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PubMed1990

Syringomyelia and arachnoiditis.

Five patients with chronic arachnoiditis and syringomyelia were studied. Three patients had early life meningitis and developed symptoms of syringomyelia eight, 21, and 23 years after the acute infection. One patient had a spinal dural thoracic AVM and developed a thoracic syrinx 11 years after spinal subarachnoid haemorrhage and five years after surgery on the AVM. A fifth patient had tuberculous meningitis with transient spinal cord dysfunction followed by development of a lumbar syrinx seven years later. Arachnoiditis can cause syrinx formation by obliterating the spinal vasculature causing ischaemia. Small cystic regions of myelomalacia coalesce to form cavities. In other patients, central cord ischaemia mimics syringomyelia but no cavitation is present. Scar formation with spinal block leads to altered dynamics of cerebrospinal fluid (CSF) flow and contributes to the formation of spinal cord cystic cavities.

Adult↗
PubMed2000

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.

Aged↗
PubMed1991

Syringomyelia associated with post-meningitic spinal arachnoiditis due to Candida tropicalis.

A 63 year old man who suffered from syringomyelia related to post-meningitic spinal arachnoiditis caused by Candida tropicalis is reported. The clinical syndrome of syringomyelia developed gradually and a definite diagnosis was delayed for more than 10 years. The patient has partially recovered after surgical treatment. This form of fungal infection and its delayed neurological complication in the form of syringomyelia has not been reported previously, to our knowledge.

Arachnoiditis↗
PubMed2003

Hereditary aspects of occipital bone hypoplasia and syringomyelia (Chiari type I malformation) in cavalier King Charles spaniels.

A database of over 1300 cavalier King Charles spaniels spanning 20 generations was established by obtaining pedigree information from 45 dogs with syringomyelia secondary to occipital bone hypoplasia. These data were supplemented with published information from the breed club. The incidence of syringomyelia was very high in certain families and lines which had been extensively inbred. The affected dogs could be traced back to one bitch born in 1956 and the two offspring from her single litter. Four key dogs representing four major breeding lines consistently occurred within the individual pedigrees. If a dog had more than five of its eight great-grandparents descended from these four lines there was a greater chance of it having syringomyelia. The data from this preliminary study suggest that occipital bone hypoplasia is hereditary in the cavalier King Charles spaniel and that its inheritance is more likely to be autosomal recessive because both dam and sire must be inbred descendants from certain lines. However, the inheritance is more likely to be of variable penetrance or oligogenic than simple.

Animals↗
PubMed1983

Spinal cord size in syringomyelia: change with position on metrizamide myelography.

Change in the anteroposterior diameter of the cervical spinal cord was demonstrated on metrizamide myelography in 20 out of 40 cases of cervical syringomyelia. Twelve of these were normal-sized cords, four were small, and four enlarged. Comparable lateral cervical myelograms in the prone and supine positions were sufficient to show size change for normal-sized or small cords. Additional myelograms in the erect and Trendelenburg positions were necessary to show size change in enlarged cervical cords, especially with associated Chiari malformation. A localized change in cord size suggests syringomyelia. If the change in cord size is diffuse, then change less than 10% is not necessarily significant. In undiagnosed myelopathy, metrizamide myelography has the potential to detect unsuspected syringomyelia even when the cord is normal-sized or small. Myelographic study may also be useful as an adjunct to delayed computed tomographic studies.

Humans↗
PubMed1996

Acute and atypical presentations of syringomyelia.

Syringomyelia is characterized by a longitudinal cavity or several cavities in the spinal cord, and syringobulbia demonstrates similar pathology in the brainstem. Both conditions typically present with slowly progressing dissociative sensory deficits as well as upper and lower motor neuron deficits. We recently encountered 3 patients who presented with the rapid onset of such signs and symptoms and had cervical syringes. All 3 patients would have been diagnostic dilemmas had it not been for MRI, which identified a syrinx in each patient, as well as an Arnold-Chiari malformation in 2 of the 3. Syringomyelia and syringobulbia should be considered in the differential diagnosis of patients who have acutely evolving brainstem or cervical spinal cord deficits. MRI is the imaging modality of choice for detecting syringomyelia or syringobulbia presenting atypically.

Adult↗
PubMed1992

Surgical treatment of syringomyelia based on magnetic resonance imaging criteria.

The treatment of syringomyelia includes many surgical options. We report a retrospective study of 65 patients with cavitary lesions of the spinal cord in whom the results of magnetic resonance imaging were used to develop specific treatment strategies. Intramedullary cavities were classified into three general types: 1) communicating syrinxes, which occurred with hydrocephalus and were anatomically continuous with the 4th ventricle (9 patients); 2) noncommunicating syrinxes, which were separated from the 4th ventricle by a syrinx-free segment of spinal cord (42 patients); and 3) atrophic syrinxes, which occurred with myelomalacia (14 patients). Noncommunicating syrinxes were further subdivided according to type: Chiari II malformations with hydrocephalus (5 patients), Chiari I malformations without hydrocephalus (11 patients), extramedullary compressive lesions (12 patients), spinal cord trauma (6 patients), intramedullary tumors and infections (6 patients), and multiple sclerosis (2 patients). Of the 65 patients, 39 underwent surgical treatment for progressive symptoms. Syrinxes occurring with hydrocephalus were treated empirically with a ventriculoperitoneal shunt. Excellent results were achieved in 7 of 7 patients with communicating syrinxes and in all 5 patients with Chiari II malformations. Two approaches were used in the treatment of syrinxes occurring with Chiari I malformations: Posterior fossa decompression improved symptoms but did not reduce syrinx size in 2 of 3 patients. In the third patient and in 3 patients who were not treated with decompression, shunting from the syrinx to the cerebellopontine angle cistern collapsed the cavity and resolved symptoms over the interval of follow-up (average follow-up, 1.5 years). Excision of extramedullary obstructions at the rostral end of noncommunicating syrinxes resulted in collapse or disappearance of the cavity in 6 of 7 patients. The remaining patient was treated effectively by a syringocisternal shunt. In all 4 patients with posttraumatic syringomyelia, good results were achieved by a spinal or syringocisternal shunt. Syrinxes associated with intramedullary masses were managed by biopsy or excision of the causal lesion and appropriate adjunctive therapy (6 patients). Patients with atrophic syrinxes were not operated upon except to relieve symptoms referrable to the causal lesion (4 patients). Recurrent syrinxes were not encountered in the 35 surviving patients over an average follow-up of 2.5 years. It is concluded that syringomyelia is a complex pathological disorder with several mechanisms of pathogenesis that requires a number of different treatment strategies.

Adolescent↗
PubMed1992

Syringomyelia in Chiari malformation: relation to extent of cerebellar tissue herniation.

In 47 patients with Chiari malformations (41 with Type I, 6 with Type II) diagnosed by magnetic resonance imaging, syringomyelia was found significantly more frequently in those who had a herniation of 9 to 14 mm (9 of 16, 56%) than in those who had a smaller (2 of 15, 13%) or larger (2 of 16, 13%) herniation. Syringomyelia associated with the Chiari malformation is generally considered to be due to mechanical factors induced by altered cerebrospinal fluid hydrodynamics. On the basis of the present study, one may speculate whether these mechanical factors affect the cord most efficiently at an intermediate level of herniation, which may prove to be a risk factor for the development of syringomyelia.

Adolescent↗
PubMed1995

Median nerve somatosensory evoked potentials in cervical syringomyelia: correlation of preoperative versus postoperative findings with upper limb clinical somatosensory function.

Median nerve somatosensory evoked potentials (SEPs) were recorded in 30 patients with cervical syringomyelia before and after surgery. The different SEP components were compared with clinical somatosensory findings. The N13 potential (generated in the dorsal horn at C5-C6) was pathological in 85% of the upper extremities, or 90% of the patients, and correlated with pain/temperature as well as vibration/joint position sense; it was of higher sensitivity in syringomyelia than any other clinical symptom or SEP component. P14 (brain stem) and N20 (postcentral cortex) were less often affected and correlated with only vibration/joint position sense. Short-term postoperative clinical or SEP changes were most often seen after syringoendoscopy and less often after syringostomy, resection of cerebellar tonsils, or tumor extirpation. Alterations of SEPs after surgery occurred in more patients (60%) than did changes in clinical condition (approximately 27%); there was, however, no general correlation between these findings. We conclude that median nerve SEP testing with a proper recording technique identifying the different subcortical components is a valuable supplement in the pre- and postoperative diagnostic evaluation of syringomyelia and is of higher sensitivity than clinical somatosensory examination alone.

Adult↗
PubMed1979

Orthopaedic features in the presentation of syringomyelia.

The orthopaedic surgeon is often the first consultant to whom a patient with syringomyelia is referred. The disease is not as rare as he may suppose, but its early presenting features are very variable; if he relies solely on such familiar features as pes cavus and scoliosis, he may well miss the diagnosis. The commonest presenting symptom is pain in the head, neck, trunk or limbs; headache or neckache made worse by straining is particularly significant. A history of birth injury also may suggest the possibility of syringomyelia, especially if any spasticity subsequently worsens. Neurological features which may be diagnostic include nystagmus, dissociated sensory loss, muscle wasting, spasticity of the lower limbs or Charcot's joints. Radiographic features include erosion of the bodies of cervical vertebrae and widening of the spinal canal; if, at C5, the size of the canal exceeds that of the body by 6 millimetres in the adult, pathological dilatation is present. The presence of basilar invagination or other abnormalities of the foramen magnum, of spina bifida occulta and of scoliosis are further pointers. Thermography is a useful way of showing asymmetrical sympathetic involvement in early cases. A greater awareness of the prevalence of syringomyelia may lead to earlier diagnosis and to early operation, which appears to hold out the best hope of arresting what is all too commonly a severely disabling and progressive condition.

Adolescent↗
PubMed2004

Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases.

We analyze the results of surgical treatment of 60 patients presenting syringomyelia (SM) associated with Chiari malformation (CM) who were operated in the period 1982-2000. For each case, analysis covered 15 signs and 16 symptoms included in a protocol that separated SM signs and symptoms from those of CM. A score system was established in parallel with the protocol to make the evaluation of treatment results easier. All cases were submitted to craniovertebral decompression by C1 and eventually C2 laminectomy and cerebellar tonsillectomy with duramater graft. To evaluate the results, statistical proportion difference tests and variance analyses were made to a reliability index of 95% (p=0.05). We conclude that the statistical improvement of CM signs and symptoms was very significant (p=0). Syringomyelia signs and symptoms also improved significantly, except for "upper limb hyporeflexia", which did not improve. No statistical difference in the improvement of SM symptoms as compared to CM symptom was found. Syringomyelia signs improved statistically more than CM signs. In half of patients, the percent improvement of signs and symptoms ranged between 40% and 60%.

Adolescent↗
PubMed1993

Asymptomatic syringomyelia associated with cerebellopontine angle meningioma--case report.

A 66-year-old female with a 3-year history of left trigeminal neuralgia presented with an unusual left cerebellopontine angle meningioma associated with asymptomatic syringomyelia at the C2 to C4 levels diagnosed by magnetic resonance (MR) imaging. Two months after total tumor removal, the syringomyelia had diminished without shunting. MR images are useful as a basis for early diagnosis of syringomyelia.

Aged↗
PubMed2002

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.

Adult↗
PubMed2004

Terminal syringomyelia: is it as innocent as it seems?--Case report.

A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.

Female↗
PubMed2006

Spontaneous resolution of syringomyelia without Chiari malformation.

A 30-year-old woman presented with a cervical syrinx manifesting as hemihypesthesia. Neuroimaging found no evidence of Chiari malformation or tight cisterna magna. Serial magnetic resonance imaging studies over a 6-year period demonstrated spontaneous and complete resolution of the syrinx accompanied by an asymptomatic clinical course. The natural history of syringomyelia is highly unpredictable. The outcome of surgical treatment for patients with syringomyelia is not always satisfactory, so the indications for surgery are controversial. Spontaneous resolution of syringomyelia unrelated with foramen magnum lesion has various causes. Close follow up of the patient is necessary to monitor for recurrence.

Adult↗
PubMed1987

The MR appearance of syringomyelia: new observations.

Fifty-eight patients with spinal cord cavities were studied with MR imaging. Patients were separated into four groups, and the appearance of the cavities were compared. There were 24 patients (41.4%) with communicating syringomyelia (associated with the Chiari I malformation). Sixteen patients (27.6%) had posttraumatic syringomyelia, nine patients (15.5%) had associated tumors, and nine patients (15.5%) had idiopathic syringomyelia. The characteristics of each syrinx, the spinal cord, and the appearance of the cerebellar tonsils were analyzed on T2- and T1-weighted images. There is a striking similarity in the appearance of many syrinx cavities regardless of the cause. Characteristics that were found in some patients in every group included areas of increased intensity on T2-weighted images, the presence of the CSF flow-void sign (CFVS) in the syrinx cavity, eccentric cavities, "beaded" cavities, and cord enlargement. Tonsillar ectopia alone does not indicate that a syrinx is of the "communicating" type, since it was present in two of 16 patients (13%) with trauma and in two of five patients (40%) with tumors. T1-weighted images were most useful in evaluating the anatomic characteristics of the syrinx and the cerebellar tonsils. Most syrinx cavities involved the cervicothoracic junction. The average length was between five and nine vertebral segments (depending on category) but varied between one and 20 vertebral segments. T2-weighted images revealed areas of increased intensity in the spinal cord in 13 patients without tumors. Two of these cases were shown to represent gliosis on histopathologic review. The CFVS was present in the syrinx cavities of 23 patients (40%), probably reflecting pulsatile movements of the syrinx fluid. It has been proposed that such movements are a cause of syrinx propagation, and the observation of the CFVS may have prognostic significance. The development and progression of the CFVS was documented in serial MR examinations in one patient over an 18-month period. The theories of syrinx development and propagation are reviewed.

Adolescent↗
PubMed1987

CSF-dynamics in syringomyelia: intracranial pressure and resistance to outflow.

The several theories on the pathogenesis of syringomyelia have not resulted in the satisfactory selection of those patients who can be treated by CSF diversion. In the present paper three types of syringomyelia are described by case studies. The classification is made by investigation of CSF-dynamics, a measurement of CSF pressure and resistance to outflow of CSF. It is proposed that in a subgroup of patients with syringomyelia the cause is defective CSF resorption and that this group may be selected out and treated accordingly.

Adult↗
PubMed1992

Syringomyelia in children: six case reports and review of the literature.

We present six cases of syringomyelia in children below the age of 16 years seen during the last 8 years at the neurosurgical departments in the west of Yorkshire. The clinical presentation of syringomyelia in children is different from that in adults and magnetic resonance imaging is the investigation of choice. Because of the natural history of syringomyelia, the long-term results of surgical treatment are very difficult to evaluate accurately. Although our series is small, it does highlight the presentations and the methods of investigation. The diversity of pathology is noted; in particular any associated hydrocephalus or intramedullary tumour.

Adolescent↗