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PubMed1993

[The use of NMR tomography for the dynamic observation of patients with disseminated sclerosis].

NMR-tomography data and clinical topographic parallels were followed up in 27 multiple sclerosis patients on treatment. Primary NMR tomography identified disseminated foci of more intensive signal in the brain and spinal cord of 78% of the patients. In 6 patients the diagnosis was not confirmed, in 21 patients there was tomographic evidence of various disorders: foci of hyperintensity associated with ventricular system enlargement, cerebral atrophy, hyperintensity zones. The number of more intensive signal foci correlated with the disease severity. Hemispheric foci of hyperintensity were clinically most important. Most of the foci were localized in periventricular region. Changes in the above parameters were observed only 1-2 years after the treatment. It is inferred that NMR tomography is both diagnostic and follow-up tool in multiple sclerosis.

Adult↗
PubMed1985

[Epidermal nevus syndrome (of Solomon, Fretzin and Dewald)].

Epidermal naevus syndrome is an entity which has been well established by Solomon, Fretzin and Dewald since 1968, presenting cutaneous, visceral, ocular, osseous and neurological malformations. We report a case with multiple osseous manifestations affecting the entire skeleton, but mainly the skull (osteolysis with absence of frontal bone), the vertebral column (scoliosis with triple deviation), the upper right limb (demineralisation and multiple pathological fractures) and lower members (fractures of the right and left femur) with enlargement of the entire ventricular system and well marked scissures, signs of an cortico-subcortical atrophy shown by scanogram.

Adolescent↗
PubMed1997

[Cerebral positron emission tomographic study in systemic lupus erythematosus].

The cerebral glucose metabolism in eight patients with systemic lupus erythematosus (SLE) and in five healthy controls were examined by positron emission tomography (PET) using 18-F-labeled deoxy-glucose (FDG) as tracer. One of the eight patients had no abnormality by magnetic resonance imaging (MRI), three of them had cerebral atrophy and four patients had multiple white matter hyperintensities and vascular infarcts in the striatum as assessed by MRI. With FDG-PET, inhomogeneous multifocal cerebral glucose hypometabolism was detected, more frequently in the temporal lobe of right hemisphere. The PET findings did not correlate always with the neurological symptoms. Abnormalities in brain metabolism can be detected more frequently by PET, than morphological changes by MRI, indicating the involvement of the central nervous system.

Adult↗
PubMed2000

Blue rubber bleb nevus syndrome with central nervous system involvement.

An 11-month-old female patient presented with focal seizures. She was born at term with multiple scattered tiny protuberances and dark purple soft compressible nevi over her entire body. She had clumsiness and decreased muscle power in her left extremities. Magnetic resonance imaging and magnetic resonance angiography revealed cerebral atrophy of the right central area around the anomalous venous sinus and multiple small enhancing lesions throughout the brain with gadolinium-diethylenetriamine pentaacetic acid enhancement. Despite right partial neonatal seizures, she had no further seizures for 3 years while receiving carbamazepine, and no change was observed in the number and size of the nevi. At last evaluation, language and psychomotor development were normal for her age.

Anticonvulsants↗
PubMed1996

Patients with amyotrophic lateral sclerosis and other neurodegenerative diseases have increased levels of neurofilament protein in CSF.

In the present study we describe an ELISA to quantify the light subunit of the neurofilament triplet protein (NFL) in CSF. The method was validated by measuring CSF NFL concentrations in healthy individuals and in two well-characterized groups of patients with amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD). The levels were increased in ALS (1,743 +/- 1,661 ng/L; mean +/- SD) and AD (346 +/- 176 ng/L) compared with controls (138 +/- 31 ng/L; p < 0.0001 for both). Within the ALS group, patients with lower motor neuron signs only had lower NFL levels (360 +/- 237 ng/L) than those with signs of upper motor neuron disease (2,435 +/- 1,633 ng/L) (p < 0.05). In a second study patients with miscellaneous neurodegenerative diseases were investigated (vascular dementia, olivopontocerebellar atrophy, normal pressure hydrocephalus, cerebral infarctions, and multiple sclerosis), and the CSF NFL level was found to be increased (665 +/- 385 ng/L; p < 0.0001). NFL is a main structural protein of axons, and we suggest that CSF NFL can be used to monitor neurodegeneration in general, but particularly in ALS with involvement of the pyramidal tract.

Aged↗
PubMed2005

Changes observed in multiple sclerosis using magnetic resonance imaging reflect a focal pathology distributed along axonal pathways.

Multiple sclerosis has long been recognized as a multifocal inflammatory demyelinating disease of the central nervous system. The fact that patients with multiple sclerosis can develop a secondary progressive phase of their disease which is resistant to anti-inflammatory therapies, together with the fact that brain atrophy can develop in patients with a relatively low volume of white matter lesions, has led to suggestions that multiple sclerosis may be a degenerative disease. However, primary degenerative disorders are not usually associated with recurrent episodes of inflammatory demyelination. Support for neurodegeneration in MS being associated with focal lesions comes from topographical mapping of the spatial relationship of axonal injury and tissue loss to lesions using advanced image analysis methods.

Atrophy↗
PubMed2005

Gray and white matter volume changes in early RRMS: a 2-year longitudinal study.

BACKGROUND: Brain atrophy, in excess of that seen with normal aging, has been observed early in the clinical course of relapsing-remitting multiple sclerosis (RRMS). Previous work has suggested that at this stage of the disease, gray matter (GM) atrophy progresses more rapidly than the white matter (WM) atrophy. OBJECTIVES: To characterize the evolution of GM and WM volumes over 2 years, and their associations with lesion loads in a cohort of patients with clinically early RRMS. METHODS: Twenty-one patients with RRMS (mean age 37.5 years, mean disease duration from symptom onset 2.1 years) and 10 healthy control subjects (mean age 37.1 years) were studied. Tissue volumes, as fractions of total intracranial volumes, were estimated at baseline and 1- and 2-year follow-up. Brain parenchymal fractions (BPF), GM fractions (GMF), and WM fractions (WMF) were estimated. In subjects with MS, brain lesion loads were determined on conventional T2-weighted along with pre- and post-gadolinium (Gd) enhanced T1-weighted images at each timepoint. RESULTS: A decrease in GMF was observed in subjects with MS vs normal controls over the 2 years of the study (mean -2.1% vs -1.0%, p = 0.044), while no change was seen in WMF over the same period (mean -0.09% vs +0.09%, p = 0.812). However, when the MS cohort was divided in half, dependent upon change in Gd-enhancing lesion load over 2 years (n = 20), a decrease in WMF was seen in the group (n = 10) with the largest decline in Gd volume, whereas WMF increased in the other half (n = 10) concurrent with a net increase in volume of Gd-enhancing lesions (difference between groups: p = 0.034). CONCLUSIONS: Increasing gray matter but not white matter (WM) atrophy was observed early in the clinical course of relapsing-remitting multiple sclerosis. Fluctuations in inflammatory WM lesions appear to be related to volume changes in WM over this time period.

Adult↗
PubMed1999

Acute encephalopathy: a new toxicity associated with high-dose paclitaxel.

The purpose of this study was to describe acute encephalopathy as a new toxicity associated with paclitaxel, when it is delivered at high doses (> or =600 mg/m2) with stem cell support. A total of 129 patients, included in clinical trials of paclitaxel-containing high-dose chemotherapy, were analyzed. A total of 114 patients received paclitaxel at a dose of > or =600 mg/m2. Six patients presented acute encephalopathy starting between 7 and 23 days after paclitaxel treatment; two of them had received prior whole-brain irradiation. Paclitaxel was given alone (one patient), with cyclophosphamide and cisplatin (two patients), and with cyclophosphamide and cisplatin plus 1,3-bis(2-chloroethyl)-1-nitrosourea (three patients). Central nervous system toxicity consisted of rapid obtundation and coma (five patients) and severe confusional picture with paranoid ideations (one patient). Brain magnetic resonance imaging showed diffuse white matter atrophy (one patient) or multiple small infarcts (one patient), or it was normal (four patients). Other complementary tests, including cerebrospinal fluid analysis and electroencephalography, were nondiagnostic. An effect from concomitant psychotropic medications or from other organ toxicities was excluded in all patients. Three patients recovered after 8-15 days, either spontaneously (two patients) or after high-dose steroids (one patient). Three patients died of irreversible coma. Necropsy, performed in two patients, showed generalized white matter atrophy and multiple brain parenchymal infarcts, respectively. No pharmacodynamic correlation between the occurrence of encephalopathy and a pharmacokinetic parameter of paclitaxel could be identified. Paclitaxel-containing high-dose chemotherapy can cause severe acute encephalopathy. An aggravating effect from prior brain irradiation or concurrent 1,3-bis(2-chloroethyl)-1-nitrosourea seems possible.

Antineoplastic Agents, Alkylating↗
PubMed1983

Occupational reproductive hazards: necessary steps to prevention.

Although some attention has recently been given to the study of occupational reproductive hazards, little is known about measures being taken to prevent exposure to substances having the potential to cause such problems. In the past, OSHA has promulgated a standard to lower the permissible exposure level to a reproductive toxin as the major focus only after experimental evidence was confirmed in humans. The method of selection of appropriate substitutes is also cause for concern. A chemical known to cause testicular atrophy, cancer, and gene mutations in subhuman test systems and to cause sterility in male workers was replaced with a substance known from experimental studies to cause testicular toxicity, cancer of multiple sites, and gene mutations. Experimental test results need to be addressed from the standpoint of determining presumptive risk to humans. A scheme for setting priorities for reduction of exposure or for consideration of regulation of occupational reproductive hazards also needs to be developed. A policy addressing occupational reproductive hazards may serve as a stimulus for protecting workers from these hazards and for improving scientific research protocols.

Ethylene Dibromide↗
PubMed1998

Idiopathic peripheral neuropathy in the horse with knuckling: muscle and nerve lesions in additional cases.

We have previously reported a pathological investigation of peripheral neuropathy in a horse with knuckling. This report describes details of the muscle and peripheral nerve lesions in two additional cases of light horse yearlings with knuckling. The skeletal muscles showed neurogenic atrophy characterized by scattered single angular fibers, fiber grouping, and fiber-type grouping. The severity of muscle lesions increased distally; that is, both fore- and hindleg muscles were affected more severely than cervical and dorsal muscles. In the peripheral nervous system, a number of Renaut bodies appeared to be common in the nerve fascicles. Pathological alterations indicating demyelination, remyelination and regeneration of nerve fibers were occasionally observed. The most common abnormality was myelin ovoids or myelin debris infiltrated by macrophages. Occasionally, myelinated axons were seen containing accumulations of organelles, often associated with buckling of the myelin. The myelin sheath occasionally formed axonal outpouching containing accumulations of mitochondria and dense lamellar bodies. Histochemically, intramuscular nerve fibers presented multiple arborization and collateral ramification, indicating relapsing denervation and reinnervation. Also seen were the fibers with myelin balloons or swollen segments considered as being degenerative processes. The distribution patterns of muscular lesions in the affected animals were indicative of systemic distal denervation atrophy. In addition, peripheral nervous lesions that selectively involve the distal parts of axons and an absence of abnormalities in neuronal cell bodies in the spinal cord suggest a dying-back neuropathy. It was concluded that this disease should be classified as a distal axonopathy.

Animals↗
PubMed2000

Quantitative ultrastructural analysis of a single spinal cord demyelinated lesion predicts total lesion load, axonal loss, and neurological dysfunction in a murine model of multiple sclerosis.

Infection of susceptible mice with Theiler's murine encephalomyelitis virus results in neurological dysfunction from progressive central nervous system demyelination that is pathologically similar to the human disease, multiple sclerosis. We hypothesized that the development of neuropathology proceeds down a final common pathway that can be accurately quantified within a single spinal cord lesion. To test this hypothesis, we conducted quantitative ultrastructural analyses of individual demyelinated spinal cord lesions from chronically infected mice to determine whether pathological variables assessed within a single lesion accurately predicted global assessments of morphological and functional disease course. Within lesions we assessed by electron microscopy the frequencies of normally myelinated, remyelinated, and demyelinated axons, as well as degenerating axons and intra-axonal mitochondria. The frequency of medium and large remyelinated fibers within a single lesion served as a powerful indicator of axonal preservation and correlated with preserved neurological function. The number of degenerating axons and increased intra-axonal mitochondria also correlated strongly with global measures of disease course, such as total lesion load, spinal cord atrophy, and neurological function. This is the first study to demonstrate that functional severity of disease course is evident within a single demyelinated lesion analyzed morphometrically at the ultrastructural level.

Animals↗
PubMedDate not supplied

[Premature aging from the biological dynamic viewpoint].

Contrary to normal and harmonic aging, the multiple dimensional and mainly nonsynchronous proceedings of premature aging mostly include a pathological process. The phenomenon of aspectual premature aging is accentuated and its specific structure is elaborated. Within the scope of a multiple systemical biological correlation matrix, modern neurophysiological points of view discussed, which specially target the central nervous infra-structure.

Aged↗
PubMed1997

Screening for Leber's hereditary optic neuropathy associated mitochondrial DNA mutations in patients with prominent optic neuritis.

Previous case reports demonstrated the presence of Leber's hereditary optic neuropathy (LHON) associated mitochondrial (mt) DNA mutations in patients presenting with prominent optic neuritis (PON). By screening the mtDNA, we have excluded the presence of these mutations in 22 patients with PON, indicating that the frequency of these mutations is less than 4.5% in our selected patient population. Reviewing the clinical data of these patients revealed that severe optic nerve atrophy developed in association with both the benign and the severely disabling form of Multiple Sclerosis (MS). This observation suggests that the prominent feature of ON in MS may be related to local factors or to a selective vulnerability of the optic nerve in some patients. However, it also may be consequence of a deleterious process associated with inflammatory demyelination in the central nervous system (CNS) of another, genetically probably distinct subgroup of severely disabled patients.

Adult↗
PubMed1976

Cerebro-hepato-renal syndrome of Zellweger. Ocular histopathologic findings.

An infant who was the product of a full-term pregnancy was born with multiple congenital defects, including diffuse bilateral corneal opacification. He failed to thrive and died at the age of 4 days. Autopsy findings were consistent with those seen in the cerebro-hepato-renal syndrome. Histopathologic examination of both eyes, obtained at autopsy, demonstrated similar changes. The main pathologic findings in the anterior segment were bilateral, diffuse corneal edema associated with paracentral iridocorneal adhesions and focal attenuation of Descemet membrane. Both retinas disclosed a selective degeneration of the outer nuclear layers and photoreceptors, confined mainly to the macular region. Atrophy of the optic disc and nerve, with reactive gliosis and demyelination, were consistent with findings in the central nervous system described in this syndrome.

Abnormalities, Multiple↗
PubMed2003

Volumetric quantitation by MRI in primary progressive multiple sclerosis: volumes of plaques and atrophy correlated with neurological disability.

In primary progressive multiple sclerosis (PPMS) abnormalities in brain magnetic resonance imaging (MRI) differ from abnormalities in other subtypes of multiple sclerosis (MS). It was investigated whether the extent of brain and spinal cord MRI abnormalities is reflected in the neurological disability in PPMS. Focal and diffuse changes and atrophy in central nervous system (CNS) in patients with PPMS (n = 28) and healthy controls (n = 20) were assessed by semi-automatic MRI segmentation and volumetric analysis. The measurements were related to neurological disability as expressed by the expanded disability status scale (EDSS), the regional functional scoring system (RFSS), the arm index and the ambulation index. Plaques in T1- and/or T2-weighted images were seen in all brains, while spinal plaques were detected in 23 of 28 patients (82%). The total volumes of brain and spinal cord were significantly smaller in patients than in controls (P = 0.001 and 0.000, respectively). The volumes of T1 or T2 lesions in the brain correlated to the ambulation index (r = 0.51, P = 0.005 and r = 0.53, P = 0.004, respectively). No correlations were detected between MRI measurements and total EDSS score, but relative brain atrophy correlated inversely with the total RFSS scores, poor arm index and higher cerebral disturbances (r = -0.53, P = 0.004; r = -0.53, P = 0.004; and r = -0.52, P = 0.005, respectively). Although the number of spinal T2 lesions correlated with sensory disturbances (r = 0.60, P = 0.001), no correlations were found between EDSS subscores and spinal cord atrophy. These findings show that marked atrophy of brain and spinal cord detected by volumetric quantitation correlates with neurological disability. This observation indicates the importance of neurodegenerative events in PPMS.

Activities of Daily Living↗
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↗