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Anooj Chatley, Raj Kumar, Vijendra K. Jain, Sanjay Behari and Rabi Narayan Sahu

Object

The presence of intramedullary T2 high signal intensity changes in patients with cervical spondylotic myelopathy (CSM) indicates the existence of a chronic spinal cord compressive lesion. However, the prognostic significance of signal intensity changes remains controversial. The purpose of this study was to evaluate the effect of spinal cord T2 signal intensity changes on the outcome after surgery for CSM.

Method

In a prospective study, 64 patients with CSM who underwent surgical treatment between October 2006 and April 2008 using an anterior approach were included. Based on the clinical symptoms and signs present, the severity of neurological deficits of all patients was scored according to a modified Japanese Orthopaedic Association scale score for CSM just before the surgery and at 6 months follow-up. Recovery rates were calculated at 6 months.

Results

There were 22 patients who did not have spinal cord intensity changes on MR imaging and 44 who demonstrated high-intensity signal changes on T2-weighted images (focal or segmental). No statistically significant differences were found in recovery rates between cases with T2 signal intensity changes and those with no signal intensity changes. However, the postoperative modified Japanese Orthopaedic Association scale scores and the recovery rates were much lower in patients with multisegmental signal intensity changes compared with those without these changes or those with focal signal intensity change, and ANOVA demonstrated this difference to be statistically significant (p < 0.05).

Conclusion

Multisegmental spinal cord signal intensity changes on T2-weighted MR imaging are predictors of a poor outcome in terms of functional recovery rate in patients undergoing operations for CSM.

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Vijendra K. Jain, Piyush Mittal, Deepu Banerji, Sanjay Behari, Rajesh Acharya and Devendra K. Chhabra

✓ Between 1989 and 1994, 50 patients suffering from congenital atlantoaxial dislocation with either an assimilated atlas or a thin or deficient posterior arch of the atlas were treated with occipitocervical fusion using the technique described by Jain and colleagues in 1993 with a few modifications. An artificial bridge created from the occipital bone along the margin of the foramen magnum was fused to the axis using sublaminar wiring and interposed strut and lateral onlay bone grafts. Ten patients (20%) also underwent atlantoaxial lateral joint fusion by intraarticular instillation of bone chips. In 22 patients (44%) with irreducible dislocation, posterior fusion was preceded by transoral odontoidectomy. In seven patients (14%) with ventral compression, who showed marked clinical improvement on traction despite radiological evidence of persisting atlantoaxial dislocation, occipitocervical fusion was performed without ventral decompression. Seven patients (14%) underwent a single-stage transoral odontoidectomy and posterior fusion. There was no perioperative mortality and the osseous fusion rate was 88%. Of the 43 patients available at follow-up examination (range 3–12 months), 31 patients (72.09%) improved, seven (16.28%) remained the same, and five (11.6%) deteriorated in comparison with their preoperative status. Hence, this technique achieves a stable occipitocervical arthrodesis without supplemental external orthoses and facilitates early postoperative mobilization.

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Harsh Deora, Kuntal Kanti Das, Awadhesh Jaiswal and Sanjay Behari

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Sanjay Behari, Himanshu Krishna, Marakani V. Kiran Kumar, Vijay Sawlani, Rajendra V. Phadke and Vijendra K. Jain

✓ Basilar artery (BA) aplasia when unaccompanied by a primitive carotid—vertebrobasilar anastomosis is exceedingly rare. The association of BA aplasia with two aneurysms on the dominant posterior communicating artery (PCoA) has not been previously reported.

This 40-year-old man presented in a state of drowsiness and responded to simple commands only after being coaxed. He had complete left cranial third nerve palsy, right hemiparesis, and persisting signs of meningeal irritation. A computerized tomography (CT) scan revealed subarachnoid and intraventricular hemorrhage. An angiogram revealed BA aplasia. The right PCoA followed a sinuous course with multiple loops and provided the dominant supply to the posterior circulation. This vessel harbored two aneurysms, one at the origin of the PCoA from the internal carotid artery and the other at the looping segment just proximal to the brainstem. The left PCoA was extremely thin. The pterional transsylvian approach was used to clip the two aneurysms on the PCoA.

The hemodynamic changes produced by the BA aplasia may have produced alterations in the cerebral vasculature leading to aneurysm formation and consequent subarachnoid hemorrhage.

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Mandakini Pradhan, Sanjay Behari, Samir K. Kalra, Priti Ojha, Sarita Agarwal and Vijendra K. Jain

Object

Genetic mechanisms of atlantoaxial dislocation (AAD) have not previously been elucidated. The authors studied association of polymorphisms in the methylenetetrahydrofolate reductase (MTHFR) gene, which encodes enzymes of the folate pathway (implicated in causation of neural tube defects [NTDs]), in patients with AAD.

Methods

Molecular analysis of MTHFR polymorphisms (677C→T, cytosine to thymine and, 1298A→C, adenine to cytosine, substitutions) was carried out using polymerase chain reaction and restriction enzyme digestion in 75 consecutive patients with AAD and in their reducible (nine patients, 12%) and irreducible (66 patients, 88%) subgroups. Controls were 60 age- and sex-matched patients of the same ethnicity. Comparisons of genotype and allele frequencies were performed using a chi-square test (with significance at p < 0.05).

Results

The CT genotype frequency of MTHFR 677C→T polymorphism was significantly increased in the full group of patients with AAD (odds ratio [OR] 3.00, 95% confidence interval [CI] 1.28–7.14, p = 0.005) as well as in the irreducible subgroup (OR 2.81, 95% CI 1.17–6.86, p = 0.01). The frequency of T alleles was also higher in the AAD group (25.3%) than in controls (15%). The comparison of the combined frequency of CT and TT genotypes with the frequency of the CC genotype again showed significant association in AAD (OR 2.63, 95% CI 1.98–5.90, p = 0.009) and the irreducible (OR 2.5, 95% CI 1.1–5.74, p = 0.016) subgroup. There was, however, no significant association of MTHFR 1298A→C polymorphism with AAD.

Conclusions

Both MTHFR 677C→T polymorphism and higher T allele frequency have significant associations with AAD, especially the irreducible variety. Perhaps adequate supplementation of periconceptional folic acid to circumvent effects of this missense mutation (as is done for prevention of NTDs) would reduce the incidence of AAD.

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Pramod Janardhan Giri, Manish Singh Sharma, Awadhesh Kumar Jaiswal, Sanjay Behari and Vijendra Kumar Jain

✓ Lumbar disc herniation and spinal epidural hematomas (SEHs) are highly unusual causes of secondary lumbar canal stenosis in the adolescent population. The authors report a unique concomitant occurrence in a 16-year-old boy who presented with left-sided L-5 radiculopathy. Magnetic resonance imaging T1-weighted sequences revealed a left-sided posterolateral prolapsed L4–5 disc with an isointense extruded fragment lying behind the L-5 body. On T2-weighted sequences a hyperintense area was seen in the region of the extruded disc fragment with thecal compression. At surgery the extradural encapsulated hematoma was removed, together with the extruded disc fragment and the L4–5 disc. The characteristics of the biopsy specimen from the epidural collection were consistent with those of a hematoma. At 6 months’ follow up, the patient had returned to his normal activities. An SEH may result from tearing of delicate epidural veins following disc extrusion. It can occur at any age, regardless of whether there is a history of significant trauma. Magnetic resonance imaging allows preoperative characterization of the lesion. Results after surgical evacuation are excellent. Distinguishing between a solitary SEH and one caused by a lumbar disc extrusion has significant implications, as the former may resolve completely with conservative management.

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Pravin Salunke, Sanjay Behari, Markani V. Kirankumar, Manish S. Sharma, Awadesh K. Jaiswal and Vijendra K. Jain

Object

Clinicoradiological differences and outcome following surgery in pediatric patients (≤ 16 years of age) with congenital irreducible atlantoaxial dislocation (IAAD) and reducible atlantoaxial dislocation (RAAD) were analyzed.

Methods

Ninety-six patients (57 with IAAD and 39 with RAAD) were categorized as follows: Grade I, no deficits except hyperreflexia or neck pain (six patients); Grade II, minor deficits but independent for activities of daily living (25); Grade III, partially dependent (30); and Grade IV, totally dependent (35). Patients with RAAD underwent direct posterior fusion, and those with IAAD were treated with transoral decompression and posterior fusion. Patients with good outcomes included those who could walk unaided, with improvement in spasticity and weakness, and those who maintained Grade I status. The category of poor outcome included patients with the following conditions: postoperative deterioration or lack of improvement; inability to ambulate regardless of neurological improvement at a minimum follow-up duration of 3 months; or perioperative death.

A significantly higher incidence of C-1 assimilation, C2–3 fusion, asymmetrical occiput–C2 facet joints, and basilar invagination were seen in patients with IAAD, and os odontoideum was noted in those with RAAD (p < 0.05). A good outcome was recorded in 35 patients with IAAD and 22 with RAAD, whereas 14 with IAAD and nine with RAAD had a poor outcome (eight patients in each category were lost to follow up).

Conclusions

Radiological differences in the anatomy of patients with IAAD and those with RAAD may be due to improper segmentation of the occipital and upper cervical sclerotomes in the former and dysfunction of the transverse ligament in the latter. A significantly better outcome was noted in completely dependent patients with IAAD compared with those with RAAD. Respiratory compromise was an important prognostic factor.