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A, 21-years-old, male, presented with acute onset, gradually progressive, predominantly distal, symmetrical weakness of both upper and lower limbs with arreflexia. He had impaired sensations in glove and stocking distribution with distal gradient. He was found to have absolute CD4 + cell count of 188 cells/µL, absolute CD8 cell count, 532 cells/µL and CD4: CD8 ratio of 0.35. Electrophysiology revealed reduced to absent CMAP amplitude as well as SNAPs in various nerves of upper and lower limbs, along with normal conduction velocity and normal F wave latencies. Pattern evoked visual potentials were prolonged, on both sides, P100 being 130 ms, on right and 108 ms, on left side. In the follow up of 2 years, he showed spontaneous but gradual clinical improvement but his electrophysiological parameters as well as CD 4+ cells count did not show any significant improvement. [ABSTRACT FROM AUTHOR]

The blood-nerve barrier ( BNB) is a peripheral nerve-specific capillary barrier system that blocks the entrance of pathogenic leukocytes and toxic soluble mediators, such as cytokines, chemokines and immunoglobulin, from the circulating blood to the parenchyma of the peripheral nervous system ( PNS). The pathological breakdown of the BNB might play pivotal pathophysiological roles in the onset of various immune-mediated neuropathies, including Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy ( CIDP), multifocal motor neuropathy ( MMN) and a subset of diabetic neuropathy. However, fewer studies have so far been carried out regarding the cell biology and clinical significance of the BNB compared with the total number of studies of the blood-brain barrier because of the lack of adequate human cell lines derived from the BNB. In recent studies, we successfully established human immortalized cell lines of BNB origin in our laboratory and describe their detailed cellular properties. Although CIDP is now recognized as involving several clinical phenotypes, including both typical CIDP and atypical CIDP, disruption of the BNB could determine the clinical phenotype, severity and course of disease in the case of CIDP. Although the underlying pathomechanisms of MMN have not yet been clarified, BNB breakdown can cause persistent demyelination at sites of conduction block in patients with MMN. In the present review article, we highlight the recently acquired insight regarding the pathology and pathophysiology of BNB breakdown in the setting of the immune-mediated neuropathy. In addition, we discuss how this knowledge can be used to develop novel therapeutic strategies for treating immune-mediated neuropathies by manipulating the BNB. [ABSTRACT FROM AUTHOR]