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Adult tethered cord syndrome presenting with refractory diarrhoea
Adult tethered cord syndrome presents with various neurological symptoms such as bladder/bowel disturbance, motor and sensory disturbance, and pain.1–7 A common bowel disturbance is constipation and incontinence, which may recover depending on the severity or duration of the symptoms.2,3 In adult tethered cord syndrome, recovery of bladder disturbance is generally only seen in patients with a relatively short clinical history; therefore early diagnosis and treatment are essential to achieve a good functional recovery.
We present a rare case of a patient who had suffered from refractory diarrhoea for years without a definite diagnosis and who was finally found to have tethered cord syndrome after bladder symptoms became apparent.
A 22 year old woman was admitted to our department of gastroenterology with a complaint of severe diarrhoea which had been occurring five to 10 times a day since she was 19. The cause of the diarrhoea could not be determined, even after a barium enema and endoscopy. The diarrhoea did not improve with drug treatment and diet modification. The patient also began to suffer from a urinary disturbance. Incomplete emptying of her urine had been verified when she was 21 years old. After this history, the patient was admitted to the department of urology, where a urodynamic study showed a neurogenic bladder. There was sphincter dysfunction and hypertonus of the external sphincter. Lumbar magnetic resonance imaging (MRI) was done and showed a thick tethering filum with intradural spinal lipoma in the sacral region (fig 1). There were no abnormal signs over the skin at the lumbosacral region. The spinal cord was untethered (fig 2), after which the refractory diarrhoea improved promptly and had almost completely resolved after six months. However the urinary disturbance remained and the patient had to perform self catheterisation.
Tethered cord syndrome with spinal lipoma usually presents with deterioration of motor, sensory, and autonomic nervous function caused by rostrocaudal traction on the spinal cord. The onset of symptoms commonly occurs in childhood.1–4,7 There have been a few reported cases of adult onset tethered cord syndrome, and the mechanisms of late onset are generally attributed to the degree of tethering and the cumulative stress of repeated microtrauma from exercise, especially any requiring a flexion position.2–3,5,6 The mechanical stress resulting from a tethered cord is usually localised in the lumbosacral region; thus most sensorimotor deficits appear in the lower limbs or in relation to bladder and bowel function. In urinary disturbances, some cases show a spastic small capacity bladder caused by supranuclear interruption, while others present with a hypotonic, large capacity bladder caused by dysfunction of the sacral autonomic nuclei.5 This suggests that longitudinal tensile stress within the cord may be transmitted even to remote regions of the cord, and various symptoms may appear that reflect the damaged part of the cord. Bowel dysfunction can also be attributed to this mechanism. Although most previous reports have mentioned either constipation or incontinence, in this case the diarrhoea was possibly caused by irritation of the digestive system related to parasympathetic upregulation or sympathetic inhibition. The surgical procedure of untethering the spinal cord and loosening the longitudinal tensile stress may alter the balance of sympathetic and parasympathetic bowel supply and affect the symptoms of bowel hypermotility. Because it is more difficult to evaluate bowel function objectively than to evaluate urinary dysfunction, bowel disorders—especially diarrhoea—are often regarded as functional disorders (irritable colon) if there is no obvious underlying disease. This is the probable reason why we could find so few reports on bowel dysfunction in tethered cord syndrome.2,5 The improvement in our patient’s diarrhoea after untethering the conus medullaris strongly suggests that the tethered cord was the primary cause of the symptom.
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