Posterior reversible encephalopathy syndrome as a presentation of acute intermittent porphyria

Gudkova V.V.; Shanina T.V.; Kimelfeld E.I.; Salimov K.A.; Orekhova M.A.; Fedotov E.V.; Stakhovskaya L.V.

doi:https://doi.org/10.17116/jnevro202012003160

Gudkova V.V.

Rossiĭskiĭ natsional'nyĭ issledovatel'skiĭ meditsinskiĭ universitet im. N.I. Pirogova, Moskva

Shanina T.V.

NII tserebrovaskuliarnoĭ patologii i insul'ta Rossiĭskogo gosudarstvennogo meditsinskogo universiteta

Kimelfeld E.I.

Pirogov Russian National Research Medical University, Moscow, Russia

Salimov K.A.

GBOU VPO "Rossiĭskiĭ natsional'nyĭ issledovatel'skiĭ meditsinskiĭ universitet im. N.I. Pirogova" MZ RF, Moskva

Orekhova M.A.

Eramishantsev City Clinical Hospital, Moscow, Russia

Fedotov E.V.

Eramishantsev City Clinical Hospital, Moscow, Russia

Stakhovskaya L.V.

Pirogov Russian National Research Medical University, Moscow, Russia

Posterior reversible encephalopathy syndrome as a presentation of acute intermittent porphyria

Authors:

Gudkova V.V., Shanina T.V., Kimelfeld E.I., Salimov K.A., Orekhova M.A., Fedotov E.V., Stakhovskaya L.V.

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Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(3): 60‑66

DOI: 10.17116/jnevro202012003160

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To cite this article:

Gudkova VV, Shanina TV, Kimelfeld EI, Salimov KA, Orekhova MA, Fedotov EV, Stakhovskaya LV. Posterior reversible encephalopathy syndrome as a presentation of acute intermittent porphyria. S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(3):60‑66. (In Russ.)
https://doi.org/10.17116/jnevro202012003160

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова (S.S. Korsakov Journal of Neurology and Psychiatry)

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Abstract:

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.

Keywords:

acute intermittent porphyria

epileptic seizures

polyneuropathy

posterior reversible encephalopathy syndrome

Authors:

Gudkova V.V.

Rossiĭskiĭ natsional'nyĭ issledovatel'skiĭ meditsinskiĭ universitet im. N.I. Pirogova, Moskva

Shanina T.V.

NII tserebrovaskuliarnoĭ patologii i insul'ta Rossiĭskogo gosudarstvennogo meditsinskogo universiteta

Kimelfeld E.I.

Pirogov Russian National Research Medical University, Moscow, Russia

Salimov K.A.

GBOU VPO "Rossiĭskiĭ natsional'nyĭ issledovatel'skiĭ meditsinskiĭ universitet im. N.I. Pirogova" MZ RF, Moskva

Orekhova M.A.

Eramishantsev City Clinical Hospital, Moscow, Russia

Fedotov E.V.

Eramishantsev City Clinical Hospital, Moscow, Russia

Stakhovskaya L.V.

Pirogov Russian National Research Medical University, Moscow, Russia

Close metadata

References:

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