Scleroderma, stroke, optic neuropathy : a rare association.
Posted Sep 11 2009 4:56pm
By Das CP, Prabhakar S, Lal V, Kharbanda PS
A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.
The term 'scleroderma' refers to a group of disorders that share the common feature of thickening (sclerosis) of the skin. Although the cutaneous manifestations are the most striking, generalized scleroderma is a systemic disorder that can affect virtually any organ system and is more appropriately referred to as 'systemic sclerosis'. The pathology of different organ system involvement was first demonstrated by Goetz. The American college of Rheumatology has laid down the diagnostic criteria for systemic sclerosis. Certain medical diseases, and disorders in response to environmental, mental or extrinsic factors may result in a scleroderma like syndrome and are termed as secondary scleroderma. Rarely systemic sclerosis may not have skin changes ('systemic sclerosis sine scleroderma'), or may coexist with other connective tissue disorders e.g. systemic lupus erythematosis, polymyositis etc. ('overlap syndrome'). Lungs, kidney, gastrointestinal tract, joints and the heart are the common organ involvement in scleroderma besides the skin. Cerebrovascular ischemia is very rarely seen in patients of scleroderma. Scleroderma 'per se' is a rare disease with an incidence of 0.1-14 per 100,000 of general population. It is the rare manifestation of a rare disease that has prompted us to report this case.