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Biomedical subjects

J M Brucher

Publications and source records attributed to J M Brucher.

At least 73 records · Page 4Linked to original sources

[Papilloma of the choroid plexus of the lateral ventricle without generalized hydrocephalus].

A benign papilloma of the choroid plexus of the left lateral ventricle is found in a five-month old infant. An important dilatation of the isolated occipital horn is observed, without generalized enlargement of the ventricles. The clinical signs are limited to a moderate macrocephaly with deviation of head and eyes to the left. The absence of global hydrocephalus is rather exceptional in such cases, where overproduction of cerebrospinal fluid, in an amount proportional to the tumoral mass, is generally admitted. Nevertheless, the literature offers no absolute proof of the role of oversecretion alone in the origin of hydrocephalus, whilst an eventual obstruction of the circulatory pathways and resorption areas of the C.S.F. must be kept in mind. The absence of hydrocephalus, in the present case, suggests that the subarachnoid spaces and arachnoid villi remained patent.

Cerebral Ventricle Neoplasms↗

Stereotactic brain biopsies in presenile dementia.

Early detection and precise diagnosis of presenile dementias are important since they determine subsequent medical care. Presently the only way to classify these syndromes is by neuropathological examination. Between 1972 and 1987, 222 stereotactic serial brain biopsies were performed in 25 selected demented patients. Comparison between preoperative clinical diagnosis and histological finding gives the following results: in 32% the clinical diagnosis has been confirmed, in 60% the diagnosis has been corrected or specified and in 8% the stereotactic brain biopsies only showed unspecific pathological gliosis.

Biopsy↗

Early AIDS cases originating from Zaïre and Burundi (1962-1976).

Three retrospectively diagnosed fatal AIDS cases are reported and discussed together with 4 other HIV seropositive patients, who all apparently contracted the HIV infection prior to the beginning of the present outbreak, without any other risk factor than heterosexual exposure in Central Africa. These data and simultaneous information from other sources contribute to the assumption that AIDS is an old disease in Central Africa. AIDS, having presented itself as a sporadic and ill defined entity, remained unrecognized in Central Africa until the outset of the present outbreak.

Acquired Immunodeficiency Syndrome↗

[Association of cerebral cysticercosis and multifocal glioma. Discussion of interactions].

Diagnosis in a case of cerebral parenchyma cysticercosis was based on conventional arguments described in the literature. The patient died after progression of the disease over 6 months, despite two courses of Praziquantel. Results of neuropathologic examination confirmed diagnosis of cysticercosis and the discovery of a multifocal astrocytic tumor involving mainly the periventricular regions. Possible interactions between parasitic infection and glioma are discussed but it is difficult to determine the precise role played by the parasitosis in the evolution of the tumor, the hypothesis of a simple coincidence being most likely. Periventricular images of an inflammatory nature observed after PZQ treatment should not be accepted unreservedly, and when an unfavorable course is apparent the periventricular histopathologic lesions should be compared with results of CT scan imaging qualified as periventricular in origin.

Brain Diseases↗

Spinal arachnoiditis due to aspergillus meningitis in a previously healthy patient.

A 30-year-old, previously healthy, non-addicted man presented with a chronic spinal meningitis complicated by arachnoiditis and spinal cord compression. Biopsy showed a chronic granulomatous leptomeningitis, in which some cells contained branching septate organisms that were immunostained with an antiserum to Aspergillus fumigatus. Precipitins to A. fumigatus were detected in cerebrospinal fluid (CSF), but not in blood, and aspergillus infection was apparently restricted to the leptomeninges. Clinically successful treatment led to the disappearance of CSF precipitins and oligoclonal bands.

Adult↗

Rupture of the arterial wall in two cases of recent cerebral embolism: morphological expression of cerebral vasospasm?

Previously undescribed morphological changes are reported in two cases of recent, non-septic cerebral embolism. These consisted of an arterial rupture involving the whole thickness of the wall. A direct tear by a sharp embolus seems unlikely but, as proposed by Villaret et al. in 1937, the arterial spasm may have occurred at the time of cerebral embolism. The embolic occlusion would prevent narrowing of the vascular lumen, so that focal spasm would rupture the arterial wall even at the level of a healthy and relatively thick media.

Arteries↗

The neurovirulence of human and animal rotaviruses in cercopithecus monkeys.

Cercopithecus monkeys were inoculated according to the specifications of neurovirulence safety test for live rubella virus vaccine with RIT 4237, a rotavirus vaccine candidate. RIT 4237 is a high passage level of the Nebraska Calf Diarrhoea Virus (NCDV). The histological findings in the first test indicated some involvement of the central nervous system. The same test was therefore repeated with RIT 4237, with a lower passage level of the NCDV strain, and with the 'Wa' strain, a human virus grown in tissue culture. Clinical signs and histological findings were concordant and demonstrated that all the viruses were moderately neurovirulent. As in the poliovirus neurovirulence test, the histological lesions depended mainly upon a correct inoculation in the lumbar cord. RIT 4237 was found to have the same degree of neurovirulence as the low-passage NCDV or as the 'Wa' strain.

Animals↗

Gamma-aminobutyric acid-transaminase deficiency: a newly recognized inborn error of neurotransmitter metabolism.

Cerebrospinal fluid aminoacid analysis in a girl with severe psychomotor retardation, hypotonia, hyperreflexia and growth acceleration showed highly increased levels of free gamma-aminobutyric acid (4.8 mumol/l; range in twenty controls 0.04-0.12, median 0.08), homocarnosine, a dipeptide of gamma-aminobutyric acid and histidine (23.4 mumol/l; control range 4.0-8.7, median 7.6) and of beta-alanine, an alternative substrate for gamma-aminobutyric acid-transaminase (0.48 mumol/l; control range 0.02-0.06, median 0.05). Liver gamma-aminobutyric acid-transaminase activity was deficient (0.07 mumol/mg protein h; range in ten controls 0.31-0.69, median 0.38). Fasting plasma growth hormone levels were increased (7.9-38.4 ng/ml; nl less than 5). Brain evoked responses were suggestive of leukodystrophy. A brother of this patient, showing a similar clinical picture, had died at one year. Postmortem examination of his brain showed leukodystrophy of the type seen in amino acidopathies such as phenylketonuria. This appears to be the first report of gamma-aminobutyric acid-transaminase deficiency.

4-Aminobutyrate Transaminase↗

Cerebral amyloid angiopathy as a cause of multiple intracerebral hemorrhages.

Clinical and pathologic findings in six autopsies and five biopsies of cerebral amyloid angiopathy associated with cerebromeningeal hemorrhages are presented. One patient had experienced a previous meningeal hemorrhage. Only two had chronic hypertension; the multiple fresh hematomas found in all the autopsied brains always spared the basal ganglia and brainstem, as did vascular lesions, which were mostly cortical and meningeal. Extensive lesions of Alzheimer's disease were found in the autopsied cases with dementia. The most significant feature for clinical diagnosis of hemorrhagic cerebral amyloid angiopathy is the presence of multiple hemorrhages in unusual locations in the absence of hypertension.

Aged↗

Low grade astrocytoma in children treated by surgery and radiation therapy.

Results of treatment of low grade astrocytoma by surgery and postoperative irradiation in 35 children under 15 were retrospectively analysed. The actuarial overall 5 and 10 year survival was 94 and 82 per cent, the actuarial relapse-free survival 87 and 70 per cent, respectively. The male patients fared better, although not significantly, than the females. No significant difference in relapse rate was found between cystic and solid tumors. The cystic processes had a more indolent course, but a fatal outcome was noted in one case (after 21 years). No relapse occurred in totally removed tumors (10 cases), which throws doubt on the need for postoperative irradiation in this subgroup. In the subtotally resected processes (24 cases) radiation therapy seemed to be beneficial, as in 3 of 4 local recurrences geographic misses of tumour remains probably existed. No dose response relationship was apparent. The performance status was severely impaired in 11 per cent (3/27 patients). Growth retardation was observed in 2 patients.

Actuarial Analysis↗

[Infarction of the olivary area of the medulla oblongata].

The two first cases of an infarct involving the arterial olivary territory of the medulla oblongata are reported. In the first one, an old infarct of the medial territory and a recent infarct involving the whole inferior olivary nucleus at its middle third level were associated. Rostrally this infarct was reduced to the medial reticular formation and internal half of the olive. Caudally it was reduced to its external half. In the second case the infarct involved only the external half of the right olive, but involved also the external half of the left olive with the lateral territory. From these two cases and from the anatomic, pathologic and clinical data reviewed in the literature, the authors propose as "total" olivary territory a triangular area including all the inferior olivary nucleus, the dorsal accessory olivary nucleus and partly the medial reticular formation, and as "minimum" olivary territory the external half of the olive. The many important medial, lateral and cranio-caudal arteries which supply the olivary territory account for these "total" and "minimum" territories, as well as for some cases in the literature. They account also for the pyramid-like cranio-caudal extension of the olivary territory infarction. The olivary territory is likely to be supplied by two short circumferential arterial groups, i.e. inferior bulbar and superior medullo-pontine. Nevertheless, the cause of the olivary territory infarction is an occlusion of the vertebral artery.

Arteries↗

Idiopathic recurrent myoglobinuria and persistent weakness.

The case of an adolescent with idiopathic recurrent myoglobinuria is reported. The following features are emphasized: (1) persistence of limb weakness and elevated CK levels between the attacks, (2) a constant myopathic pattern in EMG, and (3) chronic morphologic abnormalities of myopathy in two muscle biopsies. All known causes of myoglobinuria were investigated and ruled out.

Adolescent↗

[Anatomo-clinical study of a neuronal form of Charcot-Marie-Tooth disease].

A 79 years-old man suffered gait disturbance for more than ten years. During the last year the gait worsened. Twenty days after having developed a right hemiparesis, he died from cardiorespiratory failure. Neurological signs were characterized by cogwheel rigidity, distal paresis and amyotrophy of the legs. E.M.G. demonstrated a denervation; ulnar conduction velocity was 38 m/s. The sural nerve biopsy showed a loss of myelinated fibres and no onion-bulb-like formations. A sister (72 years) and two nephews (51 and 39 years) also suffer gait disturbance. The E.M.G.'s demonstrate denervation of the legs. The amplitudes of the motor and sensory nerves are reduced and conduction velocities range between 39 and 59 m/s. Pathologic examination of the propositus showed recent left frontal subcortical infarction, partial neuronal depopulation of the substantia nigra and Lewy inclusions. Microscopic examination of the spinal cord showed integrity of the posterior columns, anterior and posterior spinal roots, cauda equina, and of a thoracic posterior root ganglion. The anterior horns showed a mild gliosis and a slight neuronal depopulation with a few pale or atrophic neurons and very rare neuronophagias. This is believed to be the first autopsy report of a dominant sensory motor neuropathy of the neuronal type. The relative integrity of the spinal cord suggests that the peripheral motor and sensory neurons are affected to the greatest degree at their distal extremity.

Aged↗

Progressive pontobulbar palsy with deafness: clinical and pathological study of two cases.

Among the hereditary affections of the nervous system associated with deafness, a rare condition called "progressive pontobulbar palsy with deafness" has been described. In this slowly progressive condition, hearing loss and vestibular are-flexia are almost always the first symptoms, occurring in late childhood or early adulthood. Only 18 cases-some sporadic, several familial-have been published without a full report of pathological findings. The clinical and pathological data of two new cases-one familial, one sporadic-are described here. There are differences from other forms of bulbar paralysis, lower motor neuron diseases, and some spinocerebellar hereditary affections. In view of the homochrony and homotypy in familial cases and the pathological findings, progressive pontobulbar palsy with deafness appears to be an abiotrophic process wih autosomal recessive inheritance.

Adolescent↗