FA appears to be the best marker for the epileptogenic zone. Surgery should be contemplated with caution if DEE is the first ictal change. However, a widespread/bilateral PED at onset is common and should not discourage surgery.
Diagnostic accuracy for FNAB was very high. No clinical or radiological factors improved the positive LR of FNAB alone. Liberal use of FNAB of PGM is recommended.
A 51-year-old man gave a 2-year history of worsening mobility, cognitive decline and headaches. He had a history of thromboembolic stroke, recurrent transient ischaemic attacks and a spontaneous intraventricular haemorrhage. On examination, he had livedo reticularis and perniosis and a systolic murmur. Catheter cerebral angiography showed peripheral small-vessel and medium-vessel vasculopathy resulting in pruning of the distal cortical vessels and tortuous irregular distal collaterals. Skin biopsy showed subtle vasculopathy with ectasia of capillaries and postcapillary venules but no frank vasculitis or arterial thrombosis. Repeated serum antiphospholipid antibody titres were negative. The clinical features, skin biopsy and angiogram findings strongly supported a diagnosis of Sneddon's syndrome. Clinicians should consider Sneddon's syndrome in patients with livedo reticularis and stroke. There are treatment dilemmas in this situation when ischaemic and haemorrhagic cerebral events coexist.
A 69 year old man developed two month progressive short term memory difficulty and then presented with vomiting and confusion. He had bilateral lung transplants for bronchiectasis, immunosuppressed with tacrolimus and mycophenolate for two years. Examination revealed dry thoracic shingles.MRI demonstrated two intra-axial frontal lesions with faint peripheral enhancement, diffusion restriction and vasogenic oedema. Additionally, small foci of diffusion restriction were seen in the upper cervical cord and brainstem. Cerebrospinal fluid showed 340/mm3 lymphocytes, protein 1.2 g/L, normal glucose ratio and positive Varicella zoster virus (VZV) PCR.For possible VZV vasculopathy, he received steroids and acyclovir but unfortunately died of multi organ failure approximately two weeks from admission. Post mortem examination demonstrated necrotic frontal lesions with angiodestructive neoplastic B cell proliferation and EBV positivity. An infarct within the upper cervical cord was also seen with arteritis of one of the posterior spinal arteries. There was widespread perivenular inflammatory cell infiltrate. These findings were consistent with lymphomatoid granulomatosis with vasculitic changes; a rare EBV-driven lymphoproliferative disorder that affects the lungs, skin and central nervous system. Aetiology in this case is felt to be secondary to immunosuppression. However, the role of varicella is unclear and the native lungs have not been examined.
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