Idiopathic cervical cord infarction in a young girl presenting with acute neck pain and flaccid paralysis: a case report

Abstract

Introduction

Spinal cord infarction (SCI) is a rare disease representing nearly 1% of all strokes with a wide variety of symptoms at presentation. SCI diagnosis is very challenging owing to its low incidence and the variety of symptoms, and could be misdiagnosed with neuromyelitis optica spectrum disorders (NMOSD).

Case presentation

We describe the case of an 18-year-old girl who presented to the emergency department with acute neck pain and flaccid paralysis of the left upper and lower extremities. Few hours later, she developed apnea and was endotracheally intubated. Brain MRI was normal but spinal cord MRI revealed non-enhancing longitudinal abnormal high T2 signal intensity extending from C1 to C5. The patient underwent steroid therapy with methylprednisolone (1 g daily for 7 consecutive days) combined with physiotherapy. She was extubated after 3 weeks and discharged after 30 days of hospitalization with a muscle force of 4/5 in her left extremities.

Discussion

Idiopathic SCI in adolescence is a rare but often devastating disorder with unknown pathophysiology, however, some specific conditions in adolescent such as mechanical stresses on the immature spine can be considered as risk factors for SCI development. Early diagnosis and treatment can improve outcomes in SCI.

Introduction

Spinal cord infarction (SCI) is a rare disease representing nearly 1% of all strokes but it may leave the patient with devastating neurological complications such as paraplegia or quadriplegia [1]. SCI diagnosis is made based on clinical symptoms and magnetic resonance imaging (MRI) findings including focal cord swelling and hyperintensities on T2-weighted images [2]. The neurologic manifestation of SCI depends on the affected level of the spinal cord and involved vascular territory. However, the abrupt onset of back and limb pain depending on the dermatomal level of the affected spinal cord followed by the development of neurological symptoms are the most common presentations [3]. From the pathological point of view, SCI is reported secondary to trauma, aortic disease, systemic hypoperfusion, cardiogenic embolism, vertebral artery dissection, and pathologies of the spine but the exact etiology is not identified in most cases [4].

Childhood SCI is less common and no epidemiologic data are available for SCI in children and young people. Because of its rarity, knowledge of the natural history of childhood SCI is based mostly on the evidence of case reports or studies mixing children and adults [5]. There are differences between adults and children in pathogenesis of SCI and the underlying etiologies in children are not well understood. Moreover, SCI can happen in healthy children in the absence of recognized risk factors and without any explanation [6]. A limited number of studies on early manifestations and outcomes of this idiopathic type of SCI in children exist and therefore its clinical course and MRI findings remain largely unknown. We herein present a case of acute SCI syndrome with an atypical presentation and no known risk factors in a young girl who presented to the emergency department and its management.

Case presentation

A 18-year-old girl presented to the emergency department (ED) complaining of acute neck pain and subsequent sudden onset weakness in the left side of the body about one hour before admission to the ED. Her past medical history was not significant and she reported no strenuous physical activity or stretching prior to the onset of pain. On the admission time to ED, the patient was alert and oriented with the ability to communicate verbally while she complained weakness of left side extremities. Her vital signs were normal including blood pressure of 130/71 mmHg, pulse rate of 98 beats per minute, regular rhythm, temperature of 36.9° C, and O2 saturation of 99%. In the physical examination, right side extremities had normal tone with 5 out of 5 strength but she had flaccid paralysis of the left upper and lower extremities with a decreased tone (3/5). Sensation was intact throughout and the result of electrocardiography was normal. Few hours later, she developed apnea and was intubated endotracheally with a 7.0 mm tracheal tube in the ED.

Initial diagnostic imaging including brain, cervical, and thoracolumbar MRI was performed. Brain MRI showed no inflammatory or hemorrhagic lesion but spinal cord MRI revealed a non-enhancing longitudinal abnormal high T2 signal intensity with minimal expansion in cervical cord extending from C1 to C5 (Fig. 1). The patient was visited by an attending neurologist in the ED and the initial neurologic differential was formulated as neuromyelitis optica (NMO), inflammatory/infectious myelitis, SCI, and acute transverse myelitis. Subsequently, she was transferred to the neurological ICU to initiate pharmaceutical therapy and work-up. Routine hematology and biochemistry laboratory data along with serum vitamin B12 were in the normal range but erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and interleukine-6 (IL-6) showed a significant increase. Meanwhile, specific lab tests were performed to rule out autoimmune diseases or hypercoagulable states including antinuclear and antineutrophil cytoplasmic antibodies (ANCA), lupus anticoagulant, and antiphospholipid antibodies that all were negative. Serum cell-based anti-aquaporin-4 (NMO) and anti-myelin oligodendrocyte (MOG) antibodies were negative and there were no oligoclonal bands (Table 1). Serologic markers for systemic vasculitis (MPO) and viral panel showed no abnormalities and cerebrospinal fluid (CSF) assessment revealed normal levels of protein and glucose without white or red blood cells. Intravenous methylprednisolone (1 g daily for 7 consecutive days) combined with physical rehabilitation was started and resulted in a significant improvement in muscle forces over the course of treatment.

Fig. 1.

Sagittal and axial T-2 weighted MRI images of the spine revealing a non-enhancing longitudinal hyperintensity in cervical cord at C1-C5 level.

Table 1.

Laboratory biomarkers of the patient at admission.

Variable	Reference value	Result
IL-6	< 50 pg/ml	2456
Anti-thrombin-3	17–30 mg/dl	25.8
Factor 5 Liden	> 120 s	144 (negative)
R.A. Factor	0–10 Iu/ml	< 10
Protein C	70–140%	125
Protein S	55–160%	94
MOG antibody	±	Negative
NMO antibody	±	Negative
MPO antibody (PANCA)	±	Negative
PR3 antibody (CANCA)	±	Negative
Anti ds-DNA antibody	<12 AU/ML	8
CH50	50 to 150%	42
Serum IgG	700–1600 mg/dl	1010
Serum IgM	40–230 mg/dl	123
ANA antibody	<0.8 index	0.405
Cardiolipin IgG	<12 GPLU/ml <3	<3
Cardiolipin IgM	<12 GPLU/ml	<3
Anti TPO antibody	<31.5 IU/ml	<10
Anti SSA-RO antibody	<12 U/ml	3
Anti SSA-LA antibody	<12 U/ml	2
B2 Glycoprotein IgG	<20 Eu/ml	2.5
B2 Glycoprotein IgM	<20 Eu/ml	8
Anti phospholipid IgG	<12 GPL/ml	1.6
Anti phospholipid IgM	<12 GPL/ml	1.8

Cardiology consolation was requested and performed for evaluation of possible cardiovascular anomalies. Echocardiography findings were not significant and levels of troponin and CK-MB were normal. In addition, computed tomography (CT) of the chest cavity was done to rule out aortic dissection and returned unremarkable. Two neurologists and a neuroradiologist re-discussed the case and they agreed that imaging findings and results of laboratory tests were more suggestive of cervical cord infarction with no identifiable causes. The patient developed nosocomial pneumonia as a complication of mechanical ventilation during hospitalization, with no associated respiratory dysfunction, having completed antibiotic therapy with clinical improvement. After 3 weeks, she was successfully extubated, weaned from the ventilator, and underwent rehabilitation to restore limbs motor function. The patient was discharged after 30 days of hospitalization while muscle force in left extremities was 4/5 and referred to the neurology clinic for monthly follow-up.

Discussion

SCI is among the infrequent causes of acute non-traumatic ischemic stroke and accounts for 1.2% of all neurovascular events [7]. About 25% of SCI cases are idiopathic and some of these cases are associated with variable gene mutations [8]. SCI patients develop symptoms quickly with maximal symptomatology reached within 12 and 72 h respectively for 50% and for most cases [9]. Children typically exhibit a rapid onset and painful acute myelopathy with a T2-hyperintense signal on MRI. Clinical presentation in SCI is determined by the involved vascular territory and the severity of the impairment is varied from paraplegia to minor weakness [10]. Interestingly, the onset to peak time of symptoms in our cases was ~10 min presented with sudden onset weakness of left side limbs. Focal pain is commonly reported as one of the initial symptoms in patients with SCI, at a frequency ranging from 59 to 73% [11]. The mechanism of pain may be related to ischemia of the local meninges, the vertebral body or the nerve root [10].

SCI diagnosis is very challenging owing to its low incidence and the variety of symptoms that can be present at admission. In acute myelopathies, SCI could mimic neuromyelitis optica spectrum disorders (NMOSD) because of several overlaps in the clinical symptoms and MRI findings. However, the treatment and prognosis may be quite different in SIC and NMOSD, therefore, the correct diagnosis of each disease can prevent delays in management and treatment [12]. There are no guidelines for the treatment of spinal strokes, and they should be treated as cerebral strokes. Nevertheless, steroid therapy may be prescribed with the suspicion of post-stroke edema to reduce medullary edema and/or cerebrospinal fluid drainage [13].

Conclusion

Idiopathic SCI in adolescence is a rare but often devastating disorder while the exact pathophysiology of this disease still remains largely unknown. However, some specific risk factors for this age such as mechanical stresses on the immature spine must have an important effect on the SCI pathogenesis. Early diagnosis and immunomodulatory treatment such as steroids can result in improved outcomes in SCI.

Author contributions

MS and HBG conceived the presentation of the case study for publication. MT and NT agree to be accountable for all aspects of the work related to accuracy and integrity of the work. MS collected information, reviewed information, and wrote the manuscript.

Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Competing interests

The authors declare no competing interests.

Consent for publication

Consent was obtained from the patient.