title: [Rapidly progressing facio-scapulo-humero-femoro-peroneal muscular dystrophy with sensorineural deafness].
abstract: The paper concerns a family in which father and sibs (two brothers and a sister) suffered from rapid-progressing myodystrophy. Clinically, the disease was marked by a debut in the pubertal period (at 12 to 13 years), derangement of the muscles of the limb girdles, proximal parts of the legs and arms, and distal parts of the legs. The given case was also characterized by the impairment of the cervical and facial muscles as well as by associated neuromuscular lesions and neurosensory hypoacusis. The case described supports the clinical pleomorphism of the facial scapulohumeral form of Landouzy-Dejerine myopathy.
authors.forename: L O
authors.initials: LO
authors.lastname: Badalian
authors.forename: P A
authors.initials: PA
authors.lastname: Temin
authors.forename: K Iu
authors.initials: KIu
authors.lastname: Mukhin
authors.forename: M Iu
authors.initials: MIu
authors.lastname: Nikanorova
authors.forename: N V
authors.initials: NV
authors.lastname: Bulaeva
authors.forename: L A
authors.initials: LA
authors.lastname: Kret
authors.forename: V L
authors.initials: VL
authors.lastname: Lysov
mesh_terms: D000293:Adolescent; D000328:Adult; D001132:Arm; D002648:Child; D003638:Deafness / Q000150:complications* / Q000151:congenital / Q000235:genetics; D004576:Electromyography; D005152:Facial Muscles; D005260:Female; D006801:Humans; D007866:Leg; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D010375:Pedigree; D012540:Scapula; D013997:Time Factors
vernacular_title: Bystroprogressiruiushchaia litse-lopatochno-pleche-bedrenno-peroneal'naia myshechnaia distrofiia s neĭrosensornoĭ tugoukhost'iu.