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Show I ' " I . S I i p | ) I I K . i I I W l l l l . m i s \ VV l l k l l l v I ' h l l . u l l ' l | ) l l l . l Alfred Bielschovvsky's 1940 Otto Lipp Objectives: The author was stimulated to write tins article In a ll)° d \ isit to the l'ni\ ersii\ where 1' rol'essor AHVed biclschowsky was Cliairinan of Ophthalmology in Ihe 1930s. Dr. Hiclschowsky was one of ( he founders t)( neuro ophthalmologv. This re\ iew. with hiograplncal notes, is presented in his honor. Dr. Hiclschow skv and the author had similar disruptive experiences, ol historic interest, during the Hitler regime in Na/ i Germany. Review: Professor Hielseliowsk) \ legacv begins wnh his contributions to oculai phvsiolog). Lor instance. Ins aller- iniagc lest estahlishcs Ihe presence of retinal correspondence, important tor stereoscopic vision. Alfred Hiclschow skv taught how an ocular examination is critical for iicuro- ophthalmologic di agnosis. locali/ ation. prognostication, and treatment. Much of our knowledge is linked with his name. Hxaniplcs include " Hiclschow skv \ Phenomenon", explaining dissociated vertical movements, and " BicNchow skv \ Doll's Head Phenomenon" ( Doll's I'ves). describing proprioceptive reflexes important for localizing intracranial lesions. Dr. liielsehowskv eni-phasi/ ed manv pitfalls in the differential diagnosis of ocu\ o motor anomalies, lor example, he cautioned against mistaking the compensatory head position in congenital fourth cranial nerve paresis for neck muscle disease. Conclusion: Dr. Hielseliowsk) \ emphasis on the clinical examination remains critical despite lodav's advanced diagnostic equipment. His legacy is the application of physiology to patient care. Key Words: Ocular movements Intracranial lesions Ocular paresis Lesion locali/ ation \ euro- ophlhalniolog\ Alfred Hiclschowsky. This review was initiated by an oral presentation in 1996 at my medical school, the University of Breslau. ( iernianx. where Professor Alfred Bielsehow sk\ had been the cliairinan ol the Ophthalmology Department in the 1930s. Under Bielschovvsky's guidance at this institution. I decided to speciali/ e in Ophthalmology and wrote my first ophthalmologic publication in 1934 ( I). following the Potsdam Treaty in 1945, my Alma Mater became the Medical Academy ( Katedra I Klinika Ocu lislyki) ol W r o c l a w . Poland. In 1996. the Ophthalmology building remained miraculously intact despite the dc- Mamisuipl received June I1) 1)'.', accepted March I1)')* The author is a retired ophthalmologist. Address correspondence to: Olio I ippmann. . M. I).. v MM I'eirv Lane. \ iistin. lev. is " s~ sl. I S. A. gacy for Neuro- Ophthalmology aim, M. D. struction of SO' < of the cily during a siege by the Russian . Army in World War II. My memories go back to my senior year as a medical student 64 vears ago. when 1 was beaten bv Na/ i storm troopers in Bielschovvsky's lecture hall. Both Professor Biclschowsky and I were thrown out of the room because we were of " non- aryan descent."' This happened in late 1933. after Hitler became Germany's leader earlier that year. After completing medical school in 1934, I received a certification that 1 had " met all requirements to graduate with honors and to obtain licensure in Germany." but that " the license to practice medicine cannot be issued." No explanation was given. At about the same time. Prolessor Bielsehow skv \ academic appointment was terminated by the Na/ i Government. bortuiialely. both my teacher and 1 found a stile and productive haven in the United Stales. Dr. Biclschowsky is considered to be one ol the founders of the held of ncuro-ophihalmology. This paper is written in his honor. IHOGUAPHU Al. SKKTCH Allreil Biclschowsky was born in 1S7I in Namslau. a small low n in Silesia, at that lime a prov incc of ( icrniany ( 2i. Most of his family moved to Breslau. the capital of Silesia, and became respected citizens of that community. He received ophthalmology training in l. eip/ ig under and in association with the renowned physiologist Lvvald llcring and many ol ophthalmology's most prominent Luropean personages of the laic nineteenth anil early twentieth centuries. Larh in his career. Professor Bielsehow skv focused his medical studies on ocular neurophysiology and observations of eye motility. His clinical eye examination techniques provide teaching and guidance for neuro-ophthalmologic diagnosis anil treatment. After academic advancement in Leipzig, he became head of the Ophthalmology Departments in Marburg and later in Breslau. Germany. In 1918. German Lmperor \\ ilhclm II he-slowed on Allreil Biclschowsky the extraordinary honorary title of " Gehcinirat" ( privy counselor) ( sec big. h. Dr. Biclschowsky gave lectures in the United Stales in 1934/ 33; these presentations were published in book form by the American Medical Association ( 3). Adalbert . Ames invited him to join the Dartmouth LAC Institute and he spent d months there, but he fell obligated to 228 (). LIP PM ANN FIG. 1. Alfred Bielschowsky, 1871- 1940. ( Reproduced with permission from Duke- Elder S, Textbook of Ophthalmology, Vol IV, St. Louis: C. V. Mosby Co., 1949: 4030.) return lo Germany. By that time Nazi persecution had intensified and his chairmanship at Breslau was seized by the Vice- Chairman. Professor Bielschowsky then accepted Dr. Ames' offer and became Professor of Ophthalmology and Director of the Dartmouth live Institute in 1937. The Institute is known for studies on anisokonia and its relation to ophthalmologic practice. Settling in Hanover. New Hampshire, he became a trusted citizen of his adopted town. He pursued his career again in association with outstanding researchers of physiology and optics ( e. g.. Adalbert Ames. Paul Boeder, et til.). In this country, he sustained productivity in teaching, writing, and lecturing as a popular professor and authority. This included a ground- breaking series of discussions on motor anomalies of the eye. visual physiology, and neuro- ophthalmology. He died unexpectedly in 1940 in New York City while visiting his daughter following another academic lecture tour in Los Angeles. He had been suffering signs of a left frontal cerebral lesion, and he died of an intracranial hemorrhage that occurred shortly after undergoing pneumoencephalography. Dr. Bielschowsky is buried in New Hampshire, just yards away from the grave of Eliazar Wheelok, the founder of Dartmouth College ( see Fig. 2). A complete list of Alfred Bielschowsky's I 32 publications are listed in his obituaries ( 2). There are many medical terms linked lo his name ( see ' fable l). BIELSCHOWSKY'S LLCACY Lwald Hering and Alfred Bielschowsky formulated the concept of the two eyes acting as one double organ ./ Nnmi- OiilillHilniiil. Vol. IS. Nn. .(, / W. S' for visual perception and as a motor apparatus ( 3). The concept of corresponding retinal points of each eye explains localization of vistial stimuli and stereoscopic vision. The presence or absence of retinal correspondence is significant in the prognosis of treatment for ocular deviations. Bielschowsky devised an ingenious, yet simple apparatus for testing retinal correspondence. He utilized the after- images of each eye. exposing one to a horizontal linear light source and the other to a vertical one. If retinal correspondence is present, the after- images form a cross. The primary function of the ocular musculature is to bring lo the fovea retinal images of objects observed in the periphery. These movements are automatic, but are nol true reflexes. Motor innervation from the brain flows equally to each eye; one cannot control a single eye alone. An exception occurs when convergence and lal-eral movements are simultaneous. Similar phenomena of nonassociated movements occur in fusion of the eyes or in prism- induced duction movements. The limits of fusion movements are not rigid and can be increased by successive, repealed stimulation. Fusion movements, too. arc automatic, but are also not true reflexes. Double, alternating dissociated hyperphoria has puzzled physiologists, yet Professor Bielschowsky explained the process ( 3). It is nol caused by the true position of rest or by paresis; rather, il is a frequent complication of concomitant strabismus, as well as of paretic deviations. In an assessment, upon covering one eye, the covered eye is observed underneath the cover lo move slowly up and clown, yet never below the horizontal plane. II' one puts progressively darkening red glasses over the other eye, the covered eye also moves below the horizontal plane. The amount of movement below the horizontal plane is proportionate to the darkness of the red glass in front of the fixating eye. If the covering and red glass are changed to the other eye, the same vertical movements occur on the opposite side. Bielschowsky explained that these alternating, dissociated vertical movements are due to alternating, intermittent excitations of the vertical elevator muscles. These movements are now called " Bielschowskv's Phenomenon." To rule FIG 2. Professor Bielschowsky's tombstone. Dartmouth College Cemetery, Hanover, New Hampshire. IUEESC1IOWSKY. S LEGACY FOR NEURO- OEIEEIIAEMOEOC. Y 22' J TABLK 1. Examples of Iliclsi'liuusk\' s ICIHICY In ophthalmology in icnns linked In his name Bielschowsky's Phenomenon ( now also called Dissociated Verlical Divergence) Alternating or double illuminalion- dcpcndcnl anomaly of ocular motility with upward movement of one eye. often complicating concomitant slrahismus as well as ocular paresis Biclschowsky's Sign in Trochlear Palsy ( also called Hiclschow sky's Head- lilting Test) I'pwanl dCMalion of the paielic eye. increasing wilh homolateral rotation and lilting of the head Biclschowsky- lail/-(' ogan Syndronie ( synonymous with Inlernuclear Ophthalmoplegia) Describes lesions of ( he medial longitudinal fasciculus iniereonnecling die third, louiih and sixth ncr\ e nuclei (- 1 - 6 i Biclschowsk\ ' s Doll's I lead Phenomenon Hn ( lei man- Puppenkoplphaenoniciii or Doll's l i es Proprioceptive i cl lex eye nnwements clieited by head rotation Rolh- Biclschowsky Syniiromc ( synonymous with Pscudo- ophtlialmoplegia) A loss of horizontal and vertical vohuilarv ocular movements with preservation n{ Pursuit Movements and/ or with preset \ ation of rcllcv nioM'iiicnts under lab\ rinthine control i7, S) Hielscliowskv ( iesellschalt ( Society] An association whose membership is open lo scientists and health care providers concerned wilh slrabismology and ncuro- ophthalmology ( I leadi| uarlcrcd in Wiesbaden. Germany) out the occurrence of such alternating hyperphoria, he performed a simple lest. A red glass is put over the right eve: the red light is localized below the white fixation light. II ihe red glass is over the left eye, the ted light would also be localized below the white fixation light. He found an SO'/ i incidence of hetcrophoria in people who report eye strain: among more than 4()'/ i of those eases of hetcrophoria, there was a combination of hetcrophoria with dissocialed vertical movements. Consequently, surgical intervention for such changing hyperphoria would be strictly contraindicated. If one corrects the horizontal deviation of a concomitant strabismus, any dissocialed hyperphoria will disappear. Other pitfalls of muscle surgery were noted by Dr. Bielschowsky ( 3). I- or example, high horizontal exophoria could be corrected by surgery, but surgery cannot influence convergence insufficiency. In paresis of ocular muscles, there tire many factors that can influence diagnosis. They include: I) Paresis of the medial rectus muscle can be hidden because it is overcome by convergence impulses. 2) Very strong fusion movements can make recognition of horizontal deviation difficult, yet fusion can overcome only small amounts of vertical deviation. 3) Preexisting hetcrophoria can either increase or decrease the amount of deviation. 4) If a paresis has existed for a long time, the antagonist of the paretic muscle may develop a secondary contracture, making the deviation more equal in all directions of gaze and thus mimicking a concomitant strabismus. 5) Normal movements do not require maximal innervation of a muscle, but a paretic muscle, by maximal innervation, can reach normal rotational limits in uniocular testing. Therefore, one tests binocular measurements of movements before diplopia occurs. 6) In fresh paresis, the patient avoids the range of the paretic muscle by turning the head in the direction of the paretic muscle. By maintaining fixation on objects straight ahead, the eyes then move into the range of the normal antagonist. 7) Other head positions are sometimes chosen by the patient so as lo be in Ihe least inconvenient position; this not only provides relief for the paretic muscle, but also decreases contracture of the antagonist. Convergent deviation is slightly diminished by supravcrsion. whereas it is increased b\ inlraversion. Thus, in longstanding lateral rectus paresis, the patient's head is sometimes turned down with the chin pressed toward the chest. 8) The nonparelic eye is usually the fixating eye, yet, the paretic one may be so used because ol" preexisting amblyopia of the nonparelic eye. In 4th cranial nerve paralysis, the compensating head position is the most obvious diagnostic sign. In right superior oblique paresis, the head is turned to the left side and lilted toward the left shoulder: this position avoids the direction of gaze in which inlraversion and intorsion by Ihe right superior oblique muscle is most deficient. Bielschowsky demonstrated that congenital superior oblique paresis with such compensating head position is often misdiagnosed as neck muscle pathology, for the same reason, turning and tilting the head toward the paretic side increases the deviation of the paretic eye. This increase in the manifestations of the paresis is used as a diagnostic aid in trochlear paresis and is known as " Bielschow skv " s Sign." In 3rd cranial nerve paresis, rarely is there evidence of paresis of a single muscle, but, more often, there is a loss ol" conjugate bilateral movements. In localizing causative lesions. Bielschowsky drew attention to the medial rectus because of its dual action in adduction and in convergence. Me pointed out the different localizations in cases of loss ol'adduction with intact convergence versus those of loss of convergence with intact adduction. In combined elevator muscle paresis of one or both eyes, normal elevation in Bell's Phenomenon may be observed. This proves thai the elevator muscles are intact, leaving only the supranuclear pathway interrupted. Associated paralysis of gaze behaves quite differently, it presents equal displacement of the eves in till directions of gaze and is always caused by supranuclear lesions. There tire many special situations lhal enable us lo localize the intracranial pathology. I) Bielschowsky con-dueled special stttdies of the so- called ' T'uehrungsbewe-gungen" or " following movements," now called " smooth pursuit movements." for a patient who cannot, on simple command, look to the side, the eyes tire able to follow an object when it is very slowly moved lo the side. Dr. Bielschowsky explains these following movements as resembling fusion movements. 2) If paiients cannot move their eves on command, sudden jerky head ./ . V, in,' Oiililhiilm. il. Y,, l. IS. x,, I. IWS 230 (). U PPM ANN movement can induce short version. This can be explained as being caused by the vestibular innervation and is a line reflex. 3) In the conscious patient, vestibular stimulation by cold water car irrigation causes nystagmus with the fast phase to the opposite side. Absence of nystagmus proves that the causative lesion is either nuclear or more peripheral. 4) Bell's Phenomenon evidences supranuclear lesions. If absent, while vestibular reflexes are intact, lesions are located in the central brain stem pathways between the 3rd and 7th cranial nerve nuclei. 5) Bielschowsky described and named special proprioceptive reflexes the " Puppenkopf Phaenomen" or " Doll's Head Phenomenon," commonly called Doll's Eyes. In such a case, the patient having conjugate elevator paralysis cannot supravert the eyes, but a passive jerky head movement downward produces a quick su-praversion. The eyes then slowly return to their original position. Similar movements occur in paralysis of infra-version or laleroversion. The Doll's Eyes Phenomenon pi'oves a supranuclear causative lesion located more centrally than the vestibular reflex pathway. This test is very useful in neurologic evaluations of comatose patients. 6) In pseudo- ophlhalmoplegia. patients cannot move the eyes on command; but if a big object is brought into the peripheral field of vision and attracts attention, they can automatically move the eyes. If vestibular reflexes are intact, the lesion is located more centrally. In convergence insufficiency, the angle of deviation is constant in all directions of gaze and diplopia for near vision occurs. Convergence is a type of fusion movement, but convergence insufficiency also can be voluntary by suppressing convergence and usually is due to a functional, not organic etiology. Surgical correction is not advisable. Bielschowsky suspected the existence of a subcortical convergence center. In divergence paralysis, similar diagnostic criteria apply. Bielschowsky stressed that there must be a center of divergence located near the 6th cranial nerve nucleus. Shortly after his death, a paper on divergence paralysis described a patient with an intracranial tumor; an autopsy documented its location as predicted ( 8). This rare finding supports the location of a divergence center as suggested by Bielschowsky. In surgery of paretic deviations, Dr. Bielschowsky stressed that the first step should always be to assist the efficiency of the paretic muscle. For example, right ab-ducens nerve paralysis calls for advancement of the right lateral rectus. Surgery in paresis often requires multiple operations and is more difficult than in concomitant strabismus. Results are less predictable than for surgery of concomitant deviations. CONCLUSION An astute clinician, knowledgeable in ocular anatomy and physiology, can diagnose, localize, and treat many neurologic lesions. In this age of technologic advances in diagnostic equipment, the clinical examination remains an important diagnostic technique for neuro- ophthalmol-ogists. Research was Bielschowsky's original foundation and clinical practice became his expertise. He is famous for combining physiology and medical care. Professor Alfred Bielschowsky's legacy was enhanced by his excellence in teaching. REFERENCES 1. Lippmann (). Die Lntartungszeichen unci die Ucbcrwcrtigkeit cincr Koerpcrhaell'le in Hirer Bedeulung Inertias Schielen. ( Dominance. . . . its significance for slrabismns). MM /•' Ahk 1934; 92: 370- 384. 2. Lancaster WB, Ames A. Obituaries- Alfred Bielschowsky, 1871- 1940. Arch Ophthalmol 1940: 34: 1 354- 65. 3. Bielschowsky A. Lectures on Motor Anomalies of the lives. Chicago: American Medical Association. 1944. 4. Bielschowsky A. Die Innervation der niusculi recti inlemi als Seitcnwaender. Her '/. tisaiiiiuenkimfl Dtsch Ophthalmol ( les 1902; 30: 164- 171. 5. Lutz A. Leber cinscilige Ophthalmoplegia internuclearis anterior. Craefes Arch Ophthalmol 1924; I I 5: 692- 7 1 7. 6. Cogan DO. Kubik OS. Smith L. Unilateral internuclear ophthalmoplegia: report of eight clinical cases with one postmortem study. Arch Ophthalmol 1950: 44: 783- 96. 7. Roth W. Demonstration von Kranken mit Ophthalmoplegic. Neurol Ceitlralhl 190l; 2(): 921. 8. Bielschowsky A. Das klinische Bild der assoziierten Blicklaeh-mung uml seine Bcdeutung fuer die topische Diagnostik. Mneiicheiier Med Woehenschr 1903; 50: 1660- 70. 9. Lippmann O. Paralysis of divergence due to cerebellar tumor. Arch Ophthalmol 1944; 31: 299- 301." ./ Neuw- Ophthiilmol. Vol. I, S. No. I IWX |
