Clinical Analysis Of Fahr Disease

Objective: To analyze the nosogenesis, clinical characteristics, diagnosis and treatment of Fahr diseaseMethods: The clinical data of 7 cases with Fahr disease were analyzed, including the pathogeny, clinical manifestations, family history, imaging characteristic, laboratory investigation, diagnosis, differential diagnosis, treatment methods and outcome.Results: Fahr disease is a rare and slowly progressive disorder manifested by amentia, progressive mental retardation, dementia, dysphonia, severe eccyliosis, epilepsy, ectrapyramidal disorders ( rigidity, tremor, mask face, dysmyotonia, chorea, torsion spasm, athetosis ), pyramid sign ( weakness, hyermyotonia, tendon hyperreflexia ), ataxia, anarthria. CT scan shows encephalic general calcifications and CT scan plays an important role in the right diagnosis. All 7 Fahr disease cases had bilateral and symmetric calcifications of the basal ganglia, 4 of 7 had calcifications of the cerebellum, 1 of 7 had calcifications of the thalamus, 3 of 7 had calcifications of the cortex, 3 of 7 had calcifications of the brainstem,. All 7 Fahr disease cases had normal level of serum calcium, phosphorus, magnesium, FT3, FT4, thyrotropin, parathormone and of serum parastic examinination. All 7 Fahr disease cases had misdiagnosis. There is no special treatment expect for symptomatic treatment.Conclusion:The bilateral and symmetric calcifications of the basal ganglia, including the lentiform nucleus, caudate nucleus and thalami are characteristic of Fahr disease on CT scan while the normal level of serum calcium, phosphorus, magnesium, thyrotropin, parathormone are the markers for Fahr disease to differentiate from other diseases.