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Cerebrospinal fluid (CSF) leakage is known to cause orthostatic headache. Spontaneous CSF leakage occurs under several conditions, such as lumbar puncture, spinal surgery, and fracture of the spine. Intrasacral meningocele is an anomaly caused by an abnormal prolongation of the meninges in the sacral spinal canal. Typical symptoms of this anomaly are low back pain, bladder dysfunction, and sciatica, possibly caused by tethered cord. We describe CSF leakage from an intrasacral meningocele without tethered cord syndrome, which caused severe orthostatic headache.
A 23 year old woman was admitted to hospital with the complaint of severe headache. She described a similar episode two years previously. There was no aura or trigger, nausea, vomiting, or fever. Non-steroidal anti-inflammatory drugs were not effective. By lying down she could get some relief from her splitting headache. Cranial magnetic resonance imaging (MRI) showed no abnormalities. On the first occasion, the headache gradually disappeared after lying down for several days.
On the day before admission, she experienced the headache again, and it was again relieved by lying down. Several days before the headache began, she had fallen on her buttock during skiing. No prominent external injury was found. Her temperature was normal. On neurological evaluation, her neck was not stiff. Kernig and Lasegue signs were negative. There was no muscular weakness or sensory disturbance. She had never experienced bladder or rectal disturbance.
Lumbar puncture yielded an opening pressure of 60 mm H2O. The CSF contained 7 mononuclear cells/mm3; the glucose concentration was 95 mg/dl and the protein concentration was 85 mg/dl.
Cranial magnetic resonance imaging (MRI) using gadolinium (fig 1, lower right panel) did not show dural enhancement, subdural haematoma, or Chiari malformations. The cervical and thoracic regions were normal. However, a meningocele in the sacrum was found, and the caudal spinal canal was enlarged (fig 1, left). Computed tomographic (CT) myelography failed to show CSF leakage from the enlarged spinal canal (not illustrated). On the other hand, radioisotope cisternography demonstrated leakage of CSF around the meningocele. Twenty four hours after the injection, strong activity was found around the spinal canal just caudal to the most enlarged portion (fig 1, upper right panel). In the most enlarged portion of the meningocele, radioisotope accumulation was also found, and it seemed that clearance of the CSF was impaired in comparison with the upper portion of the spinal cord. Isotope clearance around the brain was normal.
The headache gradually improved without any treatment, and none was subsequently required.
Postural headache began after a fall during skiing. An intrasacral meningocele was found, and CSF leakage from the meningocele detected. CSF pressure was low normal. Intrasacral meningoceles are now detected more commonly because of the advent of MRI scanning. Although some are truly asymptomatic, many cause pain in the low back. Pain in the buttocks and legs may be the main symptom, and this seems to be caused by root compression or tethered cord syndrome.3 In our case, headache was the principal symptom, and the patient did not complain of anything else. It was not clear whether the fall precipitated the CSF leakage from meningocele. The patient could not remember any trauma when she experienced the first attack of the headache. However, a meningocele could be vulnerable to minor injury as it is surrounded by thin bone.
In this case, however, cranial MRI did not show any abnormalities commonly associated with intracranial hypotension.4 CT myelography is reported to be sensitive for detecting CSF leakage along the nerve root sleeves of the spine,2 but was negative in this case, although it helped establish the diagnosis. In our case it is probable that low volume but continuous CSF leakage, as demonstrated by radioisotope cisternography, was responsible for the intracranial hypotension. This case adds congenital meningocele to traumatic meningocele1 as a cause of CSF leakage and intracranial hypotension. It has been reported that CSF leakage from a meningocele was detected in a patient with a gunshot wound.1