[Show abstract][Hide abstract] ABSTRACT: Syringomyelia is a centromedullary syndrome that can be treated conservatively or with various neurosurgical procedures. We hypothesized that different clinical subgroups of patients exist, which would necessitate the need for individualised neurosurgical intervention and maintenance to achieve optimal quality of life (QoL). Using both the short-form 36-item (SF-36) questionnaire and the Syringomyelia Disability Index, clinical and QoL data was prospectively assessed in 142 patients with syringomyelia. Cluster analysis was then performed on the subscale results of the SF-36. The SF-36 scores of those with syringomyelia were significantly lower than those of the general German population, as well as when compared to those patients suffering from other chronic diseases. The SF-36 scores were independent of the syringomyelia patients' underlying syrinx pathology. Cluster analysis of the QoL patterns revealed four indicative patient groups. Syringomyelia is a chronic, progressive disease, and the syrinx itself appears to be the source of the symptoms, rather than the underlying pathology. The identified QoL subgroups in syringomyelia patients indicate the necessity of appropriate diagnosis and treatment of the pathology so that expansion of the syrinx cavity is reduced, maintaining QoL and functionality of these patients.
[Show abstract][Hide abstract] ABSTRACT: With the frequent use of magnetic resonance imaging (MRI), patients with subtle and diffuse symptoms due to small syrinx cavities increasingly present to neurosurgical care. In this respect, a dilated central canal, hydromyelia, must be separated from patients with true syringomyelia with an underlying disorder, as they do not share clinical and radiological features. We hypothesize that a differentiation of these two entities with distinct diagnostic tools is possible.
To describe the entity of hydromyelia, we excluded all patients from the syringomyelia database (n = 142) with any obvious cause of a syringomyelia, any objective neurological deficits on clinical examination, pathological results on electrophysiological monitoring (SSEP, MEP, silent periods) or a widening of the spinal cord cavity of more than 6 mm on MRI [routine acquisitions with FLAIR, T1/T2-weighted images, Cine and CISS (constructive interference in steady-state) studies]. Life quality was assessed through SF-36 questionnaires and an individualized questionnaire for the clinical history, pain and alternative therapies.
Forty patients (15 males/25 females) matched the criteria of a hydromyelia. With a mean age of 36.7 years (range 11-62), they almost all presented with pain (79%) or dysaesthesia of the limbs, with some having been an incidental finding (10%). Over a follow-up time of 36.9 months (range 6-93) there was no neurological or radiological deterioration.
Patients with a hydromyelia do not share clinical or radiological characteristics with patients harbouring a true syringomyelia. As hydromyelia does not represent a disease with an underlying pathology, no clinical or radiological progression has been seen. With sophisticated diagnostic tools to rule out any pathology this subset of patients can be identified.