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Call Fleming Syndrome

HISTORY

30 year old male presented with severe, sudden onset of throbbing orbito-frontal thunderclap headache

FLAIR axial images show focal subarachnoid hemorrhage – blood in the precentral and superior frontal sulci (arrow). The cisterns and Sylvian fissures are free of blood.

MRA TOF images show vasospasms in right A1 and left A1.

Oblique view under DSA shows vasospasm in left A1 and the frontal view shows vasospasm in right supraclinoid ICA, M1 and A1, confirming our MRA findings.

Follow up after 12 weeks demonstrates normal MR angiogram of the brain. 

DIAGNOSIS : Call Fleming Syndrome 

DISCUSSION : Thunderclap headache is most commonly seen secondary to subarachanoid haemorrhage (SAH), the various other causes are cerebral vasculitis, carotid artery dissection, cerebral dural venous thrombosis and pituitary apoplexy and Reversible cerebral vasoconstriction syndrome (RCVS). 

Call Fleming Syndrome, is an idiopathic part of the spectrum of reversible cerebral vasoconstriction syndrome (RCVS), which is a clinic-radiological entity, encompassing a large group of conditions which are characterized by, as the name suggests, intracerebral vascular spasms that are reversible. The classical radiological presentation is that of multi-segmental vasospasm in the vessels of the circle of Willis. The post-stenotic areas classically show dilatation. The resolution of this occurs within 8 to 12 weeks. 

DIFFERENTIALS: 

  1. Aneurysmal rupture – Vasospasm associated with SAH tends to be in the vessels closest to the site of ruptured aneurysm and there tends to develop about 1-2 weeks post hemorrhage. In various aneurysmal rupture, cisterns/sylvian fissures are inevitably involved. Our case had involvement of a focal right frontal cortical sulcus. 
  2. Traumatic SAH – history of trauma needs to be availed. 
  3. Primary angiitis – Diffuse white matter lesions, multiple infarctions in various vascular territories, multiple intraparenchymal hemorrhages and associated with dull aching pain of insidious onset and progressive in nature, unlike our case where a sudden and sever onset of headache occurred and MRI revealed no parenchymal changes. 
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