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Neurology (Expert Forum)
Farr's Disease
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Farr's Disease
by L.B., Feb 08, 2000 12:00AM
Can you explain Farr's Disease and/or offer a good web site where
I can find information? Thanks.
I can find information? Thanks.
Doctor's Answer
by CCF Neuro[P] MD, RPS, Feb 08, 2000 12:00AM
Dear LB:
Fahr disease is a encephalopathy and progressive calcification of the basal ganglia. It may be familial or sporatic. When familial it may be automsomal recessive or dominant. Those with dominatnt inheritance are usually associated with hypoparathyroidism or pseudohypoparathyroidism. The calcification is thought to be from a defective iron transport. There is a child form and adult form. The child form usually ard short stature, senile appearance, and retinitis pigmentosa. Mental deterioration and choreothetosis are constant features. Ataxia and dysarthria, spasticity and seizures are variable. Currently, there is no treatment.
I hope this helps.
Sincerely,
CCF Neuro MD
Fahr disease is a encephalopathy and progressive calcification of the basal ganglia. It may be familial or sporatic. When familial it may be automsomal recessive or dominant. Those with dominatnt inheritance are usually associated with hypoparathyroidism or pseudohypoparathyroidism. The calcification is thought to be from a defective iron transport. There is a child form and adult form. The child form usually ard short stature, senile appearance, and retinitis pigmentosa. Mental deterioration and choreothetosis are constant features. Ataxia and dysarthria, spasticity and seizures are variable. Currently, there is no treatment.
I hope this helps.
Sincerely,
CCF Neuro MD
