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Biomedical subjects

J M Brucher

Publications and source records attributed to J M Brucher.

At least 55 records · Page 3Linked to original sources
PubMed1992

[Anatomical modification of the levator muscle of the eyelid in congenital ptosis].

Congenital ptosis of the upper lid is classified as a developmental anomaly of the levator muscle. We report the histochemical findings in 38 specimens of levator muscle removed in patients with congenital ptosis and operated on by a resection of the levator muscle. Results demonstrated a close relationship between the presence or the absence of muscular fibres in the specimen and the degree of ptosis and the levator function. We suggest that congenital ptosis may result from a failure of differentiation and/or fusion of the anterior portion of the superior orbital mesoderm complex.

Adolescent↗
PubMed1992

Transgenic mice expressing polyoma virus large T antigen in astrocytes develop severe dysmyelination of the central nervous system.

Transgenic mice were generated using a construct that encodes mouse polyoma virus large T antigen, one of three oncogenic products of the "early region" of the polyoma viral genome. Of 16 transgenic families developed, 1 was characterized by a neurologic disorder consisting of constant tremor and recurrent seizures. Morphologic analysis of the central nervous system (CNS) of affected transgenic mice included: classical light and electron microscopic examination; immunohistochemical assessment of the presence and localization of myelin-specific proteins, of the astrocyte marker glial fibrillary acidic protein, of the oligodendrocyte marker galactosyl cerebroside, and of large T; double immunolabeling of glial fibrillary acidic protein or galactosyl cerebroside and large T to identify the CNS cell type in which large T is expressed; and in situ hybridization to study myelin basic protein gene expression. Our results suggest that polyoma large T is expressed in astrocytes, possibly resulting in altered glial-glial interactions causing impaired oligodendroglial development and secondary dysmyelination. Transgenic oligodendrocytes exhibit features of immaturity, failing to myelinate axons properly and producing morphologic phenotypes of early stages of myelination, such as numerous mesaxonal profiles. Myelin proteins are markedly reduced in transgenic CNS, and myelin basic protein transcripts, while present, are generally decreased. We believe that expression of large T in astrocytes could influence the complex and dynamic interactions between astrocytes and oligodendrocytes, perhaps with regard to the molecular (trophic) signals in the local CNS environment, bringing about arrested oligodendroglial maturation and hypomyelination. This raises intriguing questions concerning the importance of glial-glial interactions in the CNS and the complex levels of control involved in biological expression of genetic information in glial cells.

Animals↗
PubMed1992

[Primary meningioma of the geniculate ganglion: apropos of a case].

Based on a clinical observation and a literature review, etiologic hypothesis, clinical, radiological and therapeutics characteristics of intratympanic primitive meningioma are described. Those tumors arise from arachnoid cells, some may be carried along the nerve sheath within the Fallopian canal during embryology.

Adult↗
PubMed1992

[Gerstmann-Sträussler-Scheinker disease. Pathologal and genetic study].

Gerstmann-Sträussler-Scheinker's disease is a familial spongiform encephalopathy whose pathological hallmark is the existence--especially in the cerebellum--of numerous amyloid plaques. We report here the third clinicopathological case in a French family. Brain tissue from one of its members--initially described as familial Creutzfeldt-Jakob's disease--has been reported as successfully inoculated to monkeys. We present the currently accumulating data favouring the hypothesis of a common etiology for familial Creutzfeldt-Jakob's disease and Gerstmann-Sträussler-Scheinker's disease. The familial characteristics, resulting in different durations of incubation and evolution, could lead to different clinical and histological expressions.

Amyloidosis↗
PubMed1992

[Intracranial plasmocytoma manifesting multiple myeloma: apropos of a case].

Neurological complications of myeloma are multiple and various. Cranial and intracranial locations have been rarely reported. They can be classified into three clinical groups: (1) cranial nerve palsies secondary to single or multiple lesions in the base of the skull, (2) intraorbital tumors, (3) intracranial tumors, either cranial myeloma extending intracranially or pure intracranial tumor (dural and/or cerebral). In our case, macroscopic and microscopic examination of the brain showed dural and cerebral involvement, confirmed by immunohistochemical studies. The radiological features are discussed (CT scan, MRI, angiography). To our knowledge, a single report of intracranial plasmacytoma documented by MRI has been reported in the literature.

Brain Neoplasms↗
PubMed1991

Lacunar infarctions due to cholesterol emboli.

BACKGROUND AND PURPOSE: Hypertension is commonly considered the major cause of lacunar infarctions. However, in some cases, it has been suggested that lacunes could be caused by cerebral emboli from cardiac or carotid sources. Cholesterol cerebral emboli have been rarely reported as a cause of lacunes. CASE DESCRIPTION: We describe a 79-year-old patient with a progressive multi-infarct dementia who developed transient motor aphasia and paresis of the right arm. Computed tomography showed lacunar infarcts in the right caudate nucleus, left thalamus, and left putamen, as well as an old right frontal infarction. Neuropathological examination demonstrated no prominent vascular hyalinosis, but did show multiple cholesterol emboli occluding small arteries around lacunar infarcts and leptomeningeal arteries near cortical infarcts. The cholesterol material presumably originated in the extended atheromatous changes along the aortic arch. CONCLUSIONS: Our report confirms that lacunes can be caused by cholesterol emboli in some patients. Small cerebral emboli should not be overlooked as a cause of lacunes.

Aged↗
PubMed1990

Paraganglioma of the cauda equina. Report of 2 cases and review of 59 cases from the literature.

Paragangliomas of the cauda equina are not so rare as said in the literature. Two additional cases are presented with a global analysis of the 59 cases from the literature. The diagnosis of this pathology greatly benefit of the use of immunostainings as the cells are often neuron-specific enolase, neurofilament protein and somatostatin positive so that electron microscopy is thus no longer mandatory for establishing the diagnosis. In addition, we report the first magnetic resonance images of this tumor at this location.

Adult↗
PubMed1990

Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET.

Regional brain glucose utilisation was investigated with positron emission tomography (PET) and fluorodeoxyglucose (FDG) in four siblings with neuronal ceroid-lipofuscinosis. A consistent pattern was found, namely a decrease of glucose utilisation in all grey structures but more marked at the level of the thalamus and posterior association cortex. The severity of metabolic anomalies was correlated with the degree of clinical impairment and with disease duration; they were the most severe in the oldest patient, who was also the most affected clinically, intermediate in two others, and minimal in the subject with the shortest period of development of the disease. These observations suggest that PET is useful for the definition of anatomical targets of metabolic diseases and for the investigation of their pathophysiology.

Age Factors↗
PubMed1990

Contribution of stereotactic brain biopsies to the diagnosis of presenile dementia.

Precise diagnosis of presenile dementia is a great challenge. The only valuable classification of dementia is the neuropathological one. We present the first series of stereotactic biopsies in 28 presenile patients (18 males, age 39 less than or equal to 58 less than or equal to 69 years, and 10 females, age 23 less than or equal to 52 less than or equal to 66 years). The results demonstrate that the clinical and paraclinical diagnosis (including positron emission tomography scan, neuropsychological examinations, computed tomography scan, electroencephalogram) in only one third of all cases and only in 50% of the Alzheimer and Creutzfeld-Jakob cases. This is very important to be kept in mind when epidemiology or efficacy of drugs are investigated without any neuropathological confirmation.

Adult↗
PubMed1990

[Posterior cortical atrophy: anatomo-clinical variant of Alzheimer's disease].

The authors report the case of a 66-year-old woman who developed progressive occipital dysfunction and lately a dementing illness. Brain CT revealed posterior cerebral atrophy. Post-mortem examination showed the characteristic features of Alzheimer's disease, mainly in the posterior areas, relatively sparing the amygdala and Ammon's horn. The occurrence of focal signs and lesions in Alzheimer's disease is emphasized.

Aged↗
PubMed1990

Acute polyneuropathy after malathion poisoning.

We report a patient in whom intoxication with a commercial preparation of malathion led to a severe acute cholinergic crisis, protracted cardiac arrhythmias, an acute respiratory distress syndrome, and an acute sensorimotor axonal polyneuropathy. The occurrence of the polyneuropathy was associated with a novel malathion transformation product, S-(1-ethoxycarbonyl, 2-isopropoxycarbonyl) ethyl 0,0-dimethyl phosphorodithioate (isopropylmalathion), an impurity present in the pesticide residue. The role of isopropylmalathion in the pathophysiology of the polyneuropathy remains to be determined.

Acetylcholinesterase↗
PubMed1990

[Bilateral, symmetrical and simultaneous putamino-capsulo-caudal infarction].

The first case, to our knowledge, of bilateral, symmetric and simultaneous infarction in the basal territory of the middle cerebral arteries is reported. The patient presented with a pseudo-rostral brainstem syndrome, including loss of oculo-vestibular reflexes, severe tetraparesia and akinetic mutism-like disturbance of consciousness. The lesions were due to embolism, caused by atrial fibrillation, occluding the ostium of the lenticulostriate arteries. It involved only the putamino-capsulo-caudal regions, concerning the whole anterior and posterior dorsal capsules. The term tetraparetic mutism is proposed to describe this new clinico-pathological syndrome, which is the sum of a capsular tetraparesia, due to the lesion of the geniculate and cortico-spinal fibres at the level of the genu and posterior limbs, and of a capsular akinetic mutism, due to the lesion of the reticular thalamo-cortical tracts at the level of the anterior limbs. These total capsular infarcts moreover involved all the oculomotor cortico-reticular tracts, which could explain the absence of oculo-vestibular reflexes.

Aged↗
PubMed1988

Subacute sensory neuronopathy associated with Sjögren's sicca syndrome.

Sensory subacute neuropathies associated with sicca syndrome without any systemic involvement have been reported rarely. A sixth case is described with what appears to be the first report of muscle and nerve biopsy findings. The histological studies revealed axonal degeneration without vasculitis in the sural nerve, and a slight denervation process and a discrete myositis in the gastrocnemius muscle, reflecting a subtle systemic disorder. The clinical course of a long-standing subacute sensory neuropathy, the biopsy-documented axonal degeneration, and the neurophysiological findings suggest involvement of the dorsal root ganglia.

Evoked Potentials, Somatosensory↗
PubMed1988

Antiserum to scrapie-associated fibril protein reacts with amyloid plaques in familial transmissible dementia.

Scrapie-associated fibrils (SAF) are disease-specific markers for the unconventional agent-induced, transmissible spongiform encephalopathies. Polyclonal rabbit antiserum to SAF protein was reacted with brain sections from scrapie-infected mice, two familial cases of transmissible dementia, and three cases of Alzheimer's disease (AD). Specific immunostaining of cerebral amyloid plaques occurred in the scrapie-infected mice and in the two familial cases of transmissible dementia. No immunoreactivity was detected in senile plaques or neurofibrillary tangles in the three cases of AD. Our results suggest that SAF, the causative pathogenic agent, and extracellular deposits of amyloid in the brain are closely related. Immunohistochemical detection of SAF protein could serve as a useful diagnostic adjunct in the postmortem evaluation of difficult cases of dementia. The identification of SAF protein in the brains of two affected members of a family combining the clinical and pathological features of Creutzfeldt-Jakob disease (CJD) and the Gerstmann-Straüssler syndrome (GSS) substantiates earlier conclusions of a nosological relationship between the two. Our study provides further evidence of the similarity of SAF protein to prion protein (PrP 27-30).

Adult↗