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Biomedical subjects

J Servan

Publications and source records attributed to J Servan.

At least 19 recordsLinked to original sources

[Peripheral neuropathy and hepatitis C virus infection: more than cryoglobulinemia].

PURPOSE: To study characteristics of peripheral neuropathies associated with hepatitis C virus infection. METHODS: A study of two groups of four patients with peripheral neuropathy and infected with hepatitis C virus, and a literature review. RESULTS: We observed four cases of peripheral neuropathy in patients with hepatitis C virus infection with persistent negativity of mixed cryoglobulinemia, and with no purpura, rheumatoid factor, or low C4 level. Neuropathy improved with treatment in three patients between 8 and 28 months after treatment was begun. We report four other observations of peripheral neuropathy with mixed cryoglobulinemia. Worsening of peripheral neuropathy was observed 2 to 13 weeks after initiation of interferon alpha despite improvement of hepatic parameters. After stopping interferon, peripheral neuropathy worsened (one case), stabilized (one case), or improved (two cases). DISCUSSION: In patients infected with hepatitis C virus, peripheral neuropathy can be due to mixed cryoglobulinemia, periarteritis nodosa, and perhaps hepatitis C virus itself as suggested by our first observations. Low doses of interferon alpha may worsen peripheral neuropathy in patients with mixed cryoglobulinemia, as suggested in our later observations.

Adult↗

Hepatitis C virus infection with peripheral neuropathy is not always associated with cryoglobulinaemia.

OBJECTIVES: To describe cases of peripheral neuropathy associated with chronic hepatitis C virus infection without mixed cryoglobulinaemia. METHODS: Four cases of peripheral neuropathy associated with chronic hepatitis C virus infection with persistent negativity of mixed cryoglobulinaemia were found. RESULTS: All patients had small increases of transaminase levels and a positive viraemia. Liver biopsy showed chronic active hepatitis in all but one case (Knodell 4-9, Metavir A0F0-A3F3). Neuromuscular biopsy showed axonal neuropathy associated with lymphoid infiltrates around small vessels in two cases. Rheumatoid factor was always negative and C4 complement level was always normal. In three patients, neuropathy improved with interferon alpha, interferon alpha + ursodesoxycholic acid, or steroids + plasma exchange. CONCLUSION: Peripheral neuropathy may be associated with hepatitis C virus infection without mixed cryoglobulinaemia.

Adult↗

Systemic vasculitis in patients with hepatitis C.

OBJECTIVE: To analyze the main characteristics of patients infected with hepatitis C virus (HCV) presenting with different types of vasculitis syndrome. METHODS: We retrospectively compared 2 groups of patients with HCV presenting with systemic vasculitis: 10 with biopsy proven polyarteritis nodosa-type systemic vasculitis (PAN, Group 1) and 7 with mixed cryoglobulinemia syndrome (MC, Group 2). RESULTS: Patients of Group 1 presented with different features than Group 2: life threatening systemic vasculitis (10 vs 0; p < 0.01), severe multifocal sensorimotor mononeuropathies versus distal moderate sensory polyneuropathies, malignant hypertension (5 vs 0; p = 0.04), cerebral angiitis (2 vs 0), ischemic abdominal pain (2 vs 0), kidney and liver microaneurisms (2 vs 0), increased erythrocyte sedimentation rate and C-reactive protein (7 vs 0; p < 0.01), renal insufficiency (5 vs 0; p = 0.04), HCV genotype 1b (3 vs 6; p = 0.06), and lower activity of chronic hepatitis (p = 0.02). Neuromuscular biopsies showed lesions of vasculitis in all patients, but the type of vasculitis was different in Group 1 compared to Group 2: medium size artery involvement (7 vs 0; p < 0.01), necrotizing vasculitis (10 vs 0; p < 0.01), and mononuclear cell infiltrate in perivascular areas (0 vs 7; p < 0.01). Using prednisone, plasma exchanges, and interferon-alpha, complete recovery was obtained in all PAN-type patients except one. In Group 2 patients, interferon-alpha did not have any effect on the peripheral neuropathy. CONCLUSION: HCV infection may be associated with different types of systemic vasculitis, i.e., polyarteritis nodosa or mixed cryoglobulinemia. Because of differences in clinical and pathological features and therapeutic strategy, PAN-type vasculitis should be distinguished from MC-type vasculitis in HCV patients.

Adult↗

[Familial orthochromatic leukodystrophy: clinicopathological study of two cases].

This paper reports the clinico-pathological data in a French family with orthochromatic leukodystrophy. The parents were first cousins and had seven children. Among those, two sisters and one brother presented with neurological signs, with onset around the 5(th) decade, including a dementing syndrome of frontal type, a tetrapyramidal syndrome, seizures, and, in one sibling, a cerebellar syndrome. CT scan or MRI showed diffuse involvement of the white matter. The neurological signs worsened progressively leading to death within 11 and 22 months. Neuropathological examination was performed in two cases. It revealed characteristic orthochromatic leukodystrophy. In one case, the presence of pigmented macrophages and astrocytes was suggestive of Van Bogaert and Nyssen disease. However there were some atypical features including the absence of pigmented cells in the second case whose clinical course was shorter, and the cavitary appearance of the white matter changes with a relative increase in the number of oligodendrocytes raising the issue of a possible link between this condition and cavitary orthochromatic leukodystrophies.

Aged↗

Measuring HIV-1 RNA and interferon-alpha in the cerebrospinal fluid of AIDS patients: insights into the pathogenesis of AIDS Dementia Complex.

The aim of this work was to study the role of HIV replication and the role of endogenous secretion of interferon-alpha in the pathogenesis of AIDS Dementia Complex (ADC). To accurately establish the diagnosis of ADC, 39 consecutive HIV-positive patients who presented with immune and intellectual deficiency underwent an extensive neurological evaluation. This included magnetic resonance imaging, neuropsychological testing and a lumbar puncture. The levels of HIV-1 RNA were measured in the cerebrospinal fluid (CSF) and blood by HIV Monitor (Roche Diagnostics) and those of interferon-alpha by an in-house biological assay. The diagnosis of ADC was established in 22 cases, which included nine out of the ten patients who had a high CSF viral load (above 4 log HIV-1 RNA copy per ml). Patients receiving highly active antiretroviral therapy had low viral loads in blood and CSF. In all 22 ADC patients, viral load in the CSF correlated with the staging of ADC (r=0.46, P=0.03), the CSF level of interferon-alpha (r=0.42, P=0.05) and with the bicaudate ratio (r=0.43, P=0.06), a measure of cerebral atrophy in the region of the caudate nucleus. No correlation was observed between CSF and plasma HIV-1 RNA. These results show that HIV may play a role in the neurological impairment of ADC patients possibly in part through the deleterious effect of interferon-alpha on the central nervous system and that highly active combination therapy should reduce or prevent these complications.

AIDS Dementia Complex↗

[Cerebral hemosiderosis related to hereditary ceruloplasmin deficiency. Clinical familial case study].

A 59-year-old patient progressively developed dementia, hallucinations and facial dyskinesia. Brain T and T2-weighted MRI images showed low signal intensity on basal ganglia specially striatum, posterior thalamic and dentate nuclei. He had no evidence of ceruloplasmin and a high level of ferritin in the serum. Liver biopsy confirmed accumulation of iron in the cytoplasm of many hepatocytes. Similar clinical and biological signs were also observed in two brothers. All the three siblings were homozygous for a hereditary ceruloplasmin deficiency. This new clinico-pathological entity, first described in 1987, is different from Wilson's disease, Hallervorden-Spatz's disease and idiopathic hemochromatosis and linked to a mutation of the ceruloplasmin gene located on chromosome 3.

Brain↗

[A contribution of brain imaging techniques to the diagnosis of psychiatric emergencies].

This study was performed in the psychiatric emergency unit of René-Dubos General Hospital (Pontoise, France). Two cases of female patients with motor deficiencies suggesting a diagnosis of conversion disorder because of preceding psychological stressors and lability of symptoms, are presented. In both cases, the diagnosis was reconsidered after the use of RMI for multiple sclerosis in one case, HIV related encephalopathy in the other. Recent studies have shown that organic diseases with a psychiatric presentation represent 1,2 to 4% of patients examined in emergency setting. Criteria for the diagnosis of conversion disorders are discussed (they are classified as dissociative disorders in ICD-10 and as somatoform disorders in DMS-IV). A diagnosis of conversion disorder should not be made before a thorough clinical and neurological evaluation has been performed.

AIDS Dementia Complex↗

[Interhemispheric disconnection syndrome caused by partial infarction of the corpus callosum: neuropsychological study and MRI].

A partial syndrome of hemisphere disconnection was observed in a 63 year-old woman, following an anterior and middle corpus callosum infarct on MRI. Notably, we found left ideomotor apraxia, diagonistic apraxia, left-year extinction on dichotic listening, but no left-hand anomia nor left visual field anomia. A left tactile extinction in dichaptic condition is interpreted as resultant of a dysregulation of the attentional balance between the two hemispheres. This detailed neuropsychological study permits a correlation between the callosal syndrome and the lesion. We suggest that an occlusion of the anterior callosal artery could explain this limited ischemia.

Cerebral Infarction↗

[Meningomyelitis disclosing Behçet's disease].

The authors report a patient who developed a meningo-myelitis attributed to Behçet disease. Spinal MRI assessed spinal involvement. Among neurological manifestations of Behçet disease, isolated spinal cord involvement is infrequent. Dramatic improvement of neurological symptoms was obtained after steroid and immunosuppressive treatment.

Adolescent↗

Aphasia and infarction of the posterior cerebral artery territory.

Spoken language disorders are rarely mentioned in superficial infarction of the posterior cerebral (PCA) territory. Two clinical types have been reported: transcortical sensory and amnesic aphasia. Between 1979 and 1990, we studied retrospectively 76 patients suffering from an occipitotemporal infarction located in the superficial territory of the posterior cerebral artery, all well documented by CT. Aphasia was one of the first and prominent signs in 18 cases. Middle cerebral artery concomitant infarction could have been the cause of language impairment in 10. In 8 patients aphasia was only explained by a PCA territory infarct. Three patients showed features of transcortical sensory aphasia. CT localization showed internal lobe and thalamic involvement of the dominant hemisphere. Five patients exhibited word finding impairment with various degrees of amnestic syndrome. The dominant internal temporal lobe was always affected. Dominant thalamus involvement was found in one case only. Some correlations between clinical features and anatomical support (vascular supply and anatomical structure) might be suggested in our 8 cases of aphasic disorders due to PCA infarcts. They are discussed and compared with data in the literature.

Adult↗

[Narcolepsy disclosing neurosarcoidosis].

A 37-year-old man developed excessive daytime sleepiness, sleep attacks and cataplexy revealing an hypothalamic tumour. Multiple Sleep Latency Tests (MSLT) were characteristics of narcolepsy. Tissue typing was positive for HLA DR2 and DQ1. Most cases of narcolepsy are idiopathic without any evidence of brain pathology. Although symptomatic narcolepsy may occur occasionally with diencephalic lesions. The relationship between narcolepsy with diencephalic lesions is unsettled and will be discussed.

Adult↗

[Amnestic syndromes and confabulation in infarction of the posterior cerebral artery area].

The Posterior Cerebral Artery (PCA) supplies the greatest part of the limbic system. Several authors have reported amnestic syndromes in PCA infarcts. Amongst 76 patients with a CT proven PCA infarct, 21 suffered anterograde amnesia. Confabulations were associated in 5 cases. We studied CT scans according to previously reported templates in the 21 amnestic patients: all patients with confabulations had a paramedian or a tuberothalamic infarct associated with a cortical infarct within the superficial territory of the PCA. None of the 16 remainders had a thalamic lesion. These results emphasize the role of the thalamus especially of the dorsomedial nucleus in the pathogenesis of confabulation. The underlying mechanism could imply fronto-cingular deafferentation.

Adult↗

[Post-traumatic crossed cerebellar atrophy].

A case of crossed cerebellar atrophy after cranial trauma, with a 12-year neuroradiological follow-up is reported. This uncommon cerebellar degeneration has previously been reported only as an autopsic finding. It results from a lesion affecting the contralateral cerebral hemisphere. The role of different cerebro-cerebellar pathways is discussed.

Atrophy↗

Yersinia enterocolitica among small wild mammals in France.

A survey of Yersinia enterocolitica was made in two French areas (Indre and Alsace) in several components of the terrestrial ecosystem. Y. enterocolitica has been isolated from several habitats and from numerous species of small mammals. The trap line method enabled us to estimate the density of the two abundant small mammal species, Microtus arvalis and Apodemus sylvaticus, and the densities of Y. enterocolitica carriers. The frequency of isolation was high when temperatures were low, but did not seem to be in direct relationship with animal densities. Infectious lesions were never found in spleens or intestines of 1893 captured animals. Samples of fresh water, fresh-water fish, soil and earthworms were occasionally collected. A total of 163 strains were isolated. Of these, 99 were of biochemical type 1; 20 different 0 serogroups were found.

Animals↗