Sjogren syndrome accompanying pulmonary amyloidosis and lymphoproliferative disease manifests as multiple, large, thin-walled cysts; multiple nodules; parenchymal opacity; and bronchiectasis. These findings are caused by the interstitial or peribronchial infiltration of mixed amyloid and lymphoproliferative cells.

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Background: Mild encephalopathy with a reversible splenial lesion (MERS) is a clinico-radiological syndrome characterized by a reversible isolated lesion with transiently reduced diffusion in the central portion of the splenium of the corpus callosum (SCC). The reason for MERS is unknown. however, infectious-related MERS (in particular virus) remains the most common cause of reversible splenial lesions. Acute intermittent porphyria (AIP) is an autosomal dominant disorder caused by a partial deficiency of porphobilinogen deaminase (PBGD), the third enzyme in the of hemebiosynthetic pathway. It can affect the autonomic, peripheral, and central nervous system. Result: In this study, we report a 20-year-old woman with AIP who presented with MR manifestations suggestive of MERS, she had a novel PBGD splicing mutation, a G to A mutation in base 594 resulting in tryptophan to a stop codon (W198*). Conclusion: To the best of our knowledge, this is only one published case of MERS associated with AIP.

Background: Acute intermittent porphyria (AIP) is an inherited disorder of heme biosynthesis, a porphyric attack can affect the autonomic, peripheral, and central nervous systems. The neurological clinical manifestations of which are incompletely understood. The neuroimaging findings of AIP could be reversible. Methods: In this report, we describe 29 cases of AIP, focusing on neurological clinical features and neuro-imaging. Results: In this study, we showed two cases of PRES, two cases of ODS, two cases of porphyric encephalopathy (cortical laminar necrosis), one case of RESLES. We divided 29 cases into 2 groups ,the blood sodium levels of who with MRI/CT abnormality were significantly lower than which with normal MRI/CT(110±43.15mmol/L and 117±57.02mmol/L, p=0.01).Conclusions: To the best of our knowledge, these are the biggest series of MRI in AIP in China, and the only published cases of ODS associated with AIP in China. Hyponatremia may be an important mechanism in porphyric encephalopathy.