1/63. Inflammatory demyelinating polyradiculoneuropathy associated with interstitial lung disease.A 58-year-old woman presented with inflammatory demyelinating polyradiculoneuropathy accompanied by sensory and motor disturbance and interstitial lung disease. Corticosteroid therapy led to a marked amelioration of both the neuropathy and the lung disease. We suggest that a demyelinating neuropathy is associated with an interstitial lung disease.- - - - - - - - - - ranking = 1keywords = inflammatory demyelinating, polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
2/63. Chronic relapsing multifocal sensory-motor neuropathy with conduction block.A 47 years old man had 13 episodes of relapsing and remitting sensory-motor neuropathy involving the upper limbs over the last 20 years. All but the last episode resolved spontaneously within 2 months. neurophysiology revealed multifocal motor and sensory conduction block in the upper limbs with normal terminal latencies. CSF analysis was normal and anti-GM1 antibodies were not detected. There was a dramatic clinical improvement after intravenous immunoglobulin treatment. This case represents an unusual multifocal variant of chronic inflammatory demyelinating neuropathy.- - - - - - - - - - ranking = 0.57092533122086keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/63. Recurrent myelinoclastic diffuse sclerosis: a case report of a child with Schilder's variant of multiple sclerosis.Myelinoclastic diffuse sclerosis (MDS, Schilder's disease) is a rare CNS demyelinating disorder affecting mainly children and usually presenting as an intracranial mass lesion. We report the first case of recurrent intracranial MDS where the third episode of demyelination involved the cervical spinal cord. This may represent a subset of the disease, which should be considered as Schilder's variant (childhood form) of multiple sclerosis.- - - - - - - - - - ranking = 0.015531962493937keywords = demyelinating (Clic here for more details about this article) |
4/63. Demyelinating disorder of the central nervous system occurring in black South Africans.OBJECTIVES: To investigate the nature and cause in eight black South African patients of a recurrent (multiphasic), remitting, and relapsing demyelinating disease of the CNS. methods: The clinical and laboratory investigations and radiological manifestations of these patients were documented. RESULTS: Each patient had two or more acute attacks of demyelinating disease affecting the CNS. The clinical presentations of the patients were predominantly those of multiphasic neuromyelitis optica (NMO). brain MRI in these patients showed features consistent with those described for acute disseminated encephalomyelitis (ADEM), as well as lesions that are described in multiple sclerosis. There was involvement of the corpus callosum in addition to typical ADEM lesions. Laboratory investigations excluded all other known causes of multiphasic CNS demyelination. Oligoclonal antibodies were not detected in these patients at any time. The patients were all from a population with a low risk for MS (black South Africans). CONCLUSION: The patients described here represent a new phenotypic expression of a recurrent (multiphasic), steroid sensitive, inflammatory demyelinating disorder of the CNS occurring in black South Africans. The disorder is either a distinct inflammatory demyelinating disorder of the CNS of as yet unknown aetiology, or a varied form of MS (ADEM/NMO) occurring in a population with a low risk (where the genetic trait and environmental risk factors for MS do not exist) for MS.- - - - - - - - - - ranking = 0.46560760358872keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/63. Hereditary neuropathy with liability to pressure palsies: a case report.Hereditary neuropathy with liability to pressure palsy is a rare autosomal dominant disorder characterized by multiple episodes of focal demyelinating neuropathies after minor trauma to peripheral nerves. It usually appears in early adulthood with recurrent attacks of pain, numbness, and muscular weakness along the distribution of the clinically affected nerve. Segmental demyelination and thickenings of the myelin sheath are the pathologic findings. Electrophysiologic studies show a nonuniform mild demyelinating neuropathy with prolonged distal latencies. Genetic tests are available to aid in diagnosis as molecular analysis has identified a deletion in the chromosome 17p11.2 in the majority of these patients. There is a paucity of information in the orthopaedic literature regarding hereditary neuropathy with liability to pressure palsy. A case report is presented of a patient with this disorder to promote awareness and recognition that this entity should be considered in patients with multiple nerve palsies.- - - - - - - - - - ranking = 0.031063924987873keywords = demyelinating (Clic here for more details about this article) |
6/63. recurrence of acute disseminated encephalomyelitis at the previously affected brain site.BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. OBJECTIVE: To examine the frequency, nature, and outcome of recurrent ADEM. DESIGN: review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. RESULTS: Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. CONCLUSIONS: Recurrent ADEM may be more prevalent than previously recognized. patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.- - - - - - - - - - ranking = 0.015531962493937keywords = demyelinating (Clic here for more details about this article) |
7/63. Detection of human herpesvirus 6 and jc virus in progressive multifocal leukoencephalopathy complicating follicular lymphoma.Progressive multifocal leukoencephalopathy (PML), a demyelinating infectious disease caused by jc virus (JCV), occurs almost exclusively in immunocompromised patients usually with malignant diseases. We report here a Japanese female with follicular lymphoma who subsequently developed PML. In addition to JCV, human herpesvirus 6 (HHV-6) was detected in the affected brain lesions of the patient by polymerase chain reaction and by in situ hybridization. HHV-6, recognized as a neurotropic virus, is known to be reactivated during immunosuppression and can cause fatal complications such as encephalitis/encephalopathy. It is likely that impaired immunity associated with lymphoma and the additional immunosuppression following cytopenia-inducing chemotherapies predisposed the patient to reactivated HHV-6 infection. Although it remains to be clarified whether HHV-6 plays an important role as a co-agent with JCV in causing demyelination of the brain, our observation alerts physicians to the possible association of HHV-6 with the pathogenesis of PML.- - - - - - - - - - ranking = 0.015531962493937keywords = demyelinating (Clic here for more details about this article) |
8/63. ophthalmoplegic migraine: a recurrent demyelinating neuropathy?The demonstration by magnetic resonance imaging (MRI) scanning of thickening and enhancement of the cisternal part of the oculomotor nerve in patients diagnosed as "ophthalmoplegic migraine" prompts reconsideration of this uncommon disorder. The case histories of five patients, three male and two female, varying in age from 6 to 30 years, are presented here. Recurrent painful ophthalmoplegia started in infancy in two cases, childhood in two instances and adult life in one. One child had his first attacks at 3, 5 and 12 months of age, on each occasion 10 days after an injection of triple vaccine. The possibility of this condition being a recurrent demyelinating neuropathy is considered and its possible relationship to migraine explored.- - - - - - - - - - ranking = 0.077659812469683keywords = demyelinating (Clic here for more details about this article) |
9/63. Pure motor chronic inflammatory demyelinating polyneuropathy.We describe four patients affected by chronic inflammatory demyelinating polyneuropathy (CIDP) in a pure motor form. Selective involvement of motor fibers was suggested by the absence of sensory symptoms, normal sensation at neurological examination and normal findings on electrophysiological testing of sensory fibres and sural nerve biopsy. The onset of the disease occurred at a young age (3-29 years) and the clinical course was relapsing-remitting. Over a follow-up period of 1.5-14 years, periodical clinical and electrophysiological examinations showed that selective involvement of motor fibers remained a constant feature. electromyography and nerve conduction studies continued to show a purely demyelinating neuropathy without signs of axonal impairment. All patients were steroid-unresponsive, whereas they considerably improved after being treated with immunoglobulins. Two patients were treated with interferon alpha and showed a good response. In conclusion, the occurrence in our four patients of pure motor involvement over a long period of time during which several relapses occurred, suggests that pure motor CIDP may represent the result of a specific immunological process rather than of a random distribution of inflammation throughout peripheral nerves.- - - - - - - - - - ranking = 2.8701586185983keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/63. Progressive multifocal leukoencephalopathy in a child with hyperimmunoglobulin E recurrent infection syndrome and review of the literature.Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease due to infection with polyomavirus JC (JCV). PML occurs almost exclusively in immunocompromised patients, and although it has increased markedly in relation to AIDS, remains exceptional in children. We present the case of an immunocompromised child with hyperimmunoglobulin E recurrent infection syndrome (HIES) and pathologically-proven PML. HIES is a rare congenital immunodeficiency that to our knowledge has never before been reported in association with neurological complications. Following a recurrence of bronchopneumonia, the child's motor and cognitive functions deteriorated progressively in parallel with alterations on cerebral MRI. The neurological onset coincided with lymphocyte subset changes. PCR for JCV dna did not detect the virus in CSF, and brain biopsy was required to secure the diagnosis. Antiviral treatment with cidofovir produced no benefit. autopsy revealed the typical neuropathological findings of PML which were associated with inflammatory eosinophilic infiltrate (a marker of HIES). In accordance with the few pediatric PML cases reported and here reviewed, the child died five months after neurological onset.- - - - - - - - - - ranking = 0.015531962493937keywords = demyelinating (Clic here for more details about this article) |
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