Revisiting cruciate paralysis: A case report and systematic review.

OBJECTIVE: Cruciate paralysis is a rare, poorly understood condition of the upper craniovertebral junction that allows for selective paralysis of the upper extremities while sparing the lower extremities. Reported cases are few and best treatment practices remain up for debate. The purpose of this study was to conduct a systemic literature review in an attempt to identify prognostic predictors and outcome trends associated with cases previously reported in the literature.
MATERIALS AND METHODS: We conducted a systematic literature review for all cases using the term "Cruciate Paralysis," reviewing a total of 37 reported cases. All outcomes were assigned a numerical value based on examination at the last follow-up. These numerical values were further analyzed and tested for statistical significance.
RESULTS: Of the 37 cases, 78.4% were of traumatic causes. Of these, there were considerably worse outcomes associated with patients over the age of 65 years (P < 0.001). Those patients undergoing surgical treatment showed potentially worse outcomes, with a P value approaching significance at P = 0.08.
CONCLUSION: Numerous cases of trauma-associated cruciate paralysis have been reported in the literature; however, there remains a strong need for further study of the condition. While certain risk factors can be elicited from currently reported studies, insignificant data exist to make any sound conclusion concerning whether surgical intervention is always the best method of treatment.

Introduction

Termed first by Bell in 1970, “Cruciate Paralysis” is a rare neurological disease of the cervicomedullary junction.[1] Cruciate paralysis often presents with bilateral paresis of the upper extremities while sparing the lower extremities.[2] Patients may also present with difficulty breathing, cranial nerve deficits, or a comatose state.[345] While trauma is the most common cause of cruciate paralysis, the exact mechanism for these symptoms is not entirely understood.[36] The leading hypothesis involves disruption of the anatomy of the pyramidal decussation at the cervicomedullary junction.[3] The anatomical decussation extends longitudinally, spanning from the cervicomedullary junction to the C-2 level. Within this region, the motor tract fibers of the upper extremities cross both ventrally and superiorly to the fibers supplying the lower extremities. By crossing at a spatially different location, the independent upper extremity fibers provide a way for lesions to preferentially damage upper extremity fibers while sparing those of the lower extremities.[3] However, cruciate paralysis is a rare condition with few reported studies; hence, treatments have been variable and are often without supportive evidence.

Materials and Methods

In this report, we conducted a systematic literature review from 1966 to the present of patients diagnosed with cruciate paralysis to identify potential prognostic predictors for the outcome. Using MEDLINE and PubMed Central, a comprehensive search for all papers under MeSH and keyword term “Cruciate Paralysis” was performed. Additional information and cases were obtained through Google and Google Scholar, and appropriate search of relevant sources was performed using the same keywords. A case was included if it met the following criteria: (1) The paper under review demonstrated appropriate signs and symptoms of cruciate paralysis as defined above; (2) a mechanism of injury was noted; (3) the type of intervention and treatment was noted; and (4) a follow-up examination was documented. Cases with patients presenting in a comatose state were excluded along with papers written in languages other than English. Our study focused on patients who were noncomatose and carried the diagnosis of cruciate paralysis. This is due to the fact that different states of coma may affect appropriate examination of the upper and lower extremities and hence the diagnosis. We were able to identify 38 cases from the literature. One additional case treated at our institution was also added. Of the 39 cases initially found, 37 of them met our criteria [Table 1]. Follow up results were classified into three categories of recovery: Insignificant recovery, moderate recovery, or full recovery. A case was considered to have made a full recovery if, at the time of the last documented follow-up, upper extremity neurologic deficits had completely resolved. A case was considered to have made a moderate recovery if, at the time of the last follow-up, symptomatic improvement was documented, but residual upper extremity neurologic deficits still remained. Finally, a case was considered to have made an insignificant recovery if, at the time of the last follow-up, there was little to no change in upper extremity neurologic deficits since the time of initial presentation. Each category was assigned a numerical score of 1, 2, or 3, respectively, for simplicity of analysis. Treatments were further characterized into two groups: Those who underwent surgical intervention and those who did not. Each of the 36 cases was analyzed for outcome trends based on cause (trauma or nontrauma related), and an appropriate ANOVA test was run using the mean numerical scores of each category [Table 2]. The 28 cases associated with trauma were further analyzed, and respective ANOVA tests were run to determine trends and associations of outcomes categorized by age, gender, and type of intervention [Table 3].

Table 1

Clinical studies investigating the management of cruciate paralysis

Table 2

Percentage of cases making a full recovery, moderate recovery, or insignificant recovery by cause of symptoms

Table 3

Percentage of trauma cases (29 patients) making a full recovery, moderate recovery, or insignificant recovery by age, gender, and type of correctional intervention

Results

In patients who carried the diagnosis of cruciate paralysis and who were not comatose, the overall reported outcome was favorable with 54% of patients achieving full recovery and 29.7% of patients achieving moderate recovery.

The overall outcomes associated with cruciate paralysis secondary to trauma did not differ significantly from other nontraumatic causes, P = 0.5 [Table 2]. Since the majority of cases of cruciate paralysis were traumatic (29 patients, 78.4%), we analyzed factors that might impact outcomes of traumatic cruciate paralysis [Table 3]. Patients over the age of 60 years showed significantly worse outcomes as compared to those under the age of 60, P < 0.001. Similarly, patients in the both 0–20 and the 20–40 age ranges had statistically better outcomes when compared to the rest of the cohort, P = 0.02 and P = 0.02, respectively. Male patients also seemed to have slightly better outcomes on average than female patients, P = 0.08. Finally, patients treated without surgical intervention had better prognoses than those treated surgically but did not reach statistical significance, P = 0.08 [Table 3]. We included the details of the patient with traumatic cruciate paralysis that was treated at our institution in Figures 1–3.

Figure 1

A 59-year-old woman suffered a motor vehicle accident. She was intubated at the scene. Her neurological examination showed a motor strength of 1/5 in the upper extremities and 3/5 in the lower extremities. Sagittal T2-weighted sequence magnetic resonance imaging of the cervical spine demonstrating a Type III odontoid fracture with posterior subluxation causing compression of the cervicomedullary junction with upper cervical spine signal cord change

Figure 3

The patient underwent tracheostomy and percutaneous endoscopic gastrostomy tube placement. Her neurological examination continued to improve. Ultimately, the tracheostomy and the percutaneous endoscopic gastrostomy tubes were removed. She was kept in a crown halo vest for 6 weeks, followed by 6 weeks of rigid collar placement. During her 6-month follow-up visit, she was ambulating with a walker. Her motor strength in her proximal upper extremities improved to + 4/5. Her intrinsic hand function was 3/5 and she her speech was dysarthric. Sagittal computed tomography of the cervical spine revealed complete healing of the fracture

The patient was placed in crown halo traction and her fracture fragment was reduced as demonstrated by the lateral cervical spine X-ray (a). The patient then was placed in crown halo vest, and a magnetic resonance imaging of the cervical spine was done revealing reduction and realignment with decompression at the cervicomedullary junction as demonstrated with a sagittal T2-weighted sequence (b)

Discussion

Cruciate paralysis is a syndrome that results from cervicomedullary compression. It resembles central cord syndrome of the subaxial cervical spine, in that it usually affects the upper more than the lower extremities; however, since it is localized to the upper cervical spine, it is also associated with various degrees of lower cranial nerve palsies and at times states of coma.[15] Our review demonstrated that most cases are traumatic in nature with 78.4% of the cases reported. Overall, in the absence of coma, the outcome following this injury is favorable with 54% of patients achieving full recovery and 29.7% of patients achieving moderate recovery. Patients who were older than 60 years had a worse outcome than younger patients suffering from traumatic cruciate paralysis. While no concrete treatment recommendations have been suggested in the literature, due to the presence of a neurological deficit, severe cruciate paralysis has traditionally warranted surgical intervention.[312] Our review, however, showed that patients who were treated nonsurgically may have better outcomes with a P value of 0.08. This may reflect that patients who warrant surgical intervention may be sicker due to other associated injuries or suffer from a biomechanically unstable fracture requiring surgical intervention. Similarly, Dickman et al. recommended surgical intervention only be used for patients having more severe fractures with associated ligamentous instability of the atlantoaxial complex. Recommended procedures include posterior atlantoaxial arthrodesis and occipital-cervical fusion.[34814]

Our study has a few limitations; the cohort size is small in size since our search focused only on papers that included cruciate paralysis as a keyword and hence some papers that may have included patients with cruciate paralysis secondary to atlantooccipital dissociation and combination atlas and axis fractures were not included. Moreover, patients who were in a comatose state were excluded as well since it would be hard to ascribe coma due to an intracranial or upper cervical spine injury.

Conclusion

While numerous cases of trauma-associated cruciate paralysis have been reported in the literature, there remain insignificant data to make any sound conclusion concerning whether or not surgical intervention is always the best method of treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.