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1/10. Demyelinating sensorimotor neuropathy with congenital cataract, mental retardation, and unique, dysplastic perineurial cells within the endoneurium.

    We report on a 27-year-old Caucasian female with congenital cataract and mental retardation complaining of progressive paresis and atrophy of the lower legs beginning at the age of 16 years followed by atrophy of the thighs and small hand muscles. Motor and sensory conduction velocities (CV) of the upper and lower limbs were reduced (distal peroneal nerve: 21 m/s; median nerve: motor CV: 28 m/s, sensory CV 30 m/s). In the sural nerve biopsy specimens there were unique endoneurial cells immunoreactive for antibodies against the epithelial membrane antigen with multiple surface indentations and projections considered to be dysplastic perineurial cells. To the best of our knowledge these cells have not been reported in any other type of human peripheral neuropathy. The present case with the above clinical and structural findings appears to represent a new, complex, demyelinating type of a sporadic or possibly recessively inherited motor and sensory neuropathy. ( info)

2/10. central nervous system disease in patients with macrophagic myofasciitis.

    Macrophagic myofasciitis (MMF), a condition newly recognized in france, is manifested by diffuse myalgias and characterized by highly specific myopathological alterations which have recently been shown to represent an unusually persistent local reaction to intramuscular injections of aluminium-containing vaccines. Among 92 MMF patients recognized so far, eight of them, which included the seven patients reported here, had a symptomatic demyelinating CNS disorder. CNS manifestations included hemisensory or sensorimotor symptoms (four out of seven), bilateral pyramidal signs (six out of seven), cerebellar signs (four out of seven), visual loss (two out of seven), cognitive and behavioural disorders (one out of seven) and bladder dysfunction (one out of seven). brain T(2)-weighted MRI showed single (two out of seven) or multiple (four out of seven) supratentorial white matter hyperintense signals and corpus callosum atrophy (one out of seven). evoked potentials were abnormal in four out of six patients and CSF in four out of seven. According to Poser's criteria for multiple sclerosis, the diagnosis was clinically definite (five out of seven) or clinically probable multiple sclerosis (two out of seven). Six out of seven patients had diffuse myalgias. deltoid muscle biopsy showed stereotypical accumulations of PAS (periodic acid-Schiff)-positive macrophages, sparse CD8 T cells and minimal myofibre damage. Aluminium-containing vaccines had been administered 3-78 months (median = 33 months) before muscle biopsy (hepatitis b virus: four out of seven, tetanus toxoid: one out of seven, both hepatitis b virus and tetanus toxoid: two out of seven). The association between MMF and multiple sclerosis-like disorders may give new insights into the controversial issues surrounding vaccinations and demyelinating CNS disorders. deltoid muscle biopsy searching for myopathological alterations of MMF should be performed in multiple sclerosis patients with diffuse myalgias. ( info)

3/10. central nervous system sjogren's syndrome in a child: case report and review of the literature.

    We describe a case of pediatric sjogren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with melkersson-rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary sjogren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering sjogren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke. ( info)

4/10. Osmotic demyelination syndrome after correction of chronic hyponatremia with normal saline.

    Rapid correction of severe chronic hyponatremia with hypertonic saline has been known to cause osmotic demyelination syndrome (ODS). Less recognized are the dangers of rapid correction with normal saline. A 60-year-old woman on thiazide diuretics for hypertension presented with profound hyponatremia (94 mmol/L) and hypokalemia (1.9 mmol/L) associated with volume depletion. Normal saline (2 L/day) and (KCl 40 mmol/day) were given for 5 days. serum Na concentration rose to 106 mmol/L within 18 hours. With improvement of her hyponatremia, she became more alert although the hypokalemia persisted. However, she developed progressive obtundation, quadriplegia, and respiratory failure 6 days later. magnetic resonance imaging of the brain clearly showed typical features of pontine and extrapontine myelinolysis. We suggest that the aggressive KCl supplement would have been the first-line therapy for this patient presenting with chronic hyponatremia and hypokalemia associated with volume depletion. ( info)

5/10. Evidence for the role of demyelination, HLA-DR alleles, and cytokines in the pathogenesis of parvovirus B19 meningoencephalitis and its sequelae.

    OBJECTIVE: To review the clinical and pathological features of parvovirus B19 meningoencephalitis and its sequelae in 12 previously published cases, and to perform additional tests to determine the pathogenesis of the disease. methods: Cases were reviewed and available serum and cerebrospinal fluid (CSF) tested for antiganglioside antibodies and a range of cytokines. In situ hybridisation for parvovirus B19 dna was performed on postmortem brain tissue in two cases. HLA-DRB1 typing was undertaken on genomic dna extracted from peripheral blood leucocytes. RESULTS: Cerebellar involvement was suggested either clinically or pathologically in four cases. In the two cases with postmortem histology, there was marked atrophy of the molecular and granular layers of the cerebellum with focal loss of purkinje cells. brain scanning by MRI or CT was done in six cases during the acute phase. Three were abnormal with evidence of demyelination. Three had markedly enlarged ventricles, in two of which there was high signal intensity from the white matter on both T1 and T2 weighted images. The three cases with abnormal brain scans had long term neurological sequelae (mental retardation, personality change, altered affect). In situ hybridisation on available postmortem brain tissue was negative in the two cases tested. All cases in which HLA-DR alleles were determined carried at least one of the following alleles: HLA-DRB1*01, *04, *07, *09, *15, *16. Available serum and CSF was tested for antiganglioside antibodies (all negative) and for a panel of cytokines, which had a similar profile in both serum (n = 5) and CSF (n = 1) during the acute phase. cytokines that were consistently detectable were IL-6 (mean 726.20 pg/ml), TNFalpha (50.64 pg/ml), IFNgamma (39.64 pg/ml), GM-CSF (216.12 pg/ml), and MCP-1 (154.43 pg/ml); IL-1beta, IL-5, and IL-13 were undetectable. CONCLUSIONS: HLA-DR associations, an increased cytokine response, and benefit from immunomodulatory treatment (in one case) support a role for the immune response in the pathogenesis of parvovirus B19 meningoencephalitis. ( info)

6/10. Inflammatory demyelinating disease mimicking malignant glioma.

    The differential diagnosis between inflammatory demyelinating disease and malignant glioma is difficult based only on neuroimaging methods. methods: Four patients with inflammatory demyelinating disease who presented with clinical and neuroimaging findings strongly suggestive of malignant glioma were examined. RESULTS: MRI showed a mass lesion with prolonged T1 and T2 values and gadolinium enhancement in all cases. Proton MR spectroscopy and (201)Tl SPECT showed findings supportive of the diagnosis of malignant glioma in all cases. However, surgical biopsy revealed inflammatory demyelinating disease. After the diagnosis, 2 patients were treated by steroid administration and 2 were just observed. The gadolinium enhancement of all lesions decreased and finally disappeared. CONCLUSION: Such cases illustrate the importance of considering a demyelinating lesion in the differential diagnosis of a mass lesion. The difficulties encountered in establishing the correct diagnosis of inflammatory disease are related to the variations in the radiologic appearance, which require exclusion of gliomas or other brain tumors by surgical biopsy before the therapeutic strategy can be selected. ( info)

7/10. central nervous system involvement in autoimmune polyglandular syndrome.

    We present a 33 year-old man, admitted because of transient deterioration of visual acuity. magnetic resonance imaging showed diffuse central nervous system (CNS) demyelination, which largely resolved spontaneously within 4 months. The patient fulfilled the diagnostic criteria of APS type III, having autoimmune thyroiditis and alopecia universalis. In this patient, autoimmune activation against CNS antigens is thought to be the cause of reversible CNS demyelination. ( info)

8/10. plasma exchange in episodes of severe inflammatory demyelination of the central nervous system. A report of six cases.

    The standard therapy for episodes of severe acute inflammatory demyelinating disease of the central nervous system is high dose intravenous corticosteroids. A small proportion of patients fail to improve with this regime and their prognosis can become grave. A recent sham controlled double blind crossover trial in this group of patients demonstrated a significant benefit from plasma exchange. We report six patients with severe acute steroid-insensitive inflammatory demyelinating disease of the central nervous system treated with plasma exchange. We observed a clear improvement in five of these six patients. Whilst complications of plasma exchange occurred these did not outweigh the benefits. Our study supports the use of plasma exchange in severe acute steroid-insensitive inflammatory disease of the central nervous system. ( info)

9/10. Neuropathology of paraneoplastic neuropathy with anti-disialosyl antibody.

    We report a paraneoplastic neuropathy with severe motor involvement following sensory-ataxic disturbance. Anti-disialosyl immunoglobulin m (IgM) antibody was detected in the course of malignant lymphoma of diffuse large B-cell type, which usually spares the motor system. Onset was subacute, with relapsing and remitting sensory ataxia, muscle weakness, bulbar palsy, respiratory paralysis, and ophthalmoplegia; only neck rotation was retained in the terminal stage. autopsy showed no lymphoma cells infiltrating the nervous system. motor neurons survived in the spinal cord, but mean diameter of the ventral spinal nerve roots was reduced considerably. The gracile fasciculus and the sural nerve were more markedly degenerated than proximal portions. Morphometric study showed that most of the proximal motor and sensory axons did not extend distally. This autopsy report provides further definition of a neuropathy associated with malignant lymphoma and IgM antibodies against disialosyl residues. ( info)

10/10. CNS demyelination in autoimmune diseases.

    autoimmune diseases represent a diverse group of disorders that have generally of unknown etiology and poorly understood pathogenesis. They may be organ-specific or systemic, giving rise to overlapping syndromes; more than one autoimmune disease may occur in the same patient. Numerous case reports have documented that multiple sclerosis (MS) may be present concurrently with other autoimmune diseases, most commonly rheumatoid arthritis, autoimmune thyroid disease, type I diabetes mellitus and pernicious anemia. case reports of disseminated encephalomyelitis (DEM) coincidental with other autoimmune diseases are rare. Many of systemic autoimmune diseases cause central nervous system (CNS) demyelination and are frequently then diagnosed as MS, whereas they often are instances of DEM, the result of vascular, granulomatous or postinfectious manifestations. We have reviewed 15 patients with autoimmune diseases and CNS demyelination in order to determine the nature of the demyelinating process. ( info)
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