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1/474. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.

    BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. methods: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients. ( info)

2/474. Spontaneous resolution of a cervicothoracic syrinx in a child. Case report and review of the literature.

    A child with near complete spontaneous resolution of a cervicothoracic syrinx and improvement in a Chiari type I malformation without surgical intervention is presented. The child was followed clinically with serial magnetic resonance (MR) imaging and has remained neurologically stable over an 11-year period. To our knowledge, only 3 pediatric cases of spontaneous resolution of a spinal cord syrinx as documented by MR imaging without surgical intervention have been reported. This case contributes to the literature on the natural history of syringes. ( info)

3/474. Headache in Chiari malformation: a distinct clinical entity?

    A relatively common group of developmental anomalies of the posterior fossa and its contents make up the Chiari malformations. The most common of these anomalies are the Chiari malformations type I and type II. Many patients with these defects are asymptomatic, but many of them suffer from headaches. Although the prevalence of common primary headache disorders in patients with Chiari malformations is similar to that of the general population, a particular type of occipital headaches occurs more frequently in patients with a Chiari malformation. In the two patients described in the illustrative case presentations, the major complaint was headache. Both had Chiari malformation type I diagnosed. Chiari malformation should be considered in the differential diagnosis of patients who complain of exertional headache. Exertional headaches or worsening of pain during Valsalva's maneuver should prompt clinical suspicion of an underlying intracranial lesion and warrants further evaluation by appropriate neuroimaging studies. ( info)

4/474. Arnold Chiari Type I malformation presenting as a trigeminal neuralgia: case report.

    OBJECTIVE AND IMPORTANCE: Arnold Chiari Type I malformation usually presents as headache, arm numbness, dysesthesia, upper weakness, or gait difficulty. We report a case of Chiari malformation presenting as a left trigeminal neuralgia. CLINICAL PRESENTATION: A patient with a history of 29 years of trigeminal neuralgia was admitted. He was treated with three thermocoagulations. Microvascular decompression was planified. magnetic resonance imaging was performed, and it demonstrated an Arnold Chiari malformation. After surgery, the patient was asymptomatic. INTERVENTION: Posterior fossa decompression by enlarging the foramen magnum and aspiration of the cerebellar tonsils was performed. CONCLUSION: The trigeminal neuralgia could be attributable to a compression of the trigeminal nucleus. The compression of the nucleus could explain both the pain and the regression after surgery. This is the second reported case of pure trigeminal neuralgia in Arnold Chiari malformation. ( info)

5/474. Pseudo Chiari type I malformation secondary to cerebrospinal fluid leakage.

    cerebrospinal fluid (CSF) leakage may occur spontaneously, iatrogenically or from spinal trauma. Postural headache is the cardinal symptom; dizziness, diminished hearing, nausea and vomiting are additional symptoms. In neurological examinations cranial nerve palsies may be found. Due to low CSF pressure neuroimaging studies may reveal dural enhancement and vertical displacement of the brain. We describe a patient with the history of an uncomplicated lumbar discectomy at the level L4-5 and the typical clinical symptoms of intracranial hypotension. MRI of the craniocervical junction showed typical features of a Chiari type-I malformation. After neurosurgical ligation of a CSF leak at L4-5 caused by lumbar disc surgery, the patient was free of orthostatic headache. A repeated MRI showed a striking reduction of the previous downward displacement of the cerebellar tonsils and pons. ( info)

6/474. The Chiari II malformation: lesions discovered within the fourth ventricle.

    Structural lesions associated with the Chiari II malformation have been identified within the fourth ventricle in 8 patients. During the 42-month period encompassing the operations of the 7 patients treated surgically, only 9 other patients were explored without the discovery of some associated structural lesion. The patients ranged in age from 2 to 26 years. The following lesions were identified: glial or arachnoidal cysts (3 patients), glial or choroidal nodules (3 patients) and subependymoma (2 patients). These lesions were all situated in the roof of the fourth ventricle adjacent to or interspersed with the choroid plexus. Only the cystic lesions were identified by preoperative imaging. In only 1 case did the associated lesion, a 2-cm cyst, seem to contribute to the patient's clinical presentation. Structural lesions of the fourth ventricle associated with the Chiari II malformation are common in patients who are submitted to decompression. These lesions may be dysplasias of developmental origin, or they may be reactive lesions related to chronic compression and ischemia. They do not necessarily required biopsy or excision. ( info)

7/474. Respiratory sinus arrhythmia of brainstem lesions.

    In this pilot study we investigated the hypothesis that intrinsic and extrinsic brainstem lesions situated within the pontomedullary region would effect the integrity of respiratory sinus arrhythmia. The study sample consisted of three patients with anatomic brainstem abnormalities associated with isolated Chiari I malformation, Chiari II malformation with syringobulbia, and achondroplasia with cervicomedullary compression. They were compared to an age- and sex-matched control group of nine patients. Each subject's electrocardiogram was recorded in a quiet room and digitized by a personal computer during five 1-minute periods. R-R intervals within each 1-minute period were converted to heart rate in 120 successive 0.5-second intervals. The resultant heartrate time series was converted to its underlying frequency composition by a fast Fourier transform and averaged across minutes. Respiratory sinus arrhythmia was defined as the variability in the time series over a frequency range (0.096 to 0.48 Hz) corresponding to a range of respiratory rates from 6 to 30 breaths per minute. Analysis revealed a significant reduction in respiratory sinus arrhythmia (P < .05), defined as the summated area under the curve, with a mean for controls of 35.42 /-28.13 SD and for subjects of 17.20 /-11.50 SD. There was a gradient of abnormality noted, with the mildest deviation in respiratory sinus arrhythmia for the patient with isolated Chiari I malformation and maximum deviation seen in the patient with extrinsic cervicomedullary compression. ( info)

8/474. lip biting in a patient with Chiari type II malformation: case report.

    self mutilation of lips and tongue is considered a common type of Self-Injurious behavior (SIB). Treatment of SIB in the form of lip-Biting in developmentally disabled individuals has been the focus of several related reports using different oral appliances preventing or inhibiting the SIB. In this paper we report a case of SIB in the form of lip-Biting on an infant with Chiari Type II Malformation which was treated with a lip-bumper. The lip-bumper demonstrated to be a viable option in treating transient and acute episodes of SIB involving the lower lip and buccal mucosa. ( info)

9/474. shoulder enlargement as the presenting sign in syringomyelia. Report of two cases and review of the literature.

    Two adult patients had painful enlargement of the shoulder and complete destruction of the humeral head. Angiograms of the shoulder showed highly vascular lesions in both patients. Both patients had syringomyelia, for which the swollen shoulder was the first manifestation. ( info)

10/474. Unusual late responses in a patient with an arnold-chiari malformation.

    We studied a young man with spastic right hemiparesis, in whom supramaximal stimulation of the left posterior tibial nerve produced toe movements of the both feet and associated late responses in the flexor hallucis brevis muscle bilaterally. These findings indicate that, in this patient, there are central connections between peripheral afferents and contralateral alpha-motor neurons. It may be that such connections are normally present but that they are too weak in normal subjects to produce firing of the alpha-motor neurons by themselves. If so, the loss of cortical inhibition in our patient may have allowed these connections to produce movement. ( info)
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