addition to the classical stigmata of severe bone sclerosis, growth failure, anemia, thrombocytopenia, and visual impairment with optic atrophy. In addition we analyzed the brain morphology and histology of the grey lethal mutant mouse. RESULTS: The analysis of the OSTM1 gene in two patients, both from Kuwait, showed homozygous two nucleotide deletion in exon 2, leading to a frameshift and premature termination. The third (Lebanese) patient showed a single point mutation in exon 1, leading to a nonsense mutation. The clinical neurological evaluation of the two Kuwaiti patients by CT and MRI scans showed a defect in the white matter, with a specific diagnosis of severe cerebral atrophy. The gl brain showed a diffuse translucent appearance with loss of the normal demarcation between the white and the grey matter, features consistent with myelin loss or hypomyelination. Histological and myelin staining analysis evidenced an atrophy of the corpus callosum with loss of myelin fibers, and in cortical areas, loss of the normal lamination consistent with multiple foci of cortical dysplasia. CONCLUSIONS: These findings suggest that OSTM1-dependent ARO defines a new subset of patients with severe central nervous system involvement leading to a very poor prognosis. The fact that central nervous system involvement is also present in the gl mouse mutant suggests that this mouse is a good model to test possible therapies.

Animals↗
PubMed1999

Sporadic amyotrophic lateral sclerosis with multiple system degeneration: a report of an autopsy case without respirator administration.

This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with unusual clinical and neuropathological findings. The patient was a Japanese man without hereditary burden who was 49 years old at the time of death. His clinical manifestation included dysarthria at age 48, followed by dysphagia, atrophy and fasciculation of the tongue, muscle weakness in the four extremities, tremor, rigidity, increased deep tendon reflexes in the upper and lower extremities, and incoordination of the four extremities. He died of respiratory failure 12 months after the disease onset. No respirator administration was performed throughout the clinical course. The neuropathological examination revealed not only degeneration of upper and lower motor neuron systems, including the presence of Bunina bodies and ubiquitin-immunoreactive neuronal inclusions in the lower motor neurons, but also prominent degeneration of the substantia nigra and dentate nucleus with slight neuronal loss in the locus ceruleus and pontine nucleus. To our knowledge, this is the first reported case of sporadic ALS without dementia and respirator support, showing degeneration of the substantia nigra and dentate nucleus. This report may contribute to the resolution of the question concerning the neuropathological heterogeneity of sporadic ALS with respiratory support.

Amyotrophic Lateral Sclerosis↗
PubMed2001

Cyclooxygenase-2 is up-regulated in proliferative inflammatory atrophy of the prostate, but not in prostate carcinoma.

Cyclooxygenase-2 (COX-2) is the inducible isoform of the rate-limiting enzymes that convert arachidonic acid to proinflammatory prostaglandins as well as a primary target for nonsteroidal anti-inflammatory drugs. Accumulating evidence suggests that up-regulation of COX-2 is associated with carcinogenesis in multiple organ systems including the large bowel, lung, breast, and prostate. In this report, we examine the expression of COX-2 protein and mRNA in prostate tissue containing various lesions and in prostate cancer cell lines. In the cell lines, LNCaP, DU145, PC-3, and TSU, COX-2 protein expression was undetectable under basal conditions but could be induced transiently by phorbol ester treatment in PC-3 and TSU cells, but not in DU145 and LNCaP cells. Immunohistochemical analysis of 144 human prostate cancer cases suggested that, in contrast to several previous reports, there was no consistent overexpression of COX-2 in established prostate cancer or high-grade prostatic intraepithelial neoplasia, as compared with adjacent normal prostate tissue. Positive staining was seen only in scattered cells (<1%) in both tumor and normal tissue regions but was much more consistently observed in areas of proliferative inflammatory atrophy, lesions that have been implicated in prostatic carcinogenesis. Staining was also seen at times in macrophages. Western blotting and quantitative RT-PCR analyses confirmed these patterns of expression. These results suggest that if nonsteroidal anti-inflammatory drugs are indeed chemopreventive and/or chemotherapeutic for prostate cancer, their effects are likely to be mediated by modulating COX-2 activity in non-PCa cells (either inflammatory cells or atrophic epithelial cells) or by affecting a COX-2-independent pathway.

Atrophy↗
PubMed1999

The intrarenal vascular lesions associated with primary antiphospholipid syndrome.

Even 10 yr after the identification of the antiphospholipid syndrome (APS), renal involvement in the course of APS is still relatively unrecognized, and is probably underestimated. The association of anticardiolipin antibodies and/or lupus anticoagulant with the development of a vaso-occlusive process involving numerous organs is now confirmed. In a multicenter study, 16 cases of "primary" APS (PAPS) were found and followed for 5 yr or more, all with renal biopsy. In all 16 cases of PAPS, there was a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries (12 patients), recanalizing thrombi in arteries and arterioles (six patients), and focal cortical atrophy (10 patients). In combination, these led to progressive destruction of the kidney, accelerated by acute glomerular and arteriolar microangiopathy in five patients. Focal cortical atrophy is a distinctive lesion, present in 10 biopsies, and likely represents the histologic and functional renal analogue to the multiple cerebral infarcts detected on imaging studies. The clinical hallmark of this vascular nephropathy in PAPS is systemic hypertension, only variably associated with renal insufficiency, proteinuria, or hematuria. The ensemble of histologic renal lesions defined in this study should aid in the separation of the lesions found in cases of secondary APS, especially systemic lupus erythematosus, into those lesions related to APS and those related to the underlying disease.

Adult↗

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