1/6. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/6. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/6. 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.- - - - - - - - - - ranking = 1.25keywords = nervous system (Clic here for more details about this article) |
4/6. 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.- - - - - - - - - - ranking = 1.75keywords = nervous system (Clic here for more details about this article) |
5/6. 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.- - - - - - - - - - ranking = 0.25keywords = nervous system (Clic here for more details about this article) |
6/6. 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.- - - - - - - - - - ranking = 0.25keywords = nervous system (Clic here for more details about this article) |