historical neurology
NEUROLOGY 42;1992:1820-1822
Midbrain syndromes of Benedikt, Claude, and Nothnagel: Setting the record straight
-
G.T. Liu, MD; C.W. Crenner, AM; E.L. Logigian, MD; M.E. Charness, MD; and M.A. Samuels, MD Current neuro-ophthalmology textbooks vary considerably in their definitions of the eponymic oculomotor fascicular syndromes-Benedikt’s, Claude’s, and N~thnagel’sl-~ (table, first three columns). By contrast, Weber’s syndrome is universally accepted as ipsilateral oculomotor palsy and contralateral hemiparesis due to a peduncular 1esi0n.l.~ Because eponyms have both historical a n d humanistic value, unified descriptions for these syndromes are necessary to preserve their integrity and usefulness. Therefore, we studied Benedikt’s, Claude’s, and Nothnagel’s original descriptions in an attempt to clarify the syndromes attributed to them.
Benedikt’s syndrome. Moritz Benedikt (18351920), a Viennese physician, attempted to correlate tremors, such as those seen in Parkinson’s disease o r multiple sclerosis, with midbrain lesions. I n 1889, the French Bulletin Medical published a summary of a lecture in which Benedikt reported his finding^.^,^ Benedikt described three patients, each manifesting a n oculomotor palsy a n d contralateral hemiparesis, tremor, and involuntary movements. The first patient, age 4, developed a right oculomotor paralysis, left facial a n d leg weakness, unsteady gait, left hand jerks resembling a tremor, and continuous involuntary movements of the left leg. Autopsy revealed a tuberculoma “the size of a pigeon’s egg” within the right cerebral peduncle in the mesencephalon, obliterating the third nerve. The other two patients had similar clinical presentations but no postmortem examinations. Supported by the clinicopathologic correlation in the first case, Benedikt localized the tremors to the cerebral peduncle. Charcot7 was the first t o publicize Benedikt’s symptom complex by highlighting its importance as one of the “crossed paralyses.” In 1893, a t the Salpetrikre, Charcot discussed a 37-year-old man
who developed left-sided ptosis, mydriasis, and adduction weakness as well as involuntary, wandering movements of the right arm. The next day the patient suffered from intermittent right leg weakness, and a tremor at rest, similar to “that of paralysis agitans,” replaced the right arm movements. Honoring the original description, Charcot named the constellation of symptoms “Benedikt’s syndrome” and attributed it to a lesion within the inferior medial p a r t of the cerebral peduncle. Charcot emphasized that the tremor distinguished the syndrome from Weber’s. de la Tourette and Charcot,Bin 1900, added two more cases. The first, with a right hemiplegia, involuntary movements, and bilateral ophthalmoparesis, fit the definition only loosely. The second individual was a 20-year-old woman with childhood onset of a hemiplegia involving her left face, arm, and leg, tremor of the left arm with proximal and distal oscillations, and a right exotropia and mydriasis. The left patellar reflex was hyperactive, and t h e left plantar response was extensor. Thirty years later, Souques et a19 described this woman’s autopsy, which revealed an old “softening”affecting the ventral and lateral portions of the right red nucleus, medial substantia nigra, and third nerve root. The cerebral peduncle was spared, and there were no degenerative changes within the pyramids o r s p i n a l cord. T h e a u t h o r s reasoned t h a t Benedikt’s syndrome was due solely to damage to the red nucleus, and that this patient was hemidystonic due to the rubral lesion rather than hemiparetic from corticospinal involvement. It is incontrovertible, however, that Benedikt’s first patient had pathologically demonstrated . ~ account involvement of the cerebral p e d ~ n c l e To for the movement disorder, it is likely that the substantia nigra and red nucleus were also involved, given the size of the lesion. Therefore, Benedikt’s original combination of symptoms could be attributed to a mesencephalic lesion involving ipsi-
From the Division of Neurology (Drs. Liu, Logigian, Charness, and Samuels), Brigham and Women’s Hospital, Harvard-Longwood Neurology Program, and Harvard Medical School (Drs. Liu, Logigian, Charness, and Samuels, and C.W. Crenner), Boston, MA; and the Department of the History of Science (C.W. Crenner), Harvard University, Cambridge, MA. Presented in part a t the 44th annual meeting of the American Academy of Neurology, San Diego, CA, May 1992. Received January 6, 1992. Accepted for publication in final form February 17, 1992. Address correspondence to Dr. G.T. Liu, Bascom Palmer Eye Institute, P.O. Box 016880, Miami, FL 33101. Reprints not available. 1820 NEUROLOGY 42 September 1992
Table. Midbrain syndromes of Benedikt, Claude and Nothnagel as defined in current neurophthalmology textbooks and as originally described Syndrome
Miller (Walsh and HoytY
Glasel.2
Leigh and Zee3
Original description
Benedikt’s (1889)
Oculomotor palsy
Oculomotor palsy
Oculomotor palsy
Oculomotor palsy
Contralateral chorea, tremor, ballismus or athetosis
Contralateral ataxia and intention tremor
?
Contralateral involuntary movements or tremor
L S Third nF, RN
W. Third nF, RN
W.Third nF, RN, CP
L S Third nF, CP, SN?, RN?
Oculomotor palsy
Not mentioned
Oculomotor palsy
Oculomotor palsy
Contralateral ataxia and slow rubral tremor
Contralateral ataxia, asynergy, dysdiadochokinesia
Contralateral hemiparesis
Claude’s (1912)
Contralateral asynergia, ataxia, dysmetria, dysdiadochokinesia
+I- trochlear palsy, +/- sensory loss
LS: Third nF, RN, BC Nothnagel’s ( 1879)
Oculomotor palsy
Not mentioned
LS: Third nF, RN
LS: Third nF, RN, SCP, (+/-fourth n, ML, MLF)
Oculomotor palsy with vertical gaze palsy
Bilateral oculomotor palsies of varying degree and usually asymmetric +I- nystagmus
Ipsilateral ataxia
Gait ataxia
LS: Third nF, BC LS Third n F RN CP SN
Lesion site. Third nerve fascicle. Red nucleus. Cerebral peduncle. Substantia nigra.
BC SCP ML MLF
L S Third n F and ?
LS: superior and inferior colliculi
Brachium conjunctivum. Superior cerebellar peduncle. Medial lemniscus. Medial longitudinal fasciculus.
lateral oculomotor nerve, red nucleus, substantia nigra, and cerebral peduncle (figure).
Claude’s syndrome. In 1912, the French psychiatrist and neurologist Henri Claude (1869-1945) described a house painter who developed left arm and leg asynergy, and ataxia with extreme gait instability.l0 He tended t o fall t o the left, and there was left hand dysdiadochokinesia. Complete ptosis of the right eye accompanied virtual ophthalmoplegia, sparing only some adduction and abduction and involving the superior oblique. The right pupil was fixed and dilated. The left eye had only some limitation of adduction, but convergence of both eyes was absent. Strength and sensory function were normal. The pathologic examination1’ demonstrated a paramedian mesencephalic infarction on the right involving a portion of the crossing of the superior cerebellar peduncles and the medial half of the red nucleus. The medial longitudinal fasciculus was also involved, but the substantia nigra and cerebral peduncles were unaffected. The authors did not comment on the fourth nerve nucleus. More rostrally, the oculomotor fibers and the entire red nucleus were infarcted. The medial lemniscus was spared. Ten years later, Claude and Levy-Valensi12summarized the syndrome with slight modification: “associated difficulties of sensation more or less marked, of hemiataxia, of ocular paralysis (third and fourth nerve) or of conjugate movement of the
Figure. Anatomic localization of the syndromes of Benedikt, Claude, and Nothnagel, as originally described. Drawing depicts the mesencephalon at the level of the superior colliculus.
eyes.” His localization involving the red nucleus and superior cerebellar peduncle remained unchanged (figure).
Nothnagel’s syndrome. In his monograph, Localization of Diseases of the Brain13 (1879), Nothnagel (1841-1905), an Austrian physician, delineated the clinical features of tumors involving the quadrigeminal bodies. He restricted his discussion to neoplasms, which in his view were more September 1992 NEUROLOGY 42 1821
localized to this region than were cerebrovascular infarctions. Nothnagel presented a 2 1-year-old craftsman who developed severe gait ataxia, drowsiness, dilated pupils, and an ill-defined bilateral ophthalmoparesis. Autopsy revealed hydrocephalus and a sarcoma invading the pineal gland and all four colliculi and extending dorsally to the cerebellum. After an extensive review of the literature, Nothnagel drew several conclusions: (1) lesions of the superior colliculi may be associated with abnormal pupillary responses and visual loss without papilledema o r optic atrophy, and (2) tumors involving the inferior colliculi can lead to gait ataxia and bilateral oculomotor paralysis of varying degree in each eye. A decade later, Nothnagel refined his thoughts. l 4 He described a 15-year-old boy who first developed papilledema and a wide-based, unsteady gait, then bilateral superior and lateral rectus weakness, ptosis, and mydriasis 3 weeks later. Over the next 2 months, Nothnagel noted horizontal nystagmus, optic atrophy, blindness, deafness, and left superior oblique weakness. Postmortem examination demonstrated hydrocephalus and a “papillomatous epithelial growth” the size of “a small apple” compressing the quadrigeminal bodies, the aqueduct of Sylvius, and the cerebellum and its crura. Based on his and others’ experience, he argued that the ataxia resulted from involvement of the colliculi and not the cerebellum, or from hydrocephalus. He retracted his views that the colliculi per se were necessary for vision a n d eye movements. Compression of the oculomotor nerves or fascicles, h e reasoned, led to t h e ophthalmoparesis. Nothnagel proposed that the combination of gait ataxia and bilateral oculomotor paresis, varying in degree and usually asymmetric, localized a tumor to the quadrigeminal region. In a modern interpretation of Nothnagel’s symptom complex, a quadrigeminal neoplasm might first produce bilateral supranuclear ophthalmoparesis. The ataxia may result from extra-axial involvement of the cerebellum or cerebellar peduncles. An enlargmg tumor could invade or compress the oculomotor nuclei or fascicles, or the brachium conjunctivum. Nothnagel’s syndrome, therefore, may not necessarily include an oculomotor fascicular lesion and may be a variant of the dorsal midbrain syndrome (figure).
Discussion. The last column of the table summarizes the features and localization of Benedikt’s, Claude’s, and Nothnagel’s syndromes as the original authors described them. The figure illustrates the associated lesions.
1822 NEUROLOGY 42 September 1992
Merton,15 a sociologist, has identified the role that eponyms play in the reward systems of science. In addition to providing an elegant incentive to research, eponyms often help to organize scientific information and t o establish patterns for its orderly extension. Clinical localization practiced by 19th century neurologists is still valuable today. A clearly defined eponymic symptom complex with neuroanatomic correlation reminds us of the long, fruitful heritage of this method.6 In this review of the syndromes of Benedikt, Claude, and Nothnagel, we have recovered the rich detail of these elegant clinicoanatomic studies from the original publications.
Acknowledgments The authors wish to thank H. Richard Tyler, MD, for lending his copies of references 6 and 12, Virginie Chaves-Vischer, MD, for translating some of the French references, and Stefan Ries for translating the German reference.
References 1. Miller MR. Walsh and Hoyt’s clinical neuro-ophthalmology. 4th ed. Baltimore: Williams and Wilkins, 1985:661-663. 2. Glaser JS, Bachynski B. Infranuclear disorders of eye movement. In: Glaser JS, ed. Neuro-ophthalmology. 2nd ed. Philadelphia: J B Lippincott, 1990:374. 3. Leigh RJ, Zee DS. The neurology of eye movements. 2nd ed. Philadelphia: FA Davis, 1991:331. 4. Weber H. A contribution to the pathology of the crura cerebri. Medico Chirurg Trans 1863;46:121-139. 5. Benedikt M. Tremblement avec paralysie croisee du moteur oculaire commun. Bull Med Paris 1889;3:547-548. 6. Wolf JK. The classical brain stem syndromes. Translations of the original papers with notes on the evolution of clinical neuroanatomy. Springfield, IL: Charles C Thomas, 1971. 7. Charcot J-M. Le syndrome de Benedickt. MCdecine Moderne 1893;4:194-195. 8. de la Tourette G. Charcot J-M. Le svndrome de Benedikt. Semaine Medicale 1900;20:127-133. “ 9. Souques, Crouzon, Bertrand I. Revision du syndrome de Benedikt. A propos de l’autopsie d’un cas de ce syndrome. Forme tremo-choreo-athetoide et hypertonique du syndrome du novau rouee. Rev Neurol (Paris) 1930:2:377-417. 10. Claude H. Syndrome pedonculaire de la region du noyau rouge. Rev Neurol (Paris) 1912;1(23):311-313. 11. Claude H, Loyez. Ramollissement du noyau rouge. Rev Neurol (Paris) 1912;2(24):49-51. 12. Claude H, Levy-Valensi. Maladies des pedoncules cerebraux. In: Maladies du cervelet et de l’isthme de I’encephale (pedoncule, protuberance, bulbe). Paris: Bailliere, 1922:184-211. 13. N o t h n a g e l H. Corpora q u a d r i g e m i n a . I n : Topische Diagnostik der Gehirnkrankheiten. Berlin: A Hirschwald, 1879:204-220. 14. Nothnagel H. On the diagnosis of diseases of the corpora quadrigemina. Brain 1889;12:21-35. 15. Merton RK. Priorities in scientific discovery. Am Sociol Rev 1957;22:635-659.
Midbrain syndromes of Benedikt, Claude, and Nothnagel: Setting the record straight G. T. Liu, C. W. Crenner, E. L. Logigian, et al. Neurology 1992;42;1820 DOI 10.1212/WNL.42.9.1820 This information is current as of September 1, 1992 Updated Information & Services
including high resolution figures, can be found at: http://www.neurology.org/content/42/9/1820.citation.full.html
Citations
This article has been cited by 3 HighWire-hosted articles: http://www.neurology.org/content/42/9/1820.citation.full.html##ot herarticles
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Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions
Reprints
Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus
Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1992 by AAN Enterprises, Inc.. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
NEUROLOGY 42;1992:1820-1822
Midbrain syndromes of Benedikt, Claude, and Nothnagel: Setting the record straight
-
G.T. Liu, MD; C.W. Crenner, AM; E.L. Logigian, MD; M.E. Charness, MD; and M.A. Samuels, MD Current neuro-ophthalmology textbooks vary considerably in their definitions of the eponymic oculomotor fascicular syndromes-Benedikt’s, Claude’s, and N~thnagel’sl-~ (table, first three columns). By contrast, Weber’s syndrome is universally accepted as ipsilateral oculomotor palsy and contralateral hemiparesis due to a peduncular 1esi0n.l.~ Because eponyms have both historical a n d humanistic value, unified descriptions for these syndromes are necessary to preserve their integrity and usefulness. Therefore, we studied Benedikt’s, Claude’s, and Nothnagel’s original descriptions in an attempt to clarify the syndromes attributed to them.
Benedikt’s syndrome. Moritz Benedikt (18351920), a Viennese physician, attempted to correlate tremors, such as those seen in Parkinson’s disease o r multiple sclerosis, with midbrain lesions. I n 1889, the French Bulletin Medical published a summary of a lecture in which Benedikt reported his finding^.^,^ Benedikt described three patients, each manifesting a n oculomotor palsy a n d contralateral hemiparesis, tremor, and involuntary movements. The first patient, age 4, developed a right oculomotor paralysis, left facial a n d leg weakness, unsteady gait, left hand jerks resembling a tremor, and continuous involuntary movements of the left leg. Autopsy revealed a tuberculoma “the size of a pigeon’s egg” within the right cerebral peduncle in the mesencephalon, obliterating the third nerve. The other two patients had similar clinical presentations but no postmortem examinations. Supported by the clinicopathologic correlation in the first case, Benedikt localized the tremors to the cerebral peduncle. Charcot7 was the first t o publicize Benedikt’s symptom complex by highlighting its importance as one of the “crossed paralyses.” In 1893, a t the Salpetrikre, Charcot discussed a 37-year-old man
who developed left-sided ptosis, mydriasis, and adduction weakness as well as involuntary, wandering movements of the right arm. The next day the patient suffered from intermittent right leg weakness, and a tremor at rest, similar to “that of paralysis agitans,” replaced the right arm movements. Honoring the original description, Charcot named the constellation of symptoms “Benedikt’s syndrome” and attributed it to a lesion within the inferior medial p a r t of the cerebral peduncle. Charcot emphasized that the tremor distinguished the syndrome from Weber’s. de la Tourette and Charcot,Bin 1900, added two more cases. The first, with a right hemiplegia, involuntary movements, and bilateral ophthalmoparesis, fit the definition only loosely. The second individual was a 20-year-old woman with childhood onset of a hemiplegia involving her left face, arm, and leg, tremor of the left arm with proximal and distal oscillations, and a right exotropia and mydriasis. The left patellar reflex was hyperactive, and t h e left plantar response was extensor. Thirty years later, Souques et a19 described this woman’s autopsy, which revealed an old “softening”affecting the ventral and lateral portions of the right red nucleus, medial substantia nigra, and third nerve root. The cerebral peduncle was spared, and there were no degenerative changes within the pyramids o r s p i n a l cord. T h e a u t h o r s reasoned t h a t Benedikt’s syndrome was due solely to damage to the red nucleus, and that this patient was hemidystonic due to the rubral lesion rather than hemiparetic from corticospinal involvement. It is incontrovertible, however, that Benedikt’s first patient had pathologically demonstrated . ~ account involvement of the cerebral p e d ~ n c l e To for the movement disorder, it is likely that the substantia nigra and red nucleus were also involved, given the size of the lesion. Therefore, Benedikt’s original combination of symptoms could be attributed to a mesencephalic lesion involving ipsi-
From the Division of Neurology (Drs. Liu, Logigian, Charness, and Samuels), Brigham and Women’s Hospital, Harvard-Longwood Neurology Program, and Harvard Medical School (Drs. Liu, Logigian, Charness, and Samuels, and C.W. Crenner), Boston, MA; and the Department of the History of Science (C.W. Crenner), Harvard University, Cambridge, MA. Presented in part a t the 44th annual meeting of the American Academy of Neurology, San Diego, CA, May 1992. Received January 6, 1992. Accepted for publication in final form February 17, 1992. Address correspondence to Dr. G.T. Liu, Bascom Palmer Eye Institute, P.O. Box 016880, Miami, FL 33101. Reprints not available. 1820 NEUROLOGY 42 September 1992
Table. Midbrain syndromes of Benedikt, Claude and Nothnagel as defined in current neurophthalmology textbooks and as originally described Syndrome
Miller (Walsh and HoytY
Glasel.2
Leigh and Zee3
Original description
Benedikt’s (1889)
Oculomotor palsy
Oculomotor palsy
Oculomotor palsy
Oculomotor palsy
Contralateral chorea, tremor, ballismus or athetosis
Contralateral ataxia and intention tremor
?
Contralateral involuntary movements or tremor
L S Third nF, RN
W. Third nF, RN
W.Third nF, RN, CP
L S Third nF, CP, SN?, RN?
Oculomotor palsy
Not mentioned
Oculomotor palsy
Oculomotor palsy
Contralateral ataxia and slow rubral tremor
Contralateral ataxia, asynergy, dysdiadochokinesia
Contralateral hemiparesis
Claude’s (1912)
Contralateral asynergia, ataxia, dysmetria, dysdiadochokinesia
+I- trochlear palsy, +/- sensory loss
LS: Third nF, RN, BC Nothnagel’s ( 1879)
Oculomotor palsy
Not mentioned
LS: Third nF, RN
LS: Third nF, RN, SCP, (+/-fourth n, ML, MLF)
Oculomotor palsy with vertical gaze palsy
Bilateral oculomotor palsies of varying degree and usually asymmetric +I- nystagmus
Ipsilateral ataxia
Gait ataxia
LS: Third nF, BC LS Third n F RN CP SN
Lesion site. Third nerve fascicle. Red nucleus. Cerebral peduncle. Substantia nigra.
BC SCP ML MLF
L S Third n F and ?
LS: superior and inferior colliculi
Brachium conjunctivum. Superior cerebellar peduncle. Medial lemniscus. Medial longitudinal fasciculus.
lateral oculomotor nerve, red nucleus, substantia nigra, and cerebral peduncle (figure).
Claude’s syndrome. In 1912, the French psychiatrist and neurologist Henri Claude (1869-1945) described a house painter who developed left arm and leg asynergy, and ataxia with extreme gait instability.l0 He tended t o fall t o the left, and there was left hand dysdiadochokinesia. Complete ptosis of the right eye accompanied virtual ophthalmoplegia, sparing only some adduction and abduction and involving the superior oblique. The right pupil was fixed and dilated. The left eye had only some limitation of adduction, but convergence of both eyes was absent. Strength and sensory function were normal. The pathologic examination1’ demonstrated a paramedian mesencephalic infarction on the right involving a portion of the crossing of the superior cerebellar peduncles and the medial half of the red nucleus. The medial longitudinal fasciculus was also involved, but the substantia nigra and cerebral peduncles were unaffected. The authors did not comment on the fourth nerve nucleus. More rostrally, the oculomotor fibers and the entire red nucleus were infarcted. The medial lemniscus was spared. Ten years later, Claude and Levy-Valensi12summarized the syndrome with slight modification: “associated difficulties of sensation more or less marked, of hemiataxia, of ocular paralysis (third and fourth nerve) or of conjugate movement of the
Figure. Anatomic localization of the syndromes of Benedikt, Claude, and Nothnagel, as originally described. Drawing depicts the mesencephalon at the level of the superior colliculus.
eyes.” His localization involving the red nucleus and superior cerebellar peduncle remained unchanged (figure).
Nothnagel’s syndrome. In his monograph, Localization of Diseases of the Brain13 (1879), Nothnagel (1841-1905), an Austrian physician, delineated the clinical features of tumors involving the quadrigeminal bodies. He restricted his discussion to neoplasms, which in his view were more September 1992 NEUROLOGY 42 1821
localized to this region than were cerebrovascular infarctions. Nothnagel presented a 2 1-year-old craftsman who developed severe gait ataxia, drowsiness, dilated pupils, and an ill-defined bilateral ophthalmoparesis. Autopsy revealed hydrocephalus and a sarcoma invading the pineal gland and all four colliculi and extending dorsally to the cerebellum. After an extensive review of the literature, Nothnagel drew several conclusions: (1) lesions of the superior colliculi may be associated with abnormal pupillary responses and visual loss without papilledema o r optic atrophy, and (2) tumors involving the inferior colliculi can lead to gait ataxia and bilateral oculomotor paralysis of varying degree in each eye. A decade later, Nothnagel refined his thoughts. l 4 He described a 15-year-old boy who first developed papilledema and a wide-based, unsteady gait, then bilateral superior and lateral rectus weakness, ptosis, and mydriasis 3 weeks later. Over the next 2 months, Nothnagel noted horizontal nystagmus, optic atrophy, blindness, deafness, and left superior oblique weakness. Postmortem examination demonstrated hydrocephalus and a “papillomatous epithelial growth” the size of “a small apple” compressing the quadrigeminal bodies, the aqueduct of Sylvius, and the cerebellum and its crura. Based on his and others’ experience, he argued that the ataxia resulted from involvement of the colliculi and not the cerebellum, or from hydrocephalus. He retracted his views that the colliculi per se were necessary for vision a n d eye movements. Compression of the oculomotor nerves or fascicles, h e reasoned, led to t h e ophthalmoparesis. Nothnagel proposed that the combination of gait ataxia and bilateral oculomotor paresis, varying in degree and usually asymmetric, localized a tumor to the quadrigeminal region. In a modern interpretation of Nothnagel’s symptom complex, a quadrigeminal neoplasm might first produce bilateral supranuclear ophthalmoparesis. The ataxia may result from extra-axial involvement of the cerebellum or cerebellar peduncles. An enlargmg tumor could invade or compress the oculomotor nuclei or fascicles, or the brachium conjunctivum. Nothnagel’s syndrome, therefore, may not necessarily include an oculomotor fascicular lesion and may be a variant of the dorsal midbrain syndrome (figure).
Discussion. The last column of the table summarizes the features and localization of Benedikt’s, Claude’s, and Nothnagel’s syndromes as the original authors described them. The figure illustrates the associated lesions.
1822 NEUROLOGY 42 September 1992
Merton,15 a sociologist, has identified the role that eponyms play in the reward systems of science. In addition to providing an elegant incentive to research, eponyms often help to organize scientific information and t o establish patterns for its orderly extension. Clinical localization practiced by 19th century neurologists is still valuable today. A clearly defined eponymic symptom complex with neuroanatomic correlation reminds us of the long, fruitful heritage of this method.6 In this review of the syndromes of Benedikt, Claude, and Nothnagel, we have recovered the rich detail of these elegant clinicoanatomic studies from the original publications.
Acknowledgments The authors wish to thank H. Richard Tyler, MD, for lending his copies of references 6 and 12, Virginie Chaves-Vischer, MD, for translating some of the French references, and Stefan Ries for translating the German reference.
References 1. Miller MR. Walsh and Hoyt’s clinical neuro-ophthalmology. 4th ed. Baltimore: Williams and Wilkins, 1985:661-663. 2. Glaser JS, Bachynski B. Infranuclear disorders of eye movement. In: Glaser JS, ed. Neuro-ophthalmology. 2nd ed. Philadelphia: J B Lippincott, 1990:374. 3. Leigh RJ, Zee DS. The neurology of eye movements. 2nd ed. Philadelphia: FA Davis, 1991:331. 4. Weber H. A contribution to the pathology of the crura cerebri. Medico Chirurg Trans 1863;46:121-139. 5. Benedikt M. Tremblement avec paralysie croisee du moteur oculaire commun. Bull Med Paris 1889;3:547-548. 6. Wolf JK. The classical brain stem syndromes. Translations of the original papers with notes on the evolution of clinical neuroanatomy. Springfield, IL: Charles C Thomas, 1971. 7. Charcot J-M. Le syndrome de Benedickt. MCdecine Moderne 1893;4:194-195. 8. de la Tourette G. Charcot J-M. Le svndrome de Benedikt. Semaine Medicale 1900;20:127-133. “ 9. Souques, Crouzon, Bertrand I. Revision du syndrome de Benedikt. A propos de l’autopsie d’un cas de ce syndrome. Forme tremo-choreo-athetoide et hypertonique du syndrome du novau rouee. Rev Neurol (Paris) 1930:2:377-417. 10. Claude H. Syndrome pedonculaire de la region du noyau rouge. Rev Neurol (Paris) 1912;1(23):311-313. 11. Claude H, Loyez. Ramollissement du noyau rouge. Rev Neurol (Paris) 1912;2(24):49-51. 12. Claude H, Levy-Valensi. Maladies des pedoncules cerebraux. In: Maladies du cervelet et de l’isthme de I’encephale (pedoncule, protuberance, bulbe). Paris: Bailliere, 1922:184-211. 13. N o t h n a g e l H. Corpora q u a d r i g e m i n a . I n : Topische Diagnostik der Gehirnkrankheiten. Berlin: A Hirschwald, 1879:204-220. 14. Nothnagel H. On the diagnosis of diseases of the corpora quadrigemina. Brain 1889;12:21-35. 15. Merton RK. Priorities in scientific discovery. Am Sociol Rev 1957;22:635-659.
Midbrain syndromes of Benedikt, Claude, and Nothnagel: Setting the record straight G. T. Liu, C. W. Crenner, E. L. Logigian, et al. Neurology 1992;42;1820 DOI 10.1212/WNL.42.9.1820 This information is current as of September 1, 1992 Updated Information & Services
including high resolution figures, can be found at: http://www.neurology.org/content/42/9/1820.citation.full.html
Citations
This article has been cited by 3 HighWire-hosted articles: http://www.neurology.org/content/42/9/1820.citation.full.html##ot herarticles
Permissions & Licensing
Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions
Reprints
Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus
Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1992 by AAN Enterprises, Inc.. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
