Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression. 2000

Z Gil, and S Rao, and S Constantini
Department of Physiology, The Zlotowski Center for Neuroscience, Faculty of Health Science, Ben Gurion University, Beer-Sheva, Israel.

Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). OBJECTIVE Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. METHODS Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. CONCLUSIONS This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI.

UI MeSH Term Description Entries
D007405 Intervertebral Disc Displacement An INTERVERTEBRAL DISC in which the NUCLEUS PULPOSUS has protruded through surrounding ANNULUS FIBROSUS. This occurs most frequently in the lower lumbar region. Disc Herniation,Disc Protrusion,Disc, Herniated,Disk Herniation,Disk Protrusion,Disk, Herniated,Intervertebral Disc Herniation,Intervertebral Disc Protrusion,Intervertebral Disk Displacement,Intervertebral Disk Herniation,Intervertebral Disk Protrusion,Prolapsed Disk,Protruded Disc,Protruded Disk,Slipped Disk,Disk Prolapse,Herniated Disc,Herniated Disk,Prolapsed Disc,Slipped Disc,Disc Displacement, Intervertebral,Disc Herniations,Disc Protrusion, Intervertebral,Disc Protrusions,Disc Protrusions, Intervertebral,Disc, Prolapsed,Disc, Protruded,Disc, Slipped,Discs, Protruded,Disk Displacement, Intervertebral,Disk Herniations,Disk Prolapses,Disk Protrusion, Intervertebral,Disk Protrusions,Disk, Prolapsed,Disk, Protruded,Disk, Slipped,Herniated Discs,Herniated Disks,Herniation, Disc,Herniation, Disk,Herniation, Intervertebral Disc,Herniation, Intervertebral Disk,Intervertebral Disc Displacements,Intervertebral Disc Herniations,Intervertebral Disc Protrusions,Intervertebral Disk Displacements,Intervertebral Disk Herniations,Intervertebral Disk Protrusions,Prolapse, Disk,Prolapsed Discs,Prolapsed Disks,Prolapses, Disk,Protruded Discs,Protruded Disks,Protrusion, Disc,Protrusion, Disk,Protrusion, Intervertebral Disc,Protrusion, Intervertebral Disk,Protrusions, Intervertebral Disk,Slipped Discs,Slipped Disks
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003388 Cranial Fossa, Posterior The infratentorial compartment that contains the CEREBELLUM and BRAIN STEM. It is formed by the posterior third of the superior surface of the body of the sphenoid (SPHENOID BONE), by the occipital, the petrous, and mastoid portions of the TEMPORAL BONE, and the posterior inferior angle of the PARIETAL BONE. Clivus,Cranial Fossas, Posterior,Fossa, Posterior Cranial,Fossas, Posterior Cranial,Posterior Cranial Fossa,Posterior Cranial Fossas
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001139 Arnold-Chiari Malformation A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46) Arnold-Chiari Deformity,Arnold-Chiari Malformation, Type 1,Arnold-Chiari Malformation, Type 2,Arnold-Chiari Malformation, Type 3,Arnold-Chiari Malformation, Type 4,Arnold-Chiari Malformation, Type I,Arnold-Chiari Malformation, Type II,Arnold-Chiari Malformation, Type III,Arnold-Chiari Malformation, Type IV,Arnold-Chiari Syndrome,Chiari Malformation Type 2,Chiari Malformation Type I,Chiari Malformation Type II,Malformation, Arnold-Chiari,Type I Arnold-Chiari Malformation,Type II Arnold-Chiari Malformation,Type III Arnold-Chiari Malformation,Type IV Arnold-Chiari Malformation,Arnold Chiari Deformity,Arnold Chiari Malformation,Arnold Chiari Malformation, Type 1,Arnold Chiari Malformation, Type 2,Arnold Chiari Malformation, Type 3,Arnold Chiari Malformation, Type 4,Arnold Chiari Malformation, Type I,Arnold Chiari Malformation, Type II,Arnold Chiari Malformation, Type III,Arnold Chiari Malformation, Type IV,Arnold Chiari Syndrome,Deformity, Arnold-Chiari,Malformation, Arnold Chiari,Syndrome, Arnold-Chiari,Type I Arnold Chiari Malformation,Type II Arnold Chiari Malformation,Type III Arnold Chiari Malformation,Type IV Arnold Chiari Malformation
D013595 Syringomyelia Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269) Hydrosyringomyelia,Morvan Disease,Morvan's Disease,Myelosyringosis,Syringomyelus,Hydrosyringomyelias,Morvan Diseases,Morvan's Diseases,Morvans Disease,Myelosyringoses,Syringomyelias
D018908 Muscle Weakness A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251) Muscular Weakness,Muscle Weaknesses,Muscular Weaknesses,Weakness, Muscle,Weakness, Muscular,Weaknesses, Muscle,Weaknesses, Muscular
D019299 Decompression, Surgical A surgical operation for the relief of pressure in a body compartment or on a body part. (From Dorland, 28th ed) Surgical Decompression,Decompressions, Surgical,Surgical Decompressions

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