Biomedical subjects
Publications and source records attributed to H F Piper.
Morbus Kniest was described by Springer and Wiedemann as an early form of dysplasia spondylo-epi-metaphysaria. The syndrome is combined with vitreoretinal degeneration. It is an autosomal dominantly inherited disorder, like Morbus Wagner. We report on a case with dwarfism and cataracta complicata. The diagnosis must be differentiated to Morbus Morquio.
Case report on a 47-year-old man who got blind during the last 10 years because of dysgenesis mesodermalis iridis et corneae Rieger. The patient showed an oculopupillomotor syndrome consisting of direction-changing spontaneous nystagmus and simultaneous changes of pupil diameter and eyelid interspace. The specific modification of direction, form, frequency, and amplitude of nystagmus under central influences such as emotional excitement, change of gaze, eye closure, head tilt, and other vestibular stimuli was registered electronystagmographically and is described in this paper. The unique oculomotor disturbance is compared pathogenetically with periodic oculomotor phenomena such as periodic alternating nystagmus, other types of direction-changing nystagmus, and uninhibited oculomotoricity of the blind. Loss of cerebellar inhibition mechanisms in the central pathogenesis of this syndrome is discussed hypothetically.
Two pairs of albinotic brothers, a severely myopic albino and an aged albinotic woman patient were examined; visual acuity was 0.2. In the nystagmogram there were varied rhythmic wave-forms with 1) oscillations of high frequency; 2) slow arrhythmic movements; 3) deviation tendencies with a fast nystagmus component. In three cases the better eye was used in adduction (latent nystagmus), in another in abduction (alternating nystagmus), and in two cases the direction of the jerks changed in accordance with the intended range of vision. The head was turned to the side of the fast component by motoric control or moved quickly, synchronized with the nystagmus. Another two patients presented with an atypical form of head-tilting, looking upward at an extreme angle or closing the weaker eye. Lowering of the eyelids and compensatory movements of the head were caused by photophobia or myopia, i.e., by sensory control.
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Some people are able to abduct and adduct just one of their eyes from maximum convergence position. Such a distribution of different tonus on both sides has a binocular-symmetric innervation pattern carried out on two chronological orders and a monocularasymmetric innervation pattern carried out on one chronological order. The latter is trainable. The voluntary nystagmus ist comparable to the muscle tremor (on coldness). It is mediated by the parasympathic-innervated reflex for near focussing. The relief of the brain stem automatism is a 'stigma degenerationis' and not trainable because it is a basic rhythm. An optokinetic nystagmus could be performed without stimulus by conditioned reflexes.
On two female patients it was shown that intoxication by sedatives containing part bromecarbamide may result in various ocular diseases. The two patients complained of a sudden inexplicable deterioration of vision; semi-dilated slow-responding pupils were diagnosed. Only in one case could a sudden vertical nystagmus be observed. Many possible symptoms were shown. In most cases a discontinuation of drug together with therapy lead to a complete withdrawal of symptoms.
Septo-optical dysplasia and Optic nerve hypoplasia often are combined with pendular nystagmus in the horizontal, vertikal or rotatory direction. Our patient, 26 year old, showing discret neurological symptoms, added the nystagmus giratoire, perhaps similar to see-saw-nystagmus: Vision was about 0.2. Nystagmus and the whole state did not change within 6 years. The nystagmus was influenced by drugs. There was found also an aplasia of the fovea zentralis. Pneumencephalography revealed in the midline a dilatated single ventricle; the septum pellucidum was absent. Te X-rays of the atlantooccipital axis showed a foramen arcuale atlantis.
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In cases of paralytic ectropion the slackened skin must not be resected but has to be used for the lifting of the lower eyelid. This aim can be reached by an antidromic skin shifting with an ypsiliform incision or in less difficult cases by a hook-shaped skin flap, who becomes effective as a curb of the lower eyelid by the exchange of the flaps.
A study of visual habits consisting of a questionaire and an examination was carried out on 70 unilaterally aphakic patients. These patients wore correcting glasses and had better vision in the cataract operated eye. It was observed that the patients, aided by senile convergence weakness on one side and senile divergence weakness on the other, were able to develop a form of monocular vision without complications and in particular without double vision. These patients who wore glasses developed a type of "multiple choice" vision, which was dependent on the intelligence and the determination of the individual.
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