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Biomedical subjects

J Julien

Publications and source records attributed to J Julien.

At least 145 records · Page 8Linked to original sources
PubMed1984

IgM demyelinative neuropathy with amyloidosis and biclonal gammopathy.

A 59-year-old man developed a sensorimotor neuropathy of the upper and lower limbs, associated with a biclonal gammopathy, within the space of a few months. Each of two paraproteins was coupled with a distinct IgM kappa IgG lambda light chain. Examination of a nerve biopsy specimen by electron microscopy revealed a demyelinative process with a widening of the interlaminar space in the myelin sheath, as well as deposits of amyloid substance between nerve fibers. Direct immunofluorescence revealed the presence of IgM and of the kappa light chain in certain Schwann cells, while the lambda IgG was fixed to the amyloid deposits. Immunoperoxidase histochemistry showed a positive reaction in normal human nerve tissue to the immune serum IgM and kappa light chain. The findings suggest that the widening of the interlaminar space of the diseased myelin corresponds to an active fixation of immunoglobulin on the sheath of the Schwann cell. The presence of two light chains in this patient's gammopathy caused a dual pathology: the kappa chain, a demyelinative neuropathy, and the lambda chain, a primary amyloidosis, with deposits in the peripheral nerve and in the kidney.

Amyloidosis↗
PubMed1984

Expression of a spore-specific gene in Dictyostelium discoideum.

The expression of a previously cloned Dictyostelium discoideum spore-specific gene (Julien et al., EMBO J. 1, 1089-1093 (1982)) was investigated in wild type and mutant strains. In vitro translation of this spore-specific mRNA gave a protein of a molecular weight consistent with the mRNA size. Expressed at a low level during vegetative growth development and in stalk cells, the accumulation of this mRNA reached high values only in spore cells.

Cloning, Molecular↗
PubMed1984

Cavernous sinus syndrome due to lymphoma.

In both the cases described, painful ophthalmoplegia was the first indication of infiltration of the cavernous sinus by a lymphosarcoma. The onset of symptoms and the course of the disease were different in the two cases. CT scan which has been the crucial investigation for detecting lymphomas in the cavernous sinus, was normal in the early stages. Symptomatic remission could be obtained with treatment, although the prognosis remained poor.

Aged↗
PubMed1984

Chronic demyelinating neuropathy with IgM-producing lymphocytes in peripheral nerve and delayed appearance of "benign" monoclonal gammopathy.

A chronic demyelinating neuropathy with "benign" IgM gammopathy was followed for 6 years in a 63-year-old man. The clinical, biologic, and EMG aspects were similar to those already reported, but a lymphoplasmocytic infiltrate in the nerve connective tissue of this patient has only rarely been observed in benign IgM gammopathy. The paraprotein was not evident in the serum until 5 years after symptoms of the neuropathy started.

Demyelinating Diseases↗
PubMed1984

[Sex-linked familial form of progressive spinal amyotrophy in adults].

An X-linked spinal muscular atrophy is reported in one family. Four of the five patients were examined. In three, electromyography, conduction nerve velocities and muscle biopsy were consistent with anterior horn cell disease. Similar families in the literature were reviewed and characteristic data were: 1) adult-onset, 2) proximal, bulbar and facial involvement, 3) prominent perioral fasciculations, 4) frequent association of cramps, tremor and sexual dysfunction (hypogonadism and gynecomastia), 5) very slow progression and favorable prognosis. These characteristics define, among the spinal muscular atrophies, a distinct entity named by several authors Kennedy's disease.

Adult↗
PubMed1983

Uncompacted myelin lamellae in two cases of peripheral neuropathy.

Peripheral nerve biopsies from two patients with chronic sensorimotor neuropathy were studied. The first case was a non-Hodgkin malignant lymphoma and did not show any dysglobulinemia. The second case had a benign monoclonal gammopathy IgG, Lambda type. Direct immunofluorescence showed no deposits in the first case and slight deposits of anti IgG sera on a few myelinated fibers in the second case. There were numerous fibers showing uncompacted myelin lamellae, 7% in the first case and 4% in the second case. Some of these fibers had axons containing more tubules than filaments. The very few cases reported on neuropathies showing that uncompacted myelin lamellae were frequently associated with dysglobulinemic neuropathy. However, this ultrastructural abnormality of the myelin sheath can be observed without any dysglobulinemia.

Aged↗
PubMed1983

Nuclear inclusions in oculopharyngeal dystrophy. An ultrastructural study of six cases.

Nuclear inclusions in striated muscle from patients with oculopharyngeal dystrophy have been detected recently. We carried out ultrastructural examinations of biopsy specimens on 5 patients with oculopharyngeal dystrophy and we also reexamined a former case. In these 6 cases we found filamentous inclusions in a few nuclei. These inclusions seem to be characteristic of this disease as they have never been seen elsewhere.

Aged↗
PubMed1983

[Familial amyloid neuropathies in 3 families of French origin].

Clinical, electromyographic and neuropathological studies were carried out at different stages of evolution of a familial amyloid neuropathy in 6 members of 3 families of French origin. The clinical onset was marked by sensory symptoms and signs in limb extremities, primary manifestations being alterations in pain and thermal sensitivity. This was followed by motor and amyotrophic disorders predominant in the lower limbs. Autonomic nervous system disorders were frequent later. Early electromyographic signs were diminished amplitude and increased duration of sensory potentials. Progression of the disease is shown by the onset of signs resulting from severe axonomyelinic lesions. Neuromuscular biopsy demonstrated the presence of amyloid deposits in the endoneurium in 5 of the 6 cases. Transmission appeared to be dominant autosomal. These cases pertain to group I of the amyloid neuropathies. The axonal lesions marking the onset of the affection could be secondary to biochemical alterations in prealbumin, responsible for amyloid formation. This hypothesis affords a basis for plasmapheresis which has been used in 3 patients.

Adult↗
PubMed1983

[Recurrent cerebral hemorrhage and amyloid angiopathy].

A patient with no previous relevant history presented with recurrent cerebral hemorrhages of which he died 4 years 5 months after the onset. On autopsy, an amyloid angiopathy was present and localised to the cerebrovascular system. Hemorrhages were associated with some small infarcts in the subcortical regions. The leptomeninges and the cortical arteries were mostly involved by an infiltration mainly in the adventitia and media. There were no senile plaques in the cortex. The amyloid deposit present in the senile plaques and in the vessel walls of the amyloid angiopathy could be the result of two distinct pathological processes.

Amyloidosis↗
PubMed1982

Cloning and analysis of a genomic fragment of Dictyostelium discoideum hybridizing to an RNA specifically accumulated in spore cells.

A marker for Dictyostelium discoideum spore RNA differentiation has been isolated from a genomic DNA library by differential screening and a recombinant plasmid containing a genomic sequence complementary to spore specific RNA has been characterized. Only a small portion ( approximately 1 kb) of the 4.7-kb genomic insert is transcribed. The genomic organization of this spore specific gene shows a unique sequence flanked by reiterated sequences.

Journal Article↗
PubMed1982

Inclusion body myositis. Clinical, biological and ultrastructural study.

A 48-year-old patient presented for the past 4 years an amyotrophy of the quadriceps and moderate involvement of the truncal and pelvic girdle muscles. The CK level was elevated (10 times the normal rate) and the EMG revealed a fibrillation pattern on relaxation, myotonic bursts on needle insertion and reduced activity during contraction. The histological study of the muscle biopsy showed nuclear cytoplasmic inclusion bodies and pseudo-myelinic membranes. The case was classified in the inclusion body myositis group. Analysis of the other published cases underlines the variety of the clinical, biological and electromyographical aspects and abnormalities.

Biopsy↗
PubMed1982

[Comparative study of fungicidal activity in vitro of various quaternary ammonium compounds used in ophthalmology].

A study was conducted to evaluate the antifungal activity in vitro of 5 quaternary ammonium compounds. N cetylpyridinium was found to be particularly active against Candida albicans and Aspergillus fumigatus. The association of N cetylpyridinium and of neosynephrine, found in various eye drop compositions, resulted in potentation of the antifungal activity of this quaternary ammonium.

Antifungal Agents↗
PubMed1980

Guillain-Barré syndrome and Hodgkin's disease--ultrastructural study of a peripheral nerve.

A 46-year-old male patient developed the Guillain-Barre syndrome and recovered completely within 3 months. He had been treated 5 years previously for Hodgkin's disease, and during the neurological syndrome, relapse of the malignant lymphoma was discovered. On neuro-muscular biopsy, lymphocytes were observed penetrating Schwann cells. These neuropathological aspects confirm the auto-immune character of the nervous involvement.

Hodgkin Disease↗
PubMed1980

[Adrenomyeloneuropathies].

An adrenomyeloneuropathy is observed in a patient died at 24 years old after an illness of five years duration. These case is peculiar by the presence of lamellar cytoplasmic inclusions in the Schwann cells. Adrenomyeloneuropathy is an adult variant of adrenoleucodystrophy. This sex linked recessive disorder is related to an excessive amount of long chain fatty acids.

Adrenal Insufficiency↗