Table 1 Case reports of PRES with MCTD reported till now
Case | Age | Sex | Associations/Medications | Hypertension | Clinical features | MRI findings | Outcome/sequale |
|---|---|---|---|---|---|---|---|
Case-1 | 15 years | Male | Mixed connective tissue disease on cyclophosphamide pulse therapy, prednisolone and HCQ | Yes | GTCS | High signal intensities in the posterior areas of his brain. | Uncontrollable respiratory distress and eventually died |
Case-2 | 20 years | Female | Mixed connective tissue disease | No | Headache and intermittent vomiting along with two episodes of GTCS | Bilateral hyper intense signal in occipital region on T2 weighted MRI and FLAIR, and bilateral hypo intense signal in T1 weighted MRI | Completely recovered. Follow up MRI Brain was normal |
Case-3 | 12 years | Male | Mixed connective tissue disease, secondary membranous glomerulopathy | No | GTCS Aphasia Fever Generalized body swelling | Hyper intense signal in the parieto-occipital regions | Completely recovered |
Our patient | 30years | Female | Mixed connective tissue disease | Yes | Headache Fever Visual disturbances Sudden drop in sensorium | Hyperintense signals in fronto parietal and parieto temporal region | Completely recovered |