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Biomedical subjects

E Ellie

Publications and source records attributed to E Ellie.

At least 19 recordsLinked to original sources
PubMed2005

JC virus meningitis in a patient with systemic lupus erythematosus.

The human neurotropic JC virus (JCV) is most commonly acquired during childhood, and, because no clinical illness has been associated with primary infection, is presumed to be asymptomatic. In the immunocompromised host, JCV is responsible for progressive multifocal leukoencephalopathy (PML). We describe a patient with longstanding systemic lupus erythematosus who presented with acute meningitis without encephalitis or PML. JCV was the only pathogen found in the cerebrospinal fluid suggesting a primary infection or symptomatic reactivation. Our observation demonstrates the expanding clinical importance of JCV in autoimmune diseases, and diagnostic tests for JCV should be included in the investigative work-up for meningitis or encephalitis in these patients.

Adult↗
PubMed2001

Recurrent subarachnoid hemorrhage associated with a new transthyretin variant (Gly53Glu).

CNS involvement is rare in systemic amyloidoses due to transthyretin (TTR) mutation and manifests as a combination of dementia, seizures, and myelopathy. The authors report two French siblings who experienced recurrent subarachnoid hemorrhages as the main clinical feature. Brain specimens showed that the leptomeningeal vessels walls were thickened by amyloid deposits, and sequencing of the TTR exons showed a heterozygous single base-pair transition from G to A (codon 53), resulting in a glycine for glutamic acid substitution (G53E).

Adult↗
PubMed1999

[Acrodystrophic neuropathy of Bureau and Barrière].

INTRODUCTION: The acrodystrophic neuropathy described by Bureau and Barrière in the 1950s is a rare trophic complication of chronic, analgesic neuropathy due to alcohol abuse, which is at the origin of perforating ulcers of the foot, vasomotor disorders with dysautonomia, and leads to mutilating arthropathy of the lower limb. This neuroacropathy, also termed vagabonds' or vagrants' disease, usually occurs in subjects with a debilitated condition, chronic alcoholism, and unfavourable socioeconomic conditions. EXEGESIS: We report four cases of Bureau-Barrière disease which occurred in male subjects who were on average 55 years of age. The clinical presentation was close to that reported in the literature. Indeed, all four patients were alcoholic, nondiabetic and lived under conditions of precarious hygiene. Therapeutic management of the patients was difficult due to bad compliance with the treatment and persistence of alcohol abuse. Immobilization of the foot is considered to be the primary treatment. Local care including baths and bandages with hydrocolloids must be continued during several months, and associated with antibiotic therapy, administered by infusion when necessary. The outcome is often chronic, with poor prognosis. Given the limited therapeutic possibilities, acrodystrophic neuropathy is an invalidating disease with high morbidity. CONCLUSION: Bureau-Barrière disease is a rare, serious invalidating disease. The clinical symptomatology is usually based on the diagnostic triad: analgesia of the foot, perforating ulcers of the foot, and deforming and mutilating arthropathy of the lower limb. Treatment is often hampered due to poor compliance with local care and persistence of alcohol abuse.

Alcoholism↗
PubMed1999

[Group B streptococcal meningitis revealing an inner ear congenital malformation. A case report in an adult].

INTRODUCTION: Group B streptococcal meningitis is unusual in adults. It occurs in patients with a chronic disease. EXEGESIS: We report a case of group B streptococcal meningitis that occurred in a 32-year-old woman, uncovering inner ear congenital malformation associated with cerebrospinal fluid fistula. There was no recurrent episode of meningitis during a 2-year period following surgical correction of the malformation. CONCLUSION: Clinical signs suggesting cerebrospinal fluid fistula should be considered in any adult patient with severe group B streptococcal infection.

Adult↗
PubMed1999

Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus.

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.

Adrenal Cortex Hormones↗
PubMed1997

Three different anti-myelin antibodies in a case of demyelinating dysglobulinemic peripheral neuropathy.

We report the case of a patient with a severe, predominantly motor, demyelinating neuropathy associated with an IgM kappa biclonal gammopathy. Immunoblot studies showed IgM reactivity against MAG, and IgG reactivity against a peripheral nerve myelin-specific protein of approximately 35 kDa. Immunodetection by thin layer chromatography showed IgM reactivity towards GM1 and GD1b, as well as towards SGPG and SGLPG. This case illustrates the existence of overlapping syndromes among dysglobulinemic neuropathies, and points to an interaction of different autoantibodies in the pathogenesis of the nerve lesions.

Aged↗
PubMed1996

In vivo and in vitro expression of rat galactose-1-phosphate uridyltransferase (GALT) in the developing central and peripheral nervous system.

Galactose-1-phosphate uridyltransferase (GALT) is a key enzyme in the metabolism of galactose. GALT activates the galactose-glucose interconversion and enables the synthesis of glucose-1-phosphate and UDP-galactose (UDP-Gal). UDP-Gal is the galactosyl donor for the incorporation of galactose into complex oligosaccharides, glycoproteins and glycolipids. The expression of GALT was characterized both in vivo and in vitro during late embryonic and postnatal development of the brain and peripheral nerve of the rat. Assays of GALT mRNA and protein showed that it is weakly expressed during late embryonic development with a second peak of expression concomitant with myelinogenesis. GALT was prominently expressed in myelinating Schwann cells in a rat dorsal root ganglia culture system. GALT deficiency in humans results in galactosemia, a disease characterized by long-term intellectual impairment, and probably dysmyelination. The developmentally regulated pattern of GALT expression during maturation of the nervous system may provide a molecular basis for these neurological complications which seriously compromise the outcome of many galactosemic patients.

Animals↗
PubMed1996

Neuropathy associated with "benign" anti-myelin-associated glycoprotein IgM gammopathy: clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases.

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46-81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.

Aged↗
PubMed1995

Differential expression of inducible nitric oxide synthase mRNA in human brain tumours.

Messenger RNA encoding the inducible form of human nitric oxide synthase (iNOS) was quantified by reverse transcription and polymerase chain reaction (RT-PCR) in tissue samples from glioblastomas and meningiomas. iNOS mRNA expression was considerably higher in the glioblastoma than in the meningioma specimens (mean +/- s.e.m., 41.18 +/- 11.5, n = 25, vs 5.31 +/- 0.98, n = 21; p < 0.0001). Moreover, iNOS expression appeared as a polymorphic character among glioblastomas, as individual tumours expressed either high (n = 6), intermediate (n = 10) or low (n = 9) levels of iNOS mRNA.

Base Sequence↗
PubMed1995

High-grade B-cell cerebral lymphoma in a patient with anti-myelin-associated glycoprotein IgM paraproteinemic neuropathy.

A 74-year-old woman with a sensory neuropathy and IgM M-protein monoclonal gammopathy of undetermined significance developed a fatal B-cell cerebral lymphoma. CSF protein immunofixation revealed intrathecal secretion of a paraprotein of the same heavy- and light-chain isotypes as the serum monoclonal component (IgM-lambda). Reactivation of Epstein-Barr virus was present in the lymphoma cells. Different factors may be involved in the preferential malignant development of the monoclonal B-cell clone within the CNS.

Aged↗
PubMed1994

[Mental deterioration and hypertension: uncommon manifestation of corticotropin insufficiency and functional carboxymethyl oxidase block].

We report a new case of muscle contractures associated with adrenocortical deficiency. Outstanding features were the diffusion of the contractures, rhabdomyolysis and an encephalopathy which disappeared with hormonal therapy. Endocrinological investigations revealed a functional carboxymethyl oxidase type II defect which could, in part, explain our patient's neuromuscular symptoms.

Abdominal Muscles↗
PubMed1994

Severe axonal polyneuropathy with onset in the postpartum period.

We report two patients who presented severe polyneuropathy in the postpartum period. Electrophysiological studies evidenced an axonal process which was associated with proximal demyelination in the second patient. In both cases, a peripheral nerve biopsy showed severe axonal Wallerian-like degeneration and no feature of demyelination. The first patient had a dramatic loss of myelinated fibres, and severe disability persisted for several months. These two patients are different from cases of acute or chronic inflammatory demyelinating polyradiculoneuropathy previously reported in relation with pregnancy.

Adult↗
PubMed1994

[Multifocal motor neuropathy].

We report the case of a patient with multifocal motor neuropathy. Electrophysiological studies showed typical multifocal conduction blocks, but the search for anti-GM1 antibodies was negative. This case provide the heterogeneity of this clinical entity.

Adult↗