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Arnold-Chiari malformation was diagnosed in five patients with vertigo. Four were of anatomical type I. A case report is given of one patient who presented features of a unilateral cerebellopontine angle lesion. Downward beating vertical nystagmus was an important finding in three of the five patients. Other clinical and radiological features of the condition are discussed.
The position of the pons, determined angiographically, can be related to the clivus and sella. In adult patients with the Chiari malformation, a caudal dislocation of the pons is frequently associated with the well known anomalously low position of the cerebellar tonsils. This finding may aid in the angiographic diagnosis of the Chiari malformation.
We have compared the efficacy of computed tomography (CT) and air ventriculography (VGM) in the diagnostic evaluation of progressive nontumoral infant hydrocephalus where both examinations have been done without significant interventing time or treatment in infants under 12 months of age. CT alone was judged to be adequate for diagnosis and treatment 21 of 30 cases reviewed, provided that cerebrospinal fluid studies were available to complete diagnosis where necessary, and provided that the question of ventriculocisternal communication was not a factor in treatment selection. The ability to visualize cerebran aqueduct and 4th ventricle on CT was not always a reliable indicator of ventriculocisternal communication. CT was most adequate as the sole radiographic study in cases of myelodysplasia with Arnold-Chiari malformation, and in premature infants with intraventricular hemorrhage.
A case of spontaneous disappearance of an intrathoracic meningocele following insertion of a ventriculo-venous shunt is reported. The shunt was performed for treatment of hydrocephalus secondary to Arnold-Chiari malformation. The intrathoracic meningocele reappeared when the shunt obstructed and decreased in size when the obstruction was relieved.
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The morphological findings in 12 cases of Arnold-Chiari and 3 cases of Dandy-Walker malformations are described and compared to those in 2 cases of congenital hydrocephalus of unknown origin, 1 case of isolated meningo-myelocele and 2 cases of normal newborn brains. Lesions common to both types of malformation indicate a same time-related embryogenetic defect of the roofplate of the rhombencephalon, that does not allow the inferior vermis and the choroid plexus to turn inward into the fourth ventricle.
Computerized Metrizamide ventriculomyelography consists of CT exploration of the head and neck performed one hour after introduction of Metrizamide into the lateral ventricle via a frontal burr hole. This procedure permits visualisation of all soft-tissue anomalies present in Chiari malformation with hydromyelia as seen in two cases.
Ventriculography with meglumine iocarmate (Dimer X) or Metrizamide (Amipaque was carried out in 15 infants with myelomeningocele and progressive hydrocephalus. In all cases, an Anold-Chiari-Malformation type II was diagnosed with compression of the structures of the posterior cranial cavity and their displacement within or below the level of the foramen magnum. The aqueduct was always patent but laterally compressed. In the region of the third ventricle there was often encountered an enlargement of the massa intermedia with indentation of the lamina terminalis and enlargement of the recessus suprapinealis. Aspects of pathogenesis of the malformation and possible causes of hydrocephalus originating from these findings are discussed. Methods and advantages of positive ventriculography using water-soluble media are presented.
In the years 1973--1975 20 patients with syringomyelia were treated surgically. The surgical interventions were preceded by careful radiological examinations which was often indispensable for confirmation of diagnosis, establishing of indications to operation and choice of surgical method. On plain radiograms in 16 cases cervicothoracic scoliosis was found, in 11 cases the vertebral canal was dilated in its cervical part, in 6 cases atlanto-occipital malformations were disclosed. The basic diagnostic examination was ascending myelography which was performed in 17 cases. In 3 cases the contrast medium failed to pass to the atlanto-occipital junction because of marked dilatation of the spinal cord in the cervical part. In 9 out of the remaining 14 cases radiological findings were compatible with the diagnosis of Arnold-Chiari syndrome which was confirmed during the operation. In 1 case positive contrast central pneumoencephalography was performed demonstrating a communication between the 4 th ventricle and the cavity in the spinal cord. Early detailed neuroradiological diagnosis is indispensable in syringomyelia for early surgical treatment.
Chiari I malformation (CM1), the most common structural hindbrain disorder in humans, is traditionally characterized by the downward displacement of the cerebellar tonsils through the foramen magnum. However, this definition does not reflect the variability in clinical presentation, natural history and treatment response of this disorder. Some individuals with minimal tonsillar descent have severe neurological symptoms and syringomyelia, whereas others with extensive descent remain asymptomatic. Emerging evidence from neuroimaging, developmental biology and human genetics indicates that CM1 is not a single anatomical entity but a spectrum of disorders resulting from disruptions in coordinated growth and homeostasis across the cerebellum, posterior fossa, craniocervical junction, cerebrospinal fluid and neurovascular systems. CM1 may be best understood as a disorder of disrupted developmental scaling, in which the tightly regulated relationships between cerebellar growth and cranial accommodation are altered within a dynamic neurovascular and cerebrospinal fluid environment. In this context, tonsillar herniation is a geometric consequence rather than the primary disease process. This Primer synthesizes current knowledge on the epidemiology, mechanisms, diagnosis and management of CM1 across the lifespan. We highlight advances in neuroimaging, genomics and phenomics that support a shift from anatomy-based definitions towards an integrated genomic-phenomic classification.
Forty-three infants and children underwent conventional or CT ventriculography after instillation of metrizamide into the ventricular system. The procedure had few complications. Anatomic detail of intraventricular obstruction was good. With CT additional physiologic information can be obtained.
42 ventriculographies (PV) were carried out in infants between 10 days and 10 months of age using positive contrast medium. Indications, technique of PV and complications are discussed; PV is compared with CT and it is concluded that PV is a simple method of clarifying the causes of a macrocephalus in infancy without great technical effort and that the roles of CT and PV are valuable diagnostic modalities which are complementary.
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A study of the C.S.F. circulation by intrathecal injection of radioactive tracers has become a common technic of neurological exploration. Among the molecules used at present, Indium111 DTPA has numerous advantages for this type of study. It is a chelating agent, the renal clearance of which is rapid and which has no toxicity at the dose injected. Indium111 is a product of the cyclotron and the physical period (2.8 days) is compatible with the duration of the examination. Finally the dose of radioactivity delivered by this isotope is less than that Iodine131, Technetium99m and Ytterbium169. In normal subjects after injection by the lumbar route, the average biological period measured by external counting lies between 20 and 28 hours. A study of the circulation of the C.S.F. is particularly useful in patients suspected of hydrocephalus. It permits finer diagnosis and shows the indications and type of by pass operation that may be neccssary. A marked increase in the biological period of Indium111-DTPA seems to be a good indication for such an operation. Our experience of 300 patients has shown the interest of the use of Indium111-DTPA which now seems to be the best radio-isotope for the study of the subarachnoid space.
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