Naslov
Rare etiology of postural orthostatic tachycardia syndrome

Autori
Didović, Diana ; Habek, Mario

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Skup
5. STUDENTSKI KONGRES NEUROZNANOSTI NEURI 2015.

Mjesto i datum
Rab, Hrvatska; Rijeka, Hrvatska, 24.04.2015. - 26.04.2015

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Nije recenziran

Ključne riječi
postural orthostatic tachycardia syndrome; tilt table test; anti-VGKC antibodies

Sažetak
Postural orthostatic tachycardia syndrome (POTS) implicates a heterogeneous group of disorders clinically manifested by similar symptoms. POTS symptoms include tachycardia, palpitations, fatigue, headache, nausea, syncope, exercise intolerance, diminished concentration and tremor. Diagnostic criteria for POTS are a heart rate increase of ≥30 beats/minute during 10 minutes upon standing up from a supine position without excessive decrease in blood pressure. POTS etiology is mostly idiopathic. We present a patient who, during the last 4 years, had frequent attacks of fainting, headaches, nausea and vertigo, without convulsive symptoms. A comprehensive diagnostic assessment (brain and cervical MRI, EEG and transcranial Doppler) was performed but showed no pathological deviations. Cerebrospinal fluid analysis showed a regular cell number but with blood-brain barrier dysfunction and positive oligoclonal IgG bands. The patient's difficulties were understood as epileptic seizures and treated with antiepileptics. The symptomatology worsened and the patient was referred to tilt table testing which showed positive for POTS. With additional diagnostic assessment (quantitative sudomotor axon reflex test, quantitative sensory testing and adrenaline and noradrenaline plasma values) a diagnosis of a neuropathic variant of POTS was made and fludrocortisone therapy began. As the disease progressed, several partial complex epileptic seizures were observed and anticonvulsive therapy was modified. Due to poor therapy response, an extended assessment was performed which revealed an increased anti-VGKC antibodies titer (anti-voltage-gated potassium channel antibodies) and immunosuppressive treatment began. Variants of autoimmune POTS connected with anti-ganglionic acetylcholine antibodies are described. In the reported case, POTS was connected with anti-VGKC antibodies which is, according to the literature published so far, the first case of this kind. Recent researches expanded the spectrum of symptoms and conditions with which increased anti-VGKC is usually connected. To conclude, while treating POTS patients who exhibit a poor response to therapy, it is necessary to keep in mind the autoimmune variants of this syndrome.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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