Fibrous Dysplasia of the Right Cranium Orbit and Frontosubcortical Deficits
Golnoush Akhlaghipour1, Leila Parand2
1Neurology, Saul R. Korey Department of Neurology, Montefiore Medical Center, Albert Einstein College of Medicine, 2Neurology, UCLA Memory Clinic, David Geffen School of Medicine
Objective:
To describe a patient and the relationship of frontoparietal functioning changes after injury of the brain from extension of fibrous dysplasia of the right cranium orbit.
Background:
Fibrous Dysplasia is a rare skeletal developmental anomaly that occurs when normal bone is replaced by an excessive growth of fibrous connective tissue intermixed with irregular bone tissue. It may occur as a single lesion, multiple lesions, and part of a syndrome (McCune-Albright) with incidences ranging from 1:4000–1:10,000 although incidents may go undetected as not everyone experiences symptoms. Craniofacial fibrous dysplasia may affect the bone structures such as the mandible, maxilla, cranial base and vault. The disease manifests from a missense mutations in Gs alpha gene in pluripotent embryonic stem cells. The symptoms tend to start in childhood but may present at any stage. For craniofacial dysplasia the sphenoid, temporal and frontal bones are less often involved compared to maxilla and mandibular involvement. Less information has been reported on the possible cognitive changes associated with fibrous dysplasia.
Design/Methods:
We present a case of a 59-year-old, right-handed, African American woman with past medical history of fibrous dysplasia, bilateral frontoparietal and right orbitozygomatic craniectomy for subtotal resection, external/epidural hydrocephalus, and left ventriculoperitoneal shunt surgery presenting with memory complaints, persistent depression, and migraines. The patient’s neurocognitive tests' results are indicative of a frontal-subcortical profile with variable deficits in attention, working memory, and processing speed, and fluctuation in executive functioning(deficits in inhibition, learning, attentional set-shifting). Based on objective neuropsychological findings and her difficulties with performance at work, she met criteria for Mild Neurocognitive Disorder. Brain MRI showed Evolving postsurgical changes of right frontoparietal and orbital frontal craniotomy with cranioplasty flap.
Conclusions:
This case shows that fibrous dysplasia, a rare disease, can affect aspects of behavior and cognition. Comprehensive neuropsychological assessments are helpful to monitor changes in cognitive status.
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