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Neurophysiologic assessment of urinary dysfunction in children with thoracic syringomyelia.

Isolated syringomyelia of the thoracic portion of the spinal cord is relatively uncommon. In children, signs and symptoms may involve only the urinary system. Five children who presented for evaluation of urinary tract dysfunction were found to have syringomyelia of the thoracic cord not associated with any other central nervous system abnormality on radiographic imaging. Each child underwent a complete neurologic examination and a complete electrophysiologic/urodynamic evaluation. One patient underwent surgical drainage of the syrinx due to progression of neurologic deficit, with slight improvement; three patients were treated pharmacologically to control the urinary dysfunction.

Child↗

Metrizamide ventriculography in syringomyelia.

Computed tomography (CT) both with and without contrast agents has been used to diagnose syringomyelia. We report a case in which the diagnosis was made by injection of metrizamide into a ventriculoperitoneal shunt. Continuity of the syringomyelic cavity with the ventricular system was demonstrated. This case supports the hydrodynamic concept of syringomyelia formation and illustrates a potentially useful approach to its diagnosis.

Biomechanical Phenomena↗

Torticollis in three children with syringomyelia and spinal cord tumor.

There are numerous well-known causes of acquired torticollis. However, an extensive review of the literature reveals only a rare, brief remark that associates torticollis with syringomyelia. We present three children found to have syringomyelia and spinal cord tumor, with torticollis as an early finding in each.

Astrocytoma↗

Abdominal wall weakness due to thoracic syringomyelia.

We describe a patient who presented with unilateral abdominal herniation due to weakness of abdominal musculature, associated with extensive syringomyelia. Electromyographic evidence of focal denervation and reinnervation was present in the weak muscles consistent with anterior horn cell loss. We compare these unusual clinical features with the more typical presentations of syringomyelia and with other neurologic causes of abdominal weakness.

Abdominal Muscles↗

Syringomyelia and syringobulbia associated with an ependymoma of the cauda equina involving the conus medullaris: case report.

A patient with syringomyelia and syringobulbia secondary to an asymptomatic ependymoma of the cauda equina involving the conus medullaris is described. Delayed metrizamide computed tomography myelography was decisive for making the diagnosis of a highly extended syrinx associated with the tumor. Syringomyelic symptoms improved remarkably following the removal of the tumor, and the syrinx was not visualized in a postoperative study. The pathogenesis of syringomyelia associated with a caudally located spinal cord tumor is discussed.

Adult↗

Syringomyelia in a man with sarcoidosis.

Sarcoidosis, generally a systemic ailment, uncommonly affects the central nervous system. It only rarely affects the spinal cord alone or in combination with other areas. Syringomyelia has never been associated with sarcoidosis. We report the case of a 32-year-old man with sarcoidosis who presented with surgically verified syringomyelia. The pertinent literature is reviewed. The mechanisms by which sarcoidosis could cause a syrinx are discussed.

Adult↗

Magnetic resonance imaging and intraoperative neurosonography in syringomyelia.

Treatment of syringomyelia remains a difficult and controversial problem. However, the recent advent of magnetic resonance imaging (MRI) and intraoperative ultrasound allows a more precise approach to the diagnosis and management of this disorder. Experience with 27 cases of syringomyelia has shown that MRI is superior to all other forms of diagnostic imaging for the exact anatomical delineation of syrinxes and other spinal cavities. One-third of the syrinxes demonstrated by MRI were either not adequately visualized or missed by myelography and/or computed tomographic scanning. Intraoperative ultrasound has been used to allow more precise operative approaches to the syrinx as well as to guide the exact placement of shunt tubes into syrinx cavities. A method has also been developed to allow intraoperative ultrasound of the spinal cord with patients in the sitting position.

Adult↗

Thecoperitoneal shunt for syringomyelia: report of three cases.

During 8 months, three consecutive cases of syringomyelia were treated by the placement of thecoperitoneal shunts. In the first two patients, the cord cavitation was idiopathic in one and thought to be related to spinal trauma in the other. In the third case, there was associated anomaly of the craniocervical junction of the Chiari I type. Surgery was followed by clinical improvement in all patients. Postoperative magnetic resonance images showed definite diminution of the syringomyelic cavity. The results of treatment seem to support the use of the technique as a primary treatment of syringomyelia associated with progressive myelopathy.

Adult↗

Surgical indication and results of foramen magnum decompression versus syringosubarachnoid shunting for syringomyelia associated with Chiari I malformation.

Several surgical procedures have been used for the treatment of syringomyelia associated with Chiari I malformation at our institute. The purpose of this article is to evaluate the results of two major surgical procedures, foramen magnum decompression (FMD) and syringosubarachnoid (SS) shunting. The series consisted of 70 patients with syringomyelia associated with Chiari I malformation who were surgically treated. Their ages ranged from 3 to 59 years (median, 29.4 yr). FMD was performed on 33 patients, and SS shunting was performed on 37 patients. The follow-up period ranged from 6 months to 12.5 years, with a mean of 60 months. The clinical and radiological outcomes were analyzed comparing the two groups. We principally performed FMD in patients with symptoms of Chiari I malformation and/or a small syrinx. We prefer to use SS shunting in patients with large syringes. Postoperative magnetic resonance imaging demonstrated that the syrinx had collapsed or decreased in size in 94% of the patients who underwent FMD and in 100% of the patients who underwent SS shunting. Neurological improvements were observed in 82% and in 97% of the patients who underwent FMD and SS shunting, respectively. In particular, the relief of pain was more fully achieved after SS shunting than after FMD. The average time for the syrinx to collapse was 6.3 weeks after surgery in the FMD group and 1.8 weeks in the SS shunting group. These results indicate that clinical symptoms and radiological findings improved much more quickly in the SS shunting group than in the FMD group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation: case report.

OBJECTIVE AND IMPORTANCE: Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION: We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. Magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6-C7, associated with a Chiari I malformation. INTERVENTION: Foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION: Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. Magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.

Adult↗

Syringomyelia and bilateral vocal fold palsy.

This is a case of a 35-year-old who had complained of noisy breathing for 15 years, and had been on treatment for 'chronic asthma'. She presented to the Accident and Emergency department with an acute episode of difficulty with breathing, and on admission was found to have stridor and bilateral abductor vocal fold palsy. Further workup revealed a syringomyelia with an associated Chiari type 1 malformation. It is important to consider the above diagnosis in the differential of young adults with breathing difficulties. A discussion of syringomyelia, Chiai malformations and bilateral vocal fold palsy follows.

Adult↗

Acquired cervical syringomyelia secondary to a brainstem meningioma in a maltese dog.

A 15-year-old female maltese was referred to us because of a 3-month history of ataxia, circling, and acute blindness. A mass was noted in the brainstem on brain magnetic resonance images. A cerebellar herniation was also detected on T1-weighted sagittal images. The lateral, third and fourth ventricles and central canal of the cervical spinal cord were enlarged. Based on diagnostic imaging findings, cervical syringomyelia secondary to a brainstem tumor was suspected. The clinical signs were controlled well by lomustine and the dog survived for 8 months after the initial diagnosis. The mass was diagnosed as a meningioma based on histopathological findings. This report describes the clinical findings and imaging characteristics of an acquired syringomyelia resulting from a brainstem meningioma.

Animals↗

Natural history of scoliosis in children with syringomyelia.

We performed a retrospective review of 27 scoliotic patients with syringomyelia using MRI. Their mean age at the first MRI examination was 10.9 years, and at the final review 15.8 years. The mean ratio of the diameter of the syrinx to the cord on the midsagittal MRI (S/C ratio) decreased from 0.49 to 0.24; 14 patients showed a decrease of 50% or more (reduction group). In this reduction group, the cerebellar tonsillar herniation decreased from a mean of 11.3 mm to 6.0 mm, and some improvement in dissociated sensory disturbance was seen in nine of 13 patients. The scoliosis improved by 5 degrees or more in six patients in the reduction group. Our results indicate that spontaneous shrinkage of syringomyelia in children is not unusual and is associated with improvement in the tonsillar herniation, the scoliosis and the neurological deficit.

Adolescent↗

Obstructive sleep apnea leading to increased intracranial pressure in a patient with hydrocephalus and syringomyelia.

Rises in intracranial pressure from normal baseline values up to 50 cm H2O occurred shortly after the onset of obstructive sleep apnea in a patient with myelomeningocele, hydrocephalus, Arnold-Chiari malformation, and syringomyelia. Tonsillar hypertrophy caused the airway obstruction during sleep, because the obstructive sleep apnea and also the periodic elevation of intracranial pressure disappeared after tonsillectomy. Only one report from Japan has previously described three patients with elevated cerebrospinal fluid pressures during obstructive sleep apnea. It is conceivable that episodic airway obstruction and concurrent intracranial hypertension may have contributed to the development of syringomyelia in our patient.

Adolescent↗

Syringomyelia: current concepts in pathogenesis, diagnosis, and treatment.

Syringomyelia is a condition that results in fluid-containing cavities within the parenchyma of the spinal cord as a consequence of altered cerebrospinal fluid dynamics. This review discusses the history and the classification of the disorder, the current theories of pathogenesis, and the advanced imaging modalities used in the diagnosis. The intramedullary pulse pressure theory (a new pathophysiologic concept of syringomyelia) also is presented. In addition, the current understanding of the painful nature of this condition is discussed and the current trends in medical and surgical management are reviewed.

Animals↗

Isolated partial growth hormone deficient short stature with syringomyelia not associated with birth injury.

A 59-year-old female with 20-year history of slowly progressing muscle atrophy and sensory disturbance of upper extremities showed short stature, scoliosis, hunger type of sensory dissociation of the upper extremities and pyramidal tract sign of the lower extremities. Magnetic resonance imaging (MRI) clarified hypoplasia of the anterior pituitary lobe, Arnold-Chiari malformation and cervical syringomyelia. Insulin and arginine stimulating tests revealed partial type of isolated growth hormone (GH) deficiency but GH gene analysis detected no defects of GH genes. It was considered to be a rare case of non-hereditary hypopituitarism with Chiari malformation and syringomyelia not associated with perinatal injury, namely a midline anomaly syndrome.

Arnold-Chiari Malformation↗

Growth hormone insensitivity syndrome associated with syringomyelia and type I Chiari malformation.

A 49-year-old man with syringomyelia and a Type I Arnold-Chiari malformation (Chiari-I) was diagnosed with growth hormone insensitivity syndrome (GHIS). He was short in stature, had high circulating levels of GH, and low circulating levels of insulin-like growth factor-I (IGF-I) and IGF binding protein-3 (IGFBP-3). His GH responses to the administration of growth hormone-releasing hormone (GHRH) and L-DOPA were normal, but his levels of IGF-I and IGFBP-3 did not increase after the administration of exogenous GH. Direct genomic DNA sequencing revealed neither a mutation nor deletion in this patient's GH receptor (GHR) gene, though one polymorphism was detected, indicating that his GHR gene was normal. This is the first reported case of an association of GHIS with syringomyelia and Chiari-I malformation.

Arnold-Chiari Malformation↗