Introduction

Varicella zoster virus can remain latent in ganglionic neurons; upon reactivation, the virus can travel peripherally to cause herpes zoster. The virus can also travel centrally to produce several neurological complications including transverse myelitis (Gilden et al. 2013). Transverse myelitis is a segmental, full-thickness inflammation of the spinal cord with many etiologies including autoimmune, neoplastic, vascular, and infectious. This case report highlights a patient who developed long-segment transverse myelitis after a varicella zoster virus infection confirmed with positive titers of cerebrospinal fluid (CSF) varicella zoster virus (VZV)-antibody immunoglobulin M (IgM) which is incredibly rare. Of note, this case is unique in its presentation of long-segment transverse myelitis after VZV infection which has not been previously reported in the literature.

Case report

A 63-year-old African-American male with a past medical history significant for hypertension and hyperlipidemia presented with a 1-week history of left-sided numbness, weakness, urinary incontinence, and recurrent falls. Weakness was gradual on onset, progressive, and associated with sudden urinary incontinence. The patient denied any history of trauma, fevers, rigors, or chills. The patient had left-sided upper chest shingles in the T5 dermatome 2 weeks prior to the onset of his current symptoms which had been treated with valacyclovir. On presentation, his vitals were the following: temperature 98.1 °F, pulse 74/min, blood pressure 142/94, respiratory rate 14/min, and oxygen saturation (SpO2) of 99% on room air. Neurologic exam on admission showed normal strength in the right upper and lower extremities with 3/5 strength in the left lower extremity and 4+ strength in the left upper extremity. Patient had crusted lesions on the left upper chest from the recent shingles episode. The remainder of the physical examination was within normal limits. Computed tomography (CT) head and brain magnetic resonance imaging (MRI) were negative for acute infarct or hemorrhage.

The next day after presentation, the patient was unable to move his left leg which was followed by weakness of his right leg. Urgent MRI of the cervical and thoracic spine were done which showed diffuse hyperintense T2/STIR signal from the level of cervicomedullary junction up to the 9th thoracic vertebra (T9) level without any evidence of cord compression (Fig. 1). His symptoms significantly progressed and by day 4, strength was 0/5 in both lower extremities, 4/5 in the right upper extremity, and 2/5 in the left upper extremity. He had a sensory level at T7 dermatome. Cerebrospinal fluid (CSF) analysis showed a high white blood cell (WBC) count of 22 (ref. 0–5 cells/μL) with 100% mononuclear cells (ref. 0%) with a red blood cell (RBC) count of 495 cells/μL (ref. 0 cells/μL). CSF protein was high at 86 mg/dL (ref. 15–45 mg/dL) and glucose was high at 94 mg/dL (ref. 40–70 mg/dL). VZV antibody-immunoglobulin M (Ab-IgM) was positive in CSF while polymerase chain reaction (PCR) for VZV was negative. Human immunodeficiency virus (HIV) serology was negative. The patient was started on intravenous (IV) acyclovir and finished 5 days of IV methylprednisone pulse therapy without any clinical improvement. He also completed 5 sessions of plasmapheresis without significant recovery and was discharged to an extended care facility for physical therapy and neuro-rehabilitation. On the 3-month follow-up, the patient had some improvement in both his motor and sensory functions. Strength in the upper extremities was 4/5 and 2/5 in the lower extremities.

Fig. 1
figure 1

a MRI cervical spine sagittal view showing T2 hyperintensity (arrow). b MRI cervical spine cross-sectional view showing T2 hyperintensity (arrow); c MRI thoracic spine sagittal view showing T2 hyperintensity (arrow)

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Discussion

Varicella zoster virus infection is an uncommon cause of transverse myelitis in immunocompetent patients. The frequency of transverse myelitis during or after varicella infection is 0.3% (Ong et al. 2010). Early diagnosis of VZV-related myelitis is based on its temporal relationship to the rash and detection of VZV DNA or VZV-specific antibodies or both in the CSF (Ong et al. 2010). The CSF analysis usually reveals a mild mononuclear pleocytosis with a normal or elevated protein (Takahashi et al. 2013). Magnetic resonance imaging of the spine may demonstrate T2 hyperintense lesions in the spinal cord with occasional swelling and enhancement. Most of the patients diagnosed to have VZV myelitis are clinically suspicious cases with consistent imaging findings (Lee et al. 2010). Virologically confirmed cases of VZV transverse myelitis are extremely rare. This patient was unique in that he had positive titers of VZV-AB IgM, thus virologically confirming the diagnosis, as well as long-segment transverse myelitis.

Currently, there are no established treatment regimens for transverse myelitis as a complication of VZV infection. Some researchers recommend high doses of acyclovir and steroids. There is evidence that early antiviral treatment reduces the risk of VZV myelitis. Therefore, early diagnosis and antiviral treatment of VZV infection are essential to minimize the complications which include myelitis (Hung et al. 2012). Although clinical recovery is variable, many immunocompetent patients improve significantly, though fatal cases have been reported (Hung et al. 2012).

Conclusion

Transverse myelitis is a rare complication of varicella zoster virus infection but can have significant effects on patient morbidity. Thus, early and effective diagnosis and treatment with antivirals can reduce the risk of complications. This case report highlights a patient with both virologically confirmed VZV infection and long-segment transverse myelitis.