Arnold-Chiari type 1 malformation and syringomyelia. 1990

W Y Hsu, and J Y Chen, and C S Chi, and T T Wong
Department of Pediatrics, Chung Shan Medical and Dental College Hospital, Taichung, R.O.C.

A 12-year-old boy had a long history of headache since the age of 4 years. Numbness and weakness of the upper extremities and chest tightness, especially in the morning, were noted since 2 years ago. The headache became more severe and frequent and attacked even in sleep since one year ago. Arnold-Chiari type 1 malformation, cervical syringomyelia and mild hydrocephalus were demonstrated by MRI scan of the central nervous system. Headache and other symptoms disappeared immediately after surgical intervention. The formation of syringomyelia may be multifactorial, but Arnold-Chiari type 1 malformation and uneventful birth history play an important role in the underlying cause of this case. We presented this youngest case who had Arnold-Chiari type 1 malformation and onset of headache from the age of 4 years, discussed the pathogenesis of syringomyelia and reviewed the literature.

UI MeSH Term Description Entries
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
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006261 Headache The symptom of PAIN in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of HEADACHE DISORDERS. Cephalgia,Hemicrania,Bilateral Headache,Cephalalgia,Cephalodynia,Cranial Pain,Generalized Headache,Head Pain,Ocular Headache,Orthostatic Headache,Periorbital Headache,Retro-Ocular Headache,Sharp Headache,Throbbing Headache,Unilateral Headache,Vertex Headache,Bilateral Headaches,Cephalalgias,Cephalgias,Cephalodynias,Cranial Pains,Generalized Headaches,Head Pains,Headache, Bilateral,Headache, Generalized,Headache, Ocular,Headache, Orthostatic,Headache, Periorbital,Headache, Retro-Ocular,Headache, Sharp,Headache, Throbbing,Headache, Unilateral,Headache, Vertex,Headaches,Headaches, Bilateral,Headaches, Generalized,Headaches, Ocular,Headaches, Orthostatic,Headaches, Periorbital,Headaches, Retro-Ocular,Headaches, Sharp,Headaches, Throbbing,Headaches, Unilateral,Headaches, Vertex,Ocular Headaches,Orthostatic Headaches,Pain, Cranial,Pain, Head,Pains, Cranial,Pains, Head,Periorbital Headaches,Retro Ocular Headache,Retro-Ocular Headaches,Sharp Headaches,Throbbing Headaches,Unilateral Headaches,Vertex Headaches
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

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