Neurological manifestations in Sjögren syndrome.
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Female↗
Biomedical subjects
M P Chaunu
Publications and source records attributed to M P Chaunu.
[La Revue Neurologique has received the following correspondence].
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Adrenal Cortex Hormones↗
Changing pattern of headache pointing to cerebral venous thrombosis after lumbar puncture and intravenous high-dose corticosteroids.
OBJECTIVE: To emphasize the diagnostic importance of change in the headache pattern which pointed to cerebral venous thrombosis in two patients after lumbar puncture and high-dose intravenous methylprednisolone for suspected multiple sclerosis. RESULTS: Both patients had a diagnostic lumbar puncture for suspected multiple sclerosis and were treated with high-dose intravenous methylprednisolone. Both developed a postlumbar puncture headache that was initially postural, typical of low cerebrospinal fluid pressure. Three days later, the headache became constant, lost its postural component, and was associated with bilateral papilledema. Magnetic resonance imaging of the brain disclosed superior sagittal and lateral sinuses thrombosis. The diagnostic difficulties of such cases and the potential role of lumbar puncture and corticosteroids as risk factors for cerebral venous thrombosis are discussed. CONCLUSIONS: When a typical postdural puncture headache loses its postural component, investigations should be performed to rule out cerebral venous thrombosis, particularly in the presence of other risk factors.
Adult↗
[Main semeiologic characteristics of ptosis].
BACKGROUND: We report the main characteristics of blepharoptosis. MATERIALS AND METHODS: All cases of blepharoptosis treated at the Reims University Hospital from 1992 to 1997 were reviewed. Ptosis, levator function, palpebral aperture, and the position of the upper crease were recorded as well as results of the epinephrine test, acetylcholine esterase inhibitor antibodies and computed tomography findings. RESULTS: There were 96 unilateral and 34 cases of bilateral blepharoptosis (164 cases). The cases of congenital blepharoptosis (36 cases) were usually unilateral with severe ptosis and poor levator function. In cases with Claude Bernard Horner syndrome, the blepharoptosis was unilateral with minimal ptosis and a positive response to neosynephrine. In cases with oculomotor nerve palsy (31 cases) the ptosis was moderate to severe and levator function was poor. In those with myasthenia, the prostigmine test was positive. Levator aponeurotic disinsertion was observed in 16 cases with severe ptosis, positive response to neosynephrine and a high upper crease. There were three cases of trauma-induced ptosis, 5 cases of myopathy and 26 cases of idiopathic ptosis. DISCUSSION: The clinical presentation of blepharoptosis is usually related to the etiology. Bilateral blepharoptosis is most often observed in congenital forms, levator disinsertion or idiopathic cases. Male sex predominates in congenital ptosis and oculomotor palsy, and female sex in Claude Bernard Horner syndrome. Moderate to severe ptosis is observed in congenital ptosis or oculomotor palsy. The upper crease is absent in many cases of congenital blepharoptosis and is high in case of levator disinsertion. The response to neosynephrine is positive in Claude Bernard Horner syndrome and aponeurotic disinsertion. The response to prostigmine is positive in case of myasthenia. CONCLUSIONS: Clinical aspects of blepharoptosis are related to etiology. The different features guide surgical or medical treatment of blepharoptosis.
Adolescent↗
Modifications of oligodendroglial cells in spongiform encephalopathies.
Although gray matter lesions involving neurones and astrocytes are prominent in human transmissible spongiform encephalopathies (TSE), white matter lesions have also been occasionally observed. Secondary (Wallerian) degeneration and direct myelin damage have been invoked, but the physiopathology of white matter involvement is still debated. We performed an immunohistochemistry study with anti-PrP antibodies of autopsy material of four patients with Creutzfeldt-Jakob disease (CJD), together with transmission electron microscopy (TEM) studies of conventionally processed biopsy specimens of the same patients. Light microscopy immunolabeling was observed as arrays adjacent to myelinic fibers and as a clumps adjacent to oligodendroglial nuclei; both cerebrum and cerebellum were involved. At the ultrastructural level, two types of intracellular inclusions were seen in the white matter. They were associated with dense lysosomes in oligodendroglial perikarya and in their processes. The inclusions were made of finely fibrillar, paracrystalline, amorphous, or densely osmophilic material. Thus, our findings may suggest that white matter involvement in spongiform encephalopathy is due to direct modifications of oligodendroglial cell associated with abnormal metabolism of PrP.
Aged↗
Putative neurosurgical transmission of Creutzfeldt-Jakob disease with analysis of donor and recipient: agent strains.
A woman, aged 59 years, underwent a cortical biopsy that led to the diagnosis of Creutzfeldt-Jakob disease (CJD). A man, aged 46 years, underwent cranial surgery in the same department 3 days later for brain contusion, with an uneventful recovery. Twenty six months later, he developed clinical signs of CJD with a typical EEG pattern. Both cases exhibited features of the 'ataxic' form of the disease with depletion of cerebellar granule cells, without kuru plaques or PrP deposits. PrP deposits were immuno-histochemically observed in the cerebrum, spinal cord and peripheral nerve. Molecular genetic analysis performed on brain tissue revealed the codon 129 polymorphism to be Met129Met in the donor and Met129Val in the recipient. The shared 'cerebellar' phenotype and the genotypic discrepancy between the two patients lead us to postulate that the 'cerebellar' agent strain plays a major role in CJD phenotype and transmission.
Biopsy↗
[Neuropathy caused by necrotizing vasculitis in HIV-1 infection].
A 33 year-old male homosexual infected with human immunodeficiency virus type I developed an asymmetrical and painful neuropathy in the lower limbs. Neuro-muscular biopsy showed a necrotizing vasculitis. There were no clinical features indicative of systemic vasculitis. Prednisone therapy dramatically improved the neuropathy, without adverse effects. Although rare, necrotizing arteritis must be considered in HIV-1 patients with neuropathy, especially in case of mononeuropathy multiplex and when immunodepression is mild or absent, since a successful corticosteroid therapy can be prescribed.
Adult↗
[Characterization of inflammatory cell infiltrates of peripheral neuropathies in human immunodeficiency virus infection].
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HIV Infections↗
[Central and peripheral neurologic lesions in primary Gougerot-Sjögren syndrome. Clinicopathological study of a case].
A 51 year old woman presented with cerebellar ataxia and paralysis of conjugate lateral movement of the eyes. She had xerophtalmia and xerostomia. Biopsy of minor salivary glands showed mononuclear infiltrates typical of Sjögren's syndrome. Peripheral neuropathy was of the axonal type, and nerve biopsy showed a vasculitis made of a mononuclear infiltrate with only a few polymorphonuclears. The patient deteriorated despite corticosteroid therapy, plasma exchange and cyclophosphamide. Ciclosporin seemed to be transitorily beneficial. Epileptic seizures (auditory hallucinations) occurred. MRI showed hypersignal in the right temporal lobe. At post-mortem examination, a small ischaemic lesion with a large lymphocytic infiltrate was present in the left putamen. Neuronal loss and gliosis with some rod cells and glial stars were found in the brainstem, dentate nucleus and internal part of the right temporal lobe. Inflammatory changes were limited. The posterior columns of the spinal cord were pale, and some residual nodules of Nageotte were present in the spinal ganglia. These lesions were similar to those seen in encephalomyelitic syndromes associated with carcinoma. Lesions of the peripheral nerves were associated with vasculitis but changes in the central nervous system were morphologically similar to those observed as remote effects of carcinoma.
Central Nervous System Diseases↗
The spectrum of changes on 20 nerve biopsies in patients with HIV infection.
Nerve and muscle biopsies were performed on 20 patients with HIV infection and peripheral neuropathy. Nine patients had distal symmetrical peripheral neuropathy (DSPN) (six ARC and three AIDS), six had inflammatory demyelinating polyneuropathy (IDP) (three ARC, one AIDS, and two otherwise asymptomatic patients), one had mononeuropathy multiplex (MM) (AIDS), 1 had mononeuropathy (ARC), one had meningoradiculitis (AIDS), and two had areflexia-associated lymphocytic meningitides (ARC), DSPN exhibited axonal degeneration in four of nine cases and was associated with segmental demyelination in five of nine cases. IDP exhibited segmental demyelination associated with axonal degeneration in four of six cases. Demyelination was more frequent in asymptomatic patients (2 of 2 cases) and in ARC (7 of 12 cases), whereas axonal degeneration was predominant in AIDS (6 of 6 cases). Mononuclear cell infiltration was seen in 1 of 2 asymptomatic patients and in 11 of 12 ARC patients but was exceptionally found in AIDS (1 of 6 cases). Involvement of the walls of small vessels, mostly venules ("subacute microvasculitis"), was found in 1 of 2 asymptomatic patients, in 8 of 12 ARC patients, and never in AIDS. The polyclonal mononuclear cell population was composed mainly of Leu 2 (T8) positive cells in seven cases of ARC. No virions were seen in electron microscopy. HIV was isolated in two cases from the CSF or the nerve biopsy.
Acquired Immunodeficiency Syndrome↗
Peripheral neuropathies during treatment with almitrine: report of 46 cases.
Almitrine bismesylate is thought to cause sensory peripheral neuropathy. Forty-six patients are reported who received almitrine bismesylate alone for chronic respiratory failure or in combination with raubasine for various cerebrovascular diseases. Polyneuropathy appeared between 9 and 25 months after the onset of treatment. Sensory signs and symptoms were confined to the distal parts of the lower limbs and involved large and small fibres. Histological and electrophysiological findings indicated axonal degeneration. Respiratory failure could have caused the polyneuropathy in some cases but many had no chest disease. Patients began to improve between 3 and 6 months after withdrawal of the drug. Recovery was usually complete after 12 months.
Aged↗
The spectrum of polyneuropathies in patients infected with HIV.
Twenty five patients with peripheral neuropathy at different stages of human immunodeficiency virus (HIV) infection are reported. Cerebrospinal fluid (CSF) findings were available in 17 cases, electrophysiology in all and a neuromuscular biopsy in 11. Of six otherwise asymptomatic HIV+ patients, five had chronic inflammatory demyelinating polyneuropathy (CIDP) and one acute inflammatory demyelinating polyneuropathy (AIDP). CSF showed pleocytosis in all cases. Infiltration of the endoneurium and/or the epineurium by mononuclear cells was seen in biopsies from three cases. These six patients recovered either spontaneously, or with corticosteroids or plasmaphereses. Of five patients with AIDS related complex (ARC), three had distal predominantly sensory peripheral neuropathy (DSPN), one CIDP and one mixed neuropathy. Of 14 patients with AIDS, one had mononeuropathy multiplex and 13 painful DSPN. Electrophysiological studies were consistent with an axonopathy. Nerve biopsies in six cases showed axonal changes but surprisingly associated with marked segmental demyelination in two cases. Cell infiltration was present in nerve samples in two cases. Five patients died within six months after the onset of the neuropathy.
AIDS-Related Complex↗
[Myelopathy, polymyositis and systemic manifestations associated with the HTLV-I virus].
A case of HTLV-I associated myelopathy in a 51 year-old Haitian woman is reported. MRI showed high signals in the cerebral white matter on T2-weighted images. There also was clinical and electrophysiological evidence of myositis, and a biopsy of the quadriceps muscle showed dense inflammatory infiltrates surrounding several small perimysial blood vessels. The virus was not demonstrated in the muscle. The presence of several systemic abnormalities (polyclonal gammapathy, circulating immune complexes, Sjögren's syndrome) and the vasculitis suggest an immunopathological mechanism for this HTLV-I associated myositis.
Antigen-Antibody Complex↗
[A simple system for semi-automatic image analysis].
A device using a Macintosh microcomputer and a light microscope equipped with a drawing tube is described. The mirror of the drawing tube is turned to the screen of the microcomputer. An example of its use in morphometry of peripheral nerves is given. At a 1000X magnification, the resolution of the system is 10 pixels per 2.6-microns. The coefficient of variation between 20 measurements of diameter of thin (3 microns in diameter) and large (12 microns in diameter) myelinated fibers was 8% and 2% respectively. This precise and inexpensive system could be easily run in laboratories of morphology.
Humans↗
[Peripheral neuropathies with neural vasculitis. Clinical and electrophysiological aspects of 33 cases].
Lesions of vasculitis of nerve were found in 33 of 140 patients hospitalized for peripheral neuropathy over a little less than 2 years, and in 113 specimens from neuromuscular biopsies. A first group of 7 patients had histological lesions suggestive of systemic necrotizing vasculitis (periarteritis nodosa in 4 cases and Churg and Strauss disease in 2 cases); the 7th patient was treated for myeloproliferative syndrome. The clinical and electromyographic signs were those of an axonal polyneuropathy which was improved by various treatments in 6 cases. A second group of 26 patients had histological lesions of vasculitis of nerve. Ten of these patients presented with single or multiple neuritis (of unknown origin in 5 cases), 11 with distal polyneuropathy (of unknown origin in 5 cases) and 3 with chronic polyradiculoneuritis; in 2 cases, clinical and electromyographic findings were in favour of an anterior horn lesion. The duration of the disease varied from 6 months to 10 years. The principal causes identified were connective tissue diseases, solid cancer or lymphoma and isolated gammapathy. Two patients with multiple neuritis recovered spontaneously. The finding of vasculitis of nerve at biopsy therefore is of unequivocal significance outside the better known but smaller group of systemic necrotizing vasculitis.
Adult↗
[Study of antiglycolipid antibodies in IgM monoclonal dysglobulinemias associated with peripheral neuropathy].
An immunological mechanism may be responsible for the peripheral neuropathies related to IgM monoclonal gammopathies. Myelin-associated glycoprotein (MAG) has been found to be one the main target antigens for these paraproteinemias. Glycosphingolipids of peripheral nerve have been also found to be targets for these antibodies, especially a sulfated glycosphingolipid containing glucuronic acid (GLSG) specific for peripheral nerve. Crossed reactivity has been found between MAG and GLSG. Anti-glycolipid antibodies were determined in 14 patients with IgM gammopathy and polyneuropathy. Glycosphingolipids from human peripheral nerve were purified and chromatographed by thin-layer chromatography for immunodetection of the antibodies. The 12 patients who had GLSG antibody activity had a clinical status identical to the reported cases of dysglobulinemic neuropathies with anti-MAG antibodies.
Adult↗
[The peripheral nervous system and HIV infection. 13 cases].
Disorders of the peripheral nervous system occur at all stages of HIV infection. From 13 patients referred for a peripheral neuropathy, 9 were known to be HIV seropositive and 4 were found to be seropositive. All were Caucasian males aged 27 to 58. Ten were homosexual, 2 were drug-addicts. Patients fell into several groups: the first group was made of 5 patients, 4 asymptomatic and 1 AIDS-related-complex (ARC), with an inflammatory demyelinating polyneuropathy, acute in 1 case and subacute in 4; the CSF showed pleiocytosis in all cases. Motor conduction nerve velocities were markedly reduced and motor distal latencies prolonged. Three patients recovered spontaneously, 1 responded to corticosteroids, 1 to plasmapheresis. In the second group, 4 patients, 1 asymptomatic and 3 ARC, had a distal symmetrical polyneuropathy; CSF was normal in 2 cases. Electrophysiological studies and nerve biopsies indicated a mixed axonal-demyelinating polyneuropathy. Three patients recovered spontaneously, 1 is unchanged. Among both groups, an infiltration of mononuclear cells was found on nerve biopsies in 4 cases. The third group was made of 3 patients with AIDS who presented with a painful sensory polyneuropathy involving the distal lower limbs. Electrophysiological and pathological study of the nerve indicated axonal degeneration. No cell infiltration was found. The last patient with AIDS had a progressive meningoradiculopathy. These 4 patients died within 6 months after the onset of the neuropathy. These findings are close to those previously reported, and confirm the wide spectrum of disorders of the peripheral nervous system associated to HIV infection.
Acquired Immunodeficiency Syndrome↗