CASE REPORT   

Minerva Medicolegale 2001 September;121(3): 193-9

Copyright © 2001 EDIZIONI MINERVA MEDICA

language: Italian

Three cases of Fahr syndrome: clinical study and evaluation for disability

Lucrezi A., Fels A., Niola M., Buccelli C.

Three cases of Fahr syndrome have come to light in the field of civil invalidity which are interesting to report because of their relative rarity. The syndrome is caused by bi-lateral symmetrical, non arterio-sclerotic calcifications of the base nuclei, of the cerebellum, of the white encephalic matter, of the cortical, subcortical areas and of the thalamus. Its symptomatology is polymorphous and depends on the site and on the extent of the encephalic area involved, with variable appearance of mental disturbances, generalized or focal epilepsy, extra-pyramidal disturbances (coreo-atetosical or Parkinsons), cerebellum and sometimes pyramidal disturbances, dysarthria, alteration of eye movement. In the secondary forms the real symptoms of the basic illness are added. In order to obtain a correct diagnosis of the illness and an adequate estimate of its reverberations on invalidity a clinical-instrumental evaluation protocol is suggested; to be concentrated essentially on neuropsychiatric examination, cranium TC, EEG, mental tests, dosage of the serum concentration of parathormone and calcium (reduced in the case of hypoparathyroidism). The level of invalidity is estimated from the quality and grade of neuropsychical damage and, in the more frequent secondary forms, from the disabilities caused by the primary illness (which is almost always endocrinological). The above consideration also forms the basis for facing the delicate question of evaluating the requisites for admitting an accompaniment grant. Finally, the cases of interest for insurance purposes are characterized by a late appearance of the illness or by considerable deterioration in the course of time.