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Early descriptions of the Arnold-Chiari malformation

The contribution of John Cleland

Peter W. Carmel and William R. Markesbery

J ohn Cleland , F. R. S. (1835–1925), was a poet, teacher, Darwinist, surgeon, and anatomist. A contemporary described him as “the Hercules of British Anatomy, both in intellect and in physique.” 13 During his 53-year academic career he published papers on morphology, teratology, embryology, and surgery. Each is characterized by meticulous observation and logical thought. Among these writings is probably the first description of what is now termed the “Arnold-Chiarimalformation. Cleland's paper, “Contribution to the Study of Spina Bifida, Encephalocele

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William O. Bell, Edward B. Charney, Derek A. Bruce, Leslie N. Sutton and Luis Schut

A lthough the association between lower cranial nerve palsies and myelomeningoceles has been recognized for some time, 4, 9 treatment of the symptomatic Arnold-Chiari malformation in infants remains controversial. The debate centers on the underlying mechanism for the brain-stem dysfunction. 6, 8, 10–12 Although older children, adolescents, and young adults respond consistently to surgical decompression of their Arnold-Chiari malformation, 5 infants frequently have persistent postoperative symptoms. 5, 10, 13 A number of etiological mechanisms have been

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Ruben J. Saez, Burton M. Onofrio and Takehiko Yanagihara

associated with the Arnold-Chiari malformation. Clinical Material Only patients in whom the diagnosis was confirmed surgically were included. Patients with complicating associated neurological problems, such as infants with meningomyeloceles or hydrocephalus or both, were excluded from the study. If results of surgical exploration were equivocal, the patient was excluded. The 60 patients (22 males and 38 females) studied had an age range of 13 to 68 years, with a mean age of 38 ( Table 1 ). The average duration of symptoms before admission was 4.5 years, with a range

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Arnold-Chiari malformation

Review of 71 cases

Kamal S. Paul, Richard H. Lye, F. Alexander Strang and John Dutton

T here are several types of Arnold-Chiari malformation (ACM). Type I occurs mainly in adults; its occurrence is rare, and patients present with a variety of symptoms and signs suggesting a cerebellar or cervical cord lesion. The anomaly in Type I ACM consists of caudal displacement of the cerebellum through the foramen magnum. The medulla may also be displaced caudally, but this finding is variable and may be obscured by the more obvious cerebellar herniation. 27 The lower cranial nerves course upward. There may be fibrosis of the pia arachnoid around the

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Albert L. Rhoton Jr.

H ydromyelia , a condition in which the central canal of the spinal cord communicates with the fourth ventricle and is distended by cerebrospinal fluid (CSF), often accompanies Arnold-Chiari malformation in the adult. The first manifestation is often a loss of pain sensation in the cervical dermatomes followed by atrophy and weakness caused by extension of the cavity into the anterior horns. The painless, slowly progressive cord deficits suggest a diagnosis of syringomyelia that has traditionally been regarded as an untreatable degenerative process. 7 The

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Adelola Adeloye

O ne of the anatomical abnormalities described in cases of Arnold-Chiari malformation is a beaking deformity of the tectal plate. Cleland referred to it in 1883, 4 but Chiari concentrated on various hindbrain defects, and failed to mention it in his detailed description of the different types of the malformation that now bears his name. 2, 3 In recent years, this curious tectal defect has been mentioned by others, the most notable being Peach, 11, 12 who reported its occurrence in 75% of cases of Arnold-Chiari malformation. This paper describes some of

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Neurosurgical Forum: Letters to the Editor To The Editor Miguel A. Faria , Jr. , M.D. Macon, Georgia 316 317 Regarding the article by Bell, et al. (Bell WO, Charney EB, Bruce DA, et al: Symptomatic Arnold-Chiari malformation: review of experience with 22 cases. J Neurosurg 66: 812–816, June, 1987), I would like to make the following observations. With respect to the infants who underwent early treatment, the authors found a 50% rate of immediate mortality or subsequent death because of progressive

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Everett F. Hurteau

It has repeatedly been mentioned that Arnold-Chiari malformation without abnormality of the cervical spine is unusual, 1, 2 and the difficulties in diagnosing the lesion under such circumstances have been indicated. The following case is thus presented for its statistical value and may contribute something of diagnostic and therapeutic value as well. CASE REPORT A 23-year-old white female was admitted to the hospital on Feb. 22, 1948 complaining of headache which began about 1 year previously. This symptom had been progressive and accompanied by vomiting

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Neurosurgical Forum To The Editor Dudley H. Davis , M.D. Edward R. Laws , Jr. , M.D. Mayo Clinic Rochester, Minnesota 498 499 The recent article by Bell, et al. (Bell WO, Charney EB, Bruce DA, et al: Symptomatic Arnold-Chiari malformation: review of experience with 22 cases. J Neurosurg 66: 812–816, June, 1987), as well as a prior report 2 emphasize the poor prognosis for patients with myelomeningocele and an Arnold-Chiari malformation who present with symptoms at an early age. One of

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Shigekuni Tachibana, Hideo Iida and Kenzoh Yada

S ince Gardner 7 first proposed his hydrodynamic theory as a cause of syringomyelia associated with Arnold-Chiari malformation, the dynamics of cerebrospinal fluid (CSF) circulation have attracted considerable attention. However, controversy continues as to the initiating cause of syringomyelia, whether it be ventricular pulse waves 7 or transient pressure dissociation above and below the foramen magnum. 13 Williams 14 has also proposed a “sloshing” mechanism for progression of a syrinx. This confusing situation is partly due to the fact that, in patients