Primary phlebitis of central nervous system revealed by black-blood magnetic resonance imaging

Primary phlebitis of the central nervous system (PPCNS) is a rare condition that might be a subset of primary angiitis of the CNS. In this case report, the patient was a 39-year-old man with a 2-week history of anterograde amnesia and abnormal behaviours. Black-blood MRI (BB-MRI) showed contrast enhancement of the left basilar vein and cerebral superficial veins. Angiography showed unremarkable change in arteries. After a thorough differential diagnosis, we diagnosed PPCNS and then administered methylprednisolone pulse and cyclophosphamide pulse. The neuropsychological symptoms and MRI findings gradually improved, and after 2 months, the dose of prednisolone was gradually reduced to 20 mg. No recurrence was observed. This case shows that BB-MRI may be useful for diagnosing PPCNS.

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Black Blood MRI in Suspected Large Artery Primary Angiitis of the Central Nervous System

Journal of Neuroimaging ◽

10.1111/j.1552-6569.2012.00743.x ◽

2012 ◽

Vol 23 (3) ◽

pp. 379-383 ◽

Cited By ~ 39

Author(s):

Thomas Pfefferkorn ◽

Jennifer Linn ◽

Maximilian Habs ◽

Christina Opherk ◽

Clemens Cyran ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Black Blood ◽

Large Artery ◽

Black Blood Mri ◽

Primary Angiitis ◽

The Central Nervous System

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Elevated intracranial pressure requiring decompressive craniectomy in a child with progressive primary angiitis of the central nervous system: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-03005-y ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Lama S. Al-Mansour ◽

Abdulrahman A. AlRasheed ◽

Khaled R. AlEnezi ◽

Hamza M. AlAli

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Intracranial Pressure ◽

Decompressive Craniectomy ◽

Rare Condition ◽

Elevated Intracranial Pressure ◽

Decompressive Hemicraniectomy ◽

Ensure Patient Safety ◽

Primary Angiitis ◽

The Central Nervous System

Abstract Background Elevated intracranial pressure is a potentially catastrophic complication of neurologic injury in children. Successful management of elevated intracranial pressure requires prompt recognition and therapy directed at both reducing intracranial pressure and reversing its underlying cause. A rare condition that causes elevated intracranial pressure is childhood primary angiitis of the central nervous system, which is a rare inflammatory central nervous system disease that poses diagnostic and therapeutic challenges. To our knowledge, this is the first reported case of angiography-positive progressive childhood primary angiitis of the central nervous system requiring decompressive hemicraniectomy for refractory elevated intracranial pressure in children. Case presentation We report the case of a 5-year-old Saudi girl who presented to the pediatric emergency department with fever and new-onset status epilepticus. She had elevated inflammatory markers with radiological and histopathological evidence of angiography-positive progressive childhood primary angiitis of the central nervous system, complicated by elevated intracranial pressure. Despite medical management for both childhood primary angiitis of the central nervous system and elevated intracranial pressure, her neurological status continued to deteriorate and the elevated intracranial pressure became refractory. She developed right uncal, right subfalcine, and tonsillar herniation requiring decompressive hemicraniectomy with a favorable neurological outcome. Conclusion Decompressive craniectomy might be considered in cases of angiography-positive progressive childhood primary angiitis of the central nervous system with elevated intracranial pressure refractory to medication. A multidisciplinary approach for the decision of decompressive craniectomy is advised to ensure patient safety and avoid possible morbidities and mortality.

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