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Shunting for syringomyelia in patients with spinal cord injuries: self-reported, long-term effects in 8 patients.

OBJECTIVE: To investigate the long-term effects of shunting on neurologic outcome of syringomyelia, a complication of spinal cord injury (SCI). DESIGN: Retrospective data collection using telephonic survey. SETTING: University based medical center. PARTICIPANTS: Eight of 15 patients who had shunts placed between 1976 and 1999. INTERVENTIONS: Review of clinical records and self-reported telephone interview. MAIN OUTCOMES MEASURES: Initial clinical presentation, recovery after shunt placement, and subsequent neurologic status were recorded. Patients were asked to rate symptomatic improvement after surgery on a 100-point analog scale; they were also asked whether they would have the surgery again. RESULTS: Presenting symptoms were weakness in all 8 patients, pain in 5 patients, and numbness in 5 patients. After shunting, 6 patients had improved strength, all had less pain, and 2 had less numbness. Six patients experienced neurologic decline an average of 2 years after shunt placement, 3 because of shunt failure, 2 from a new syrinx, and 1 from both shunt failure and new syrinx. Two of the 3 patients who underwent second shunt placements for a new syrinx had full neurologic recovery. Six patients said they would repeat the shunting procedure if necessary, 1 was uncertain, and 1 would not. CONCLUSION: Initial improvements noted after shunting provide long-lasting effects, even though many patients may need a second procedure. Less pain and improved strength are more significant than decreased numbness.

Cerebrospinal Fluid Shunts↗

Arachnoiditis ossificans and syringomyelia: a unique case report.

A 62-year-old male presented with progressive quadriparesis. Magnetic resonance imaging of the spine revealed a spinal cord syrinx but failed to detect extensive arachnoiditis ossificans noted on insertion of a syringopleural shunt. A postoperative computed tomography scan clearly demonstrated the abnormality and its extent. We present a rare case of syringomyelia resulting from spinal arachnoiditis ossificans and review the relevant literature.

Arachnoiditis↗

[Osteoarthrosis due to syringomyelia and its misinterpretation (author's transl)].

The development of severe deforming osteoarthrosis due to syringomyelia was observed over a period of 25 years; the changes are illustrated by a series of radiographs. A striking feature of this case, as in others, was the relative lack of symptoms complained of by the patient, when compared with the extensive joint destruction. It is necessary, if there is destruction of the shoulder, elbow or carpal joints, to consider a neuropathic cause and to take a very careful history, which unfortunately was not always done in our patients. Comparative radiographs of the opposite side should always be obtained.

Aged↗

Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy.

This short paper illustrates a case with cervical myelomeningocoele, a Chiari malformation (CM), hydrocephalus (HC) and cervical syringomyelia treated by neuroendoscopic third ventriculostomy (NTV) with resolution of both the hydrocephalus and the syrinx. Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions.

Arnold-Chiari Malformation↗

[Charcot's elbow joint in syringomyelia].

Neuropathic arthropathy is a rare case in daily orthopedic practice. The case of a 43 year old technician is reported, who developed severe destruction of both elbow joints as a result of syringomyelia. The diagnostical problems as well as the therapeutical considerations are discussed.

Adult↗

[Neuropathic joint changes in syringomyelia. Problems in diagnosis and therapy based on a personal case].

This is a report of a patient with syringomyelia and septic Charcot joint of the left elbow. In cause of the inflammatory condition we performed an arthrodesis of the elbow. In spite of postoperative complications with fracture of the humerus and ulna we obtained a satisfactory result of treatment with weight-bearing arthrodesis and stop of the joint sepsis.

Arthritis, Infectious↗

[Magnetic resonance tomography in syringomyelia].

Thirteen patients with a clinical diagnosis of syringomyelia were examined by nuclear tomography (0.35 T magnet) in the spin-echo mode. In all thirteen patients, the T1 images (SE 400/35) showed a longitudinal cavity with a signal intensity of CSF. The shape and extent of the syrinx could be adequately demonstrated in 12 of the 13 examinations. Downward displacement of the cerebellar tonsils was seen in eight cases. The examination took between half and one hour. Advantages of magnetic resonance tomography (nuclear tomography) include the absence of artifacts, images in the line of the lesion and its non-invasiveness.

Adult↗

[Significance of reprocessed 3D gradient echo sequences in spinal MRT for surgical interventions in syringomyelia].

Taking a patient with advanced syringomyelia as an example, the article presents a method in which reprocessed 3D-gradient echo sequences from spinal MRI allow centred reconstructions which show all sections of the syrinx, even where the spinal column is curved. This method played an essential role in satisfactorily determining the position of the syrinx for a median myelotomy which, according to clinical criteria, brought about an effective reduction in pressure from the syrinx.

Adult↗

[Syringomyelia as a rare cause of respiratory insufficiency requiring ventilation].

For 17 years a now 45-year-old man had suffered from progressively more severe flaccid paresis of the arms and thoracic muscles, spastic paralysis of the legs and kyphoscoliosis. Artificial ventilation was required when he contracted pneumonia. Although it was being treated with antibiotics, frequent bronchoalveolar lavage had to be done because of repeated atelectases. After 6 weeks clonuses developed in the legs, predominantly on the right, stretch synergisms and opisthotonos. The pupils were small with sluggish reaction to light, and there was a positive "doll's head" phenomenon. The level of consciousness alternated between somnolence and sleepiness. Magnetic resonance imaging demonstrated cavities in cervical and thoracic spinal cord, supporting the diagnosis of an abnormal cerebrospinal circulation due to gliosis in syringomyelia. To secure cerebrospinal fluid drainage, the cerebellar tonsils were resected, together with a duraplasty and partial resection of the atlas. Following this he became fully conscious and the spastic state improved. During the following 8 weeks it became possible gradually to wean him from the artificial ventilation and achieve satisfactory mobilization so that he could be discharged to domiciliary care.

Combined Modality Therapy↗

Electrophysiologic findings in post-traumatic syringomyelia: implications for clinical management.

Traumatic spinal cord injured (SCI) patients may develop pain, new weakness and/or sensory loss due to an enlarging fluid-filled cyst in the spinal cord. The clinical history and physical exam are nonspecific and insensitive, particularly for diagnosing and monitoring recurrent or progressive post-traumatic syringomyelia (PTS). We compare the sensitivity and specificity of three electrodiagnostic tests, median and ulnar F waves, electromagnetic motor evoked potentials (MEP), and needle electromyography, with respect to syrinx by imaging (MRI or CT scan) and neurologic progression on serial clinical exams. Central motor conduction time (CMCT) calculated from scalp and spine MEP was the most sensitive diagnostic test. F waves were less sensitive and less specific than the CMCT, and did not provide evidence of syrinxes in the mid or upper cervical cord. Positive sharp wave and fibrillation potentials were the least sensitive and least specific. The CMCT is a useful adjunct to imaging studies for diagnosing and monitoring PTS.

Adult↗

Arachnoid telangiectasia causing meningeal fibrosis and secondary syringomyelia.

A case of syringomyelia secondary to arachnoiditis associated with arachnoid telangiectasia is reported in a female patient with no other stigmata of hereditary haemorrhagic telangiectasia. Such a case has not been reported before. She underwent surgical decompression of the spinal cord with successful outcome.

Arachnoiditis↗

Paget's disease complicated by hydrocephalus and syringomyelia.

The authors report a case of Paget's disease involving the skull with the rare but severe complications of hydrocephalus and syringomyelia related to basilar impression. Complications of Paget's disease of the skull are briefly discussed and current treatment strategies outlined.

Cervical Vertebrae↗

Neurolisteriosis causing hydrocephalus, trapped fourth ventricle, hindbrain herniation and syringomyelia.

Central nervous system infection by Listeria monocytogenes is relatively uncommon, but is known to be highly morbid and fatal. We describe a case of listeria meningoencephalitis, wherein the acute episode was followed by a hitherto unreported conglomeration of severe and progressive neurological sequelae, in the form of supratentorial hydrocephalus, aqueduct block, trapped fourth ventricle, hindbrain herniation and syringomyelia. Pertinent literature is reviewed and the pathogenesis of the observed sequelae is explored.

Adult↗

Cervical spondylotic myelopathy with syringomyelia.

Syringomyelia may complicate cervical spondylotic myelopathy (CSM). We describe a case of CSM with instability at C3/4 and a cervicothoracic syrinx which was demonstrated using magnetic resonance imaging. Decompression and stabilization, without drainage of the syrinx, were adequate surgical treatment.

Aged↗

Feasibility and safety of neural tissue transplantation in patients with syringomyelia.

Transplantation of fetal spinal cord (FSC) tissue has demonstrated significant potential in animal models for achieving partial anatomical and functional restoration following spinal cord injury (SCI). To determine whether this strategy can eventually be translated to humans with SCI, a pilot safety and feasibility study was initiated in patients with progressive posttraumatic syringomyelia (PPTS). A total of eight patients with PPTS have been enrolled to date, and this report presents findings for the first two patients through 18 months postoperative. The study design included detailed assessments of each subject at multiple pre- and postoperative time points. Outcome data were then compared with each subject's own baseline. The surgical protocol included detethering, cyst drainage, and implantation of 6-9-week postconception human FSC tissue. Immunosuppression with cyclosporine was initiated a few days prior to surgery and continued for 6 months postoperatively. Key outcome measures included: serial magnetic resonance imaging (MRI) exams, standardized measures of neurological impairment and functional disability, detailed pain assessment, and extensive neurophysiological testing. Through 18 months, the first two patients have been stable neurologically and the MRIs have shown evidence of solid tissue at the graft sites, without evidence of donor tissue overgrowth. Although it is still too soon to draw any firm conclusions, the findings from the initial two patients in this study suggest that intraspinal grafting of human FSC tissue is both feasible and safe.

Adult↗

Neurophysiological assessment of the feasibility and safety of neural tissue transplantation in patients with syringomyelia.

The feasibility and safety of a procedure involving fetal spinal cord tissue transplantation in patients with syringomyelia was assessed using a neurophysiological protocol designed to quantitate peripheral nerve function, spinal cord reflex excitability, and spinal cord conduction pathways essential for somatosensory evoked potentials. We report here data obtained before and for 18 months following the transplantation procedure performed on the first two patients in this study. The neurophysiological assessment protocols included measures of cortical and spinal cord evoked potentials, H-reflex excitability, and peripheral nerve conduction. Prior to the procedure, both patients had significant deficits on some of the neurophysiological measures, for example, lower extremity cortical evoked potentials. However, robust measures of intact pathways, such as upper extremity cortical evoked potentials, were also observed preoperatively in both patients. Thus, it was anticipated that conduction in these intact pathways could be at risk either from complications from the transplantation procedure and/or from continued expansion of the syrinx. Following the transplantation procedure, no negative changes were observed in any of the neurophysiological measures in either patient. In addition, patient 1 showed a decrease in the rate potentiation of tibial H-reflexes on the right side and an increase in the response probability of left tibial H-reflexes. The results of this postoperative longitudinal assessment provide a first-level demonstration of the safety of the intraspinal neural tissue transplantation procedure. However, the consideration of safety is currently limited to the grafting procedure itself, since the long-term fates of the donor tissue in these two patients remain to be shown more definitively.

Action Potentials↗

Cerebral potentials evoked by painful, laser stimuli in patients with syringomyelia.

Brief cutaneous heat stimuli generated by a CO2 laser were used to elicit late somatosensory evoked cerebral potentials (SEPc) in 10 patients with syringomyelia. For comparison, early and late cerebral potentials in response to electrical nerve stimuli (SEPn) were recorded in the same session. In 8 patients with localized impairment of pain and temperature sensitivity we found complete absence of SEPc after stimulation of the affected area; in another patient with similar sensory deficits, the SEPc was grossly attenuated and delayed. In 1 patient with intact pain sensitivity but absent temperature sensitivity, a well defined SEPc could be recorded. Both early cortical SEPn and late SEPn in response to conventional nerve stimuli were normal in all patients and thus did not differentiate control and affected areas. These data indicate that alteration of SEPc correlates with altered pain sensitivity in patients with a circumscribed spinal lesion. SEPc may thus be used as a neurophysiological test in the assessment of hypalgesic dermatomes.

Brain↗