Cases reported "Demyelinating Diseases"

Filter by keywords:



Filtering documents. Please wait...

1/6. New therapeutic perspectives for demyelinating retrobulbar optic neuritis.

    In patients with demyelinating retrobulbar optic neuritis (RON), a spontaneous or corticosteroid-induced improvement is generally observed within the first month, but this is clinically insignificant in 5%-7% of patients. We report the case histories of four patients who were considered to be "non-responders" to corticosteroids because their visus remained unchanged or had improved by only 1/10 after one month from intravenous corticoid therapy begun 2-7 days after disease onset, and who were therefore subsequently administered high intravenous doses of immunoglobulin. Three of these patients completely recovered in a period of 3-9 months; the fourth showed only a partial improvement, but this was consolidated after long-term continuation of the same therapy. These cases suggest the possible efficacy of early administration of intravenous immunoglobulin in RON patients who fail to respond to cortisone therapy. As recently demonstrated in animal models, it can be hypothesised that the result is due to immuno-mediated mechanisms of action that reduce autoimmune responses in the short- and medium-term, and in the long-term favour remyelination.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)

2/6. Cerebellar hypoplasia in Werdnig-Hoffmann disease.

    The case of an infant with Werding-Hoffmann disease, who died at the age of 4 1/2 days, is reported. At autopsy there was severe cerebellar hypoplasia, associated with degenerative changes in the brain-stem nuclei. This case supports the concept that cerebellar hypoplasia may develop as a manifestation of the neuronal abiotrophy of Werdnig-Hoffmann disease. A similarity has been noted between the cerebellar lesion found in the child reported here and that produced by viral infection in experimental animals.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)

3/6. Different molecular mechanisms leading to white matter hypomyelination in infantile onset lysosomal disorders.

    Hypomyelinating leukoencephalopathies may be related to a primary disturbance in the formation of myelin or may be caused by neuronal, oligodendrocytic or astrocytic dysfunction, leading to a failure of myelination. Abnormal myelination related to a direct metabolic damage on oligodendrocytes has been shown to occur in some animal models of lysosomal storage diseases. To demonstrate that cerebral white matter hypomyelination may occur also in humans affected by early-onset lysosomal storage diseases, we report three cases with infantile-onset lysosomal storage disorders (type 1 GM1 gangliosidosis, globoid cell leukodystrophy or Krabbe's disease, and type A Niemann-Pick disease) showing white matter hypomyelination. Hypomyelinating leukoencephalopathy may therefore represent a feature of lysosomal storage disorders with onset in the first months of life, when the process of myelination is particularly active, indicating that neuronal storage disorders may be primarily responsible for central nervous system hypomyelination.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)

4/6. Remyelination in the human central nervous system.

    Remyelination, albeit incomplete, has been demonstrated in human central nervous system (CNS). However, information about the initial stage and the final extent of such remyelination is not available. We describe the morphologic findings of a demyelinating lesion with evidence of early remyelination in a biopsy obtained from a 15-year-old boy about two weeks after the onset of neurologic symptoms. The demyelinated area appeared hypercellular with a relatively large number of oligodendrocytes frequently seen in the process of new myelin formation. In addition to the usual reactive changes, the astrocytes were often seen to contain otherwise normal-looking oligodendrocytes within their cytoplasm. In the ensuing months, the patient made apparently total functional recovery accompanied by nearly complete resolution of the white matter lesions demonstrated by the subsequent magnetic resonance studies. These observations suggested that the initial remyelination seen in the biopsy eventually succeeded in producing extensive remyelination in the lesion. Although the exact nature of the demyelinating disorder in our patient remains undetermined, this study indicates that clinically significant remyelination is possible in human CNS. Also, our findings appeared strikingly similar to those described in certain experimental animal models in which widespread remyelination is known to occur.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)

5/6. Combined central and peripheral acute demyelination.

    We describe a patient with multiple sclerosis who had a bout of central demyelination associated with an acute inflammatory demyelinating polyneuropathy. The contemporary involvement of central and peripheral nervous system due to a demyelinating disease has been reported anecdotically in humans, and can be induced experimentally in animals. It may be sustained by a common pathogenetic factor.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)

6/6. Histologic effect of doxycycline sclerotherapy on rat femoral nerve.

    BACKGROUND: This study stems from an encounter with a phrenic nerve paralysis in a patient following doxycycline sclerotherapy for treatment of chylous fistula. The purpose of this study is to identify possible histologic evidence of doxycycline-induced nerve injury. methods: The femoral nerves of CD rats were used as the in vivo animal model. The nerves were exposed to varying concentrations doxycycline and normal saline was the control. The nerves were studied at several time intervals using two different staining techniques. RESULTS: The results suggest that topical doxycycline induces tissue reactions which are different from normal saline. These reactions include stimulation of a local giant cell inflammatory reaction and disruption of the myelin sheath. CONCLUSIONS: Despite the fact that this study does not give physiologic evidence of neurotoxicity, the histologic results suggest that topical doxycycline may cause nerve damage directly or indirectly. We conclude that doxycycline should not be used for sclerotherapy where unprotected nerves are exposed to the agent until further physiologic tests are performed to prove its safety.
- - - - - - - - - -
ranking = 1
keywords = animal
(Clic here for more details about this article)


Leave a message about 'Demyelinating Diseases'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.