*30-year-old female.
What is the most likely diagnosis?
Answer
Answer:
Fahr syndrome
Case Discussion:
CT images revealed symmetric involvement of caudate, lentiform nucleus, thalamus, and dentate nuclei with bilateral subcortical white matter calcifications.
Fahr syndrome is usually initially asymptomatic in most patients. However, various neurological symptoms may develop later in life, such as psychosis, dementia & movement disorders.
Fahr syndrome, also known as bilateral striatopallidodentate calcinosis, is characterised by abnormal vascular calcium deposition, particularly in the basal ganglia, cerebellar dentate nuclei, and white matter, with subsequent atrophy. The differential diagnosis of basal ganglia calcifications is wide.
References:
1. LazăR M, Ion DA, Streinu-Cercel A et-al. Fahr’s syndrome: diagnosis issues in patients with unknown family history of disease. Rom J Morphol Embryol. 2009;50 (3): 425-8.
2. Perugula ML, Lippmann S. Fahr’s Disease or Fahr’s Syndrome?. Innovations in clinical neuroscience. 13 (7-8): 45-6.