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Aneurysmal subarachnoid hemorrhage with PFBC and beta thalassemia: a case report
Kuangyang Yu1, Jinwei Pang1,2,3, Xiaobo Yang1
1Department of Neurosurgery, the Affiliated Hospital of Southwest Medical University, Luzhou, 646000, China.
Insights
Primary familial brain calcification (PFBC) can co-occur with aneurysmal subarachnoid hemorrhage and thalassemia. Early CT angiography is crucial for diagnosing cerebrovascular conditions in PFBC patients.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Primary familial brain calcification (PFBC), also known as Fahr's disease, involves bilateral basal ganglia calcification.
- PFBC can extend to the cerebellum, brainstem, and grey-white matter junctions.
- Co-occurrence of PFBC with aneurysmal subarachnoid hemorrhage (aSAH) and thalassemia is rarely reported.
Observation:
- A patient presented with acute consciousness deterioration without prior neurological symptoms.
- Computed tomography (CT) and CT angiography (CTA) revealed PFBC, aSAH, and intracranial hemorrhage.
- Hemoglobin electrophoresis indicated beta-thalassemia.
Findings:
- The patient diagnosed with PFBC, aSAH, and beta-thalassemia underwent surgical intervention.
- Surgical procedures included craniotomy with aneurysm clipping and intracranial hematoma removal.
- This case highlights a rare clinical presentation of PFBC.
Implications:
- PFBC patients require monitoring of blood pressure and intracranial vascular status.
- CTA is essential for assessing cerebrovascular conditions in PFBC, particularly with hypertension or headache.
- Early diagnosis and management of vascular complications in PFBC are critical.
Background:
Primary familial brain calcification (PFBC), habitually called Fahr's disease, is characterized by bilateral calcification of the basal ganglia, accompanied by extensive calcification of the cerebellar dentate nucleus, brainstem cerebrum, and cerebellum at the grey-white matter junction. However, there are few reports about PFBC with aneurysmal subarachnoid hemorrhage (aSAH) and thalassemia.
Case Presentation:
We describe a patient admitted to the hospital with an acute deterioration in the level of consciousness with no history of neuropsychiatric features or movement disorders. After computed tomography (CT) and CT angiography (CTA), the patient was diagnosed with PFBC, accompanied by aneurysmal subarachnoid haemorrhage (aSAH), intracranial haemorrhage (ICH), and hemoglobin electrophoresis suggested beta-thalassemia. This patient underwent craniotomy aneurysm clipping and intracranial hematoma removal.
Conclusions:
For patients with PFBC, we should pay attention to their blood pressure and intracranial vascular conditions. The CTA is necessary to clarify the cerebrovascular conditions of the patient, especially when combined with hypertension and persistent headache or other related prodromal symptoms of cerebrovascular disease.
