1/7. Pathological findings in a patient with ventilatory failure and chronic inflammatory demyelinating polyneuropathy.Chronic inflammatory demyelinating polyneuropathy (CIDP) may cause significant disability, but severe respiratory complications are uncommon. We describe the case of a 49-year-old man with clinical features of CIDP for 5 years who died of respiratory failure. Post-mortem findings of denervation in diaphragm muscle and axonal loss in phrenic nerve are presented. Severe ventilatory failure may occur in CIDP when neuropathy affects the respiratory muscles. attention to early clinical features of respiratory insufficiency may facilitate the prevention of more severe features of respiratory failure.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/7. mycoplasma pneumoniae causing nervous system lesion and SIADH in the absence of pneumonia.A patient was admitted for fever and acute respiratory failure (ARF), rapidly progressive tetraparesis, delirium, behavioral abnormalities, and diplopia. leukocytosis and a rise in c-reactive protein were present. A syndrome of inappropriate anti-diuretic hormone secretion (SIADH) was also diagnosed. Lumbar puncture yielded colorless CFS with mononuclear pleocytosis and protein rise. electrodiagnosis revealed demyelinating polyneuropathy and axonal degeneration. serum IgG and IgM for mycoplasma pneumoniae (MP) was consistent with acute infection, and erythromycin was started with rapid resolution of symptoms. Contrarily to most reports, an associated respiratory disease was not present and SIADH in association with MP has been reported only once, in a patient without direct central nervous system (CNS) involvement. Differential diagnosis and possible pathogenic mechanisms are discussed.- - - - - - - - - - ranking = 0.0019244065377414keywords = demyelinating (Clic here for more details about this article) |
3/7. An autopsy case of chronic inflammatory demyelinating polyradiculoneuropathy with respiratory failure.An 85-year-old Japanese woman presented with progressive symmetrical proximal and distal muscle weakness, numbness in the distal extremities, and sudden onset of hemifacial weakness. The results of laboratory studies fulfilled the American Academy of neurology criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). She died of respiratory failure after two courses of intravenous immunoglobulin treatment, each resulting in transient improvement in strength and sensory symptoms. autopsy revealed multifocal demyelinative or axonal lesions of varying severity affecting the cranial and peripheral nerves and including the phrenic nerve. These findings suggest that the clinical phenotype of CIDP depend on distribution and severity of the anatomical lesions.- - - - - - - - - - ranking = 1.1868813181996keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/7. Chronic inflammatory demyelinating polyneuropathy, phrenic nerve and respiratory symptoms.Respiratory involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) has been very recently described. phrenic nerve conduction studies have been described as useful to detect respiratory impairment in these patients. This study describes two patients with CIDP, in whom neurophysiological studies of the respiratory muscles were performed. The first patient had severe respiratory insufficiency, and phrenic nerve studies disclosed no motor responses and electromyography (EMG) of the diaphragm confirmed severe loss of motor units, bilaterally. On treatment, we documented clinical and neurophysiological improvement. In the second patient, phrenic nerve studies showed abnormal results; however, EMG of the diaphragm ruled out loss of motor units. The first case represents the risk of phrenic nerve involvement in this disorder, and the potential recovery on treatment. The second case illustrates that the temporal dispersion of the motor responses can be misleading, and EMG of diaphragm should be performed to confirm the loss of motor units.- - - - - - - - - - ranking = 0.43935604540126keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/7. Cyclosporin A therapy in paraprotein-associated neuropathy.A case of IgG paraprotein-associated demyelinating neuropathy complicated by respiratory failure, which was unresponsive to standard immunosuppressive drug therapy, is reported. Cyclosporin A therapy resulted in a marked clinical recovery with objective improvement in nerve conduction and vital capacity. The beneficial response suggests that cell-mediated immunity is an important pathogenetic mechanism, and that cyclosporin A may be useful in the treatment of other refractory cases of paraprotein-associated neuropathy.- - - - - - - - - - ranking = 0.0019244065377414keywords = demyelinating (Clic here for more details about this article) |
6/7. Chronic inflammatory demyelinating polyneuropathy and respiratory failure.Neuromuscular respiratory failure is not considered to be a clinical feature of chronic inflammatory demyelinating polyneuropathy (CIDP). We present 4 patients with CIDP who required respiratory assistance and mechanical ventilation. Two patients needed emergent intubation and one patient lapsed in a stupor from hypercapnia. Respiratory failure in CIDP should be considered exceptional, but more formal studies in CIDP may be needed to assess its prevalence.- - - - - - - - - - ranking = 0.43935604540126keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/7. Severe motor-dominant neuropathy with IgM M-protein binding to the NeuAcalpha2-3Galbeta- moiety.We report the occurrence of a relapsing, severe predominantly motor neuropathy in a 75-year-old man with an IGM-K M-protein binding to gangliosides GM2, GM3, GM4, GD1a, GT1b and LM1. Motor nerve conduction velocities were slowed with conduction block. A superficial peroneal nerve biopsy specimen revealed segmental demyelination and remyelination. The patient improved after repeated plasma exchanges, and the antibody titer decreased in association with clinical recovery. This IgM M-protein has a unique, previously unreported binding specificity for terminal NeuAcalpha2-3Galbeta- moiety in common to all gangliosides bound by the antibody except GM2. M-proteins with this affinity may be involved in the pathogenesis of this and other cases of motor-dominant demyelinating neuropathy.- - - - - - - - - - ranking = 0.0019244065377414keywords = demyelinating (Clic here for more details about this article) |