Gudkova V V, Shanina T V, Kimelfeld E I, Salimov K A, Orekhova M A, Fedotov E V, Stakhovskaya L V
Pirogov Russian National Research Medical University, Moscow, Russia.
Eramishantsev City Clinical Hospital, Moscow, Russia.
Zh Nevrol Psikhiatr Im S S Korsakova. 2020;120(3):60-66. doi: 10.17116/jnevro202012003160.
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.
作者描述了一名36岁女性患者因首次发作先前未诊断出的急性间歇性卟啉病而出现的后部可逆性脑病综合征（PRES）临床病例。截至2018年，文献中仅报道了22例PRES与急性卟啉病合并的临床和放射学病例。这是俄罗斯文献中首例经磁共振成像（MRI）证实的类似关联报告。本文描述了该疾病从不明原因的腹痛发展到反复癫痫发作以及随后形成详细的脑和多神经病症状的过程。展示了神经学表现的特点、癫痫发作以及MRI在上述合并病理诊断中的意义。还探讨了导致这两种病理过程的可能机制。
