Background: Craniovertebral junction (CVJ) anomalies involve mosaic interaction of multifaceted neurovascular and bony elements. Most of them present late in the course of illness usually as acute presentations following trivial trauma. Knowing subclinical autonomic dysfunction in such anomalies when managed medically can not only indicate progression but also provide en route to early intervention for better outcomes, especially in relatively asymptomatic patients. Materials and Methods: We conducted a 6-year longitudinal prospective study including 40 consecutive patients of CVJ anomalies with clinical, radiological, and heart rate variability (HRV) parameters and found their correlation in preoperative and follow-up period. Results: Twenty-eight patients were male and the rest were female. The mean age was 32 years with the least age being 8 years and maximum age being 75 years old. Mean Nurick's grade and Barthel's index were 1.8 and 83.75, respectively. 38% had severe-to-moderate compression. The mean follow-up was 17.4 months. Both sympathetic and parasympathetic oscillator HRV indices were significantly affected in the preoperative period ( P ≤ 0.001) with no association with Nurick's grade or degree of compression although there was association with grade of Barthel's index. Poincare plots showed “fan,” “complex,” or “torpedo” patterns in 36 patients. Forty patients had both preoperative and follow-up clinical grade whereas 22 patients HRV tests in the above periods. None of the HRV indices showed significant improvement at follow-up. Nonetheless both sympathetic and parasympathetic did improve at follow-up with sympathetic tone registering better scores. Poincare plots showed improvement toward “comet” patterns in all patients. Conclusion: HRV indices not only help in prognosticating but may also help in predicting outcomes.

Background Autonomic nervous system (ANS) is invariably affected by craniovertebral junction (CVJ) anomalies. The usual presentation is sudden after trivial trauma. When symptomatic, most of this autonomic dysfunction is clearly elicited clinically with bedside tests. Nonetheless, ANS functionality in relatively less symptomatic or asymptomatic patients is not known as no studies exist. Methodology: We performed a longitudinal prospective study of 40 less symptomatic patients who underwent surgery with conventional autonomic function tests (AFT) in pre- and post-operative periods. Correlation of its association with such anomalies is studied. Results: All 40 had both pre- and post-operative clinical follow-up, pre-operative AFT, whereas only 22 patients had follow-up AFT. The mean age for the group was 32 years and male: female ratio was 2.3:1. Mean Nurick's grade was 1.8, whereas Barthel's index was 83.75%. Clinical improvement was seen in almost 98% at follow-up. Orthostatic test showed a significant association with Nurick's grade. Barthel's index was significantly associated with degree of compression. The mean follow-up was 17.4 months. Most conventional AFTs were significantly decreased in the preoperative period ( P ≤ 0.01). Both parasympathetic and sympathetic tone improved on follow-up with better improvement later. Overall clinical involvement of ANS was seen in 22.5% whereas subclinical involvement in the form of AFT impairment was seen in 100%. Conclusion: There is a definite involvement of subclinical ANS in all patients of CVJ anomalies irrespective of their symptomatology. Knowing the extent of involvement in the preoperative period can help prognosticate, prioritize regarding surgery as well as correlate with the extent of improvement.