Granulomatous angitis of the central nervous system. Case report and review

The nineteenth case report of granulomatous angiitis of the central nervous system is described in a 47-year-old patient, who survived 2 years with his disease. Problems with establishing the diagnosis are discussed. No single group of clinical symptoms or laboratory data exist for making a positive diagnosis during life, although a combination of certain nonspecific factors such as mental change, spinal fluid protein elevation and pleocytosis should be present before the diagnosis of granulomatous angiitis is entertained. Special blood tests and immunological studies and contrast procedures, as well as cortical biopsy have been of little value in establishing the diagnosis; instead, a leptomeningeal biopsy may be the procedure of choice. This patient, as well as the others given a trial of steroid treatment, demonstrated some obvious detectable clinical improvement. All patients treated with steroids have survived longer than any untreated patient suggesting that these drugs may be of some benefit.