Mokri, Bahram

Antiphospholipid antibodies are known to be associated with increased risk of venous and arterial thrombotic events, including cerebral venous thromboses. Pseudotumor syndrome can be produced by cerebral venous thrombosis. A patient with cerebral venous thrombosis associated with antiphospholipid antibodies who exhibited pseudotumor syndrome is reported. A 49-year-old man who noted visual blurring and persistent vertical wavy lines in his fields of vision was found to have papilledema. Cerebrospinal fluid values were normal except for an opening pressure increase to 510 mm of fluid. His visual symptoms improved with lumbar puncture and the use of acetazolamide. Imaging studies showed that the transverse sinus was occluded completely on the left and partially on the right and that there was a small left cerebellar cortical venous infarction. Antiphospholipid syndrome should be considered in the differential diagnosis of pseudotumor syndrome related to cerebral venous thrombosis.

BACKGROUND AND PURPOSE: The familial occurrence of intracranial aneurysms and cervical artery dissections has been described in different families and supports the hypothesis that a primary arteriopathy may play a role in the pathogenesis of these disorders. Although the basis for this arteriopathy is generally not believed to be similar among cases of intracranial aneurysms and cervical artery dissections, several similarities exist in the epidemiology of these disorders and a common underlying arterial abnormality may be suspected. SUMMARY OF REPORTS: The medical records of all 175 patients with spontaneous dissections of the cervical arteries who were seen at the Mayo Clinic between 1970 and 1989 were reviewed to identify families in which intracranial aneurysms and cervical dissections coexisted. Three families were identified in which intracranial aneurysms and cervical artery dissections were observed among siblings. These families are described in detail. CONCLUSIONS: The familial occurrence of intracranial aneurysms and cervical artery dissections within the same families provides support to the importance of a common underlying arteriopathy in the pathogenesis of both these disorders. The underlying vascular defect may, at least in some cases, be inherited.

Spontaneous intracranial hypotension (SIH) was described nearly 70 years ago, and for years it was essentially equated with post dural puncture headaches (PDPH) (1). The interest in this entity substantially increased after the magnetic resonance imaging (MRI) abnormalities of this disorder were recognized in the early 1990s (2-6), and consequently many more patients were diagnosed than before and a far broader clinical spectrum of this disorder began to become apparent. It also came to be recognized that the overwhelming majority if not all cases of SIH result from spontaneous cerebrospinal (CSF) leaks and that the independent pathogenic parameter is loss of CSF volume (7).

Recurrent dissections involving carotid, vertebral, or renal arteries have been described in patients with spontaneous cervical artery dissections, with a maximal interval between dissections of fourteen years. The authors describe 2 patients in whom aortic dissections developed twenty-five and forty years, respectively, following carotid artery dissections. These 2 patients constituted 8% of the total number of patients from Rochester, Minnesota, who were diagnosed with aortic dissection between 1987 and 1992. The first patient, a forty-five-year-old woman, presented in 1948 with right neck pain and headache, associated with several episodes of transient numbness of the right face and numbness and clumsiness of the left upper and lower extremities. Examination showed right miosis. Angiography showed a stenosis of the extracranial right internal carotid artery beginning several centimeters from the bifurcation. She died at age eighty- five from an aortic dissection. The second patient, a thirty-eight-year-old man, noted left orbital and frontotemporal headaches and drooping of the left eyelid in 1962. Examination showed left oculosympathetic palsy. Angiography showed stenosis and an aneurysm in the midportion of the extracranial left internal carotid artery. He died at age sixty-three from an aortic dissection. These cases suggest that following a carotid artery dissection the risk of a recurrent arterial dissection may remain elevated for a prolonged period of time and the recurrent dissection may involve the aorta.