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Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia
1New England Center for Headache, 778 Long Ridge Road, Stamford, CT 06902-1251, USA. medvol98@yahoo.com
Introduction:
Primary (isolated) angiitis of the central nervous system (PACNS) is a rare cause of cerebrovascular disease (CVD), and few leptomeningeal and brain biopsy (LBB)-confirmed cases have been reported from South America.
Methods:
We retrospectively reviewed charts of patients with diagnosis of cerebral angiitis admitted between March 1991 and July 2001 to a single university hospital in Medellin, Colombia. Patients with definitive diagnosis of PACNS by Alrawi et al.'s LBB criteria were selected. We excluded other causes of cerebral angiitis as well as cases without LBB confirmation.
Results:
We report five patients, four men and one woman, with a mean age at onset of 24.4 years, and an average disease progression of 12.4 days. Four presented with headache and motor weakness, three had seizures, and two had alterations of consciousness. Cerebral MRI was abnormal in all five cases; brain CT in four, and cerebral angiography in two. The cerebrospinal fluid (CSF) was abnormal in two patients. Leptomeningeal and brain biopsies revealed mononuclear infiltration in the wall of small blood vessels in all. Three had concurrent meningeal and cerebral involvement, two had necrotizing angiitis, and one had vascular and encephalitic lesions. All received only steroid treatment; the 1-year follow-up revealed good prognosis without relapses.
Conclusion:
We report five biopsy-proven cases of PACNS from Colombia associated with neurological and neuroimaging abnormalities; these patients presented a mild inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid treatment without relapses. Leptomeningeal and brain biopsy is mandatory for a definitive diagnosis.
Insights
Primary angiitis of the central nervous system (PACNS) is rare, especially in South America. Biopsy-confirmed PACNS cases in Colombia showed mild inflammation, few CSF abnormalities, and good outcomes with steroid treatment.
Area of Science:
- Neurology
- Neuroimmunology
- Vascular Neurology
Background:
- Primary (isolated) angiitis of the central nervous system (PACNS) is a rare cause of cerebrovascular disease.
- Few leptomeningeal and brain biopsy (LBB)-confirmed PACNS cases are reported from South America.
Purpose of the Study:
- To report biopsy-proven cases of PACNS from Colombia.
- To describe the clinical, neuroimaging, and histopathological features of these cases.
- To evaluate the treatment response and prognosis.
Main Methods:
- Retrospective review of patients diagnosed with cerebral angiitis between 1991 and 2001 at a university hospital in Medellin, Colombia.
- Inclusion criteria: definitive diagnosis of PACNS by Alrawi et al.'s LBB criteria.
- Exclusion criteria: other causes of cerebral angiitis and cases without LBB confirmation.
Main Results:
- Five patients (4 men, 1 woman) with a mean age of 24.4 years were identified.
- Common symptoms included headache, motor weakness, seizures, and altered consciousness.
- Leptomeningeal and brain biopsies showed mononuclear infiltration; three cases had meningeal and cerebral involvement, two had necrotizing angiitis, and one had vascular and encephalitic lesions.
- Cerebral MRI was abnormal in all cases; CT in four, angiography in two. CSF was abnormal in two patients.
- All patients received steroid treatment, with a good 1-year prognosis and no relapses.
Conclusions:
- Biopsy-proven PACNS cases from Colombia present with neurological and neuroimaging abnormalities.
- These patients exhibited mild inflammatory disease with minimal CSF abnormalities.
- PACNS responded well to single steroid treatment, showing a good prognosis without relapses.
- Leptomeningeal and brain biopsy is essential for definitive PACNS diagnosis.
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