Search

The authors describe a clinical case of posterior reversible encephalopathy syndrome (PRES) in a 36-year-old female patient due to the first onset of not previously diagnosed acute intermittent porphyria. Only 22 clinical and radiological cases of the combination of PRES and acute porphyria were reported in the literature by 2018. This is the first report of a similar association confirmed by magnetic resonance imaging (MRI) in the Russian literature. The article describes the dynamics of the disease from unexplained abdominal pain to repeated epileptic paroxysms and subsequent formation of a detailed picture of encephalo- and polyneuropathy. The particularities of neurological presentations, the significance of epileptic paroxysms and MRI in the diagnosis of the abovementioned combined pathology are shown. Possible mechanisms causing both pathological processes are considered.

The experience of anesthesia and intensive care departments of Hematological center Russia for intensive care management in 13 patients with acute porphyria, from 1996 till 2013 was summarized ion this pape4: Main causes of life-threatening complications of acute porphyria and its frequency were revealed Advantages of mechanical lung ventilation in respiratory failure, algorithms of clinical nutrition, correction of water-electrolyte disorders were represented. Importance of kinesiotherapy in successful treatment in these category of patients was revealed. It is shown that the whole complex of intensive care methods with the specific pathogenetical therapy brings success in 84,6 % of patients.

A case of acute intermittent porphyria is described in a 37-year-old female patient treated with normasang, a drug that regulates porphyrin metabolism at the last stages of the disease. Chronic renal failure with the hypertensive syndrome, severe neurological symptoms, and vascular sclerotic changes in all organs were the symptoms of the underlying disease. Infectious complications were the cause of sepsis and favoured deteriorated multiple organ dysfunction that determined lethal exitus.