Characteristics of idiopathic atlanto-axial subluxation: a comparative radiographic study in patients with an idiopathic etiology and those with rheumatoid arthritis.

OBJECTIVE: Atlanto-axial subluxation (AAS) is caused by multiple conditions; however, idiopathic AAS patients without RA, upper-cervical spine anomalies or any other disorder are rarely encountered. This study retrospectively investigated the radiographic findings in idiopathic AAS patients, and clarified the differences between those AAS patients and those due to RA. METHODS: Fifty-three patients with AAS treated by transarticular screw fixation were reviewed. The subjects included 8 idiopathic patients (ID group) and 45 RA patients (RA group). The study investigated the atlanto-dental interval (ADI) value and space available for spinal cord (SAC) at the neutral and maximal flexion position. RESULTS: The average ADI value at the neutral position in the ID and RA groups before surgery was 7.8 and 7.2 mm, respectively (p > 0.74). The average ADI value at the flexion position in the two groups was 10.3 and 11.7 mm, respectively (p > 0.06). The average SAC value at the neutral position in the two groups was 12.0 and 17.1 mm, respectively (p < 0.01). Finally, the average SAC value at the flexion position in the two groups was 10.7 and 13.5 mm, respectively (p < 0.01). CONCLUSIONS: The SAC value at both the neutral and flexion positions in idiopathic AAS patients was significantly smaller than those values in RA-AAS patients. This may be because the narrowing of the SAC in the idiopathic group easily induces cervical myelopathy. Furthermore, surgery was often recommended to RA patients, because of the neck pain induced by RA-related inflammation of the atlanto-axial joint, regardless of any underlying myelopathy.

Effect of a reduction of the atlanto-axial angle on the cranio-cervical and subaxial angles following atlanto-axial arthrodesis in rheumatoid arthritis.

PURPOSE: We retrospectively investigated the radiographic findings in patients with atlanto-axial subluxation (AAS) due to rheumatoid arthritis, and clarified the effect of reduction of the atlanto-axial angle (AAA) on the cranio-cervical and subaxial angles. METHODS: Forty-one patients, consisting of 29 females and 12 males, with AAS treated by surgery were reviewed. The average patient age at surgery was 61.0 years, and the average follow-up period was 4.0 years. We investigated the AAA at the neutral position in lateral cervical radiographs before surgery and at the last follow-up. In addition, we also investigated the clivo-axial angle (CAA) and the subaxial angle (SAA) at the neutral position before and after surgery. RESULTS: Due to pre-operative AAA, the patients were classified into three groups as follows: (1) the kyphotic group (K group), (2) the neutral group (N group), and (3) the lordotic group (L group). The average AAA values at the neutral position in the K group before and after surgery were 6.0° and 18.1°, respectively (P < 0.001). In the N group 19.7° and 21.7°, respectively (P < 0.05), and in the L group 31.6° and 27.0°, respectively (P < 0.01). However, no significant differences in the average CAA values were found before and after surgery in all groups. Furthermore, no significant differences in the SAA values were seen before and after surgery in all groups. CONCLUSIONS: A proper reduction of the AAA did not affect the cranial angles or induce kyphotic malalignment of the subaxial region after atlanto-axial arthrodesis. However, if we can obtain a significant and large reduction of AAA in patients showing kyphosis before surgery, then this reduction will be offset in the atlanto-occipital joint and we should therefore pay special attention to its morphology after surgery.

Atlanto-axial subluxation after pyogenic spondylitis of the atlanto-occipital joint.

This report presents a case of atlanto-axial subluxation after treatment of pyogenic spondylitis of the atlanto-occipital joint. A 60-year-old male had 1-month history of neck pain with fever. Magnetic resonance imaging showed inflammation around the odontoid process. Intravenous antibiotic therapy was administrated immediately. After 6 weeks, CRP had returned almost to normal. After 4 months, laboratory data was still normal, but the patient experienced increasing neck pain. Lateral cervical radiography in the neutral position showed instability between C1 and C2. Computed tomography showed a bony union of the atlanto-occipital joint and severe destruction of the atlanto-axial joint on the left side. Transarticular screw fixation for the atlanto-axial joint was performed. A lateral cervical radiograph in the neutral position after surgery showed a solid bony union. Neck pain improved following surgery. We speculate that spondylitis of the atlanto-occipital joint induced a loosening of the transverse ligament and articulation of the atlanto-axial joint. A bony fusion of the atlanto-occipital joint after antibiotic treatment resolved the pyogenic inflammation concentrated stress to the damaged atlanto-axial joint, resulting in further damage. The atlanto-axial instability was finally managed by the insertion of a transarticular screw.

Non-traumatic posterior atlanto-occipital joint dislocation.

This report presents a case of non-traumatic posterior atlanto-occipital dislocation. A 36-year-old female was referred with a history of numbness of the extremities, vertigo and neck pain for 1 year. The patient had no history of trauma. The axial rotation of range of motion of the cervical spine was severely restricted. A lateral cervical radiograph in the neutral position demonstrated a posterior atlanto-occipital dislocation. A coronal view on a computed tomography (CT) reconstruction image showed a loss of angle of the bilateral atlanto-occipital joint, and a sagittal reconstruction view of CT images also demonstrated flatness of atlanto-occipital joint. Instrumented occipito-cervical fusion was performed after reduction. A lateral cervical radiograph in the neutral position 1 year after surgery showed the reduction of atlanto-occipital joint, moreover, it was maintained even in an extended position. The patient had neurologic improvement after surgery. Flatness of the bilateral atlanto-occipital joint may have induced this instability. Occipital-cervical fusion was chosen in the present case since the patient showed restricted axial rotation of the neck before surgery. The surgery improved the preoperative symptoms including the function of cervical spine evaluated by JOACMEQ.

Impairment of spinal motor neurons in spinocerebellar ataxia type 1-knock-in mice.

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disorder caused by the expansion of polyglutamine repeats in the Ataxin-1 protein. An accumulating body of cerebellar, histological and behavioral analyses has proven that SCA1-knock-in mice (in which the endogenous Atxn1 gene is replaced with mutant Atxn1 that has abnormally expanded 154 CAG repeats) work as a good tool, which resembles the central nervous system pathology of SCA1 patients. However, the peripheral nervous system pathology of the model mice has not been studied despite the fact that the clinical manifestation is also characterized by peripheral involvement. We show here that spinal motor neurons are degenerated in SCA1-knock-in mice. Histologically, some spinal motor neurons of the SCA1-knock-in mice have polyglutamine aggregates in their nuclei and also thinner and demyelinated axons. Electrophysiological examinations of the mice showed slower nerve conduction velocities in spinal motor neurons and lower amplitudes of muscle action potential, compared to wild-type mice. Consistently, the mice displayed decrease in rearing number and total rearing time. These results suggest that the knock-in mice serve as a definite model that reproduces peripheral involvement and are therefore useful for research on the peripheral nervous system pathology in SCA1 patients.