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Hemorrhagic primary CNS angiitis and vasoconstrictive drug exposure
Mehmet A Topcuoglu1, Ruchira M Jha1, Jacob George1
1Massachusetts General Hospital and Harvard Medical School (MAT, MPF, ABS), Boston; Neurology Department (MAT), Hacettepe University Hospitals, Ankara, Turkey; Safar Center for Resuscitation (RMJ), University of Pittsburgh Medical Center, PA; and Government Medical College (JG), Kottayam, India.
Insights
Hemorrhagic primary angiitis of the CNS (PACNS) is linked to sympathomimetic drug use. This condition, characterized by thunderclap headaches and lobar hemorrhages, often resolves with treatment.
Area of Science:
- Neurology
- Vascular Neurology
- Neuroinflammation
Background:
- Primary angiitis of the CNS (PACNS) typically causes stroke-related neurological deficits.
- Intracerebral hemorrhage (ICH) is a rare PACNS complication with poorly understood risk factors.
Purpose of the Study:
- To investigate the risk factors and clinical features of hemorrhagic PACNS.
- To compare hemorrhagic and non-hemorrhagic PACNS presentations.
Main Methods:
- Retrospective analysis of 49 PACNS patients diagnosed between 1993 and 2015.
- Comparison of clinical and imaging features between hemorrhagic and non-hemorrhagic PACNS cohorts.
- Detailed analysis of patients with hemorrhagic PACNS.
Main Results:
- Five patients presented with ICH, with 4 cases showing ICH as the initial PACNS manifestation.
- All ICH patients reported recent sympathomimetic drug use.
- ICH cases exhibited higher rates of headache, particularly thunderclap headache, and eosinophilic vascular infiltrates on biopsy.
Conclusions:
- Hemorrhagic PACNS is strongly associated with sympathomimetic drug exposure.
- Shared features with reversible cerebral vasoconstriction syndrome (RCVS) suggest an overlap syndrome.
- Sympathomimetic drug-induced vasoconstriction may trigger inflammation in this PACNS-RCVS overlap syndrome.
Background:
Primary angiitis of the CNS (PACNS) typically manifests with accumulating neurologic deficits from ischemic strokes. Intracerebral hemorrhage (ICH) is an uncommon complication. There is limited knowledge about the risk factors and features of hemorrhagic PACNS.
Methods:
We identified 49 patients (20 biopsy-proven) with PACNS diagnosed at our hospital from 1993 to 2015. We compared the features of hemorrhagic and nonhemorrhagic PACNS and analyzed the hemorrhagic PACNS cases in detail.
Results:
The mean age was 51 ± 15 years; 13 patients were men. Five patients had ICH (mean age 52 ± 14 years; 4 men) including 4 where ICH was the first manifestation of PACNS. All ICH patients reported recent exposure to sympathomimetic drugs (e.g., diet pills, nasal decongestants). Patients with ICH had higher rates of headache (100% vs 43%, p = 0.022), especially thunderclap headache (60% vs 0%, p = 0.001), and eosinophilic vascular infiltrates on brain biopsy (50% vs 9%, p = 0.084). In all ICH patients, brain MRI showed lobar hemorrhages with concurrent punctate diffusion-restricted lesions, suggesting an acute inflammatory process. Four received a short course of immunosuppressive therapy. All patients showed complete clinical resolution or significant improvement within weeks.
Conclusions:
In this study, hemorrhagic PACNS was exclusively associated with sympathomimetic drug exposure. The high rate of thunderclap headache, lobar hemorrhages, and the self-limited clinical course suggests a shared mechanism between hemorrhagic PACNS and the reversible cerebral vasoconstriction syndrome (RCVS), a PACNS mimic. This RCVS-PACNS overlap syndrome may result from sympathomimetic drug-induced prolonged distal vasoconstriction, culminating in inflammation.
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