[Normolipemic diffuse xanthomatosis disclosing IgG kappa myeloma].
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Humans↗
Biomedical subjects
I Krivosic
Publications and source records attributed to I Krivosic.
[Exophthalmos in children: contribution of the scanner and MRI in diagnosing spontaneous hematoma].
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Child, Preschool↗
Pigmentary type of orthochromatic leukodystrophy (OLD): a new case with ultrastructural and biochemical study.
A 34-year-old woman with no family history of orthochromatic leukodystrophy (OLD) developed progressive intellectual deterioration, a frontal syndrome and spastic tetraparesis. She died four years after the onset of the clinical illness. Neuropathological studies included light and electron microscopy of cerebral and nerve biopsies, and a complete postmortem examination. Light microscopy demonstrated OLD with pigmented macrophages and glial cells. Electron microscopy showed electron-dense, membrane-bound intracytoplasmic lamellar inclusions with curved or straight parallel arrangement, or fingerprint pattern, in white matter macrophages, astrocytes and oligodendrocytes. Cortical cells contained lipofuscin which was normal in type and amount. This suggests that the material in white matter glial cells and macrophages is ceroid pigment, however, the distribution is not that seen in ceroid-lipofuscinosis. Similar inclusions have been found in oligodendrocytes in other forms of OLD. Biochemical study did not show evidence of demyelination. Galactolipids were normal. Polyunsaturated fatty acids were decreased. The most striking feature was an increase in plasmalogens.
Adult↗
Chondroma of the cervical spine. Case report.
The authors report a case of cervical chondroma presenting with a syndrome of spinal cord compression in a 76-year-old woman. Total surgical removal of the lesion was followed by partial neurological recovery. Chondromas of the vertebral column are rarely reported in the literature.
Aged↗
[Neuropathies and almitrine. 14 cases].
Previously reported cases of peripheral neuropathies occurring during almitrine therapy had only a few weeks follow-up after having stopped the drug. We have studied clinical and electrophysiological data 6 to 12 months after almitrine had been given up in 9 patients from a group of 14 whose epidemiologic, clinical, electrophysiological and pathological data had been registered. In 7 of them, without any chronic respiratory deficiency, almitrine was administered as almitrine bismésilate and raubasine, and in 7 others (6 with chronic respiratory deficiency) as almitrine bismesilate alone. In patients who had another possible cause of neuropathy, clinical disorders appeared after a lesser total quantity of almitrine (p less than 0.05). Clinical data were suggestive of sensory peripheral neuropathies of feet and lower third of legs. Electrophysiological data suggested distal axonopathy in spite of the absence of denervation: amplitudes of sensory potentials were reduced and nerve conduction velocities were moderately decreased. Biopsies revealed mild neurogenic atrophy of muscles and distal axonopathy. Clinical improvement was very slow and 6 to 12 months later, most of the patients still presented decreased vibration sense and ankle reflexes loss, but all of them were still improving. Amplitudes of sensory potentials and sensory nerve conduction velocities were significantly improved (p less than 0.05) but motor nerve conduction velocities were not (p greater than 0.05). Our study shows: 1) clinical, electrophysiological and pathological data similar to those previously reported; 2) subclinical disturbances of motor functions in lower limbs and sensory functions in upper limbs; 3) some patients presented with unusual signs: posture tremor (3 cases), painful legs and moving toes (1 case); 4) peripheral neuropathies may occur during almitrine therapy even in patients without any chronic respiratory insufficiency; 5) peripheral neuropathies occurred with lower doses in patients with other factors predisposing to neuropathies; 6) patients' improvement was very slow; 7) in 9 cases the imputability of these peripheral neuropathies to almitrine is plausible. We suggest not to prescribe almitrine without caution, especially in patients with other factors of neuropathy. Treatment should be regularly interrupted.
Aged↗
[A new model of a temporary non-traumatic caval filter].
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Filtration↗
[Spinal epidural dumbbell-shaped cavernous angioma].
A 23 year-old man, with a two month-history of back pain, presented with spastic paraplegia and hypesthesia of both lower limbs. A myelo C.T. revealed a spinal epidural dumb bell-shaped mass extending in the intra and extra vertebral spaces through the enlarged intervertebral foramen. Total recovery was observed three months after surgery. Pathological examination of the tumor showed it to be a cavernous angioma. Primary epidural angioma is rare: in 2 reported dumb bell-shaped spinal epidural cavernous angiomas, radiological findings were also suggesting a spinal neurinoma.
Adult↗
Pleocore disease. Multi-minicore disease and focal loss of cross striations.
We report clinical and morphological data on seven patients with a congenital myopathy as well as data concerning five parents. Classical myopathies such as rod disease, centronuclear myopathy or central core disease could be ruled out. Structural abnormalities of intracellular organelles or particulate inclusions were rare and insignificant. The most prominent and constant features were minicores and focal loss of cross striations, associated with a prevalence of type 1 fibres, increasing with the age at time of biopsy. A carrier state could not be defined in the five examined parents neither on clinical nor on morphological grounds. Although our group of patients could not clinically be distinguished from other congenital myopathies, the combination of the lesions allow their individualization as a subgroup of multicore or minicore disease under the already proposed denomination of pleocore disease [Martin and Busch, abstract in Zentralbl Allg Pathol 124:156 (1980)].
Adolescent↗
[Examination of the family of a patient susceptible to anesthetic malignant hyperthermia].
The familial nature of anaesthetic malignant hyperthermia must lead to the search for susceptibility in other members of the family of a sensitive subject. According to the literature, only in vitro studies of contraction characteristics of biopsied skeletal muscle fibres exposed to caffeine or halothane are of predictive value. Four members of the family of a patient who died as a result of malignant hyperthermia were investigated. The results expressed in accordance with the criteria defined by the European group on malignant hyperthermia, coupled with a histoenzymatic study, defined three of the subjects as HMN (negative) and one subject as HM (c) (equivocal or intermediate).
Adolescent↗
[11 cases of neuropathy induced by almitrine, of which one had optic neuropathy].
The only etiologic factor retained in 11 patients with sensory or sensory-motor neuropathy was almitrine therapy. In one patient there was in addition an optic neuropathy. The reduction in visual acuity in this patient coincided with the onset of the sensory-motor neuropathy of lower limbs after treatment with 100 mg/day of almitrine over a 2-year period. No other metabolic, inflammatory, toxic, vascular or immunologic cause was found. There was a moderate chronic respiratory insufficiency. Visual recuperation started one month after the arrest of almitrine treatment and was satisfactory 7 months later. The other 10 patients had neuropathy of limbs without visual disorders. Neuromuscular biopsy in one case showed lesions to be of the axonal type.
Aged↗
[Intraspinal hemangiopericytoma. 2 cases and review of the literature].
Two cases of intraspinal meningeal hemangiopericytomas are reported, one of which cervical and the other one dorsal with relapse. The anatomopathological features of the hemangiopericytomas are recorded and the nosological problems of their meningeal localizations are discussed. As in the case of intracranial meningeal hemangiopericytomas, the clinical, radiological and macroscopic features of intraspinal hemangiopericytomas--to the exception of a few particular cases--resemble those of other localizations of these tumours more than those of meningiomas, in the light of the review of twenty-eight cases to be found in the relevant literature. Treatment of such tumours hasn't as yet been well circumscribed. Surgical treatment may be facilitated by preoperative embolization. Radiotherapy must be associated to it, at least when removal is incomplete, because of frequent recurrence.
Adult↗
[Myocardiopathy disclosing a centronuclear myopathy in an adult].
The authors report the case of a primary myocardiopathy of the young adult, related in fact to a rare muscular congenital disease: centro-nuclear myopathy. The case is different from the classic picture in that it is revealed late, by its cardiac manifestations which dominate the clinical picture and lead to an irreducible cardiac insufficiency requiring a heart transplant. Only a muscular biopsy with histological and histo chemical study permits to make the diagnosis, while the lesions of the myocardium are non-specific.
Adult↗
An enzyme histochemical study of human sinus node, coronary sinus, and mitral valve muscle.
An enzyme histochemical study of the sinoatrial node, the coronary sinus, and the atrial muscle extending into the anterior mitral valve was performed on human hearts. Investigation of the activity and localization of the structurally bound enzymes was performed by conventional histochemical techniques. Determination of the activity of nonstructurally or weakly structurally bound enzymes was performed by histochemical techniques in which leakage of enzymes during the incubation period was reduced by the application of semipermeable membranes. The sinoatrial node is characterized by a high degree of anaerobic enzyme capacity and a relatively low degree of aerobic enzyme capacity. The discriminatory nature of these reactions allows examination of the structure of the sinoatrial node and its approaches. The presence of transitional cells was confirmed; isolated clusters of nodal cells were found in the atrial myocardium around the sinoatrial node, but no evidence of specialized tissue forming the beginning of an internodal pathway was found by this technique. The specific histochemical reactions that characterize the sinoatrial node also occur in the atrial muscle, extending into the anterior mitral valve, the anterior wall of the coronary sinus, and the atrial tissue near the orifice of the coronary sinus. These observations seem to corroborate the hypothesis that arrhythmic ectopic foci can arise in these regions.
Acetylcholinesterase↗
[Cystic meningioma. 3 cases].
The association of a meningioma and an arachnoid cyst is one of the many association of cyst and meningioma. The diagnosis could be difficult by CT scan. We report three cases of parameningiomal cysts and discuss their different mechanism of formation before stressing practical problems.
Adult↗
A giant intrasacral schwannoma revealed by lumbosacral pain.
A case of giant intrasacral schwannoma is reported. This tumour was revealed by a lumbosacral pain without any neurological deficit. It appears that the lesion is surrounded by a dense layer of nucleated cells which resemble immature cells. This corona of young cells could explain the tremendous size of this tumour.
Adolescent↗
Hemangiopericytic meningioma of the pineal region. Case report.
A case of hemangiopericytic meningioma of the pineal region is reported. We discuss the origin of meningiomas in this region and their histological nature. The rapid recurrence in situ of the tumor in this particular case seems to confirm the need to complement surgery with radiation therapy.
Adult↗
[Uveitis and cerebral lymphoma].
A 57 year-old man developed a left posterior uveitis, without obvious aetiology and unresponsive to antibiotherapy and steroids. Nine months later, a lymphoma of the left parieto-occipital area was discovered. An immunological study detected a peripheral blood T lymphocytes deficiency, a B lymphocytes excess and a monoclonal gammopathy (IgM lambda). The latter abnormality disappeared one month after neurosurgery. Tumor cells were of the B lymphoid type with lambda light chain only. Brain irradiation and chemotherapy first improved neurological signs but right uveitis developed. The patient died 22 months after the first ophthalmological symptoms. The association of primary non Hodgkin brain lymphoma (PNHBL) (reticulum-cell sarcoma) with uveitis has already been described in 51 other cases in the literature. Two possibilities are considered: first, uveitis appears with or follows the neurological signs (NS): the prognosis is the same as that of PNHBL alone; second, uveitis appears before the NS: the prognosis is better and can be improved by brain and ocular irradiation. The immunological abnormalities are discussed. T lymphocytes deficiency and B lymphocytes excess have already been described but a monoclonal gammopathy is a more unusual finding.
Brain Neoplasms↗
[Myositis disclosing systemic scleroderma].
A 61 year old patient presented with muscular atrophy and weakness, predominant at the shoulder girdle, as the first manifestation of a progressive systemic sclerosis with the signs of the CREST syndrome: subcutaneous calcinosis, Raynaud phenomenon, esophagus disorders, sclerodactyly, telangiectasia. The histological features were consistent with polymyositis. In spite of corticotherapy, total recovery was not obtained. The nosological relationships with Mixed Connective Tissue Disease is discussed. Myositis is infrequent in progressive systemic sclerosis and corticosteroid therapy is discussed.
Calcinosis↗