1/76. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
2/76. Acute sensory neuropathy in an adolescent girl following BCG vaccination.A 13-year-old girl developed a sensory neuropathy following bacille Calmette-Guerin (BCG) vaccination, consistent with acute inflammatory demyelinating polyradiculoneuropathy or acute sensory axonal neuropathy.- - - - - - - - - - ranking = 21.032312412781keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/76. Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin.Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids. We describe two patients with ADEM in whom treatment with IV methylprednisolone coincided with deterioration in their clinical status. They were subsequently treated with IV immunoglobulin and exhibited dramatic clinical improvement, with return to their previous level of functioning.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
4/76. filtration of cerebrospinal fluid for acute demyelinating neuropathy in systemic lupus erythematosus.We report a patient with systemic lupus erythematosus complicated by an acute demyelinating neuropathy. Conventional therapy with intravenous immunoglobulins and immunoadsorption complemented by pulse methylprednisolone and cyclophosphamide failed. Institution of filtration of the cerebrospinal fluid was followed by a rapid improvement of the paresis.- - - - - - - - - - ranking = 5keywords = demyelinating (Clic here for more details about this article) |
5/76. Acute axonal form of guillain-barre syndrome in a multiple sclerosis patient: chance association or linked disorders?multiple sclerosis (MS) is characterized by inflammation, demyelination and gliosis, involving the central nervous system (CNS) and commonly sparing the peripheral nervous system (PNS). Coexistence of CNS and PNS chronic demyelination has been rarely demonstrated in chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) and in MS, but the occurrence of acute polyradiculoneuropathy in a patient with MS is even more unusual. We describe the case of a woman with relapsing-remitting MS who presented with an acute severe tetraparesis. cerebrospinal fluid (CSF) examination together with neurophysiological data and sural nerve biopsy study demonstrated an axonal form of guillain-barre syndrome (GBS). It remains unresolved if the association of an axonal form of GBS and MS is fortuitous or, on the contrary, is indicative of the coexistence in some individuals of common pathogenetic mechanisms.- - - - - - - - - - ranking = 21.032312412781keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/76. Predominant arm weakness in acute idiopathic polyneuritis: a distinct regional variant.Acute idiopathic polyneuritis is a heterogeneous disorder with a number of clearly established variants. We describe four patients who present with an acute neuropathy predominantly affecting the arms which we believe should be considered as a distinct variant of acute polyneuritis. In two cases a primary demyelinating process affecting both motor and sensory nerves is suggested whereas in the other two the features were of a pure motor axonal degeneration. The relationship between these neuropathies, other variants of acute idiopathic polyneuritis and multifocal motor neuropathy is considered.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
7/76. guillain-barre syndrome: perspectives with infants and children.An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The guillain-barre syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in china. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
8/76. 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 = 5keywords = demyelinating (Clic here for more details about this article) |
9/76. Acute-onset painful upper limb multifocal demyelinating motor neuropathy.We report three patients who presented with acute onset of shoulder and upper arm pain followed within a few days by predominantly distal upper limb weakness. Nerve conduction studies showed severe and unequivocal focal motor conduction block in the forearm and/or upper arm along with slowing of motor conduction and prolonged F wave responses. Only very mild changes in sensory nerve conduction were found. One patient made partial clinical improvement after 17 months, and there was a significant improvement in the degree of motor conduction block and the motor conduction velocities. A second patient remained unchanged after 5 months. Idiopathic brachial neuritis (IBN) typically presents acutely with brachialgia and acute or subacute non-progressive weakness. Multifocal motor conduction block in nerves in the arm or forearm has not been described in patients with IBN. Multifocal motor conduction block restricted to the upper limbs has been described in focal chronic inflammatory demyelinating polyneuropathy (CIDP) and in multifocal motor neuropathy with multifocal motor conduction block (MMNCB). However, both these conditions have hitherto usually been described as largely painless chronic progressive disorders with a subacute onset. Our patients, with features overlapping MMNCB/CIDP and IBN, represent an as yet unreported clinical variant.- - - - - - - - - - ranking = 9.7080988033136keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/76. Rapid distinction of acute demyelinating disorders and central nervous system lymphoma by molecular analysis of cerebrospinal fluid cells.polymerase chain reaction (PCR) based automated high-resolution fragment analysis of rearranged immunoglobulin heavy-chain genes is a highly sensitive means for identifying clonal B-cell responses. We used this technique to distinguish polyclonal inflammatory from monoclonal neoplastic B-cell populations in the cerebrospinal fluid (CSF) of three patients with acute demyelinating disorders of the central nervous system whose clinical, magnetic resonance imaging (MRI) and CSF features did not permit unequivocal exclusion of primary central nervous system lymphoma (pC-NSL). This approach is highly suitable for detecting CNS inflammation particularly when lymphomatous involvement cannot be ruled out by noninvasive diagnostic procedures alone.- - - - - - - - - - ranking = 5keywords = demyelinating (Clic here for more details about this article) |
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