Horner syndrome as an unusual presentation of cervical radiculomyelopathy associated with cervical disc herniation: A case report

Abstract

Introduction and importance

Horner syndrome, an oculo-sympathetic pathway disorder, is a very rare manifestation of cervical spine pathologies which usually present with either axial neck pain, radiculopathy, or myelopathy symptoms (or combinations of these). It is more-likely to happen in the upper cervical level involvement.

Case presentation

A 44-year-old male presented with a complaint of right eyelid drop two months before admission. The symptom was accompanied by radicular neck pain and weakness of the right upper extremity (M4) since 1 month earlier. An MRI revealed a cervical herniated disc at the C5–6 spine level.

Clinical discussion

The patient underwent open discectomy followed by cervical disc replacement. Symptoms were significantly improved following surgery.

Conclusion

In the presence of cervical symptoms, a focused evaluation of Horner syndrome can be done, and symptom resolution can be expected after the appropriate treatment. Otherwise, a thorough examination must be obtained to find the pathology along the tract of the oculo-sympathetic pathway and prevent irreversible neurological disturbance.

Highlights

• •Cervical spine disorders usually manifest as neck pain, radiculopathy, or myelopathy.
• •Horner syndrome is an oculo-sympathetic pathway disorder.
• •Early and thorough evaluation of Horner syndrome is needed to prevent further and irreversible problem.

1. Introduction

Horner syndrome (HS), characterized by the triad of ptosis, miosis, and facial anhidrosis, is the result of oculo-sympathetic pathway disruption anywhere along the nerve pathways [1,2]. The common reported causes of HS are trauma, tumors, or iatrogenic. In the other hand, cervical nerve lesions are rarely reported as the cause of HS [3]. It typically results in a number of symptoms, including weakness, reduced sensation, decreased dexterity, and gait instability. Later symptoms include spasticity and bladder and bowel incontinence [4]. We present an extremely rare case of HS in a 44-year-old male who had previously experienced disturbing radicular pain to the right upper extremity months before admission, which was confirmed as cervical disc herniation on a magnetic resonance imaging (MRI) study. Patient granted Informed consent prior treatment for potential publication. This case is reported according to the Surgical Case Report (SCARE) Guidelines. [5]

2. Presentation of case

A 44-year-old male presented with the 3 months onset of disturbing neck pain (visual analog score/VAS 7) which radiated to the right arm, and tingling and numbness to the right thumb and index finger. The pain caused sleep disturbance and was aggravated with neck motion. There was no bladder or bowel neurological sequalae of incontinence. There was no loss of muscle power to the lower limbs. A month later, there was a sudden onset of drooping of right eyelid. No history of trauma or previous triggering events prior to the onset of symptoms. Before being referred to an orthopaedic surgeon, the patient had undergone a series of conservative treatments, including pain medication, acupuncture, and physiotherapy.

Left side ptosis, miosis, and anhidrosis of the left half of the face were found during the physical examination, which are typical symptoms of HS. The neck range of motion (ROM) was severely limited by pain, with evidence of paravertebral muscle spasm. Spurling's and neck passive stretch test were positive. Additionally, there was decreased of sensation on the left upper extremity starting from the C6 dermatome and thenar muscle atrophy. The grip strength was reduced than contralateral. The Hoffmann-Tromner test was positive. No abnormality was found on both lower limbs.

The NCS revealed C6 and C7 nerve root irritation without signs of denervation. The right median sensory nerve showed a prolonged distal peak latency (3.7 ms). The right brachioradialis, extensor digitorum, and biceps muscles showed increased motor unit amplitude and duration with slightly increased polyphasic potentials. All the remaining muscles and nerves were within normal limits.

Cervical MRI revealed right foraminal stenosis on the level of C5–6 due to disc protrusion with partial annular fissure. There was minimal spur formation but no evidence of ligamentous thickening. The T2 weighted image revealed no abnormal intensity of the spinal cord on the level of protrusion (Fig. 1).

Fig. 1.

The preoperative MRI showed disc protrusion on the level of C5–6 that compressed the spinal cord on the lateral recess.

The patient underwent C5–6 discectomy and cervical disc replacement (CDR) (Fig. 2A). A significant sequestrated disc fragment was removed during surgery. During the post operative follow up, the neck pain and arm pain were immediately subsided, the normal sensation was regained after 10 days, and the patient reported disappearance of his right eye ptosis and normal sensation at three weeks post-surgery (Fig. 2C). The muscle power grading was 5/5 at 6 month follow up.

Fig. 2.

A. Post operative X Ray. B—C. Clinical picture before (B) and 3 weeks after surgery (C) shows the resolution of Horner syndrome.

3. Discussion

The common presentations of cervical disc herniation are neck pain, radicular arm pain, myelopathic symptoms, or both (cervical radiculo-myelopathy) [3]. In our patient, the presentation of neck pain radiating to the right arm, tingling and numbness to the right thumb and index finger were pathognomonic to cervical spine/vertebrae lesions which preceded the classic features of HS. The cervical spine MRI was ordered over brain CT scan due to the history and presentation of the patient as no history of brain lesion was suspected. However, in an absence of cervical symptoms, surgical or trauma history, a thorough history taking, examinations includes brain CT and MRI must be obtained. The precis investigation ordered aids in early diagnosis thus preventing further morbidity.

HS was firstly described by Johann Friedric Horner in 1896 which represents the interruption of the oculo-sympathetic pathway either at the first (central), second (pre-ganglionic), or third (post ganglionic) order neuron (Fig. 3). The central oculo-sympathetic pathway disruption usually caused by brain pathology such as tumors or ischemic injury, and spinal and brain stem problem such as syringomyelia, myelitis, or other demyelinating diseases. The preganglionic lesion usually caused by thoracic or neck neoplasm, spinal cord injury, herniated disc at C8-T1, or iatrogenic disruption. Lastly, the postganglionic lesion usually has an unknown origin. However, vascular headaches, neoplasm or aneurysm in cavernous sinus, nasopharyngeal neoplasm, and basal skull fracture were reported as possible causes of the postganglionic sympathetic chain disruption. [2,3]

Fig. 3.

The three-neuron oculo-sympathetic pathway travels a tortuous and lengthy course from the hypothalamus to the orbit. The three neurons run along the spinal cord, brainstem, cervical sympathetic ganglia, internal carotid artery adventitia, and fifth and sixth cranial nerve branches. Preganglionic portions consist of first-order neurons (yellow) and second-order neurons (blue). Postganglionic third-order neurons (green) exit superior cervical ganglion, travel up the wall of the internal carotid artery to the cavernous sinus, and then joining the ophthalmic division of the trigeminal nerve that gives branch to innervate the orbit (naso-ciliary branch) and divides into two long ciliary nerves to reach the iris dilator muscle. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Central Horner syndrome is a rather uncommon condition, particularly if caused by cervical disc herniation. The cervical disc herniations are potentially caused HS if located at the level of C8 -T1 (preganglionic lesion). In our patient, the herniated disc was located on the level of C5–6. Although this is not the classical presentation, the first order neuron of sympathetic pathway on that level might be violated. [3] However, this associated HS does not have to be associated with myelopathy. Myelopathy generally involves wider area of spinal cord: the posterior columns, spinocerebellar tracts, and corticospinal tracts due to combination of static and dynamic mechanical factors and spinal cord ischemia. Whereas, in central lesion with spinal origin, the affected area is on the lateral column. The so-called lateral medullary syndrome (Wallenberg syndrome) commonly causes central HS which is due to posterior-inferior cerebellar artery or distal vertebral artery stroke [3,4]. In this patient, the central HS was resulted from the right lateralized disc herniation which compressed the intermediate-lateral column of the spinal cord.

HS due to cervical disc herniation can be associated with cervical radiculopathy symptoms, myelopathy (tetraparesis), or Brown-Sequard Syndrome (hemiparesis). Immediate decompression was found to be effective particularly before the evidence of myelomalacia. Several literatures of HS associated with cervical disc herniation reported resolution of symptoms after decompression [1,3,4,6,7]. In our case, symptoms resolutions were found immediately after the surgery.

4. Conclusion

Horner syndrome presents an interruption of oculo-sympathetic pathway along its route. A thorough history taking and examination must be obtained to localize the source of neurologic disturbance. The cervical spinal cord abnormality should be considered in the presence of cervical symptoms along with the Horner syndrome and immediate decompression must be planned to prevent irreversible neurological disturbance.

Ethical approval

Ethical Approval was provided by the authors’ institution.

Patient consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is

available for review by the Editor-in-Chief of this journal on request.

Disclaimer

No patient or author details are included in the figures.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

All authors contributed to data analysis, drafting or revising the article, have agreed on the journal to which the article will be submitted, gave final approval of the version to be published, and agree to be accountable for all aspects of the work.

Consent for publication

The patient provided informed consent for the case details and accompanying images to be published.

Declaration of competing interest

N/A