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PubMed2001

Respiratory failure caused by Chiari I malformation with associated syringomyelia.

The authors report a case of a 38-year-old male with a Chiari I malformation and syringomyelia that presented with acute respiratory distress. Pulmonary function testing was consistent with respiratory muscle weakness. A suboccipital craniectomy and C1, 2, 3 laminectomies were performed on an emergent basis. Postoperatively the patient had significant improvement in his respiratory status associated with a reduction in the size of the syringomyelia on follow-up imaging.

Adult↗
PubMed2001

Cutaneous and mixed nerve silent periods in syringomyelia.

OBJECTIVES AND METHODS: We studied cutaneous and mixed nerve silent periods (CSP, MNSP) in 4 patients with cervical syringomyelia documented by magnetic resonance imaging who on clinical examination presented with unilateral hypalgesia and hypothermesthesia. In addition, we recorded upper and lower extremity somatosensory and motor evoked potentials (SEP, MEP), and cortical silent periods. RESULTS: In all patients, CSP and the later portion of MNSP were absent or shortened on their affected side, while both were normal on their unaffected side. In all patients, SEP latencies were normal following both median and tibial nerve stimulation. In two patients, the amplitude N13 (median nerve SEP), and in one patient each the amplitudes N20 (median nerve SEP) and P37 (tibial nerve SEP) were reduced. Central motor conduction time was prolonged to abductor digiti minimi muscle in one patient on the affected side, but was normal to tibialis anterior muscle in all patients. Cortical silent periods where present bilaterally in spite of unilateral complete absence of CSP and MNSP in two subjects tested. Loss of CSP and MNSP were a sensitive parameter of spinal cord dysfunction in syringomyelia. The cervical median nerve SEP response N13 reflected gray matter involvement, while corticospinal tract dysfunction was less frequently observed. CONCLUSION: Our data suggest that CSP and later portion of MNSP are generated at the spinal level by the same small myelinated A-delta fibers, and that their central network is distinct from large diameter fiber afferents and efferents.

Adolescent↗
PubMed2004

Acute idiopathic syringomyelia: a case report.

There have been isolated reports in the literature of the natural history and less than complete knowledge of the epidemiology and pathophysiology of syringomyelia. This article describes a clinically acute-onset abnormal patient with localized dilatation of the central canal of the spinal cord who had no evidence of present or prior Chiari's malformation, trauma, infection, tumor, or other predisposing pathologic condition. It is suggested that this distinct condition be labeled "acute idiopathic syringomyelia".

Acute Disease↗
PubMed2003

Surgical prognosis in hindbrain related syringomyelia.

OBJECTIVES: The recommended operative treatment against hindbrain related syringomyelia is suboccipital decompression. The aim of the study was to define prognostic factors for surgical outcome in this disease. MATERIALS AND METHODS: Between 1990 and 1997, 31 patients with hindbrain related syringomyelia were treated. All patients underwent craniovertebral decompression by suboccipital craniectomy and laminectomy of C1 or C1 and C2, respectively. Additionally, in nearly half of the cases, the tonsils were treated by bipolar coagulation. Mean post-operative observation period was 35 months including clinical and radiological (MRI) examination. RESULTS: Neither there was correlation between clinical outcome and age nor correlation between clinical outcome and duration of preoperative symptoms. There was good correlation between clinical outcome and result of post-operative MRI: 63% of patients with a sufficient post-operative MRI demonstrated a significant clinical improvement, whereby only 17% of patients with insufficient MRI did so (P < 0.05). None of the patients with coagulation of the tonsils showed clinical improvement. In contrast, 77% of patients without tonsillar manipulation demonstrated clinical improvement (P < 0.01). Additionally, 88% of the patients with dorsal-tenting duraplasty experienced neurological improvement (P < 0.01), too. CONCLUSIONS: The results give evidence for the prognostic importance of creating an adequate artificial cisterna magna. Dorsal-tenting of the duraplasty is an advantageous means against scarring and adhesions, whereby intradural applications such as extensive bipolar coagulation of the cerebellar tonsils should be avoided.

Adult↗
PubMed1994

Hyperhidrosis as the presenting symptom in post-traumatic syringomyelia.

Post-traumatic syringomyelia is now a well known entity and occurs months or years after a spinal cord injury. The presenting symptoms are usually pain, progressive motor weakness, sensory changes, and increased spasticity. Profuse sweating or hyperhidrosis can be a symptom of the post-traumatic syrinx or can occur in autonomic dysreflexia provoked by peripheral stimuli. We present two patients with cervical spine fractures whose presenting symptom of post-traumatic syringomyelia was hyperhidrosis affected by posture. The pathophysiology involved and the management of these patients is discussed.

Adolescent↗
PubMed1996

Utility of early postoperative MRI in hindbrain-related syringomyelia. A preliminary prospective study on five patients.

We report a series of five patients operated on for hindbrain-related syringomyelia with posterior fossa decompression and duraplasty. In all cases an early postoperative MRI was performed (24-36 h after surgery). These images have been compared with the preoperative ones and with those obtained, 1, 3 and 6 months after operation. Both before and after surgery there was not always a tight correlation between clinical and MRI pictures. In all but one patient the early postoperative MR images showed a reduction of syrinx size and were as to those obtained later. We discuss the results observed in our small series and analyze the role of early postoperative MRI studies in patients with hindbrain-related syringomyelia.

Adult↗
PubMed1997

Syringomyelia--a complication of meningitis--case report.

Syringomyelia following meningitis is a rare condition. This report summarizes the history of a 43 year old Indian male who developed arachnoiditis as a sequalae of tubercular meningitis. This later progressed to syringomyelia. The patient was treated with a syringo-subarachnoid shunt.

Adult↗
PubMed1998

Post-traumatic syringomyelia and post-traumatic spinal canal stenosis: a direct relationship: review of 75 patients with a spinal cord injury.

This study aims to demonstrate predictive factors for post traumatic syringomyelia (PTS), and in particular to correlate the role of insufficiency of reduction of a spinal fracture with the occurrence of syringomyelia. One hundred and twenty-eight spinal cord injured patients (SCI) were studied during the years 1992 and 1993. Among them, 75 underwent a complete and reliable evaluation including: review of the initial vertebral lesion, and of the surgery report, and a radiological study of the lesion site with standard X-rays, a CT scan, and an MRI. The CT Scan included slices in sagittal reconstructions and in the axial plane at the site of injury with the calculation of a percentage of canal stenosis in the two planes of the space. An MRI was carried out with T1 and T2 weighted images, including sagittal entire cord images in addition to sagittal and axial slices centred on the site of injury. A syrinx was diagnosed in 28% of the patients. The occurrence of a syrinx is significantly correlated with spinal canal stenosis in the sagittal plane (delta D) with a P < 0.001 and in the axial plane (delta S) (P < 0.05). This present study demonstrates the major role of the insufficiency of reduction of the vertebral lesion in the genesis of a syrinx. The quality of the initial treatment of the vertebral injury is the first step in the prevention of a syrinx. The treatment of a syrinx, besides techniques of drainage, must also take into account the spinal realignment.

Adolescent↗
PubMed2000

Dural band pathology in syringomyelia with Chiari type I malformation.

Surgical material taken from the 'outer layer' of thickened dura mater (dural band) at the craniovertebral junction of eight cases of syringomyelia with Chiari type I malformation was histologically examined in comparison with four autopsy cases as controls. The dural band was thickened and there were increased numbers of collagen fibers which showed fiber splitting, hyalinous nodule, calcification and/or ossification. These changes were not observed in the four control cases. Thus, it is suggested that the thickening of the dura mater may be a causative factor of syringomyelia with Chiari type I malformation. In addition, the histology of the thickened dura mater suggests the condition may be a consequence of birth injury in these patients.

Adolescent↗
PubMed1999

[Posttraumatic syringomyelia].

The improvement of preclinical emergency medicine, better surgical and conservative therapies, and the development of intensive care units and specialized centers have improved the survival rate for patients with serious spinal cord injuries. Therefore, more sequelae of chronic spinal cord injuries such as post-traumatic spinal cord cavitations also occur. The first such case was described by Bastian in 1867. Generally, these cavitations were diagnosed from 2 months up to 32 years after the trauma. The overall prevalence of post-traumatic syringomyelia (PTS) is not known; however, with the increasing use of magnetic resonance imaging (MRI), its diagnosis has increased, ranging from 2.3% of paraplegic and tetraplegic patients in 1976 and 3.2% in 1985, to nearly 50% in a selected group of patients in 1991 and 1993. In 1995, a 4.45% incidence was reported. In our clinic we are currently treating 440 cases of syringomyelia, 140 of which are PTS. Several observations suggest more than one potential mechanism for the evolution of a post-traumatic cyst or PTS. Various factors, such as hemorrhage or, in particular, ischemia within the spinal cord, blockage of the cerebrospinal fluid (CSF) pathways around the cord or localized meningeal fibrosis either alone or in combination with other factors, may be involved. Clinically, sensory disturbances, loss of motor function, pain, and modification of the deep tendon reflexes are observed in most patients. On MRI, PTS is seen as a longitudinal, cystic cavity within the spinal cord, giving a hypointense signal on T1-weighted images and a hyperintense signal on T2-weighted images. For treatment planning it is mandatory to identify the lower and upper end of the PTS on the MRI.

Humans↗
PubMedDate not supplied

[Evoked potentials in Chiari-malformation type I with syringomyelia--a case history].

The Chiari-syndrome Type I being a malformation of the posterior cranial fossa often leads to syringomyelia. The disease becomes apparent in adolescence with kyphoscoliosis, headache, vertigo, ataxy, hearing loss, partial paralysis and other neurological disorders. The onset is typically characterized by dissociated anesthesia, due to the frequent localization of the syrinx in the neighborhood of the posterior column of the spinal cord. It is reported a case of an 15-years-old-girl suffering from intensive headache, recurrent left-sided paresthesias and progredient scoliosis. Somatosensory evoked potentials of left ulnar and tibial nerves revealed a complete loss of cortical response. Diagnostic imaging showed an Chiari-malformation I with herniation of cerebellar tonsils and secondary syringomyelia of the cervical spinal cord. After surgical treatment with posterior fossa decompression, C1 laminectomy and partial excision of cerebellar tonsils the patient had a clear improvement in symptoms. Postsurgical the width of cervical syrinx decreases. Now somatosensory evoked potentials of the left ulnar and tibial nerves show amplitude-reduced cortical responses with a normal latency.

Adolescent↗
PubMed1984

[Posttraumatic syringomyelia].

Communications on posttraumatic syringomyelia, a late sequel of complete or incomplete paraplegia or even tetraplegia, are rather infrequently in German literature. The problems of this well known complication occurring several months, years or even decades after a severe spinal cord trauma are discussed by two own cases. Both patients suffering from paraplegia developed signs and symptoms of posttraumatic syringomyelia 6 months and 3 years respectively after the accident. Case history and clinical findings correspond with reports in literature. Cystic degeneration in the cervical spinal cord could only be demonstrated in one patient by metrizamid -T-myelography combined with computerized tomography. The value of radiological and other technical investigations, pathogenesis, morphological findings and treatment are discussed.

Adult↗
PubMed1996

Third ventriculostomy for symptomatic syringomyelia using flexible endoscope: case report.

Using a flexible endoscope, we performed a third ventriculostomy to treat a patient with communicating syringomyelia accompanied by enlarged cerebral ventricles. Her clinical improvement was rapid and dramatic. Postoperative magnetic resonance scan demonstrated shrinkage of the cervical syrinx as well as the ventricles. This simple and safe flexible endoscopic third ventriculostomy could be a treatment of choice for communicating syringomyelia accompanied by enlarged cerebral ventricles.

Adult↗
PubMed1992

Cervical cord birth injury and subsequent development of syringomyelia: a case report.

A 2830 g full-term baby, born by breech delivery, exhibited weak crying and sucking and severe hypotonia of the extremities after birth. Magnetic resonance imaging (MRI) showed marked thinning of the cervical cord at the level of C4 and C5. This lesion evolved into focal syringomyelia by the fourth month after birth. In this patient, MRI was useful in detecting the initial spinal cord injury, which appeared as marked thinning, and the subsequent syringomyelia as well. The role of birth trauma in cervical spinal cord injuries is discussed.

Birth Injuries↗
PubMed1975

Computerized axial tomography in syringomyelia.

The ACTA-scanner, a device for computerized axial tomography, permits cross-sectional radiographic study of the entire human body, including the spine. In the ACTA-scan, the spinal cord appears as a roundish formation surrounded by the less dense subarachnoidal cerebrospinal fluid. The spines of 18 patients with verified (nine cases) or suspected (nine cases) syringomyelia were studied by ACTA-scanning. In seven of the verified and in four of the nonverified cases, some evidence of cord cavitation was shown. The cystic part of a cervical-cord ependymoma was also demonstrated. ACTA-scanning is an easily performed, noninvasive, innocuous procedure. This technic, which complements other radiographic methods of evaluating the spinal cord, is particularly suitable for screening and follow-up study of patients with syringomyelia. The easily accomplished recognition of a possible associated hydrocephalus is an added advantage of ACTA-scanning.

Brain Diseases↗
PubMed1996

Regression of syringomyelia after removal of a posterior fossa epidermoid tumour.

The case is reported of a 49-year-old man with a giant infratentorial extradural epidermoid tumour, with cervical syringomyelia and hydrocephalus, and partial protrusion of the cerebellar tonsils through the foramen magnum. The patient complained of symptoms of raised intracranial pressure and was admitted to our Department, where ventriculoperitoneal shunting and total removal of the epidermoid tumour were performed. One year later, MR imaging demonstrated collapse of the cervical syringomyelia.

Brain↗
PubMed1998

Dynamic CSF flow study in the pathophysiology of syringomyelia associated with arachnoid cysts of the posterior fossa.

Two patients with posterior fossa arachnoid cysts associated with syringomyelia are discussed adding to the five cases already reported in the literature. The formation and progression of syringomyelia secondary to a posterior fossa cyst and its possible pathophysiology by dynamic CSF flow studies using magnetic resonance (MR) imaging are discussed and reviewed.

Adolescent↗