Refractory convulsive syncope in pregnancy: a rare presentation of Takayasu’s arteritis - a case report and literature review

Background: Neurological manifestation of Takayasu’s Arteritis (TA) in pregnancy presenting as convulsive syncope is extremely rare, and poses a serious diagnostic dilemma due to other vast causes of fits in pregnancy. Objective: We aimed to present and shed more light on a case of TA with convulsive syncope in pregnancy refractory to anticonvulsants for seven weeks, and review the literature on the management of TA in pregnancy. Case presentation: A gravida 4 para 3+0 at 28 weeks of amenorrhea presented with repeated episodes of the sudden loss of consciousness, followed by a fall and jerking of the limbs. These were refractory to anticonvulsants that she had used for seven weeks. Physical examination revealed undetectable pulse and blood pressure (BP) in the upper limbs but elevated BP in the lower limbs. Further investigations confirmed TA and she improved on steroids and antihypertensives. Conclusion: This case typically describes the unexpected presentation of TA with convulsive syncope. It calls for meticu- lous clinical assessment of epileptic seizures in pregnancy to avoid a late diagnosis of TA and its potential poor outcomes. Keywords: Takayasu’s Arteritis in pregnancy; convulsive syncope; case report.

Neurologist, 7 years’ experience, Kenya

This chapter looks at the experience of a Neurologist who has had the privilege to be trained by internationally renowned specialists in epilepsy and Psychogenic Non-Epileptic Seizures (PNES). The diagnosis and management of PNES became more challenging, however, when the Neurologist moved countries to practice as a general Neurologist in a resource-limited regional tertiary referral center in Kenya. There, Neurology remains a poorly taught topic and “neurophobia” is strife; healthcare professionals therefore seem to label everything that falls and shakes as an epileptic seizure or, annoyingly, “unexplained seizure disorder.” Indeed, PNES in sub-Saharan Africa is not very well described in the published literature, and therefore the Neurologist’s introduction of terms such as PNES, transient loss of consciousness, and convulsive syncope were initially met with disbelief. The Neurologist realized early on that all staff, including the trainee doctors, had to be retrained and reminded of the importance of the clinical history and the witness account to make the diagnosis.

Neurologist, 5 years’ experience, Azerbaijan; Neurologist, 30 years’ experience, Turkey

This chapter highlights how Psychogenic Non-Epileptic Seizures (PNES) are frequent and difficult situations that should be distinguished from epileptic seizures. When misdiagnosed as epilepsy, patients with PNES are faced with serious and multidimensional difficulties, such as delayed appropriate treatment, adverse effects of long-term antiepileptic drugs (AED), and the financial burden they have to face. Moreover, PNES may be misdiagnosed with status epilepticus (SE), leading to interventions with potentially serious consequences, such as an admission to the intensive care unit (ICU) and intubation. The importance of continuous EEG monitoring for such episodes and evaluation by a clinical neurophysiologist is stressed. In cases such as PNES attacks triggered by convulsive syncope, multidimensional evaluation is necessary. Factors such as life events and psychological traumas should also be taken into consideration.