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Fahr's Disease in a Nigerian Pediatric Patient: A Rare Neurodegenerative Disorder

Eluehike SU, Izevbekhai OS, Irabor PFI, Oriaifo B, Oshodin E

Introduction: Fahr’s disease is a rare neurodegenerative disorder characterized by abnormal calcium deposition in the basal ganglia, thalamus, dentate nucleus, subcortical white matter and hippocampus. The disease is characterized by impaired cognitive development, seizures, motor deficits and other neuropsychiatric symptoms. It is largely hereditary, with some cases occurring sporadically. Computed tomography and Magnetic resonance imaging are pivotal in its imaging, as they usually would help in identifying bilateral basal ganglia calcification and also identify other associations.

Objectives: To report a rare neurodegenerative disorder (Fahrs disease) in a Nigerian child. To review pertinent literature about this condition. To highlight the role of diagnostic imaging especially with Computed tomography, and also the findings on this modality.

Case Report: The case of a 7-year old male who was admitted in the Children emergency unit of the Irrua Specialist Teaching Hospital, on account of seizures, fever, dyspnoea and loss of consciousness. Serum electrolytes were deranged notably; hypokalemia, hyperuricemia and reduced bicarbonate. Computed tomographic scanner revealed bilateral symmetrical basal ganglia calcification, faint subcortical calcification in the frontal lobes and diffuse brain inflammatory changes. Chest radiograph done prior to the CT showed right sided pleural effusion.

Conclusion: Fahr’s disease is a neurodegenerative entity which is very rare in children. Radiologic imaging of the brain using cross sectional modalities remains a centrepiece in unravelling its diagnosis.