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1/60. 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.
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2/60. arnold-chiari malformation with syringomyelia in an elderly woman.

    PRESENTATION: A 76-year-old woman, complaining of leg pain and unsteady gait for 3 years, presented with a spastic paraparetic gait, severe spasticity and touch, thermal and pain sensory loss limited to arms, lower thorax and upper abdomen. brain and spinal cord magnetic resonance imaging showed a large loculated syrinx. Cerebellar tonsillar herniation into the foramen magnum was also seen (arnold-chiari malformation, type I). OUTCOME: The patient had successful cervico-spinal surgical decompression which resulted in marked reduction in hypertonia and weakness, normal gait and normal joint movement at 6 months. CONCLUSION: This unusual, late clinical presentation of a congenital disease underlines the importance of a comprehensive diagnostic work-up in the elderly patients with complex neurological signs.
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3/60. Isolated Horner's syndrome and syringomyelia.

    Although syringomyelia has been associated with Horner's syndrome, it is typically associated with other neurological findings such as upper limb weakness or numbness. A patient is described who had an isolated Horner's syndrome as the only manifestation of syringomyelia. A 76 year old woman was discovered to have right upper lid ptosis and right pupillary miosis. Neurological examination was unremarkable, and pharmacological testing was consistent with localisation of the lesion to a first or second order sympathetic neuron. neuroimaging disclosed a Chiari I malformation with a syrinx extending to the C2 to C4 level. An isolated Horner's syndrome may be the presenting manifestation of syringomyelia.
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4/60. Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues.

    Chiari Malformation (CM) encompasses several patterns of congenital or acquired cerebellar herniation through the foramen magnum. This may result in brain-stem compression that impacts control of breathing and is associated with obstructive and central apneas. A high clinical suspicion for sleep-disordered breathing is needed in the care of such patients after as well as before corrective surgery. To introduce a review of CM with a focus on the relevance to sleep medicine, we present a case of a 13-year-old female who was diagnosed with CM Type 1 in the course of an evaluation of symptomatic central sleep apnea. After initial improvement following surgery there was recurrence of brain-stem compression. The only clinical expression of which was polysomnographically evident recurrence of sleep apnea.
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5/60. Cerebral dissection from syringomyelia demonstrated using cine magnetic resonance imaging. Case report.

    A 16-year-old boy presented at the authors' emergency department with a sudden deterioration of respiration. He had been paraparetic for 3 years and had become quadriplegic 2 days previously. Magnetic resonance images revealed a Chiari I malformation and a hydromyelic cavity extending from C-1 to T-11. Rostrally, a small cylindrically shaped lesion extended from the cervicomedullary junction to the left semioval center. The patient made a dramatic neurological recovery following suboccipital craniectomy and upper cervical laminectomies with augmentation duraplasties followed by placement of a syringoperitoneal shunt.
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6/60. A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report.

    The authors report on a 19-year-old man with an acquired tonsillar herniation caused by a craniocervical junction injury in which serial magnetic resonance (MR) images demonstrated patent and isolated segments of the central canal participating in the dilation and then formation of a cervical syrinx. The patient was involved in a motor vehicle accident; he developed tonsillar herniation as a complication of subarachnoid and epidural hemorrhage, predominantly observed around the cisterna magna and upper cervical canal. Repeated MR images obtained over an 11-month period indicated the for mation and acute enlargement of the syrinx. Ten months after the accident, the patient presented with sensory disturbance in both upper extremities and spasticity due to syringomyelia. He underwent craniocervical decompressive surgery and doraplasty, which reduced the size of syringomyelia. The authors postulate that the patent central canal may play a role in determining the location of a syrinx remote from a focus of cerebrospinal fluid obstruction.
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7/60. Acute spinal cord swelling in a child with Chiari II malformation.

    We report on a patient with Chiari II malformation who developed acute cervical myelopathy and brainstem dysfunction following a shunt failure. A brain and spine magnetic resonance image obtained immediately after admission evidenced swelling of the medulla and upper cervical spinal cord. After emergency placement of an external ventricular drain, the swelling receded, the child's respiratory pattern improved and the motor function of his upper and lower extremities progressively returned to its normal condition. The case reported here suggests that in patients with Chiari type II malformation, spinal cord swelling might have a similar pathophysiology to the periventricular edema observed in the supratentorial compartment, and thus may be independent of the early stages of syringomyelia or syringobulbia.
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8/60. Periodic alternating nystagmus associated with arnold-chiari malformation.

    The arnold-chiari malformation type I is a rare congenital condition characterized by herniation of the cerebellar tonsils into the upper cervical spinal tract. Various otoneurological manifestations occur in patients with the disease, which has usually been associated with a downbeat type of nystagmus. In this paper a case with this condition is reported presenting with periodic alternating nystagmus (PAN), which is a rare type of nystagmus observed in diseases of the central nervous system of different pathologies. The patient had a complete otoneurological examination and vestibular function testing. diagnosis was established by using magnetic resonance imaging (MRI).
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9/60. Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations.

    OBJECTIVE AND IMPORTANCE: A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal. INTERVENTION: Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions. CONCLUSION: This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).
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10/60. spinal cord edema preceding syringomyelia associated with Chiari I malformation--case report.

    A 38-year-old woman with Chiari I malformation presented with spinal cord edema preceding syringomyelia manifesting as a 5-month history of nuchal pain and numbness of the upper extremities. magnetic resonance imaging showed spinal cord edema, a poorly defined syrinx at the C-2 to T-2 levels, and distorted cerebellar tonsils. Computed tomography revealed cerebrospinal fluid (CSF) density in the center of spinal cord edema, and positron emission tomography revealed no uptake of L-[methyl-11C]methionine, indicating a non-neoplastic lesion. Craniocervical decompression achieved excellent clinical and neuroradiological outcomes. The success of surgical treatment supports the theory that patients with Chiari I malformation have increased transmural flow of CSF, causing spinal cord edema that progresses to syringomyelia. Early treatment of patients with spinal cord edema is indicated to prevent permanent spinal cord injury due to progressive syringomyelia.
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