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Pallido‐pyramidal degeneration, supranuclear upgaze paresis and dementia: Kufor‐Rakeb syndrome
Author(s) -
AlDin A. S.,
Wriekat A.,
Mubaidin A.,
Dasouki M.,
Hiari M.
Publication year - 1994
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1994.tb02645.x
Subject(s) - parkinsonism , atrophy , dementia , medicine , degenerative disease , paresis , cerebral atrophy , pathology , extrapyramidal symptoms , central nervous system disease , pyramidal tracts , globus pallidus , disease , basal ganglia , anatomy , psychiatry , central nervous system , schizophrenia (object oriented programming) , antipsychotic
An unusual neurological syndrome in an Arab family with five affected siblings, is reported. Autosomal recessive inheritance is suggested by having multiple affected siblings born to phenotypically normal consanguineous parents. Similar to Davison's Pallido‐pyramidal syndrome, they presented with the clinical signs and symptoms of severe parkinsonism as well as evidence of cortico‐spinal tract disease. In addition, they had dementia and supranuclear upgaze paresis. MRI studies showed significant atrophy of the globus pallidus and the pyramids, as well as generalized brain atrophy in later stages. Therapy with levodopa resulted in significant improvement in the extrapyramidal dysfunction. We suggest that this probably represents a new syndrome which is closely related but not identical to the pallido‐pyramidal syndrome.