. The practice of pediatrics. roy the tips of the nerve processes, so to speak,without involving the nerve-cell body itself. The next part attackedis the anterior cornual cell (Dana). Under these conditions the pro-gressive amyotrophy assumes a spastic form and is called amyotrophiclateral sclerosis. 508 THE PRACTICE OF PEDIATRICS Until the complex pathology which has just been briefly traced isfurther elucidated, the following neurologic conditions may be classifiedunder the general heading, progressive spinal muscular atrophy: 1. Progressive amyotrophy of the hand type {or Duchenne-Aran type
. The practice of pediatrics. roy the tips of the nerve processes, so to speak,without involving the nerve-cell body itself. The next part attackedis the anterior cornual cell (Dana). Under these conditions the pro-gressive amyotrophy assumes a spastic form and is called amyotrophiclateral sclerosis. 508 THE PRACTICE OF PEDIATRICS Until the complex pathology which has just been briefly traced isfurther elucidated, the following neurologic conditions may be classifiedunder the general heading, progressive spinal muscular atrophy: 1. Progressive amyotrophy of the hand type {or Duchenne-Aran type). 2. Progressive bulbar paralysis. 3. Progressive muscular atrophy of the leg type (peroneal type or Char-cot-Marie-Tooth type). 4. Progressive spinal muscular atrophy of the spastic type (or amyo-trophic lateral sclerosis). Symptomatology.—1. Progressive amyotrophy of the hand typetypically begins as a wasting of the muscles of one thumb. The adduc-tor pollicis, deep thenar, hypothenar, and the interossei muscles are pro-. Fig. 70.—Pseudomuscular hypertrophy. (Early case.) gressively involved; and as the paralysis extends, it may affect theflexors and extensors of the forearm, and eventually the triceps and del-toid and other shoulder muscles. The claw-hand deformity is com-mon. After several months the paralysis may become bilateral, involv-ing the trunk and rarely the leg muscles, or it may even develop intoa bulbar palsy. The paralysis in the hand type of atrophy is usuallyatonic and flaccid, but may assume a spastic character, with exaggeratedreflexes, thus simulating amyotrophic lateral sclerosis. The varyingdegrees of atony and spasticity are many. In most cases fibrillarycontractions occur. Electric responses are diminished and partialor complete reactions of degeneration may be elicited. Complete re-actions of degeneration belong, as a rule, to cases of rapid course. Oc-casionally rheumatoid pains and local paresthesias occur, but sensorydisturbances are for the m
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Keywords: ., bookcentury1900, bookdecade1910, bookid39002, booksubjectchildren
