A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia.

STUDY DESIGN: A case report. SETTING: Department of Orthopaedic Surgery, Shiga University of Medical Science, Japan. PATIENT: A 13-year-old woman presented progressive weakness in the lower extremities, with predominance on the right. Magnetic resonance (MR) imaging revealed a huge syrinx. The patient also showed scoliosis, cleft palate, hearing impairment, excessive sweating, hairiness, dural ectasia, and malformation of the skull. METHOD AND OBJECTIVES: We treated a very rare case of Arnold-Chiari syndrome, which presented with flaccid paralysis. Methods of differential diagnosis and suitable treatment are discussed. RESULTS AND CONCLUSION: Both the syrinx and muscle strength were quickly improved following placement of a syringo-peritoneal (S-P) shunt, after which the patient recovered the ability to walk. However, transient hypesthesia in the right hand occurred after the operation. The syrinx around the conus was thought to play a crucial role in the etiology of the patient case, which showed unique symptoms.

Spontaneous atlantoaxial rotatory fixation in old age after cerebral infarction: case report.

STUDY DESIGN: Case report of spontaneous Fielding and Hawkins Type I atlantoaxial rotatory fixation in a 78-year-old man after hemiplegia and homonymous hemianopsia caused by cerebral infarction. OBJECTIVES: To describe a case of spontaneous atlantoaxial rotatory fixation in old age and review previous adult cases of atlantoaxial rotatory fixation without fracture. SUMMARY OF BACKGROUND DATA: Atlantoaxial rotatory fixation in adults is a relatively rare finding and is mainly caused by trauma. To the author's knowledge, there has been no previous report of spontaneous atlantoaxial rotatory fixation in old age. METHODS: The patient's head was fixed in a 40 degrees left-rotated position. Left hemiplegia and homonymous left-side hemianopsia developed due to cerebral infarction. Computed tomography of the cervical spine clearly showed rotatory fixation of the atlas on the axis. RESULTS: Successful reduction was obtained after 1 day of skull traction. CONCLUSIONS: It was hypothesized that repeated left-rotational stress due to homonymous hemianopsia loaded to the atlantoaxial joint caused abnormal laxity of the joint.

A long-term follow-up study of cervical spondylotic myelopathy treated by "French window" laminoplasty.

We investigated 30 patients with multilevel cervical spondylotic myelopathy (22 males and 8 females) treated by "French window" laminoplasties from 1979 to 1988. Patients averaged 62.6 years of age, and were followed an average of 5 years and 2 months. The average preoperative and postoperative Japanese Orthopaedic Association (JOA) scores were assessed, rising from the original 8.8 +/- 3.4 to 11.9 +/- 3.3 (p < 0.001). Patients were divided into two groups, demonstrating poor alignment and no malalignment after surgery. There was no statistical difference regarding improvement in the neurological examinations between these two groups. The "French window" laminoplasty achieved good clinical results in the management of cervical spondylotic myelopathy irrespective of whether or not alignment deteriorated in the postoperative period.

Clinical outcomes of cervical spinal cord injuries without radiographic evidence of trauma.

We investigated 33 cervical spinal cord injury patients (25 males and eight females) without bony injury. Patients whose neurologic recovery had reached a plateau and who had evidence on imaging of persistent spinal cord compression were considered candidates for surgical decompression. When imaging did not show spinal cord compression or patients were maintaining a good neurologic recovery from the early days after injury, we pursued conservative treatment. Age at injury varied from 20 to 76 years (mean, 55.6). Average follow-up was 31 months. Twelve patients were treated conservatively (Group 1). Groups 2 and 3 had surgery. Group 2 (14 cases) had multi-level compression of spinal cord due to pre-existing cervical spine conditions such as ossification of posterior longitudinal ligament, cervical canal stenosis, and cervical spondylosis. Group 3 (7 cases) patients existed single-level compression of spinal cord by cervical disc herniations or spondylosis. We evaluated clinical results according to the Frankel classification, the American Spinal Injury Association (ASIA) scales and Japanese Orthopaedic Association (JOA) scores. Overall improvement of JOA and ASIA scores after treatment was 56.3 +/- 35.5% and 67.1 +/- 38.0%, respectively. Patients in Group 1 showed very good recovery after conservative treatment, with improvement of JOA and ASIA scores being 70.4 +/- 40.2% and 77.4 +/- 34.2%, respectively. The average interval between injury and operation was 4.3 +/- 4.4 months. The improvement of the surgically treated patients (Groups 2 and 3) in JOA and ASIA score was 48.2 +/- 30.7% and 61.2 +/- 39.6% respectively. We obtained good neurological recovery after operation, with significantly more improvement in Group 3 than in Group 2. No significant neurologic recovery had occurred preoperatively in these groups. In such patients operative intervention is essential for neurologic recovery.

Rupture of the Achilles tendon in rheumatoid arthritis with histologic evidence of enthesitis. A case report.

Spontaneous Achilles tendon rupture occurred in a 60-year-old man with a two-year history of sero-positive rheumatoid arthritis (RA). Histologic section of the ruptured Achilles tendon revealed the existence of rheumatoid granulation that consisted of lymphocytes, histiocytes, and small vascular proliferation within the tendon tissue. The enthesis of the tendon to the calcaneus also revealed lymphocyte infiltration in the bone marrow and rheumatoid granulation within the tendon. Throughout the course of his disease, the patient had not been treated with corticosteroids. These facts suggested that enthesis (attachment of tendon to bone) was one of the extraarticular sites of the rheumatoid inflammation and that enthesitis was a possible cause of Achilles tendon rupture in this RA patient.