. A treatise on the nervous diseases of children, for physicians and students. ators are apt tobe wasted ; while the del-toids, supraspinal, and in-fraspinati are either normalfor a long time or hyper-trophied. There are nofibrillary contractions andno reaction of degenera-tion ; no sensory or visce-ral disturbances. (See Fig. 9i.) A few years ago, I wasinclined to consider Erbstype a great rarity in thiscountry; it is unquestion-ably the least frequent ofall the forms of progressive muscular atrophy which wehave occasion to see in clinics or private practice; but Ihave seen at least a dozen c
. A treatise on the nervous diseases of children, for physicians and students. ators are apt tobe wasted ; while the del-toids, supraspinal, and in-fraspinati are either normalfor a long time or hyper-trophied. There are nofibrillary contractions andno reaction of degenera-tion ; no sensory or visce-ral disturbances. (See Fig. 9i.) A few years ago, I wasinclined to consider Erbstype a great rarity in thiscountry; it is unquestion-ably the least frequent ofall the forms of progressive muscular atrophy which wehave occasion to see in clinics or private practice; but Ihave seen at least a dozen cases of this form within a periodof five years, from which the reader may gather the fre-quency or infrequency of the disease; but there is no doubtthat many cases exist which have not been reported, simplybecause they have not been properly recognized. The Facio-Scapulo-Humeral, or Landouzy-Deje-rine Type.—This type includes cases in which the atrophybegins early in life, and, as a rule, in the muscles of the face,giving rise to what the authors have termed the fades my~. Fig. 94.—Patient with Landouzy-DejerineType. Indication of bouche de tapir;patient cannot show upper teeth, norclose eyes. (See also Fig. 8.) PROGRESSIVE MUSCULAR ATROPHIES. 375 opathique / the lips are considerably thickened and consti-tute the bouche de tapir, or tapir mouth. Later on in thecourse of the disease the atrophy spreads to the shoulderand arm muscles. The supraspinati and infraspinati, thesubscapularis, and flexors of the hands and lingers remainnormal. Among these muscles that remain normal it mayat once be noted are several which are distinctly hypertro-phied in Erbs type. In the Landouzy-Dejerine type themuscles of deglutition, of mastication, the respiratory andlaryngeal muscles, as well as the ocular muscles, remain nor-mal. In exceptional cases the disease may begin in theshoulder or arm muscles, or even in the lower disease is distinctly hereditary. Fibrillary contrac-
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