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Biomedical subjects

J Mikol

Publications and source records attributed to J Mikol.

At least 109 records · Page 6Linked to original sources
PubMed1980

A new case of Pick's disease. Anatomical and ultrastructural studies.

The fine structure of a cortical frontal biopsy of Pick's disease is described. Pic bodies appear made of unbranched 120 A neurofilaments, sometimes clustered in geometrical pattern. Post-mortem examination, performed 8 years later, reveals typical lesions. The characteristics of Pick bodies are discussed.

Autopsy↗
PubMed1980

Familial mitochondrial myopathy with cataract.

A 62-year-old female had severe progressive ophthalmoplegia associated with facial, pharyngeal and limb muscle involvement. When 40, she had undergone surgery for bilateral cataract present for about 20 years. Biopsies of skeletal muscles indicated myopathy; histochemistry and electron microscopy gave evidence of abnormal mitochondria in type I fibres. Bilateral cataract needing surgical treatment at 32 was the prominent symptom in her daughter, then with only mild facial weakness. Despite absence of ophthalmoplegia, similar pathological changes were observed in an inferior oblique muscle. The child of the former, a 10-year-old clinically healthy boy, had been surgically treated for a bilateral cataract at the age of 3. As indicated by a review of literature, cataract is not an exceptional occurrence in this particular type of ocular myopathy and therefore should be included within its multisystem associations. The same HLA haplotype (A2-B21) was found in the three patients.

Adult↗
PubMed1978

[Lesion of the lateral dorsal thalamic nucleus and alcoholic Korsakoff syndrome].

The authors report a clinico-pathological study dealing with the limbic nuclei of the thalamus in 11 cases of Korsakoff syndrome of alcoholic aetiology. There is no parallelism between the involvement of the latero-dorsal nucleus (L.D.), the medio-dorsal nucleus, the pulvinar and the importance of the retrograde amnesia. The mamillary bodies are always affected. The latero-dorsal nucleus was modified in 9 cases out of 11, the medio-dorsal in 7 cases and the pulvinar in 6 cases. In contrast, the anterior nucleus is normal. The frequency of the involvement of the L.D. is an argument to relate this nucleus with the limbic system. But the lesion of this nucleus is inconstant whereas that of the mamillary bodies is always observed.

Alcohol Amnestic Disorder↗
PubMed1977

Connections of latero-dorsal nucleus of the thalamus. II. Experimental study in Papio papio.

Modifications of the latero-dorsal (L.D.) nucleus of the thalamus have been observed earlier in man in relation to limbic lesion of various etiologies. Our proposal was to determine the role of L.D. in memory disturbances. We attempted to study the connections of L.D. in Papio papio baboon after surgical lesion using silver impregnations as well as traditional techniques. We found three afferent pathways: from the fornix, the posterior cingulate and the parietal cortex (area 7). The most important is the afferent system from the fornix, it terminates in the antero-dorso-medial part of L.D.; the other two afferent pathways have a postero-lateral projection in L.D. The three efferent systems to parietal cortex, cingulate and fornix were not delineated in this study. It was concluded that the antero-dorso-medial portion of L.D. is connected to the limbic system and the ventro-postero-lateral portion integrated into a large parieto-cingulo-parahippocampal circuit to which it is joined by direct and indirect projections with several relays. These connections have important implications, perhaps, in our understanding of memory disturbances.

Afferent Pathways↗
PubMed1976

Fatal systemic carnitine deficiency with lipid storage in skeletal muscle, heart, liver and kidney.

A fatal case of systemic carnitine deficiency is reported. The patient suffered from slowly progressive muscle weakness since early childhood. After the age of 17 years her weakness progressed more rapidly until her death at the age of 20. A pregnancy during the last year of the patient's life was followed by rapid deterioration in her condition. An episode of renal insufficiency occurred at the age of 17 years and hepatomegaly, increased BSP dye retention and intermittent ketoacidosis were present during the last month of her life. Biopsy and autopsy specimens of muscle showed a lipid storage myopathy. Type 1 fibers were selectively severely affected, and many Type 1 fibers were atrophic. Abundant large mitochondria, some also containing abnormal inclusions, were also present in the muscle fibers. At autopsy there was marked accumulation of sudanophilic lipid deposits in all hepatocytes, in the renal tubular epithelial cells, and a patchy increase of lipid material was found in the myocardial fibers. There was marked carnitine deficiency in the patient's liver as well as muscel, while the carnitine palmityltransferase activities in these tissues were abnormally high. The basic metabolic abnormality is assumed to be a defect in carnitine biosynthesis.

Adult↗
PubMed1976

[Familial form of centronuclear myopathy in the adult].

Two adult cases of centronuclear myopathy are described in a family from French Guyana. One of them, aged 23, has a slight weakness despite hypertrophic muscles. A typical picture of centronuclear myopathy was seen on muscle biopsy with atrophy of type I fibers and hypertrophy of II A fibers. His uncle, aged 53, had a progressive weakness of the lower limbs for the last 25 years, with also a pattern of centronuclear myopathy, but with more dystrophic features and atrophy of both type I and II A fibers. The mode of inheritance is dominant. These two cases are compared with the previously published reports. The pathogenesis of centronuclear myopathy is discussed.

Adult↗
PubMed1976

[Study of 5 cases of ocular myopathy].

The authors reported five cases of eyelid ptosis with progesssive external ophthalmoplegia. They emphasize infra-clinic diffusion, fatigability and variability impairment of extra-ocular muscles; coexistence of myogenic E.M.G. and single potential recurring at high frequency in the same patient; mitochondrial abnormalities in type I fibers, intense oxydative activity in superior oblique muscle and normal motor end-plates.

Adult↗
PubMed1975

[External temporal biopsy in a case of infiltrating limbic glioma: study of astrocytic membranous wrapping whorls (author's transl)].

An intracranial hypertension was found at autopsy to be due to a limbic infiltrating glioma. A cortical temporal biopsy had been done 14 months before, whose ultrastructural results are reported. Membranous whorls, made of two dense membranes with a variable separative space are seen around neurones, dendrites, axons, synapses and oligoglial cells; they derived from astrocytes and are joined by 2 types of cell junctions: gap-junctions and puncta adherentia. Relations between reactive and tumoral gliosis are discussed.

Adult↗
PubMed1975

[Familial case of myotonia with muscular hypertrophy, weakness corrected by effort and atrophy of type II fibers].

A 35 years old man has a non progressive muscle disease which appeared when he was 6. Clinically, there is a slight muscle hypertrophy, an important spontaneous myotonia and a curious muscle weakness, quite marked on the first efforts, but disappearing entirely after a few muscle contractions. The E.M.G. is normal but for the myotonic reaction. Muscle biopsy shows a selective atrophy of type II fibers. The disease is a genetic one, a sister and a brother of our patient having noticed the same symptom. The place of this disease among the congenital myotonias is discussed.

Adult↗