Among the space-occupying lesions within the vertebral canal, intradural extramedullary cysts of abnormal developmental origin are uncommon. In this paper, we report the occurrence of such a cyst in a young girl and review comparable lesions, cited in the literature, which caused symptoms similar to those in our case. In addition, observations are described on the presence of nuclear sex chromatin (Barr-chromatin) in the cells of such cysts and the significance of this finding with reference to the nature and origin of some of these cystic structures.
CASE REPORTA 6-year-old girl was admitted to the Neurological Unit, Boston City Hospital, in March 1955 because of pain in the back of the neck for four days. The pain had started suddenly without known preceding trauma. Episodes of relative comfort alternated with bouts of severe pain in the neck and upper back. There was no weakness of the arms, no gait disturbance, and no difficulties with bowel or bladder.The past medical history, growth and development, family history, and review of systems were normal.Physical examination on admission revealed an alert, well-built girl with normal vital signs. The child appeared anxious and uncomfortable, complaining of diffuse pain in the posterior part of the neck with the midscapular region and the inferior lateral portion of the right scapula as the areas of severest spontaneous pain. The pain had no definite pattern of radiation. The head was held stiffly, retracted and rotated to the right.General physical examination was unremarkable.'Supported by Grant RG 7855 of the United States Public HealthService.The cranium and cranial nerves were normal. There was no tenderness over the vertebral spinous processes.The patient resisted any attempt to active or passive motion of the cervical spine because of pain; attempted flexion of the neck caused considerable distress.The gait was abnormal with unsteadiness, absent associated movements of the arms, an increased lumbar lordosis, and a thoracic scoliosis with convexity to the left. There was some dragging of the right foot with circumduction. Muscle strength appeared considerably Radiological studies of the chest and skull were normal. The cervical spine showed loss of the normal curvature, the odontoid process was in the normal position, and there was no evidence of fracture. There was no evidence of anterior or posterior spina bifida.The following laboratory studies were normal: urine analysis, haemoglobin, white blood count and differential count, sickle cell preparation, sedimentation rate; Hinton, tuberculin skin test. The throat culture grew beta haemolytic streptococci.Results of three lumbar punctures during the patient's hospital stay gave the results shown below.Manometric studies during the third lumbar puncture were normal; flexion of the neck during this procedure, however, was limited because of pain. Cultures of the spinal fluid were negative.