Seizures in a patient with diffuse scleroderma.

Accepted 19 April 1995 A 31-year-old black woman, with a nine-year history of systemic sclerosis, was admitted with an episode of jerking movements of both upper extremities, followed by unresponsiveness. In the emergency room, she had an episode of generalised tonic-clonic seizures. Her husband stated that she had never had seizures prior to these two episodes and had no headaches, nausea, vomiting or prior neurological disease. On examination, she was found to be drowsy and disoriented but would move all four limbs on painful stimuli. She was afebrile with a respiratory rate of 20/min and a blood pressure of 172/78 mmHg. Fundus examination showed no evidence of any haemorrhage, exudate or papilledema. She exhibited a characteristic facies of scleroderma with marked tightness of the skin over the nose, ears, forehead and neck. She also had extensive skin tightness, thickening and non-pitting induration involving the distal parts of both upper and lower extremities with deformities of both hands. Signs of meningeal irritation were absent and she did not have any focal neurological deficit. The results of laboratory studies on admission and those carried out about one year earlier are shown in the table. The peripheral smear is shown in figure 1. Bone marrow biopsy revealed markedly hypoplastic marrow predominated by fat cells with occasional megakaryocytes. Serological studies showed positive antinuclear antibodies (titer 1:640), negative DsDNA, anticardiolipin antibodies and rheumatoid factor. Computed tomography (CT) scan of the head was normal and studies of the cerebrospinal fluid did not reveal any evidence of infection. Over the next three days her platelets dropped to 59 x 109/1 with a drop in haemoglobin to 7.6 g/dl. Lactate dehydrogenase increased to 1840 IU/1. Renal function also rapidly deteriorated with blood urea nitrogen 44 mg/dl and creatinine 3.1 mg/dl.