BMJ case reports
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Acute intermittent porphyria (AIP) is an inherited deficiency in the haem biosynthesis pathway. AIP is rare, affecting around 1 in 75 000 people. Acute attacks are characterised by abdominal pain associated with autonomic, neurological and psychiatric symptoms. ⋯ PRES is a clinicoradiological condition caused by the failure of the posterior circulation to autoregulate, resulting in cerebral oedema, headaches, nausea and seizures. This association is important because drugs used in the management of seizures may worsen an attack of AIP. This article describes a case of AIP and PRES in a young woman.
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A 44-year-old man, with a history of arterial hypertension, was referred with increasing shortness of breath due to a large pericardial effusion and imminent tamponade. Emergency ultrasound-guided pericardiocentesis resulted in the rapid withdrawal of 2760 cc of serous fluid. 3 hours later, the patient developed acute pulmonary oedema, which was successfully treated. ⋯ Although the underlying mechanisms causing PDS are not fully understood, patients with high left ventricular (LV) filling pressures are at particular risk. In other words: diastolic dysfunction of the LV is a risk factor for the occurrence of PDS.
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Posterior condylar canal dural arteriovenous fistulas (PCC DAVFs) are rare lesions that may present with pulse-synchronous bruit. In cases with venous reflux there is a risk of haemorrhage or even dementia. Diagnosis and endovascular treatment require a profound knowledge of the vascular anatomy of the craniocervical junction and comprehensive neurovascular imaging. ⋯ Symptoms resolved immediately after intervention and the patient recovered quickly without any neurological deficits. MR angiography confirmed occlusion of the DAVF. The dural sinus was patent with normal blood flow.