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1/10. Presentation of a previously asymptomatic Chiari I malformation by a flexion injury to the neck.

    Flexion injury and/or whiplash injury to the neck in car accidents are usually trivial injuries with no serious neurological deficits. Our intention was to point out the importance to proceed with diagnostic procedures if neurological deficits do occur in order to reveal the true cause of the deficit. The paper presents the case of a 35-year-old woman who sustained a flexion injury to the neck. A relatively trivial injury to the neck promoted a progressive neurological deterioration. The standard diagnostic procedures (x-ray, computed tomography scan) were normal. Further diagnostics with magnetic resonance imaging was required to reveal an underlying Chiari I malformation. Finally, the operative decompression of the craniocervical junction was performed. Following the surgical treatment, the patient's clinical symptoms regressed. One year after her discharge, she remains in good physical condition. To our knowledge, this case is the first report of the manifestation of Chiari I malformation in the adult as a result of a flexion or whiplash injury of the neck. This unusual case suggests that in a trivial flexion injury to the neck sustained in a car accident, which presents with serious neurological dysfunction, and where the standard diagnostic procedures are normal, the possibility of underlying congenital abnormality, such as Chiari I malformation should be considered.
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2/10. Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation.

    This is a case report of a male patient who presented with orofacial pain for a year as the only manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation. This article places emphasis on the clinical presentation and possible differential diagnoses. The pain was continuous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracranial pressure. Paroxysmal pain crises developed over this background of continuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with amplification of the foramen magnum.
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3/10. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years.

    INTRODUCTION: The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented. methods: Since 1985, 31 children with CM I (0.3-5.8) years of age have been diagnosed at University of iowa hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome. RESULTS: The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I. Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen. Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. syringomyelia improved in all patients. scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery. DISCUSSION: We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity. CONCLUSIONS: Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.
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4/10. arnold-chiari malformation with syrinx presenting as carpal tunnel syndrome: a case report.

    A 26-year-old administrative assistant presented with 3 years of left-hand dysesthesia involving primarily the first 3 digits. Her symptoms increased at night and with keyboard use. Through 12 visits to primary and specialty care physicians over 3 years, she experienced minimal improvement with splints and moderate improvement with gabapentin. On presentation, careful questioning revealed an abrupt onset of symptoms 3 years previously, related to a 2-week episode of gastritis associated with recurrent emesis. Examination revealed a negative Tinel sign over the median nerve at the wrist, decreased left biceps reflex, positive Spurling test, and decreased sensation over the palmar and dorsal surfaces of the left hand in the C5-6 distribution. The atypical onset of symptoms, poor response to therapy, and physical findings suggested the possibility of a radicular or central neurologic etiology for the patient's hand numbness. magnetic resonance imaging demonstrated a Chiari I malformation with a syrinx extending from C2 to T10, with the greatest diameter at C4. Neurosurgical decompression led to a decrease in symptoms. A meticulous history and physical examination should be performed on patients with presumed carpal tunnel syndrome with an atypical onset of symptoms or response to therapy.
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5/10. foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patient.

    STUDY DESIGN: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression. OBJECTIVE: To report this rare case along with a literature review. SETTING: Gifu, japan. methods: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred to the clinic. After physical and radiographical examinations, she was diagnosed with Arnold Chiari malformation type I with associated syringomyelia. A foramen magnum decompression by the removal of the outer layer of the dura mater was performed. RESULTS: At 2 years postoperatively, MRI revealed a decrease in the size of the syringomyelia. Her symptoms had also remarkably improved. CONCLUSIONS: A rare case of symptomatic Arnold Chiari malformation type I with associated syringomyelia in an elderly woman was successfully treated with foramen magnum decompression by the removal of the outer layer of the dura mater.
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6/10. Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature.

    OBJECTIVE: Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI S). CLINICAL PRESENTATION: We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia. CONCLUSION: A review of the literature reveals fewer than ten previous reports of familial CMI S in the past 30 years. Although rare, the existence of familial cases of CMI S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI S.
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7/10. Headache spectrum in arnold-chiari malformation.

    Exertional headaches in patients with arnold-chiari malformation (ACM) are well described. We report four patients with Type I ACM and recurrent headaches. These patients presented, respectively, with low spinal fluid pressure headache, migraine without aura, migraine with aura, and migraine with prolonged aura. This report suggests the need for observing patients with recurrent headaches for any physical stigmata of craniovertebral junction anomalies, and the need to exclude ACM in such patients. Possible implications of the association between ACM and different types of headaches are discussed.
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8/10. arnold-chiari malformation: a diagnostic challenge.

    arnold-chiari malformation is a disorder of embryologic development. In Type I, there is cerebellar displacement into the spinal canal, but hydrocephalus and syringomyelia are variable. Type II usually is manifested by severe hydrocephalus and myelomeningocele in infancy. Type I malformations may pose diagnostic challenges because they often produce bizarre and vague symptoms. A careful history and physical examination, coupled with neurologic testing, especially magnetic resonance imaging, will lead to the correct diagnosis.
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9/10. exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.

    We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II arnold-chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.
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10/10. Saccadic visual suppression and down-beat nystagmus: a case of arnold-chiari malformation.

    A case is reported in which visual disturbances associated with the sole physical finding of downbeat nystagmus led to investigation of a patient for a foramen magnum lesion. The arnold-chiari malformation was suggested by air study and confirmed at surgery. It is suggested that the phenomenon of saccadic suppression was responsible for the patient's visual disability. This was unaccompanied by any of the other neurological manifestations of the arnold-chiari malformation. Cases presenting in this manner, with only visual disturbances, may run the risk of being unrecognized for some time as a result.
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