Polyneuropathy associated with IgM monoclonal gammopathy and anti-myelin associated glycoprotein (MAG) antibodies is an immune-mediated demyelinating neuropathy. The pathophysiology of this condition is likely to involve anti-MAG antibody deposition on myelin sheaths of the peripheral nerves and it is supposed to be distinct from chronic inflammatory demyelinating neuropathy (CIDP), another immune-mediated demyelinating peripheral neuropathy. In this series, we have retrospectively reviewed clinical and laboratory findings from 60 patients with polyneuropathy, IgM gammopathy, and anti-MAG antibodies. We found that the clinical picture in these patients is highly variable suggesting a direct link between the monoclonal gammopathy and the neuropathy. Conversely, one-third of patients had a CIDP-like phenotype on electrodiagnostic testing and this was correlated with a low titer of anti-MAG antibodies and the absence of widening of myelin lamellae. Our data suggest that polyneuropathy associated with anti-MAG antibodies is less homogeneous than previously said and that the pathophysiology of the condition is likely to be heterogeneous as well with the self-antigen being MAG in most of the patients but possibly being another component of myelin in the others.
Objective: The objective of this study is to report a case of spinal cord compression, which is a rare complication of neurofibromatosis type 1. Observation: We report the case of a 45-year-old man, which presented a syndrome of thoracic spinal cord compression at the stage of spastic paraparesis. Its installation was gradually over 6 months associated with the inaugural back pain. He had a clinical history of neurofibromatosis type 1 with "Café-au-lait" spots. There were multiple painless nodules under the skin of different size on the chest, forearms and legs. A large isolated nodule, purplish was observed on the chest. The neuro-imaging showed a para-spinal anterior mass expansion inside the spinal canal causing spinal compression at the level of the second and third thoracic vertebra. It extends into the intervertebral foramen of the third and fourth thoracic vertebra leading to a scalloping. A second large heterogeneous left intra-abdominal mass containing cyst areas and calcifications was discovered in imaging. After a spinal decompression with laminectomy of the second and third thoracic vertebra, the reduction of pain and motor recovery was gradual. The large nodule excision was performed. Histology found a plexiform neurofibroma. Excision of the left intra-abdominal mass could not be performed because the patient's consent had not been obtained. Conclusion: The spinal cord compression is a rare complication of neurofibromatosis type 1. However, it is essential to think about it in front of any spinal cord symptoms or any atypical long term spinal pain.
Objective: The aim of this study is to report a case of arachnoid cyst due to mass effect on the thoracic marrow. Observation: A 15-year-old patient was admitted to our institution and complaining of high back pain. Three months before he developed progressive and occasional back pain with thoracic irradiation in hemi-belt, increased by the dorsal decubitus. Neurological examination revealed a spasmodic paraplegia. The muscular strength was quoted as 2 to the left and 3 to the right on a scale of 5. Magnetic resonance imaging (MRI) revealed and extradural cyst located to the dorsal spinal cord. The ablation of a voluminous translucent cyst was achieved after a vast decompressive laminectomy from the sixth to the eighth thoracic vertebra. The dural communication with the cyst was stitched. After surgery, the pain has disappeared and the neurological recovery was progressive over a period of 21 days. The diagnosis of arachnoid cyst was confirmed by histological examination. Conclusion: The extradural thoracic arachnoid cyst is a rare affection of good forecast. In the symptomatic form, the surgery as soon as possible remains the solution. The MRI keeps all its interest for the diagnostic orientation and the therapeutic strategy.
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