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Marinesco‐Sjögren syndrome in a Bedouin family
Author(s) -
Farah S.,
Sabry M. A.,
Khuraibet A. J.,
Anim J. T.,
Quasrawi B.,
AlKhatam S.,
AlBusairi W.,
Hussein J. M.,
Khan R. A.,
AlAwadi S. A.
Publication year - 1997
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.1600-0404.1997.tb00303.x
Subject(s) - axonal degeneration , biopsy , pathology , medicine , sural nerve , degeneration (medical)
Marinesco‐Sjögren syndrome is rarely reported in the Middle East. This is the 2nd report of Marinesco‐Sjögren syndrome in an Arab family. The clinical features of 2 affected brothers are described. Electrophysiological studies of the 2 patients showed primarily myopathic changes, whereas sural nerve biopsy revealed segmental demyelination and axonal degeneration. The role of tissue biopsy and the relationship to different electrophysiological studies are discussed. Both patients were noticed to have abnormally short lateral 3 metatarsals, a feature not present in other healthy members of the family. We suggest that this feature should be considered part of the syndrome profile.