Dyke-Davidoff-Masson syndrome-like picture in a case of Takayasu arteritis: an enigma

Abstract

Authors describe the case of a 16-year-old girl who presented with fever, tonic-clonic seizures, unequal arm blood pressures and pulselessness in the left upper limb. On examination, there was a systolic bruit over umbilical region, a pansystolic murmur of mitral regurgitation was found. Neurological examination was normal except for an asymmetry in brain hemicircumference one side compared with the other. She has borderline intelligence (IQ 70) according to Wechsler Adult Performance Intelligence Scale. Magnetic resonance imaging (MRI) of brain revealed atrophic of left cerebral hemisphere with mildly ventricular dilatation, prominent paranasal and mastoid air cells, suggestive of Dyke-Davidoff-Masson syndrome (DDMS). Conventional angiography showed narrowed left internal carotid artery. There was also stenosed brachial artery, absent left renal artery with narrowed infrarenal abdominal aorta. The patient was put on antihypertensive drugs. We hypothesise that Takayasu arteritis and related vascular occlusion is the cause of her acquired cerebral changes.

Figure 1

(A) Axial T1-weighted image: left cerebral hemiatrophy with ipsilateral occipital horn dilatation. In addition, ipsilateral pneumosinus dilatans (frontal) is seen; (B) axial T2-weighted fluid-attenuated inversion recovery MRI demonstrating signal changes in right temporal and occipital cortex with oedematous posterior right cerebral parenchyma; (C) axial T2-weighted image: hyperintensity in white matter represents gliotic changes (more on left side). MRI, magnetic resonance imaging.

Figure 2

(A) Conventional angiography showed complete obstruction of left subclavian artery distal to the origin of vertebral artery with development of several collaterals; (B) stenosis of left internal carotid artery (arrow) with normal left external carotid artery; (C) absent left renal artery and with narrowing of infrarenal abdominal aorta.